Lungs Behaving Badly.

The morning after the night before.

2012-01-23T11:22:00.001Z

Anticipative.

You guessed it, that's me.

Having hope is not a bad thing and it is hope that drives you on in life. Without hope, you would be a glass is half empty kind of person and we all know that isn't a fun way to live.

For over two weeks I have been clinging on to hope and as each day passed, I began to breathe a sigh of relief that maybe, just maybe this time things were going to be different.

Slightly misguided and lulled into a false sense of security allow me to indulge you all in our weekend of full blown rage. I'm not talking about any old type of rage, I mean Cipro rage.

The beast returned with a vengeance.

Sophie has been a little grumpy but no more than usual tiredness, we didn't think any more of it as most of the time she was super pleasant.

Cue Sunday, started off okay, visited her nan and grandad and all seemed okay, temper a little more short than usual but coping.
Getting ready to leave and temper more frayed, tears and generally irritable now.

By the time we arrived home, Sophie had morphed into a Cipro induced beast.
Throwing things, kicking, punching and head butting stuff, after coaxing her into her treatments and getting her bathed and ready for bed she just flipped.

Somehow she managed to find the strength to pull her mattress off her bed. (I struggle to move this) and she just continued to throw her stuff about, attacked her toys, attacked her wall and anyone who went near her. She trashed her room.

It was awful and took a very long time for her to calm down and fall off to sleep. I text her CF nurse and asked if the Cipro could be stopped as we were nearly at the three week mark any way.

I had a pit of fear in my tummy every time I climbed the stairs of what I was going to find. If it was physically possible her head would have spun last night. Everything about her had changed including her facial expressions, she was so agitated.

I just cannot imagine what thoughts were running through her head to make her go from being so friendly and lovable to completely violent and vile in such a short space of time.

I have gained even more respect for parents who deal with challenging behaviour on a daily basis. I just don't know how you do it both physically and mentally as I feel absolutely exhausted today.

Sophie's CF nurse has text me back and the Cipro is now stopped. Just hope it gets out of her system quickly!!

In other news.

CF clinic went really well last week, no issues at all which is brilliant. Her consultant would like her to have a CT scan of her lungs so we have a baseline picture in detail of what her lungs actually look like.

Although Xrays are useful, they don't show the finer details of the small airways which is typically where lung damage first occurs. In these areas, a change in health is not usually noticed either and it's this silent side of CF that scares me a little. For now we wait for the appointment and hope we can get Sophie to cooperate.

One last piece of news, Sophs sputum culture from clinic is back...

Mixed Growth.

Yep, completely normal and although I know pseudo will show again soon, it's nice to know the numbers are so low it's retreated and is in hiding for now at least.

Off to hospital for Sophs port flush tomorrow, I just hope the Cipro is leaving her system by then otherwise it may get ugly!

Take care and thank you so so much for all of the lovely messages and comments. They mean a lot to us :)

xxx

Huge catch up!

2012-01-17T16:26:00.002Z

Well hello blogging world.

I thought I'd better update the last couple of months and apologies in advance there are lots of pictures. You know how I like to use visual aids to get my point across :)

The picture above haunts me terribly but I have included it to emphasise just how poorly Soph has been and how obvious it is to know when she is ill.

Lets rewind.

Soph had completed a two week course of clarithromycin for the new bug she had cultured-strep pneumoniae, but unfortunately she didn't improve at all. In fact, she was much worse, so after speaking to her team again, it was decided to continue with a further two week course of clarithromycin as Christmas was approaching fast.

After four or five days, Soph was continually getting worse, becoming lethargic and grumpy, coughing and wheezing throughout the night so I called the team again. They felt that the wheeze was probably due to her airways being inflamed from all of the infection she was shifting. At this point her sputum was thick and green.

So added to her daily treatment regime was prednisolone (steroid) and a salbutamol inhaler to be used via a spacer.

Knowing Soph and how she reacts to new treatments we actually never expected her to use the inhaler. Her CF team just said as long as we could get some into her it would be better than nothing and the plan B would be for her to have it via a nebuliser instead. We gave the inhaler a try as I didn't fancy adding a 5th nebuliser into the day unless absolutely necessary.

To our surprise, Soph loved it!! I guess she is my daughter, which makes her an oddball too :D

Over the next week, Soph appeared to be turning a corner and was still at school with an irritating cough only. We thought the meds were kicking in and we would soon see the back of this run of illness.

Wrong.

Two days before Soph broke up for the holidays the school rang to say Sophie really wasn't well and wanted me. When I got to school she looked awful, super pale and could barely walk or speak.
I scooped her into the car and within minutes she was asleep. The very first photo in this blog was taken 10 minutes after I picked her up from school.

Scary times.

For the next few days, Soph remained the same and Ian and I even had to pick her up and put her on the toilet. She just lay on the floor or sofa and slept. Then the temperature appeared at 39.9C and we battled to keep it at 38C with calpol for a few days.

Soph then began to develop tummy ache and retching. Now at this point we were not sure if she had a stomach virus or a partial blockage as she cannot be sick due to her nissen fundo.

We just rode it out until she started having her bowels open-mustard yellow if you're curious and don't worry, no picture of that!

So the assumption is, she had picked up some sort of gastric viral bug on top of her chest infection and her poor little body just shut down and went into sleep mode.

Finally she turned a corner just after Christmas and two days before she was due to start back at school she was finally her old self again.
Clarithromycin finished and we sent in another sputum sample to make sure the strep had truly gone.

5 days of having no form of antibiotics we got a phone call to say Soph had grown pseudo again but it was a pathetic growth and her CF consultant wanted to try one last ditch attempt at eradication.

This I found incredibly strange as he has always said she was colonised but we went with it, nothing to lose. So 3 weeks of ciprofloxacin was started.

Yes the drug that has sent her psychotic in the past.

However this time round, she has been an absolute pleasure so maybe it was her age that caused such a severe response several years ago?

AND this brings us to present day.

Soph has approx 1 week left of cipro to go and when this course is complete, she will have had 9 straight weeks (minus the 5 day break) of some form of antibiotics.

The antibiotic train comprised of:

Two weeks of IVs, 4 weeks of clarithromycin and 3 weeks of cipro, it's insane, but couldn't have been helped due to how poorly she was. There was talk of bringing her into the hospital for more IVs if she didn't improve.

Thank goodness she rallied.

So after the mammoth catch up, I will wish you all a very happy new year and leave you with a few more pics of Soph looking like her old self again!

This was not long before she became really poorly, she had been to a fancy dress party and was snow white.

Taken today on the way home from CF clinic,

Playing in the frost, she loves getting fresh air and I have to drag her in when it is so cold.

I will update about CF clinic over the next week as I feel you've had enough to read for one sitting!

Take care all and thank you so much for all of the well wishes and support, we couldn't get through it without you all.

xxx

Just a quick note...

2011-12-23T14:39:00.002Z

To say that we wish every one of you a very Merry Christmas and want to thank you for your continued support.

Sophie hasn't been too well over the last few weeks but I will update after Christmas.
Things seem to finally be turning a corner and I hope to post good news when we return.

Special thoughts to those stuck in hospital or poorly.

I hope every one has a healthy, happy Christmas!

Much love to you all from our family to yours.

Xxx

A little worried.

2011-12-07T10:00:00.002Z

Well this past week has been interesting with Soph.

I phoned the CF team as she was continuing to cough, cough, cough and spoke to one of the lovely CF nurses about my concerns.

After a debate consisting of us batting back and forth the options, weighing up the pros and cons of each we settled on giving ciprofloxacin another try. Yes you heard right, Cipro.

We decided that jumping straight back in with IVs was a bit soon and the plan was for me to get Sophie to do a sputum sample and get it straight to the hospital and to start the Cipro to cover the pseudo, just in case the IVs hadn't fully sorted it out.

So that is what we did, however when Sophie managed to produce a brilliant sample for me I was slightly alarmed to see her sputum was green. Not really dark but still green.

Sputum sample was delivered and Cipro was started, with the agreement that if by Monday, Soph had developed Cipro rage that we could switch to something else.

Come Monday morning, Sophie was coughing more and more and producing sputum like I've never seen. I couldn't quite decide if her grumpiness was Monday morning blues or Cipro rage starting.
Either way I phoned the team to see if her cultures were back. I wasn't comfortable as I knew something must be going on as her symptoms were extreme for her.

I eventually got a call back from her nurse who had the results.

The sample grew no Pseudomonas. I repeat, NO PSEUDOMONAS.

This is rare for Soph and is only the second time in over 2 years she hasn't cultured it. I know we'll be seeing it again soon but clearly the IVs have done their job and kicked its ass for now. Good times.

However, a new bug has been cultured.

Streptococcus pneumoniae.

I have to admit that I didn't know much about this one but after a chat and some research it appears it's common in children and usually results in strep throat. Sophie hasn't had a sore throat though.

It can cause a few issues if not treated including pneumonia. It is often resistant to a lot of antibiotics too.
Fortunately for Soph her strain is sensitive to clarithromycin so this is what we have started.

I've been searching for someone who has experience of it and want to know how you/your child was with it?

Reason I ask is Soph has had no temperature, no sore throat and hasn't been particularly run down, however she has been VERY productive, her sputum is still green and she's also becoming wheezy, which I feel is down to the sheer amount of sputum she is shifting causing inflammation in her airways. This bug is no longer in her upper airways and has now descended down into her lungs.

I'm just worried that it may stop being a typical childhood bug and develop into something more serious if it isn't starting to already.

Any experiences would be gratefully received.

In other news I've done my very first guest post and would love for you all to take a look. It's on Kathryn's blog, Crystal Jigsaw, which is a fabulous blog and I highly recommend you check it out. She's also a published author and generally lovely lady. Her daughter Amy is a wonderful young lady who has had her own trials in life living with autism. My blog post is about hidden disabilities, and I have to be honest, it made me cry writing it as it brought back some tough emotional memories that I had suppressed for a very long time.
Let me know what you think either here or over on Kathryn's blog at the end of the post if you can :)

My first guest post is only going to be available until next week, so please go and read it :)

As soon as I have some more news on Sophie I'll let you all know and if you can keep her in your thoughts and will her to kick this without any further intervention that would be wonderful.

I have to admit I'm a little concerned and worried but I'm trying to hide that at the moment...

Take care everyone and for Magda, I really haven't forgotten you but hope you understand my delay and I'll be in touch very soon.

xxx

Tweet Tweet

2011-11-29T13:09:00.001Z

Finally I've been able to blog.

It's been a rather crazy couple of weeks so apologies for leaving you all hanging on how Soph is doing. Thank you to those of you who have been in touch to check on her.

Well Sophs IVs finished a week ago today and I'd love to say she is 100% back to her normal self, but she isn't really.
I cant entirely put my finger on it. She's got energy, she doesn't look unwell but she's still really productive and coughing quite a lot. Her sputum is clear though.

She appeared to pick up a slight cold towards the end of her IVs but it didn't really progress much, but I'm wondering if this is what is contributing to this persistent productive cough. It first appeared part way into her IVs and we looked at it as a good sign. The IVs kicking in and kicking all the bad stuff out but the fact it's still present leaves me with a few questions.

1) Was the course of IVs not long enough?
2) Is something else going on?
3) Is it just viral or change in weather?
4) Is it inflammation and a course of steroids could do the trick?
5) Would oral antibiotics help?

The simple answer is phone the CF team, which I am going to do, however it's difficult to explain to the team when I cant pin point the symptoms. Other than an increased productive cough, there's pretty much nothing else going on and it was the reason we started the IVs in the first place.

*sigh*

I'll keep you all posted and if anyone has any ideas please fire them this way.

I am also preparing the blog post with all the pictures of how we do home IVs. Warning you all now it'll be a large post but mainly all visual :) You ask, i'm going to deliver!

In other news I've ventured onto twitter, thank you to those who are following me but for the ones who had no idea I'm there, come find me as I update on there much more than here and if you have any burning questions, I answer back same day.

@Sophs_mum

Leave you with a couple more pictures of Soph using my amazing camera skills :D
She had confiscated my iphone because she wanted to listen to her favourite songs and dance to them.
I see another glimpse into the future, she was too busy to even smile properly, look at the poor effort!! :D

Take care all.
xx

Surviving home IVs (old school).

2011-11-14T13:36:00.001Z

We are almost a week into Sophie's home IVs and it is going well.

Bertie (her port) has some gauze around him this time because they have used a slightly bigger needle due to the smaller ones blocking off, and this is to just give it a little more protection.

I am pleased my mommy instinct was spot on as she definitely needed them. The amount of rubbish she has started to clear is unbelievable and she is already bouncing off of the walls with super energy. Goodness knows what she is going to be like at the end of the 2 weeks!

Anybody fancy babysitting for a while? ;)

A few people have asked me some questions both on here and via email so I thought I would try and answer what I was able.

Firstly, the reason we don't use oral Ciprofloxacin unless we absolutely have to is because it turns Sophie into an uber monster. The drug itself is well known for mood changes etc but Sophie is only able to take it in its liquid form. The tablets don't cause as many effects. After lots of research and talking to people in the know, it was discovered that a small percentage of people react to a preservative used in the brand over here.

Sophie is one of that small percentage and the result is an evil version of my daughter. I'm not talking stroppy or even just a bit naughty, I mean EVIL.
She becomes very short tempered, permanently angry and very very violent. She spits, slaps, pinches and throws things at people. She never smiles when on it and has a permanent frown on her face. We even caught her attacking her beloved teddy (Leeber) whilst on it.

Suffice to say we literally only use it if there is no other option.

Hope that helps! It's a shame really as it is such a powerful drug against pseudo but we do have other options fortunately.

The next bunch of questions I received was asking about how we are doing Sophie's home IVs and what I meant by mixing them ourselves.

Well the simple answer is, we are literally mixing the drugs from their powder form.
Most people when doing home IVs receive a delivery to their house or pick up their drugs each week from the hospital.

These drugs are already pre-prepared. Each antibiotic has been mixed and diluted and is either in a pre-filled syringe, bag or IV ball, and is basically ready to use. All saline and heparin are also ready in a syringe to be given.

We chose to do it all from scratch to save a slot for someone who really needs it basically.

It takes a little organisation but once you get there, it's quite easy to do it 'old school'.

The most important tip I was ever given was to put each dose of antibiotic into plastic tubs. I don't mean the whole course but 24 hours worth. So every time the IVs are due, you can grab your tub and everything you need is there.

This is what three individual doses of Sophie's Aztreonam look like. Everything we need to give her one dose is in each box.

And here is the box with everything to give her, her once daily dose of Tobramycin. The box to the right has the IV pump in it.

It takes approximately 10-15 minutes to mix each dose of Aztreonam, the reason for this is everything has to be cleaned such as the tray, each vial eg saline, heparin and antibiotic to prevent any infection getting into the port.

The Tobramycin also takes about 10 minutes to mix up and prepare.

Here is what Sophie's 4pm dose of IV medication looks like all prepped and ready to be given. This is where she has both Tobramycin and Aztreonam.

My dad has kindly photographed the whole procedure for preparing home IVs (old school)and if anyone would like to see it I can either do a stand alone blog or pop them into a slide show. So let me know otherwise I will keep them for a rainy day!

I'm pleased to say that Sophie has managed to attend school and has only missed it when she has had to go and have her Tobramycin levels checked at the hospital.

The school have been fantastic and are looking after her really well. We decided as Sophie was a little lively it would be best to keep her in during break times to prevent any port damage. She gets to choose a friend each day to look after her.

Initially she wasn't impressed but eventually came round to the idea!

If anyone has any burning questions then please please ask. It may just give someone else the answer they were after too.

Take care and thanks for all the lovely thoughts and comments. My thumb is much better now!

xxx

Ouch! (insert expletive)

2011-11-08T22:38:00.001Z

The Epi-pen is not my friend.

For those of you unfamiliar with what an Epi-pen is allow me to indulge you.

This pen-like object is actually a life saver, it is filled with adrenaline and used when a person has anaphalaxis(a severe allergic reaction). To use it you follow the simple instructions and basically jab the pen into the thigh, and it delivers the adrenaline to help reverse the effects of the allergic reaction and prevent death.

It works very quickly and many people carry these who have known allergies such as people allergic to bee stings, peanuts etc.

We have a couple in our house for when Sophie has home IV antibiotics in case she were to have a reaction to the drugs. As they are injected directly into her vein, if she were to react it would be quite quick and be an urgent situation.

Before we set off up to the hospital to get Sophie's port accessed to start this round of IVs, I was having a clear out of her IV box. I noticed that the Epi-pen we had was out of date. Being the conscientious nurse I am, I decided that it wouldn't suffice just putting it into our sharps box.

Oh no, what if someone was crazy enough to reach in there and pull it out and inject themselves?

So I told Ian I was going to discard the adrenaline and then I wouldn't need to worry about someone hurting themselves with it.

Easier said than done let me tell you.

For reasons unknown the pen wouldn't expel it's contents and me being the stubborn creature I am, began to fiddle with it until BAM, the pen deployed itself sounding like a gun going off, followed by a very loud squeal.

Oh yes, that is a puncture mark in my thumb..

And again, you'd be right if you thought that was a puncture mark in my nail..

Not the greatest picture but you should be able to make out where the needle entered the back of my thumb and came out through my nail on the other side..

The needle appeared to hit the bone in my thumb which made it bend allowing it to come out through my nail at the front.

Good times!! I can tell you it really hurt but I have done nothing but laugh about it. On a serious note, I am lucky in two ways.

1) Due to the needle going straight through my thumb, most of the drug didn't enter my system. If it had I risked losing full circulation in my thumb and would've had to go straight to hospital for more injections to reduce the effects of the adrenaline.

2) The pen was out of date so the drug was in a weakened form so any in my system wouldn't have had too much effect.

The doctor who did look at it for me tried not to laugh too much as he knew me and knew I was a nurse and really should know better.

The moral of this story is, do not fiddle with objects that appear to be stuck! :D

Sophie's IVs have started so officially day 1 of 14. So far so good and I will fill you all in soon.

Thank you for all the well wishes and support, it is appreciated. If anyone has any questions feel free to ask. I will be answering those that have already been asked this week.

Take care and feel free to have a good giggle about my misadventure, we all have!!

Decision made.

2011-11-01T17:08:00.001Z

Next Tuesday our house is going to look like this.

We'll be mixing these ourselves again. (No pre-mixed as we feel it's always kinder to keep a pre-mixed slot open for someone who doesn't have help to mix IVs).

To do this for two whole weeks.

Joint decision between us and the team to give Sophie two weeks of IV antibiotics before the real winter sets in despite her chest being a little frisky at times, she's still fairly well and we want to keep it that way.

There was a brief mention of Ciprofloxacin orally however Ian politely told Soph's CF consultant he could have her at his house for the duration if she was going back on it :)

He declined but feels it's sensible to give her an annual blast of IVs so this is where we are at.
They are more than happy with her weight (see previous post if you haven't already), her dietician had a little giggle at the letter and said to continue as planned.
Sophie managed to do an on demand sputum sample in a pot as well as a cough swab so hopefully only MR Pseudo will be present.

Plan is to flush port next week instead of being done today so it can be accessed in one go and then start IVs.

No overnight stay which is always a blessing :)

Wish us luck as it has been almost a year since we last did battle. Sophie will be attending school the whole time too hopefully. I have a meeting with the school tomorrow morning to break the news.

Take care all :)

xxx

Think outside the box.

2011-10-28T13:12:00.001+01:00

Sometimes, living in a world full of chart obsessed people can have its advantages. Without statistics and averages some things just wouldn't work. Statistics can help give a general overview of a multitude of things from TV ratings to education targets, from scientific projects to life expectancy predictions for people with life threatening illnesses.

The downside to this obsessive culture is many people no longer have the ability to think outside the box. If the chart says pink then in their eyes, obviously the result is pink. No other variables are taken into consideration at all even when there are clear indications that the result is actually blue.

We have had the pleasure of being in a situation where our result was 'blue' however the chart/graph/statistic/law of averages or whatever clothing it was dressed as this time says 'pink'.

Allow me to enlighten you all on one of the funniest things we've been told in a long time. A few years a go I may have reacted very differently but now I feel very empowered regarding Sophie's health and well being and I was able to laugh.

Sophie had her height and weight etc done at school by the school nurse a few weeks back and she phoned me to fill me in on her findings as per chart/graph/statistics/law of averages etc etc.

Now I must add a disclaimer for her school nurse as she clearly is rebellious to the chart and is able to factor in other variables to give a more rounded conclusion to her findings.

She started the conversation by telling me the percentiles Sophie was on for her height, exactly where we already know she is, and then proceeded to tell me where Sophie was in terms of percentiles for her weight, again exactly where we know she is. By we I mean Ian and I, her whole entire CF team, her whole entire feeding issue team and her whole entire TOF/OA team.
So on the theme of approximate statistics that means 3 dieticians, 3 specialised consultants in their chosen field, approx 6 other doctors and 3 specialised nurses.

I was expecting her to say Sophie was a little short for her age which we already know, but no that wasn't it at all.

Apparently according to the chart, my 4 year old, Cystic Fibrosis suffering, oral phobic, tube fed child is overweight and we were being sent the government approved letter inviting our child to enter a healthy eating program.

Now you may call me awkward, over protective or even sensitive to my daughters needs but am I the only person on the planet that sees something wrong with the above?

My response on the phone was to laugh to which her school nurse did see why. I mean even if we took a few variables out of the equation it is still astonishing to think that Sophie fits into the 'typical' mould here.

We celebrate excess weight round these parts, it's extra strength to fight off chest infections, it's also extra reserves while we are tube weaning where it's inevitable Sophie will lose weight. Not to mention how on earth my oral phobic child would be able to participate in a healthy eating program seeing as she DOESN'T EAT.

Now do you see why I laughed?

Luckily her school nurse has wrote a detailed account in her notes as to why the program is not appropriate for Sophie, but she is sending us the letter so we can have a laugh at CF clinic next week.

Sophie is currently back on oral antibiotics as she has been coughing up green gunk again. This time she is entirely well and now deep down in my heart I am thinking we may be heading for IV antibiotics. It's been nearly 12 months since her last tune up and seeing as she is colonised with pseudo, I think the time is drawing closer for him to have his ass kicked again.

Will let you know what her team decide next week as full CF clinic and port flush are due.

Ian and Sophie carved this, it's rather good isn't it? The first picture is an attempt to photo it at night lit up.

Sophie is steadily improving with her writing, she did me a montage while at her nans.

Take care everyone, I hope you are all okay as it's very quiet on the blogging front currently. I know we haven't been very regular lately but I'm working on improving this :). I'm also working on a post explaining what gene therapy is all about and how it could potentially change the lives of all with Cystic Fibrosis.

xxx

The real window of opportunity?

2011-10-05T10:46:00.002+01:00

As most of you are aware, Sophie doesn't just deal with one condition, no that just isn't enough is it? She has battled with two very different conditions every day since she was born.

The focus of the blog usually revolves around her day to day life and CF rearing its head every now and again currently. I do find I touch on her TOF/OA issues but it just never seems to be in the spot light often.

I'm hoping this will change in the future but in a positive light.

We met with Sophie's psychologist and dietician who deal with her oral aversion (eating phobia) last week. We had a long discussion about where we felt Sophie was at now in regards learning to eat. She has made some small advances but I often feel like I over ooze positivity in how well she is actually doing or I don't mention it at all.

Mostly the reason for me not discussing it is basically down to emotional wear and tear, period.

I can honestly say that dealing everyday with a child who just wont eat is draining. Physically and mentally, and it is one of the hardest things Ian and I have had to deal with, with Sophie.

I'm not talking fussy eating here, that's something completely different. I'm talking nothing going into the mouth and if it makes it in there by some miracle, it's spat out or retched out before given the opportunity to be chewed, sucked or swallowed.

The endless tube feeds and living life around a schedule because we have to give our four and a half year old milk throughout the day so she doesn't starve. Mixing up prescription formula into baby bottles so it's easy to measure out each individual feed.

We have been doing this everyday since she was born.

It's only when I really think about it I realise how draining the whole thing is. It was hard in the early days because all anyone wanted to do was ask how she was getting on with her eating. Ian and I found ourselves constantly repeating ourselves which in turn brought out all our anxieties and emotions about the whole situation. It felt like a constant reminder. In the end Ian just told everyone that "when she eats something we will tell you, we'll let you know if there is any change at all".

Currently the maximum Sophie will try and eat is about 3-4 teaspoons of either yogurt or ice cream that's it. She will lick and suck other things but doesn't really eat them.

You remember the failed tube wean we tried last year? She wasn't ready to take the next step then but we all feel she is now.

Last year I was very apprehensive about it, it was uncharted territory and I had no idea how she would react. This time feels very different, I'm much more relaxed about the whole thing and I know now that if it doesn't work it's fine. We can try again in the future if need be.

So we are already over a week in and this time we are doing things very slowly with milk reduction. Sophie seems to understand what we are doing and is trying her hardest to eat.

For the first time ever, Sophie ate a whole pot of yogurt. Every. Last. Morsel. And, I just sat there with my jaw on the ground in disbelief as she asked for another, which for the record she ate half of!

Tiny steps but HUGE leaps.

I'm not sure how this will play out in the long run but I feel very different this time. Is this actually the window of opportunity and last time was a warm up to the main event?

I guess only time will tell right?

I hope you will all stick with us on this journey once more as I know there may be times we need a huge pick me up!

Will leave you with a few recent pics of Soph...

She's ready to start the journey now :) Ian has started to use his bike again as rehab for his knee and Soph still isn't quite able enough to ride her bike with him. She'd never keep up so he bought this trailer for her which attaches onto his bike and she absolutely loves it. She has to wear her bike helmet for real journeys but this was just a try it out adventure. She squeals with so much excitement when out and about in it that she has cleared her own chest several times!!

"Mummy I really want one of these bags"- Sophie aged 4 clutching Mummy's Louis Vuitton!

Who knew reading was so tiring?
She wore herself out after a shopping trip and had also had her port flushed earlier on in the day.

Take care all xx

Fun, Friends and Foolishness.

2011-09-30T19:14:00.004+01:00

Sorry I majorly suck with keeping to blogging weekly right now. Life is still steaming by on full cylinders. I have no viable excuses.

Sophie's black eye has completely gone now thank goodness. It was rather impressive but it's nice not to have to explain to everyone we see how she got it.
She is continuing to enjoy school although she did have a few little setbacks due to another little girl. We'll call her Missy A.

Missy A has been causing her some bother with sly little pushes here and little slaps there. Soph was getting a little upset about it and had said a couple of times she didn't want to go to school. I did have a quiet word with her teacher and asked if she could just keep an eye on the situation in case Soph was fabricating it all. So far so good though and she hasn't had too much trouble since. I have heard that Missy A has been causing trouble for others too so it's not just isolated to Soph.

Sophie is quite sensitive and takes a lot to heart so I'm glad we have sorted it out before it became a major issue and it's also taught Sophie how to stand up for herself but not to retaliate.

She's also had a couple of play dates with her 'best friend' this week and has had a blast. Her nan took the two of them to an indoor play centre and today she spent a couple of hours at her house. I did have to give her friends mum a brief explanation about why Soph doesn't eat much and also not to be alarmed at her cough.

I'm quite proud of how Soph handled it and she had so much fun. I thought it would be much harder for me to loosen the reins, but I think I've realised that Soph needs to enjoy every second and do the same things as all of her friends.

Life is definitely for enjoying and living!!

A few recent pictures of Soph enjoying a HUGE lollipop...

In other news I decided to enter a blog giveaway that was been hosted by Rach over at Mid 30s Life
.It was for the latest Postman Pat DVD and she was also throwing in a Tinga Tinga Tales DVD too.

Well we only went and won!!! I never win!!! Sophie absolutely loves them both so she was really excited too. If you haven't already please check out Rach's blog. It isn't a health blog and it has to be one of the funniest blogs I have read. I never leave without a smile on my face. So what you waiting for? Go check it out now!!

Here's the Postman Pat DVD (how cool are my camera skills!? :) )

Random picture of Sophie goofing around, this one makes me laugh lots...

AND FINALLY...

Next years Skydive will be on the 1st of July 2012. Oh.My.Goodness.

If you remember, I foolishly declared I would be doing it alongside my husband and a few friends and my brother in law are very likely to be taking part too.

Must be completely mad but it's for a very worthy cause obviously.
Watch this space for me begging like crazy for sponsors ;)

I have other Sophie news but will leave that as a stand alone blog post for next time. Port flush is on Monday and also flu jab time for Ian, Soph and I too. Hopefully we will have no ill effects this year, it's non negotiable in my eyes and I'm not prepared to risk Sophie's health so we will all have to just go with it and see how we are.

That's all for now folks!

Take care xx

Don't mess with me.

2011-09-12T13:19:00.002+01:00

Well it's finally nice to blog in a positive way (partly why I left it so long).

Sophie has improved health wise and managed to start her first day of proper school. She's now full-time doing 08.55-15.15 Mon-Fri.
The antibiotics have shifted her cough and the temperatures have gone leaving her less spicy and hot!

CF clinic went well, here's the stats...

- Weight 18.5kgs (So 2kgs gained in two months).
- Chest sounded clear so her illness was definitely viral (good news).
- Stomach was soft and palpable.
- Height is now 100.2 cm so 3 cm increase.
- Sats were 97% so down a bit on her normal as she was still poorly.
- Cough swab taken.
- Bertie had his drink (port flush).

The fibre we have been adding to Sophs milk is working and things are finally firming up and her laxatives are down to a minimum now, sooo relieved!

Sophie is handling school very well, she's really enjoying it and cant wait to get home and do her homework. Bless her my little geek! Have a feeling it probably wont last though.

We spent her first week going up to the school at lunchtimes to spy on her through the dining hall door. This was at the schools request in case of any problems with Soph's eating.
I've explained that she could get food stuck if she was to actually eat something and not chew it properly, however we don't know when or if this will happen but it's made the school feel more relaxed and it has given us chance to answer any questions from the dinner ladies and teachers.

The school really rocks in our eyes. So far so good and Sophie happily sits with her little gang of friends and at least has a few tasters of some of the lunch we have packed her. She is also taking in some money to buy toast at morning break, which she has been spotted nibbling at :)
So watch this space! We have an appointment with her eating psychologist later this month and I'm looking forward to it.

I've handed the school all of Sophs appointment letters for the month and I think they have realised the magnitude of Sophie's medical involvement now.

And finally, Sophie was playing with an older boy at lunchtime (Ian is really impressed!!) and they were running about with each other and managed to bump heads somehow.

This was the result by evening...

The following day...

Beautiful isn't it?

Ian giving Sophie her tube feed after school and Sophie doing her best "Don't mess with me I'm hard" face :D

I feel I need to hang a sign around her neck stating we did not do this to her! She's been really good about it, didn't cry and says it doesn't hurt. It looks soooo sore though!

That's all for now folks and I'm pledging to blog at least once a week now unless I have a valid reason so you all have my permission to tell me off if I don't stick to it!

Take care all and thanks for Soph's good vibes, they've worked!

xxx

Seeing red.

2011-08-29T17:12:00.001+01:00

We had quite the scare last week.

Sophie continues to be unwell, coughing although not the meaty kind of cough we associate with typical infections. She's sleepy and grouchy and just generally not herself at all.

One night she woke up having a crazy coughing fit, she was calling for us so I ran upstairs to her. She must have coughed for a good ten minutes with no let up and bringing up lots of clear sputum.

Then I saw blood :(

Two of her blobs of sputum had lots of streaks of blood running throughout. The logical side of me knew she had probably aggravated her throat with the coughing fit and a small vessel had most likely ruptured. The crazy mum side of me panicked slightly and it has left me with a small pit of fear in my stomach.

Is Sophie likely to suffer with lung bleeds?

No one knows the answer to this but the idea of haemoptysis is one of the sides of CF that chills me to the bone.
Sophie has such a forced, extreme cough due to her TOF repair and this could mean that statistically she has a higher chance of suffering bleeds. I guess only time will tell.

Fortunately there has been nothing further and after speaking to her CF nurse we tried one treatment, which caused no further occurrence of the red stuff. So it's safe to say that this time it was almost certainly a one off and the coughing had been the aggrevater.

She's a week into her oral antibiotics now and I'm not convinced they are helping her much at the moment.
Her cough is no worse and her temperatures have stabilised but it's her lack of energy that is the concern. She can manage a few hours where she seems her normal self but then crashes on the floor and sleeps for several hours.

Sophie never sleeps in the day unless she is poorly and the amount she is sleeping at the moment is totally off the norm for her.

We have a full CF clinic tomorrow so will see what her team think. She also has her port flush so will be a long day for her. I just want to get her sorted as she starts full time school next week and it would be awful for her to miss any of it. Seems a long time for a virus to linger?

Currently this is how Sophie is spending the majority of her days...

Wakes up for a treatment and then crashes again.

I will keep you all posted and thank you so much for the positive thoughts and vibes for Soph. I'm hoping to report much more positive news next post.

One can only hope.

xxx

There's always a sting in the tail.

2011-08-23T14:12:00.000+01:00

Oh hi there!

I had absolutely no intention of leaving it so long between blog posts but a big thank you to those of you who have been in touch to check we are okay.

We have been enjoying the summer holidays as much as possible as they are flying by so quickly. Sophie starts full-time school September so not long to go now.

It's been a little bit manic round here the last week or so, Ian had his knee surgery and unfortunately there was much more damage than first thought. Rather than been up and about after a week, he is faced with not been allowed to stand on his right leg for 4-6 weeks!!
So that means crutches, no driving, and the inability to pretty much not be able to anything. He cant stand up (or should I say balance) for long enough to be able to make drinks, care for Soph etc so it's been interesting.

I've had to juggle babysitters for both Ian and Soph so I could go to work. I did leave Ian alone while I did a short six hour shift at work and my mum stopped by to check on him to find he had had to stand/wobble in the kitchen to drink a drink as he couldn't carry it anywhere :)

He has managed to adapt more now so it's not so hectic. The pain he was in was quite severe so he spent a good few days at my dads and Soph was at her nans.
I've basically had two kids with lots of extra needs to look after and juggle full-time work. It's been, well...interesting!

Things have settled now as Ian is able to get about a lot more, I'm sure cabin fever is going to set in some time soon though!

He's been going in and out of the shop to pick up bits and pieces but that hasn't gone without trouble. Obviously on crutches he doesn't have free hands to carry anything so he improvised and put his coffee and a bottle of water into his pockets to walk to the counter.
Unfortunately, a do gooder type clocked him before he could get to the counter as he takes ages and alerted the shop keeper that she should keep an eye on him as he was shop lifting :D

In hindsight I can see how it must have looked but what else was he supposed to do? Luckily our local shop know him and know he is very honest and laughed about it, but he really could have been arrested :D

Soph and I have been out and about enjoying ourselves, a small selection of photos from some of her summer fun...

Mmmmmm ice cream...

Ahh well, she did eat some!!

Silly dress up...

Fun on a giant bouncy castle...

The goat that fell in love with Soph...

How on earth does this thing work?

I think I'm getting it now...

Oh yes, result!!!

More quality time with the goat :)

Sophie's picture of me :D

She really was very proud of it, and to be fair so was I.

Look at my oral phobic little girl go with her toothbrush!!!!

She has hugely improved with this...

She has been super well for the summer and it has been a huge relief, however things have changed. Last night she had her first temperature in 3 1/2 years, a whopping 39.5 C!

She had been a little grouchy for a day or so but my brother got married Friday and we put it down to her being tired from all the goings on but we were wrong.
Her eyes started to look heavy and she became very productive.

Not a great quality picture but just look at how unwell she looks...

She has started oral antibiotics this morning and luckily she is at her CF clinic a week today and they will review her then. She'll be having her port flush done then too.

Her temp is being controlled with Calpol and it seems to be helping her mood as she hates being hot at the best of times.
Super sputum Soph is back and I'm gutted but hopeful it wont last long.
Please send her some feel good vibes that this passes without needing any IV intervention.

I wished I could end on a more positive note but I guess this is life with CF, UNPREDICTABLE.

I really hope everyone is doing good at the moment, I'll be checking in on you all later.

Take care

xxx

Schools out & a question inside...

2011-07-29T15:27:00.004+01:00

Sophie has finally broken up from pre-school for 6 whole weeks. Come September she will be starting full time school *gulp*.

We had a brief meeting with the head and deputy before they broke up, school that is not them as a couple!

Anywho, they just wanted us to try and figure out what kind of a plan to put in place regarding Sophie's eating. I think they had initially thought we would take her home for lunch but after explaining she needs to be with the other kids, we are all now on the same page. I think they are freaked at the possibility Soph could choke, we don't know if she has motility issues or how narrow her oesophagus is in places. It really is a wait and see issue and we will be exploring this during the first week of school. I am going to be lurking around at the school during lunch time just in case issues arise and then we will meet and decide if Soph needs someone paid to watch her at mealtimes. This would be someone trained to act if she was to choke but not be glued to Soph, kind of lurking to one side, ready to pounce if a sticky were to happen in her food pipe.

It's all what ifs at the moment so I guess we wait and see.

In other news, Soph had her port flush today, not as great as previous attempts and Soph chalked up another oscar winning performance to her credit. Once the needle went in she was fine but prior to that she was very dramatic and using diversion tactics to delay proceedings. Once done she was absolutely fine, I guess this is going to be her 'normal' until she relaxes more. It sure beats sedated Soph though!

She made her lovely CF nurse push her in a car up to the shop while she bought a magazine and then push her all the way back to the exit doors so we could walk to the car. Bless her, above and beyond her job description that's for sure! She asked for a tip, I told Soph to tell her not to eat yellow snow ;)

While there we checked out her sputum sample from the previous clinic. Yeah. I had forgotten to phone up for it but knowing we hadn't had a call, nothing new was lurking.
Well Mr Pseudomonas is still there however, only a small scanty growth which is FANTASTIC. Something is going on and maybe we are slowly able to fight this little sod again. For years Soph has grown moderate amounts of pseudo and since her clear culture, it has only showed in small quantities.

I will be discussing this at the next clinic. I have questions for a change ;)

Question for everyone, what are you told is the correct dose of nebulised Colomycin for someone over the age of two?

a) 1 million units twice daily.

or

b) 2 million units twice daily.

Can you let me know please it's important.

Finally just before Soph broke up for the holidays, they had bad hair day at school. Sophie wanted her hair in pigtails and red. I decided to grant her wish for one day and bought some of the red hairspray in a tin. Only problem is it says it may stain light coloured hair like Sophs.

We compromised as she is due her FIRST haircut (I will explain nearer the time) in a few weeks so just decided to do the ends of her bunches. If it stained, we could just have it cut off. Sorted!

Heres the pics, the best I could get...

Sophie loved it and so did her friends. Does anyone else think Soph is looking quite porky in the last pic?? It's fabulous!!

Take care all and sorry for the delay. We have been enjoying life as much as we possibly can. Thank you for all of the responses on my previous post, really interesting and helpful to read!

xxx

A Doctor said it, so it must be true.

2011-07-15T13:20:00.000+01:00

Ian had his appointment with his surgeon about the options for his knee. As he has a tear in the ligament, he is awaiting a date for an operation. They wont know the full extent of the damage until they get in there with the camera, and this will be the difference between getting back to normal quickly or not.

All was going well until he said something that really ruffled my feathers. He asked if there were any dates we had plans for in case a cancellation occurred. Both Ian and I said no unless our daughter was to be hospitalised, which would be something we couldn't plan for anyway.

He asked what was wrong with her and Ian explained she had Cystic Fibrosis. He turned to face us at this point and said that "Cystic Fibrosis is a very different disease now to how it used to be" to which Ian and I both agreed. He then continued to say that most people are living a normal life span. I replied by saying hopefully, although I wouldn't say 37 was a 'normal' life span. He continued talking and this is what really ruffled me,

"All Cystic Fibrosis is these days is a couple of niggly chest infections, it's manageable and not the worry it once was".

Excuse me? Manageable, maybe to an extent but not a worry? NOT A WORRY?
I wish that all Sophie had to deal with were a few niggly chest infections, that would be AWESOME. Can someone tell me which Cystic Fibrosis this is, I'd like to trade now.

Luckily Ian's consult was over at this point otherwise I may have had one of my 'speech' moments. They don't happen very often as I'm not a real believer in being negative and ramming information down peoples throats but I came very close at that moment.

It wasn't that he had little knowledge, it was the fact that he was a doctor who was having a verbal diarrhoea moment and the 'facts' that he was quoting were quite flawed. I'd love to find one family affected by Cystic Fibrosis that can honestly say it isn't a worry and know their secret.

Normally I wouldn't waste energy on this kind of thing but I guess it's because we are desperately trying to raise awareness to keep the money flowing in for the Gene Therapy trials, and for a medical professional to spout such utter nonsense just felt like a huge slap in the face.

If this is what the health professionals think, what hope have we of convincing the rest of the public that Cystic Fibrosis is a SERIOUS and WORRYING disease which still takes young lives?

Needless to say, we left quite sharpish.

On a brighter note, this blog turned one last week! Yes I have officially been blogging for a year and must thank everyone who bothers to read my ramblings and actually come back for more! THANK YOU.

I will leave you with a pile of pictures of stink bomb having fun in the sun :)

Sophie launching a ball at my dad..

Sophie cheering because she managed to hit him with the ball..

Remember the bean plant Sophie came home from pre-school with? Well this is it!!

Look, it even grew beans, she's so proud of herself...

Tunnel time..

A few minutes time out to have her milk feed via her Mic-key button...

Seriously this girl never stops running...

Playing a tune on her new Thomas guitar...

Posing...

Take care all and please read my previous post and help out the CF community if you can. Many thanks.

xxx

Please help!!

2011-07-08T14:42:00.002+01:00

Meet Sophie.

You could mistake her for being like any other typical four year old. She's sassy, bossy, loud, messy, has a wonderful imagination and loves playing with her friends. She starts full time school in September and already loves pre-school. She has a wonderful heart and will always try and help others. Her favourite toys are her Thomas the tank engine trains, peppa pig toys, her teddy Leeber and anything she can create something out of.

However, Sophie isn't like other four year olds, she has a ticking time bomb inside her that isn't always obvious to most people.

At four years of age, Sophie shouldn't have to endure hours of treatment everyday just to stay healthy or to try and minimise the damage from frequent lung infections.

At four years of age, Sophie shouldn't know what a nebuliser is.

At four years of age, Sophie shouldn't be able to name all of the medications and KNOW what they are for.

At four years of age, Sophie shouldn't know she has to stop playing to sit for up to an hour twice a day having nebulisers.

At four years of age, Sophie shouldn't have to endure pummelling and patting to loosen thick, sticky mucus from her lungs twice a day for half an hour.

At four years of age, Sophie shouldn't be mopping up her own mucus that she has coughed up so somebody doesn't stand in it.

At four years of age, Sophie shouldn't know what a port is and give it a 'pet name' (hers is called Bertie).

At four years of age, Sophie shouldn't have to miss school every month to go to hospital and have a needle shoved into her chest to flush her port, ensuring it remains working as her veins are scarred and damaged due to frequent IV access.

At four years of age, Sophie shouldn't know the names of all of the nurses on a hospital ward.

At four years of age, Sophie shouldn't have to have Intravenous antibiotics, three times a day for a minimum of 14 days for lung infections.

At four years of age, Sophie shouldn't know what a feeding tube is and be able to assemble the giving set to hook her up to milk.

At four years of age, Sophie shouldn't know the whole route to the hospital she attends, which is 20 miles away from our home.

At four years of age, Sophie shouldn't be able to assemble three different nebulisers.

At four years of age, Sophie shouldn't have to deal with chronic constipation that can leave her screaming in agony some days.

At four years of age, Sophie shouldn't have to stay indoors because the medication she is taking for an infection causes hyper sensitivity to the sun.

At four years of age, Sophie shouldn't be worrying about going to hospital and be scared because she knows what is going to happen.

At four years of age, Sophie shouldn't know that a chest xray is a picture of her lungs and will show if anything is wrong.

At four years of age, Sophie shouldn't be cheering because she has managed to produce a good sputum sample.

At four years of age, Sophie shouldn't know the words CYSTIC FIBROSIS, and be able to explain that she has it and what it is.

Sophie was born with Cystic Fibrosis. With help and luck, she may reach 37 years old.

The Cystic Fibrosis Trust needs your help. Due to cuts and the recession money is tight and the Gene therapy consortium needs to find 6 million pounds to continue its work. This is including a multi dose trial next year, which will help pave the way to finding a cure for this devastating disease.

See attached letter asking us as families to help with donations from the CF Trust, which we have all contributed. As a government, please consider helping this vital work continue by raising awareness of Cystic Fibrosis or allocating funds. Please give hope for our children and the many many people affected by this awful disease.

For once you could make a real difference.

This is our letter which will be sent to the Prime minister, David Cameron.

Another parent has asked anyone affected by CF to write a short account of what living with CF is like. Can you help??

For once we aren't asking for donations, just experiences to hopefully get our government to take notice of what living with CF is really like, learn about the vital work being done, to raise awareness and possibly gain some funding for the CF Trust.

The next phase of the gene therapy trials needs to go ahead next year. This could have a huge impact on CF across the world. See here for more details. I know I'm very keen on the Vertex drugs and believe that this will be the future for Sophie, BUT I still believe strongly in Gene Therapy and know that this is the only real shot at a cure for CF.

If any of you have a spare 10 minutes, would you be able to write a brief note about what life is like living with CF, it doesn't matter how you write it, it can be a paragraph or pictures etc and send it to feedingproblems@gmail.com. I will then print them off and send them to the parent who is collecting them all.

You can remain anonymous if you choose and it doesn't matter if you are not in the UK. Parents, grandparents, aunties, uncles, siblings and of course people with CF are all needed.

Please share this page on your own blogs, twitter or facebook pages to spread the word.

Many thanks and please consider helping us to help the CF community.

xxx

Silver lining.

2011-07-05T15:20:00.001+01:00

Hugely slacking with updates recently, apologies!

Left you all on a bit of a cliff hanger too. Well the orals did the job and little miss is now fully back to her happy self with little to no cough.

Soph had her port flushed last week which went okay. She's still a little tearful again but it was done and her CF nurse has a great aim :)
Bertie (sophs port) turned one in June also. We know he wont last forever but very pleased he has survived his first year and long may it continue.

We have been to CF clinic today and I have to say it went beautifully. Soph cooperated like an old pro and didn't refuse anything.

- Weight has increased a whopping 1kg, she now weighs in at 16.8kgs.
- Oxygen saturations were 100%
- Height is a bit variable depending on how well Soph stands but I think a small increase was there.
- Chest sounded wonderful, no signs of crackles or pops.
- Temperature fine.
- Stomach nice and soft.
- No signs of clubbing to her fingers.
- Awesome cough swab taken so I'm sure she'll grow something!

The only change to be added is some fibre to her milk. We had a small issue last week where Soph developed a partial blockage again and was complaining of tummy ache and although she passing poop fine, it smelt very stale and old and was very bitty. All classic signs of her being blocked.
We increased her laxatives and she was soon flowing well again but I'm concerned that we cant get her weaned off the laxatives and it has been a very long time. As she is pancreatic sufficient, she shouldn't really be getting too many bowel problems and after talking to her dietician, we discovered her neocate milk doesn't have too much fibre in it.

Worth a shot right? We will add some powdered fibre to her milk in the hope it will give her bowel a more normal action and help it work a little better. Hoping then we can wean the laxatives off.
When we get the prescription and start it will let you know!!

I asked what age PFT's will be introduced and was told around age 5 or 6 if she doesn't cooperate. It'll take her a while to get the technique right but will be a vital tool in knowing how the windbags are behaving.

We also saw her feeding psychologist and dietician last week and basically they are pleased with her eating progress and will re-review once she starts full-time school in September. It was kind of strange not having to speak for Sophie when they asked the usual question of what foods she likes. Sophie interrupted me and reeled off everything she likes to try. My goodness this girl has grown up FAST!

So all in all, everything is looking fairly good again for Soph. I on the other hand have a trapped nerve in my neck and having to take time off work due to the pain currently. Wish I knew what I had done as it just went twang and hasn't been right since. Ian reckons it's the 30 year curse. As soon as you hit 30 your body temporarily falls apart :D

I will leave you with some photos of Sophie's first sports day at school. She LOVED the running races, however it was a very hot day and she doesn't fair well in the heat. She had a very prominent salt line where her hat had sat!

I was limited as to the pictures I could show due to cutting out other children. I have no skill in editing pictures so you will have to make do with what's below :)

Hockey baby!!

Obstacle course...

The running race where she had to scoop up a bucket and spade...

This was a parent and child race and as I couldn't take part due to my wonky neck, Ian took part with her. I have to say the first attempt he managed to trip her up but the second time went well!

Waiting to throw bean bags into the hoop...

She soon mastered it well...

And yes, her school are insane letting her loose with a large weapon...

Take care all and it has been lovely receiving emails from parents who have found this blog helpful. I partly started it as we couldn't find anyone who had been through similar to us when really could have done with having someone to talk to. If we can help in anyway, please get in touch :)

Much love!
xxx

Poorly bean.

2011-06-20T17:56:00.002+01:00

Well so much for summer being round the corner is all I can say.

I dropped Soph at school only to receive a phone call to say that they wanted me to know that she had been coughing and retching but she was now fine. Her teacher informed me the other kids must think she is mad because she was really praising Soph for aiming her coughed up gunk into the bin and not the floor. Bless.

When I picked her up and she seemed fine in herself but over night she began coughing and by morning she was coughing, retching and clear gunk was flying all over the place.

Normally I would give her a couple of days to see how things are going to go but due to the fact she had no cold symptoms just a wet, barking, productive cough I was a bit out of my comfort zone to just leave it.

I don't like coughs that literally strike like lightening, there was no warning at all. Bam, and Soph had become a sputum fountain with a good aim!

After chatting to one of her CF nurses and a consult with her CF prof, orals have been called in. As I was on the phone, they could hear Sophie in the background it was that bad.

To the rescue...

So two weeks of orals and review after one week if no improvement. I'm guessing a pseudo exacerbation, however I'm hoping something viral may have triggered this otherwise IVs may be looming in the future if the drugs don't work fast.

I guess we can become too complacent during a long good spell. I forget sometimes just how quick infections develop and I just feel like telling CF to f**k off at the moment. It's supposed to be summer you know, the one time we can normally relax a bit.

I also turned 30 last week but wasn't going to let it overshadow the skydive as that was way more important :)
I was proper spoilt though, promise.

For anyone who missed my last post and may want to watch Ian's skydive click here to view it, let me know what you think!

Again thank you all so much for all of your support and well wishes. Total raised so far is over £1500 which is way more than we ever expected.

Take care all.
xxx

Don't look down! (updated with video link)

2011-06-13T09:51:00.006+01:00

He did it and survived ;)

My wonderful husband Ian did his first tandem skydive in aid of the Cystic Fibrosis Trust today at 08.45 and had an absolute blast.

Ian just about ready to walk to the plane...

Certificate of confirmation from the skydive centre...

Certificate of confirmation and thanks from the CF Trust...

The money shot :D...

A huge THANK YOU to everyone who has already sponsored Ian, we have exceeded our target and we are so very grateful for every ones generosity. There is still time to donate on our Just Giving page for 3 weeks if anyone would still like to.

Link to our Just Giving page.

Full video of the skydive is now available to watch here! Enjoy :)

PS Look out for Ian trying to say hello to Sophie and also his instructor looking like he was wrestling him at one point :)

Roll on Skydive 2012 when Ian will take to the skies again along with me. Yes, next year as promised I am going to do it too!!

Take care all and thank you for all of your lovely good luck messages.

xxx

An update of sorts.

2011-06-10T16:11:00.001+01:00

Thought I'd better give a quick update to you all :)

Firstly I must say a huge thank you for all of the messages related to my last blog post. I have decided to keep the blog public, for now at least so you're stuck with us for as long as you can bare it!

Sophs port flush went well, not as smooth as the previous but she was good after a few tears. On the bright side, sedation wont be needed again now so I'm super happy about that.
Sophie seems to be showing more interest in eating lately and is asking for more things to try than she ever has.

Ice cream is still her favourite though!

I know I'm naughty but I was curious so decided to dump her on our scales and she is now up to 16 kgs. Ours run the same as her clinic ones so the weight is always spot on so I'm happy with that too! Just hope she can keep on increasing between now and clinic.

She's getting her chub back since we upped her milk intake via her button.

It's one area we don't normally have to fret about but seeing her drop so much weight was very difficult and it was harder to accept because we had partly caused it with her tube wean. It's a terrible catch 22 but we seem to have found the right balance now and I'm happy with where we are at. We see her feeding psychologist and dietician in a few weeks so we shall see where we go from here.

At her port flush we managed to get Sophie to do a great cough swab. If you remember my last post, her previous culture had only a 'mixed growth' and no pseudo showing. I wasn't massively keen to rely on that result as she hadn't had a clear culture in nearly two years.

There are lots of schools of thought on Pseudomonas, some feel that once pseudo gets in, it'll never go and a few clear cultures do not mean it has gone. However there are others that say it is able to be eradicated and there is proof everywhere if you talk to others with CF who haven't grown it for years.

I am a realist though and am hoping the clear culture meant her pseudo numbers had decreased. Her usual growths are 'moderate' but when I eventually got the call from one of the CF nurses, her culture had grown Pseudomonas but the growth was only very very light.
This I AM pleased about as something is changing and Pseudo's numbers seem to be reducing at the moment. To reiterate, I really don't think we can clear it but I'm hopeful that we can maybe reduce the numbers further so it only shows from time to time and isn't too much of a troublesome visitor in Sophs lungs.

Only time will tell I guess.

This Sunday is the day Sophie's daddy will jump out of a plane. I cannot believe the skydive has come around this quickly but we have gone past our target of £1000 and we are beyond happy. We'd like to extend our thanks to all who have donated, tweeted, facebooked our link. Everyone has been so generous and we are so very grateful.

There is still time to donate or spread the word for us if you are able. The research and gene therapy in particular here in the UK are under threat due to financial issues and currently every penny counts now to help the future for people living with CF.

Click here to donate or share our link for us.

I will be in touch after the skydive on Sunday, wish Ian luck!! I hope the weather holds out!

Take care all and thank you all again.
xxx

Big brother is watching?

2011-05-30T12:53:00.003+01:00

Missed us?

I guess now is a good time to explain our two week absence and for once it isn't because Soph has been poorly.
First things first, Sophie had her four year annual review a few weeks back and it went great.

- Weight has increased from 15.1kgs to 15.8kgs, so the milk increase was what she needed.
- All her bloods were normal and apparently she has great Vit D levels.
- She allowed her Dr to listen to her chest, which was clear and also examine her tummy which was soft.
- Chest Xray was great, only the smallest of change to her lower right lung but that is normal with anyone with CF, and her Dr concluded that the Xray was normal unless he was being super picky.
- Soph finally allowed her oxygen sats to be taken and they were 100%.
- Cough swab was done well.

So all in all a fantastic annual review and her team are very happy with her. We just need to get a stool specimen to send for faecal elastase to check that her pancreas is still functioning as it should.

Back to the cough swab.
I phoned for the results and was a little shocked to hear that she hadn't grown anything other than normal flora type substances. No Mr P.

Before we all get excited, Soph is colonised with Pseudo, we don't ever expect her to not culture it and just because it doesn't grow, doesn't mean it isn't there. However, this is the first culture in nearly two years that Pseudo hasn't shown and I'm happy.

I have no doubt that the sample was good so I'm just hoping it means that Mr P's numbers have reduced a little and that I WILL take. Maybe explains why Soph has been so well lately?

We are going to re-culture when she has her port flush tomorrow though so my happiness may be short lived!

Right, on to why I haven't blogged in two weeks..
At the annual review Lung Behaving Badly was mentioned.

This blog.

Sophs dietician had been googling something and our blog kept appearing in the search and when she clicked the link she realised it was us.
It was brought up when they had their MDT meeting before seeing us and Sophs consultant talked to me about it when he saw us. Nothing negative was said and they actually praised it especially my post on the vertex drugs but obviously more of the blog has been read.
Fortunately for us, we think Sophs team are excellent and they know this otherwise it may have become a rather uncomfortable conversation but still, I needed some time to decide whether I was going to make the blog private with invitation only.

This blog is not only a journal or a point of reference for us, it is also my outlet to get my feelings out when I need to and occasionally that means a good vent too.
I have decided though, that the positives out weigh the negatives for us to keep the blog public. Without it, we wouldn't have the support from you all, support that without this blog we just wouldn't have. It's the only way in which we can really interact with other CF families and also people with CF and I refuse to let that go.

I doubt the team will be avid readers but I'm sure they may pop round from time to time, human nature?
I reassured them that no names were ever mentioned and I don't think I have ever mentioned which hospital she attends either. Her consultant actually joked that he'd have to consider getting a super injunction out :D (seeing as they are so popular at the moment).

What would you guys do? Luckily our blog doesn't revolve around her team, it's just an area that has to be mentioned from time to time, but a small part of me feels like we have been rumbled and I don't know why I feel like this?
Are other bloggers open with their/their child's team about their blogs?

Interested to know your thoughts on this.

I took this picture of a rainbow whilst driving home from Sophs annual review, it's camera phone quality though and I was driving at he time! You cant tell from the picture but it was a full rainbow and very beautiful.

PS Second lot of Thomas pictures to follow and we have also been to Cornwall for a weeks holiday so more to come on that too. Port flush tomorrow also.

Hope everyone is well and will be catching up on you all throughout the week.

xxxx

**For anyone struggling to leave comments due to blogger having a hissy fit, when it asks you to sign in, un-tick the keep me signed in box and it should allow you to post comments. It's working for me after writing out huge comments for people and then it not allowing me to post them! Grrrr. Hope that helps!**

Birthday fun part 1.

2011-05-10T14:03:00.002+01:00

Well Sophie is now four years old :)
We gave her the choice of how she would like to spend her birthday and knowing she isn't the girliest of girls, we guessed she would choose Thomas Land again(at Drayton Manor park) in favour of a party.

So Thomas Land we went!

I had to include a couple of pictures of her birthday as we went to Thomas Land a few days before the actual day as she had school. She had the HUGEST balloon going which amused her immensely. She is also wearing her gift from Gem which she absolutely loves so thank you again hun!

Sophie did end up having a party at her school as her birthday was the day before the royal wedding, they were already celebrating so we sent lots of food up and her cake and they had a great time.

My dad and step mum came with us this year and between us all we took over 200 photos, but relax people, I wont inflict them all on you. However, I will post a few blog posts to get across some of what we got up to.
Any Thomas fan would love this place it's so much fun and the rest of Drayton manor has plenty to occupy the big kids too!

Life size Thomas!!

Here is Rosie who is Sophs favourite at the moment, you can see some of the characters performing on the balcony, which was interesting!!

The fat controller!!

and the thin controller (non of us knew he existed and I'm convinced he was made up and he sang...alot)

Sophie on board Rosie, her face says it all.

Percy, who used to be Sophs favourite character, he was out of action or as Soph put it, having a rest.

We had a blast and look out for the next installment, lots more to show you all!
Thank you to everyone for Sophie's cards, presents and birthday wishes. You all contributed to making her birthday awesome this year.

To be continued...

VX770 first hand amazing experience.

2011-05-09T13:14:00.002+01:00

Just saw this on our CF forums and had to share for those that may have not seen this. It made me cry!

Amazing

Enjoy! Let me know what you think and if it affected you like me :)

(birthday photos coming this week!)

X

Happy birthday Sophie.

2011-04-28T00:01:00.001+01:00

On the 28th April 2007, Sophie Ella arrived at 18:20 weighing 2.25kgs (4lb, 13oz).
What a roller coaster of a journey it has already been.

Happy 4th Birthday sweetheart, we love you so very much (I'm struggling to believe she is 4!).

Sophie, 16 hours old...

Lots has been happening this week and I will blog in more detail soon about how she wanted to spend her 4th birthday this year.

Here's a sneak peek...

Take care all.
xxx

(sorry for the lack of posts on here and comments on your blogs, blogger has been majorly playing up for us but I am informed the issue has been fixed now, lots to catch up on).

Cheese.

2011-04-19T08:30:00.000+01:00

Well today was very interesting. Soph had her port flush and we took my mum along for the ride. I tried to mentally prepare her for how Sophie thrashes around still and that we still have to restrain her but once it is done, she's fine.

Sophie was in great spirits in the car and didn't appear too concerned about Bertie having his drink.
We arrived on the ward and went out to play for a while until her nurse was free.
After a short walk into a neighbouring ward, Sophie started to protest a little. I sat her on my knee and she allowed another nurse to remove her dressing that was holding the emla cream over her port to numb the area.

That went well.

Then her nurse grabbed her 'magic cleaning sponge' and Sophie again allowed her to clean over the skin above her port.

Again that went well.

Then onto Bertie's drink. Sophie put her own arm behind her back without us having to get all police arrest mode on her (she has to do this as she has a bit of blubber and it makes it hard to access her port otherwise).

Starting to think this is all too good to be true.

Her nurse then grabbed the needle, grabbed the port and Bertie was accessed without so much as a murmur from Soph.

Total shock and the best flush she's ever had. Not a single tear.
Soph sat there like an old pro and looked at us as if we were all mad. She could obviously see that myself and her nurse had our jaws dragging along the floor in disbelief at how well she had just behaved.

Result!

Due to how well she was cooperating, her nurse thought it may be a good time to test the waters with a chest xray. Bearing in mind that Sophie hasn't allowed one without sedation in a very very long time.

Remember the incident where she kicked the radiographer in the head and she practically threw us out of the department?

To say I was apprehensive was an understatement but we thought we'd roll with it.
To cut a long story short, Sophie stood in front of the film playing with a scooby doo toy, saying 'cheese' and allowed not one but two chest xrays to be taken.

Yes you heard right. No protest, no tears, NOTHING.

I am now one very proud and relieved momma. I think we may have turned one massive corner regarding Sophie's paediatric medical traumatic stress issue. Maybe it's age, maybe she is just getting used to it all now as she is able to understand what's occurring.

I just want to give hope to any parents who are struggling with similar. It really does get easier with time and a lot of work. I never imagined Sophie being like this and for the first time came home with no emotional or physical exhaustion.

My daughter rocks big time and is growing into a 40 year old :)

She is turning 4 next week too (28Th). There was a time when we honestly didn't think she would make it to her first birthday and I cannot believe where the time has gone. She is just like any other child her age but just happens to have CF and I hope this continues.

She is going to make a great teen, she already has the stroppy pose down to a tee!

Luckily though she still has lots of innocence and cuteness left...

Take care all.

xxx

The one with all the photo's.

2011-04-11T19:25:00.002+01:00

Thought i'd better give you all a quick update again. Reason for lack of blog posts is we have been having lots of fun and Sophie is really well at the moment. Yes, I DID just say that :)

The increase of 100 calories per day to Sophs feed seems to be doing the job. She's back to her usual self and is starting to try more oral foods which is just what we wanted. Obviously a lot will depend on her weight at clinic in May and if she has still lost weight then we may be increasing it further.

Port flush is due any time over the next week and we are just waiting for a confirmation on the date. Will let you all know how we get on.
Other than that, all is looking good this way for a change so I will bless you all with a Sophie fest from one of her days enjoying the English sunshine!

Mmmmm, ice lolly

The fluffy object on the trampoline is Lulu, Sophie's pretend cat not a real one!

This makes me laugh, this is Sophs 'just about to launch into the air' face.

Almost up (A health and safety officer would have a field day with this!)

And we are up!

This is Sophie's wooden house, which she loves.

Okay I admit it, I was spying on her through the window.

This is how we keep our sanity from time to time! (seriously I am kidding)

Emptying out her 'stuff'

Blossom picking much to my horror. Majorly allergic I am but what the heck, she was happy.

I will be catching up on everyone else this evening. Hope you are all okay and I promise I wont leave it as long next time :)

Take care and thank you all for your comments. I love reading them.

xxx

To fatten or not to fatten...

2011-04-01T13:51:00.002+01:00

That is the question.

Following on from my previous post, I spoke to Sophie's 'feeding' dietician yesterday. We talked for a good while, batting back and forth what to do. There were options and I like options...

Option 1 - leave well alone, which neither of us thought was even an option.
Option 2 - increase her feeds by a hundred calories and add this extra to her last feed of the day.
Option 3 - increase feeds by a hundred calories and add to last feed of the day but move around her day time feeds so she doesn't have such a huge gap in the day.
Option 4 - Re-introduce the bolus feed we culled last year.

We are trying out option 2 for now. Leave the times of her bolus feeds the same but just add in extra on her final feed. The thought is she has had a growth spurt, which is likely due to her shooting up in height and finally fitting into certain clothes now.
The growth spurt has obviously upped her metabolism and she has burnt off much more than she normally would, she is also super active and the two combined has made her feel starving and lose weight.
This has had a negative impact on her oral eating and all she can focus on is getting her tummy full via a tube feed as this is her normal.

When you or I are hungry, we pick up something to eat via mouth. Soph doesn't have this association due to never really eating or drinking orally so her cure for hunger is a tube feed.

We will try option 2 for 2 weeks and if it isn't enough for her, we will try option 3 and close the gap between her tube feeds. If this ultimately fails, we will add more calories to her feeds by possibly adding back in the feed we culled last year.

Lots of ifs but it's going to be trial and error. Soph is back at CF clinic the beginning of May and that weight will be so important and determine whether or not she needs her milk increasing further.

Neither I or the dietician want Soph to lose any more weight as she is now just perfect for height/weight, which is great but this means she has no reserve left in the tank should she need it.

The slimmest we've known her since her nissen fundo op..

School had also noticed her behaviour had changed, they said she seemed more short tempered and grizzly. Once I explained what was happening (she's hungry and this is her classic way of expressing this) they were on the same page. They now know the plan and are monitoring her closely to see if things improve or stay the same.

She already seems more content but it's early days. She has been waking up very late at night and crying to have a tube feed, it's been awful at times. Last night she was more settled and today she's happier for it. I'll keep you all posted.

Sophie came running out of nursery today beaming and had a gift for me...

She shouted 'happy mothers day mommy'.

She's also finally started to take an interest in trying to write her name...

Please pop by Margaux's blog who is Bens mom and watch this video. I have to be honest I balled like a baby but it shows the importance of raising money and the reason we all work so hard for our kiddos. Little Anna is so strong and beautiful and her family are amazing.

Link to our fundraising page for Ian's skydive.

Take care everyone and thank you all again for your advice and support.

xxx

Dear TOF, I'm beginning to loathe you.

2011-03-22T18:18:00.001Z

I know we haven't had to really mention you for a long time but you appear to be causing trouble again.

Time to touch on Sophie's 'other' issue.

It's more to do with her eating side or lack there of if I'm more truthful.
If Sophie hadn't been born with a TOF, she wouldn't have had horrendous reflux resulting in her oral aversion (phobia to eat).

We started her tube wean September/October of last year and although she has made huge progress, she still isn't taking anything substantial orally. She is still just tasting things, licking and nibbling, but not really eating anything as such. She hasn't had her milk increased since summer last year, just decreased and she has done really well to actually put on weight in that time.

Last CF clinic showed that although she had grown a huge 3cm, she had lost a fair few kilograms of weight.

The problem I'm now facing or call it a personal battle of what is right and how should we play this, is where do we go from here?
As hard and disappointing as it is to admit, I am feeling that we may need to add some more milk to her tube feeds again.

I'm trying not to look at this as a total failure but it is disheartening. The reason I've come to this conclusion is not only based on her weight loss. Sophie is physically hungry of an afternoon, I mean REALLY hungry. Her stomach is growling, she is becoming grouchy, which is her way of dealing with hunger and she has been asking for her bolus tube feed up to 3 hours before it is due.

Although her stats say she isn't underweight, physically she is looking skinny, her port is sticking out and she has bones poking out from her hips and shoulder blades.

I have emailed her feeding dietician for advice and to air my concerns. We are so lucky Soph is pancreatic sufficient so she doesn't have too many problems gaining weight, I honestly don't know how we'd even consider tube weaning if she wasn't. However, she still needs weight on her and definitely some in reserve in case of a lung infection. She has always been on the chunky side and I attribute her good health thus far to this and I do not want to risk her lungs suffering due to us being keen to get her off her tube feeds quickly.

It sucks but I guess we have to wait and see now what her feeding team want to do. They may be happy to leave her for the time being, especially with the warmer weather round the corner. She's so flipping active she is burning off so much more now.

I know deep down what we need to do and it is gutting but she may only need a slight increase to curb her hunger. It was hoped that in causing her to be hungry it would trigger her to try more foods but she's still not totally at that stage yet.

When I know of the plan I will let you all know.

On a brighter note, we have been awarded an inspirational blogger of the week award over at Jenelle's blog Single Mom Inspirations. I've linked to her blog before as she shares some amazing inspirational quotes and her blog always leaves me with a nice feeling of calm and happiness. Please pop over and say hello.

She told me that "I created this award to recognize those blogs that I find are inspirational & encouraging". I am really honoured and humbled that someone finds our blog inspiring and makes my rambles worth while :)

It's one I don't have to share like others and it means a lot to us.

I do have other awards to dish out and I'm still writing that post, so keep tuned to see if I'm spreading the love in your direction ;)

I'd also like to take this opportunity to thank you all for your continued support and awesomeness especially regarding my last post. Thank you for your comments, emails and texts it really has meant a lot and I feel less discouraged and resentful of others who are naturally selfish.

Take care of you and yours.
xxx

I'm not superwoman.

2011-03-18T19:52:00.001Z

Does anyone else feel that they paint on a happy face twenty four hours a day, even when you are not completely feeling, well.. happy?
The mask you wear can sometimes slip and those closest to you notice, or you hope they would.
I try to remain positive all the time as I hate too much negativity, don't get me wrong I like a good moan, but who doesn't eh?
I'm always there for others, especially my closest friends day or night, so is it selfish for me to sometimes want the same support return?

I rarely let on how I'm feeling, I'm really good at hiding most things and work through them on my own, but when my mask does slip, I would like to think that friends would notice. I don't necessarily mean just CF problems, just all things that can crop up and knock you sideways.

My closest of friends always notice, but I sometimes feel let down by others who I consider to be 'almost' close friends. I find this disappointing especially when I have been there for them, many many times, even during hospital admissions for Soph my phone is always on.

On the odd occasion I may drop a huge hint, it doesn't get acknowledged and they manage to turn the conversation back to them.

I guess people get caught up in their own lives and they can be more selfish than I have ever imagined. I know some of you fellow bloggers have touched on this subject before and I always thought I was one of the lucky ones to not experience such things. I guess I'm not so lucky eh?

I am fine though promise, just hate being let down and finding out people aren't who you think they are. I should stop being so nice maybe?

It seems to work for everyone else!

On to Soph...

Port flush was done on Tuesday without sedation, go Soph!
She was a little more animated than previously but after five minutes just sat still. She had all of her annual bloods taken at the same time as I refuse to let her be accessed just for bloods.
We didn't bother attempting an xray, she just wouldn't have coped but it's something we will try and work on.
She's super bright at the moment no cold appeared so we can breathe a sigh of relief, however we are on bowel watch though because we are slowly reducing her laxatives to see if we can get things a little more, err, firmer :)

She had to go to school in her pyjamas today for comic relief (national fundraising day for those that may not know). She was NOT impressed, she kept asking me over and over why and if she was really going to hospital to sleep. I guess she still has some anxieties and random things trigger these memories.
She ended up having a great day though and we may also have to surgically remove her space hopper as she is on it all the time, which is great except for when she needs a bath!

Both Ian and I have had hospital appointments for issues but all okay, except for Ian's knee but that is a whole other story!!

Will leave you with a pic of Soph who managed to get a supermarket manager(also pictured) to fall in love with her and allow her to 'go behind the scenes' of the bakery section. They also gave her a little paper hat and she had a blast :)

Thanks for listening to my mini rant, here is the best place to get it all out.
Hope everyone is okay and apologies for my lack of blogging, life has gotten in the way.
Thanks for all of the lovely comments too.

Take care.
xxx

There's something about Sophie...

2011-03-08T20:49:00.001Z

Well I thought I'd better give you a quick Sophie update, wouldn't want anyone to get withdrawal symptoms :)

She's doing okay, I say okay as she isn't completely herself at the minute. She seems more grouchy than normal and this sometimes can mean something is brewing. She's not particularly chesty, although she is clearing much more crap in the mornings, no cough to shout about yet either.

So for now we will just watch and wait.

Her port flush is booked for next Tuesday, I cant believe it has already been three weeks since the last. This is the real test to see how she copes without the narcotics ;)

Last time may have been a fluke or she may kick off knowing what is going to happen now that she is 'sober'. If she happens to be calm then we will also try and get her up to xray for her annual chest picture. Just to remind you all that she hasn't had one done without any form of sedation for over two years. Things may get very interesting I can tell ya!

I'll be needing the positive vibes I think.

Recent cough swab cultured pseudomonas. She's colonised, it's pretty much going to be there every time now so no major shock or emotional outbursts from me.
On the bright side, nothing else has shown up and she already has treatment to dampen Mr P down.

Picnic group went well, it was the last of her sessions and we got really messy by making pictures out of baked beans, jelly, custard, and a few other added extras and then driving toy cars through it all.
LOADS OF FUN, I highly recommend it :D

On a serious note it is all part of the actual therapy to help her oral aversion. She did get her hands in the mess for a short while, which is a huge step forward to how she would have reacted a year ago.

The sessions have been brilliant and Sophie actually has progressed. We are still trying to teach her how you actually chew food properly, but again this will take time and we are not prepared to rush things.

If you still want to read my post on the Vertex drugs, look up, I've put it as a stand alone page and please if you haven't already, would you sign the petition to stop the closure of vital services at the Brompton hospital. Link can be found on my previous blog post or on the widget over on the right.

Thank you to everyone for all of your comments, they've been great to read.

Will leave you with a few pictures of Soph goofing about on her space hopper.

Look at me!!

No really, look at me!!

BOO!! This one really makes me chuckle, I know it's all blurred but I think it's the pigtails that does it...

Sophie insisted I post this picture again, she really was proud of this sunflower...

Take care everyone
xxx

Your help is NEEDED!!

2011-03-06T21:32:00.002Z

On behalf of everyone in the UK affected by CF, we are seeking your urgent help.

Our government is threatening to cut significant services at The Royal Brompton Hospital. Although this isn't Sophie's centre, if these cuts were made it will have a knock on affect across the country. This doesn't just affect direct care, it will also affect the huge amount of research the hospital does towards CF care, both nationally and internationally.

Please can you take just 2 minutes to sign the petition. It doesn't matter if you aren't a British citizen as there is a box on the form to select your country.

From the CF trust website..

Threat to paediatric CF service at Royal Brompton Hospital

Following the recommendations of England's Joint Committee of Primary Care Trusts to cease children's cardiac care at Royal Brompton Hospital, Royal Brompton & Harefield NHS Foundation Trust is mounting a strong campaign to challenge the recommendation. Should the recommendations go ahead, the hospital could not sustain its children's intensive care or anaesthesia services - making provision for paediatric cystic fibrosis patients inadequate.

The CF Trust considers the paediatric CF service at the Royal Brompton Hospital to be vitally important in terms of the standard of care provided, its strong research base which has both national and international recognition, and also for the development of guidelines for the care of children with CF which are used throughout the UK and referenced on many of the CF Trust documents, including the recently launched CF Clinical Care Pathway. In addition it is unfeasible that the 300+ patients currently cared for at RBH could be easily or readily accommodated by any of the other London based CF specialist services without significant investment and expansion, neither of which seem to be an option in the current climate.

The public consultation began on March 1 and we would urge people to make their voice on this issue heard.

Click here to sign the petition. If you could link to this post on your own blogs I would be very grateful. Clearly our government have gone mad!!

Many many thanks.

x

Epic post-VX770/VX809. My take on it.

2011-03-02T12:32:00.006Z

***I gave up on the title for this once I saw how long this post actually is. Apologies in advance and if you make it to the bottom you deserve some sort of consolation prize***

There has been a huge buzz in the CF community for some time now but last week some results were announced that could potentially change the future for our little ones and the many many people living with Cystic Fibrosis.

Vertex have been trialling a new drug that could actually fix the route cause of CF. I say 'fix' loosely as it isn't the full cure but it's pretty damn good.

The drug is VX770.

Now there are many drugs in the pipeline all the time but not many get as far as this one has. So this is huge and has definitely been one to watch with anticipation.

Here's the science bit..

To understand how this drug will work, you have to first understand the underlying problem in people with CF. I will do my best to explain (with a little help from external sources)...

Salt is made up of sodium and chloride and our bodies are very clever at regulating the level that wont cause problems and death. In people with Cystic Fibrosis, their bodies cannot transport chloride properly. This causes a build up in the body and the only reaction their bodies can do to balance out this overload is to increase production of sodium and therefore, water doesn't get distributed as it should.

This is the reason that when a person with CF sweats, they taste very salty and kick out much more salt than someone without CF.

However this is not just happening on the outside, it's affecting the inside too.
The cells that line the lungs and airways, digestive tract, sinuses and other organs can all be affected, because the body cannot regulate the concentration of salt properly resulting in the thick, sticky mucus that causes so many problems in people with CF.

So what does all this have to do with genes huh?

Well the gene that regulates sodium and chloride transport is known as the CFTR gene (cystic fibrosis trans membrane conductance regulator). The CFTR gene provides the information needed to transport sodium and chloride ions across the cell membrane. This in turn controls and regulates the flow of water in mucus, sweat, tears, saliva, and digestive enzymes.

The CFTR gene doesn't function properly in people with CF because they have inherited TWO (sometimes more) mutations of that gene.

It gets (more) complicated though as there are over 1500 known CFTR mutations and they are grouped into six categories. Each category upsets the CFTR gene differently but all cause Cystic Fibrosis, just in varying degrees.

So will VX770 fix all of this?

The answer in simple terms is no.

VX770 has been developed to target ONE specific mutation known as G551D which is a class 3 mutation (remember the categories I mentioned earlier).

In this particular mutation of the gene, the chloride is able to travel a good distance a long the tunnel of the cell but it hits a dead end as the gate is firmly closed. VX770 has been developed to open that gate and therefore allowing the CFTR gene to function as it should.

The results are out and they read very very well, so well in fact that I got rather excited when I read them. Sweat chloride levels had dropped to NORMAL ranges, little to no chest exacerbation's whilst taking the drug, weight gain and also great improvements in lung function.

This is huge.

So where's the catch?

I hate the fact that there is a catch but it's there with huge flashing neon lights I'm afraid. It has to be approved firstly and then licenced, and most importantly it will only be of any real use to 4% of people affected with CF. It also cannot reverse any damage already caused.

Sophie isn't part of the 4%.

Her mutations are DF508, which is a class 2 mutation and D1152h, which is a class 4 mutation.

This huge breakthrough will pave the way for future drug developments that hopefully one day, will target all mutations.

The exciting news for us is that Vertex already have another drug in the pipeline called VX809, which has been designed to target specifically the DF508 mutation, which would help Sophie.

The trials haven't been as promising as the 770 ones though, but they have been trialling VX809 in combination with VX770.

The problem with the DF508 mutation is the chloride isn't able to travel along the tunnel of the cell and if even it could, the gate at the end is firmly shut tight just like the G551D mutation. So the hope is VX809 would allow the chloride to flow down the tunnel and then VX770 would unlock the gate at the end, allowing for the CFTR gene to function properly.

This trial is still in it's early days, but compared to the placebo group, there have been improvements in function and sweat chloride has been reduced. We now have the long wait as a phase 2 trial is currently underway and results are to be announced later this year.

We all campaign for a cure, but honestly, unless the gene trials here in the UK and around the world work I'm not convinced that we will see one in Sophie's lifetime, however I remain optimistic and incredibly hopeful that drugs such as the VX770 and VX809 will be the next best thing.

So the main thing to bare in mind is the importance of keeping our kiddos as healthy as possible and any lung damage to a minimum. If the combined VX drugs work as well as 770 has on it's own, Sophie and her many friends will have a very promising future.

Exercise, treatments and good nutrition it is then!

I just want to extend our thanks to every single person who has/is participating in the drug trials as without you, we wouldn't have anything to be hopeful about.

If anyone has any questions please ask. I hope my take on things isn't too confusing as I only have what I consider a basic understanding of these things!

Take care all.

xxxx

Clinic and button.

2011-03-01T14:20:00.000Z

Ooh ooh original title alert!

This post is going to do exactly what it says on the tin today.
Rewind to yesterday and Sophie's Mic-key button saga.

For those of you not in the know, Sophie's button has to have it's water checked every week, this is from inside the balloon that holds it in her stomach. Sophie's holds 5ml of water.
When we did her check on Sunday there was less than half a ml in there, so we promptly filled it back up and made sure we had a spare about, as we knew that come Monday, it would have to be changed.

Sophie still freaks out at having this changed, it doesn't particularly hurt but she does feel a bit of pressure when it goes in. We have always slipped her routine changes in when she has been sedated for her port flush, but now she allows those done without sedation we knew we'd have to test the waters with a sedation free button change too.

We had a little chat with her and explained what we needed to do the following day. It was her choice, either we do it or we have to go to hospital and have it done there. She opted for home.

So we eventually managed to get her to lie still in a relaxed state, as the more she tenses the more she turns her stomach from a wibbly wobbly jelly, to a full blown brick wall, and this makes it near impossible to insert the button into her stomach.

Luckily she decided to be a team player and the button was changed easily after she had chilled out.

Look at the leak on this little bugger!! (forgive my husbands crazy finger nails, he says they are working man hands, I beg to differ! ;) )

We have one happy little girl with her new, non leaking button and two very happy and relieved parents who managed to get to sleep at a reasonable hour.

CF clinic today went well, Sophie was an absolute angel and behaved herself beautifully, I couldn't be more proud of how far she has progressed, it was actually a pleasure to take her today.

Weight is down a little bit but expected due to her tube wean, but nothing too bad seeing as she has grown a whopping 3 cm in 2 months!

Chest is clear, she's full of energy, her consultant couldn't be more pleased with her at the moment. We have agreed to continue all of her nebulised treatments and be proactive, we are now all finally on the same page.

She did her cough swab like a pro, too independent and grown up to sit on mummy's lap now. She sat on a chair by herself, opened her mouth, stick went in and she let out a cracking cough. Job done. Awesome.

We should have some results either Friday or Monday.

On the next port flush they will grab bloods for her annual review and if she's feeling up to it, we will take her for a chest xray. Luckily this is not urgent as it is only a few months since she had one done.

Thanks for all your good thoughts, something has worked!!

Little miss fell asleep on the way home due to the warmth from the sun I think. Yes, I said sun, in ENGLAND!

Slowly coming to..

Full stretch and we are back in the room...

Wonder what will happen if I push this hula hoop onto my finger...

Oops, it breaks!!

Take care lovely people!

xxx

Touching base.

2011-02-28T19:13:00.001Z

Checking in to say we are all fine. Had a busy week so hence the lack of blog posts. I also work full time but squeeze it all to 3 or 4 days so I do disappear from time to time :)

Half term week didn't go to plan. Both Ian and I ended up with a vomiting bug at the beginning of the week but some how Sophie managed to avoid it. Huge blessing due to her not being able to be sick!

She did manage to have some fun with her nan and I was able to take her for a good run around in her favourite indoor play centre as the weather was shockingly cold and wet. Her green eye boogers have gone and her eyes are back to their normal sparkly selves.

I have several things to discuss including the results of the Vertex drug trials and a load of blog awards to dish out. Thank you to everyone who has given them to us.

We have CF Clinic tomorrow so will let you all know how that goes and please keep us in your thoughts for a non eventful appointment, and nothing too troublesome from the culture result.

Sophie was back at school today but having to miss tomorrow due to clinic.
This is a highly disjointed post but I promise normal service will resume tomorrow.

Will leave you with a few pictures of Soph having fun last week.

I'll leave it here as Ian and I are about to change Sophs Mic-key button, it appears to have sprung a leak and is about to fall out at any given moment. Wish us luck as Soph is usually sedated for this due to her traumatised brain.

Best case scenario, we'll be in bed for about 10.30.

Worse case scenario, we'll be at the hospital until the early hours of tomorrow.

Take care all.
xxx

It's all about the eyes.

2011-02-21T17:25:00.000Z

So it is officially half term here now, Sophie has one whole week off school. The plan is to get up to as much mischief as possible but the weather isn't playing ball, and it's poured with rain today.

I picked Sophie up on her last day of 'big nursery' on Friday ready for the fun of her week off. As she ran over to hand me her work of art (two bits of cardboard glued together-a masterpiece!)I happened to clock something in the corner of her eye that probably shouldn't have been there.

On closer inspection I noticed that she had a green eye booger. Lovely.

Once home, I gave her eye a good clean and was pleased to see that her eye wasn't red and maybe she just had some funky coloured sleep gunk.

Well an hour later, the white of her eye was bright red/pink and more green eye boogers were practically crawling down onto her cheek. Lovely.

Last day of school and Sophie managed to catch conjunctivitis, infective. It's been doing the rounds at school and we thought we were in the clear with half term upon us. Nope.

So I went a little into panic mode briefly. What on earth do I do?

All sense of logic left my brain, the fact I was a nurse left my brain. I came to the conclusion I had no idea what to do with a non CF problem.

I promptly contacted a friend of mine for a wee bit of advice and she put my mind at ease :)

Seriously strange, Soph hasn't had any typical childhood issues, only ever CF or TOF related and as hard as they can be, at least my common sense doesn't fail me.
Has anyone else ever experienced this or am I truly unique?

The infection is still going strong but has started to settle in the left eye, unfortunately, it has also spread to the right eye and she kind of looks like we've poked her in the eye now.

Bless.

We had to go to an eye appointment today (oh the irony) for her pre-school mandatory check. Soph was under an eye specialist a year ago as there was a query that she may have a squint, but she was in the full swing of her medical stress induced state then and they had to discharge her due to 'lack of participation and cooperation', AKA violence, high pitched screaming and the ability to sprint away from anyone who came within a few feet of her.

So today could have gone really bad but Sophie stepped up and behaved, allowing the lady to do all of her tests. The bonus being, Sophie's eye sight is absolutely fine and she doesn't have a squint, just a wide bridged nose, which her face should grow around :)

In other news, Soph ate a pile of spaghetti and jelly (not in the same dish!) over the weekend and today has eaten half a can of soup, some more jelly and a little bit of a boiled egg.

Due to her green eye boogers Soph hasn't been able to go to her weekly picnic group today but Ann has kindly sent us the plan so we can recreate it here at home.

Last week Sophie wanted to go on a bus with her nan, here's a few pictures she managed to snap.

Very pleased and excited to be on the bus! (Sophs hair looks really red here, don't know what that is all about as she's blonde, phone camera maybe?)

Oh my goodness, it actually moves too!!

Feeding the ducks in the park...

That's all for now folks, take care and I have three blog awards to accept and then dish out. If anybody prefers not to receive one would you let me know here, just in case I decide to send one your way of course.

xxx

Bertie's drink, Tuesday update.

2011-02-17T11:00:00.000Z

Today was met with a little apprehension.

Not because we were about to experience something totally uncharted, but it was to be an experience without the rose tinted spectacles AKA the funky haze AKA the good stuff, the drugs...

Today was to be the first ever attempt at flushing Bertie (Sophie's port) without any form of sedation. She would be going completely cold turkey and relying solely on her own fight or flight mechanism to determine the conclusion.

We had decided she was ready, by 'we' I mean Ian, myself and her poor CF nurse who always gets to be bad cop when it comes to Sophie.

Knowing how hard we had worked to get her desensitised to all of her negative hospital experiences, we all had a little dread in the backs of our minds. Would she just accept it like a trooper or would she kick off in spectacular style like she has in the past, finding strength The Incredible Hulk would be proud of?

This was going to go either way.

I've recycled an old port flush picture here as you really only need one to show what was going to be done don't you?

Sophie allowed me to put the Emla cream on easily after school and we made our way to the hospital. Sophie chose to take her favourite teddy Leeber (the Lemar), which she never normally would. Was this an omen?

To cut a very long build up short, we arrived, we removed the dressing that was holding the emla cream on, Sophie grumbled, squirmed, said ouch a few times and before you had time to say 'Holy smokes Batman', the needle was in and the port had been accessed and the flush went in.

Just.Like.That.

The tears were rolling but more so because Sophie wanted her top back on, but as soon as the needle was out, she was absolutely fine and the only person she wanted to cuddle was her CF nurse who had done the flush!

Sophie asked for toast and then took her nurse on a tour of the ward and was completely back to her usual self. In fact, I couldn't get her to leave and the only thing that worked was for some of the ward staff to do this...

This bag contained her toast :)

We left after Sophie had hugged and snuggled every single member of staff.

However, once we had completed the huge trek back to the car (we couldn't have found a space further away) we realised someone was missing, Leeber.

Sophie cannot and will not sleep without him, so I phoned the ward and asked them to look for him. They phoned me back to say they couldn't find him. As panic set in we ran back to the ward, the whole time I was questioning Sophie as to what she had done with him, knowing full well she had hidden him on purpose so we could go back. I knew this because she wasn't too concerned he was missing and kept saying "It's okay mummy, we will just go back and get him".

When we arrived back on the ward he was still missing and about 5 members of staff had joined the missing persons hunt.

Eventually (after a good half an hour or so) Leeber was found, buried under a pile of bricks and train track. Sophie decided to say then "That's where I put him, I knew he'd be safe there".

Oh the joys of manipulative kiddos :D

So today was awesome and Sophie has been an absolute angel (if you erase the Leeber incident from your memory) and we can now drop sedation, which will make everything much easier for all involved.

Someone was very tired after her adventures today and yes, that is a Thomas the tank engine quilt on my daughters bed, she loves it.
Sleeping with Leeber and in the background is 'angry bird' who in Sophie's words "Scares the spookies away". Gotta love imagination!

Take care.

xxx

Update from Monday.

2011-02-15T18:21:00.000Z

Well Monday was super busy this week.

I joined the meeting at Sophie's school along with her CF team, her school nurse, the deputy head, her feeding psychologist, one of her teachers and a nursery nurse.

The idea was to get everyone in one room and formulate Sophie's care plan ready for September. It went well, the school are happy to have Sophie in class if she is having home IVs and her port is accessed.

This is a huge weight off my shoulders as I was concerned they may be reluctant to have her there when she is just having a 'service'.
During a tune up, Soph isn't unwell as such, she just needs the extra boost to flatten Mr Pseudo when he is getting a little frisky in her lungs.

I'm also totally impressed with the CF team who are willing to come out at any time to speak to the school or as Sophie gets older, her class mates too should the need arise.

Her feeding psychologist was also amazing, she gave them Sophie's story and all of her needs in very understandable terms. Basically what they should and shouldn't be doing regarding meal times at school. The school really seemed to grasp why Sophie doesn't eat but also understood how much ground work we had already done and how far she has already progressed.

Basically they now know what to avoid to prevent any undoing of all of our hard work, which pleases me greatly.

Her nursery class are going to incorporate more 'food days' into class and have a vast array of characters who help explain eating etc

I was so very impressed with everyone, we have struck gold with the entourage Sophie has to help her over come her issues but also allow her to have a smooth School experience and not stand out like a man with a vacuum cleaner.

After this meeting, I picked Soph up from class and we headed over to the picnic group. We had a lot of fun. This weeks theme was 'smells'. Sophie had the opportunity to smell a lot of different foods and also taste them if she wanted.

We all had a lot of fun playing with different foods and almost having a full blown food fight at one point (all in the aid of therapy!).

Sophie made some progress this week and actually chewed on some leek, ate some garlic bread, licked some mint and sucked on some orange. She wasn't particularly impressed with a sugar doughnut though, she didn't like the feel of the sugar all over her hands.

So all in all, the sessions seem to be working, it's quite exciting.

Just before we left, Sophie was given a biscuit and a juice drink. She licked all of the creamy bit out of the middle of the biscuit and actually drank quite a bit of juice.

Looking rather pleased with herself...

and in case you didn't notice, look how much juice she drank!

It may not look like a lot but this is a significant amount for Sophie. She hasn't drank anywhere near that much in one sitting since she was 4 months old.

Progress I tells ya!

To finish the day off, Ian and I went out for a meal to celebrate being together 13 years and how could we have left Sophie behind?
She managed to do well during the meal, she tried some garlic bread, chewed on a carrot stick and dipped it in various sauces, drank some lemonade and ate a small amount of ice cream. What a star.

I will let you all know how the port flush went in my next blog post. Hope you are all okay?

Take care.
xxx

All is brighter.

2011-02-11T20:08:00.000Z

Because someone is feeling better...

Antibiotics have completed and Sophie is 100% back to her usual self. Just look at the colour in those cheeks!

We've had a fun afternoon of running around, climbing, laughing and generally just fun exercise.

She loved rolling these giant balls around and throwing them..

Busy week coming up after the weekend, including the meeting at the school with her CF team and feeding psychologist, picnic group and port flush. Will update about all.

For now, I'm just relieved to have my little girl back :)

Have a great weekend all, I'm working!

Take care

xxxx

Inspiration

2011-02-08T10:54:00.002Z

"You gain strength, courage, and confidence by every experience in which you really stop to look fear in the face.

You are able to say to yourself 'I lived through this horror. I can take the next thing that comes along".

~Eleanor Roosevelt.

I love this quote, it is so fitting for the situation we find ourselves in regularly, living alongside CF. Please pop over to Jenelle's blog, if you'd like to find some more inspiring quotes like this one.

Well again, thank you all for your love, support and prayers, something has helped. The antibiotics appear to be doing a fine job of getting little missy back to her bouncy self.
Her cough is still present but it has calmed down so much, she's stopped coughing at night, which is a major achievement.

We managed to get out and about and have some well needed exercise to help clear out those wind bags of hers. The weather is still cold here so we ventured off to an indoor play centre again, this is the best picture I could get as Sophie just wouldn't stay still for more than a second, she had energy to burn and I wasn't going to complain!!
It's taken at the bottom of a huge slide which dumps you in a deep ball pit, tons of fun!

Sophie's daddy also took her to one of her new school friends birthday party at the weekend. She had an absolute blast. It's so great to see how well she has settled into pre-school and how well she is adapting into her little social circle.

She's growing up fast.

We had to miss this weeks play picnic for reasons beyond our control, but hope to be there again next week as part of our journey to get Sophie to eat.

On Monday morning we have yet another meeting at the school. This time with the school nurse who will be writing Sophie's full care plan. The CF team and her psychology team from her eating side will also be present. I cannot get over how proactive the school are, I'm astounded by the detail and effort they are contributing to allow Sophie's school time to run fluently for all.

I'm one impressed Momma!

On Tuesday we have the port flush.

Bertie's drink wont be given the usual way though, this time we all feel that Sophie may be ready to try without any sedation. It could go either way so any positivity you have, please send our way. If it doesn't go to plan, Soph will have to have sedation and we will try again in the future.

We are doing really well with sponsorship for Ians skydive in June, we are up to £800 in donations now. I'm only updating the justgiving page when we actually receive the money from offline donations or unless someone donates online. So the total you see is alot less than we have. We are SO CLOSE to our target of £1000 so if any of you have any spare change, here is the link to donate :)...

http://www.justgiving.com/Ian-Ravenscroft

I'll leave you with one last picture. It is Sophie's homework.

Yes you heard right, homework, she's still only 3 and has HOMEWORK!

She had to get creative with different textures, I think she did really well, I only helped her with a few bits as I'm awful when it comes to anything art related.

How do you think she did??

Take care all

xxx

I need more space.

2011-02-01T11:12:00.002Z

No. Not alone time, but actual space.

Don't think I have ever posted a picture of Sophie's monthly feed delivery and medication. For the first time, the two have crossed paths so here you go.

Most of what keeps Sophie alive every month.

Our recycling has tripled since we had Sophie..

Breathing treatments, laxatives and in the large boxes are her bolus giving sets, which are used to feed her via her Mic-key button..

Spare Mic-key button and a selection of syringes used for medication and Mic-key button maintenance. Also the milk that has kept Sophie alive for the last 3 and a half years..

As some of you are aware, we monitored Sophie's cough but have had to start oral antibiotics for two weeks. The cough started to change to a wet cough and I wasn't prepared to take any chances so the orals have started...

I just wanted to say a huge THANK YOU for the overwhelming support you gave us in relation to our last post. You are all simply awesome and I am feeling much more 'stable' now. I seem to have these wobbles every now and again, I guess it's the frustration and fear that CF has bestowed on our lives. Most of the time I can handle it well and fight it head on, but sometimes it hits when you least expect and this knocks me totally off balance and I need to get my feelings out.

Sophie is doing okay, she's still coughing away and clearing lots of clear mucus during treatments but her energy levels are improving now. She has her port flush coming up in the next week or two so she'll have a once over then anyway. She's turned a corner for the better so I'm hopeful that this will pass with just orals this time. I say hope, as I thought this last time and it certainly wasn't the case.

Will keep you posted.

In other news, Sophie attended the Picnic group on Monday, which has been created to help children like Sophie who have issues with eating. It is a very innovative project and we are very excited to be part of it. We are the FIRST group in the country to have a group led by parents for parents. We are currently contributing towards the literature and ultimately, to find out if we as parents can do a better job than the dieticians to help our children over come their eating issues.

We are being closely guided by a team of expert psychologists and have a very interesting model to follow.

You can read more over on our other blog which I co-write with Ann who is running the sessions. Sophie will attend the sessions every Monday for five weeks and we even get homework!

I'll do a more detailed blog on the whole experience in the near future.

Sophie is continuing to make more steps in her journey to become an 'eater'

Licking..

Nibbling..

It's going in..

Cheese and cucumber sandwiches are overtaking beans and toast in the popularity stakes at the moment..

Take care all and thank you for all your comments and support.

You all rock.

xxx

Disconcerted?

2011-01-26T11:05:00.001Z

Possibly.

I'm feeling a little deflated at the moment. You work hard, put everything into keeping your child healthy, but in the end, it's totally out of your hands.
There is only so much you can physically do to prevent something throwing a huge jumbo sized spanner into the works.

Knowing that you need to react appropriately and the decision to delay that reaction can set a chain of events spiralling out of control if your timing is off.

Sophie is definitely brewing something, coughing most of the night. Cf mommas will know the cough I'm talking about.

The one you dread to hear.
The one that upsets you.
The one that keeps your baby awake.

Another Cf mom wrote a very interesting post, you can view it here. Margaux has a beautiful little boy called Ben who currently has a cough similar to the one Sophie has at the moment.
She brings up the subject of antibiotics and not reacting too quickly if not necessary. We try this approach too, sort of wait and see how things are going to materialise before we jump straight to antibiotics for Soph.

We will also get sputum cultured before anything is started just in case Sophie decides to bring something new to her own private lung party. Treating blind isn't always the right way, but sometimes, it cannot be helped.

It's the feeling of being in limbo I detest. How long do you leave things before you have to take action, normally we get it spot on but there is always going to be a time when we leave it longer than we should have.

For now we wait as Sophie is still bouncing round. Just a persistent cough that certainly sounds more 'throaty' than 'chesty' at the moment. Her team are aware of the current state of play.

I am hating the unpredictable nature of CF, fine one minute and then BAM, illness sets in. I just hope Sophie can fight this one off easily. I feel bad for her, she's just started pre-school and is loving it.

I don't want her to have to miss anything.

Sophie made this at her nanny's house while I was at an important meeting discussing the next stage of her eating development...

I was supposed to be discussing the eating side of things in this post but I got a little distracted. You'll forgive me this time right?

Take care and please swing by Gem's blog, she's just passed on the bad news that Lauren is not doing so good right now. Her blog is on my blogroll (Getting just a little breathless) but she hasn't updated in a while. She's a strong, beautiful young woman with CF, and could really do with all of your collective mendy thoughts right now.

Thank you xxx

First week and added extras.

2011-01-25T11:07:00.001Z

The first week is over and Sophie is already into her second week of pre-school.

Time certainly shoots by.

This is her official school picture I had to snap a copy of it, she looks so happy (although the lighting has made it look like some of her teeth have fallen out, they haven't!)

To summarise, it has been a fantastic week for Sophie, she has loved almost every minute and bounces out of bed every morning to get ready for 'big nursery'. The teachers are getting to know her now and are asking questions as and when things arise. We had one less hindrance to deal with as Sophie is still pancreatic sufficient, so we haven't had to sort out the school giving enzymes.

One issue has become apparent though. On a Thursday they do PE (sports) and are expected to get changed into their PE kits, however Sophie totally freaked at the idea of getting changed. The school are happy for her to participate in her school clothes until she gets more used to them.

Knowing Sophie, I'm not sure if she'll come round to the idea or not. She still has her anxiety issues stemming from the amount of hospital experiences she has encountered. Taking her clothes off in a strange environment usually means needles are coming or her mic-key button is being changed etc

Does anyone have any suggestions? Do we just ride with it and give her some time to adjust?

Both Ian and I have had quiet little talks with her and she is fine with everything but has absolutely no intention of allowing them to change her into her PE clothes. I even suggested one of us going up to help her and she still wasn't going to accept it.

Health wise she is doing great, however we have restarted her saline as we have noticed a slight change to her cough and productive levels. Her CF nurse is happy with this.

It's hard to decide if she is starting with something viral or whether it is the drop in this particular treatment. I guess time will tell, she's super well in herself though. I'd be surprised if she doesn't come down with something as the change in environment from nursery to school is likely to effect her. She has new germs to get used to and you always have at least one child who constantly has a green, snotty nose! Luckily Soph is still a little OCD with her hand washing :)

More to follow in my next blog post about this part of Sophie's life...

Her eating issues.

Click here to donate to Ian's skydive.

Take care everyone xxx

Linky linky.

2011-01-21T21:43:00.004Z

Hello all.

Here is the Just Giving link for Ian's Skydive on June 12th 2011.

http://www.justgiving.com/Ian-Ravenscroft

EDIT-(For non-uk residents select 'I am NOT a UK taxpayer' and it will accept it fine, any problems let me know and I'll sort out an alternative method).

I know we are a long way off, but if we don't start fundraising now, we wont get close to our target and I'd love for Ian to be throwing himself out of the plane with a good amount raised!

If you can help in any way, no amount is too small. Every penny will be appreciated.
I understand that everyone is struggling with money at the moment, and it's times like this when charities really suffer. Please help in any way you can.

I wanted to ask all of you lovely bloggers a favour. If at all possible, could you link to either our blog or the Just Giving page in one of your blog posts?

You may just help us meet our target by reaching an even wider audience.

Much love and I promise a round up of Sophie's first week at Pre-school in the next post. I have questions!!

Thank you all.

xxx

Is divorce a better option?

2011-01-19T10:48:00.001Z

There are many ways to leave your spouse but Ian has certainly thrown a trump card into the mix.
Instead of going about proceedings in the conventional way, always wanting to be eccentric, he has chosen a very odd method.

Before you all begin to wonder what on earth has happened and why I would be blogging about such intimate issues allow me to explain...

My brave and crazy husband has decided that now is the time to do some fundraising for Cystic Fibrosis.

On Sunday the 12th of June, 5 days before my 30th birthday, Ian will be doing a SKYDIVE.

Yes you read right, he'll be throwing himself out of a plane at a stupidly high altitude and relying on a small amount of cloth and some string to stop him from smashing into the ground at a high speed.

All sponsorship raised, will be given to CF research to help contribute to finding a cure for the devastating and fatal condition our beautiful daughter has been born with.

I am turning to the blogging community to help us raise as much money as possible and will be setting up a just giving page. Any amount you can donate will be worthwhile. £1, $1, anything..

Please help us to help our children, there isn't a lot we can do as parents other than all of the day to day care, but money ploughed into research WILL help.

If anyone is able to help us in anyway to raise as much money as possible we would be so grateful. Link to our page etc

Just look at my header picture, is she not worth it?

Thank you for all of your lovely comments on my previous post, Sophie is still loving pre-school!!

I will post the link to the just giving page as soon as possible.

Much love and take care.

xxx

First day of pre-school.

2011-01-17T19:30:00.002Z

Sophie started her first morning at pre-school today. She will be doing 9-12, Monday to Friday.

Slight culture shock this morning getting her up so early, she's always slept in a little later as her previous nursery was in the afternoons. We had been doing tactical training since we knew she would be starting Monday. She is now in bed by 18.30 and waking bright and breezy at 07.00.

When she came downstairs this morning, she had a very confused look on her face and kept saying "Mummy what's happening, it's not morning yet"?
To be fair it was a very dark morning today and I had to explain a little about how some mornings are darker than others.

We managed to fit all treatments in before she left and she seemed quite excited about starting 'big nursery'.

I had to be up there for half eight to meet her CF team. Yes they came out that quickly!!

We had a short meeting with a bunch of teachers and then Sophie arrived with her daddy. After 5 minutes she asked us to leave and we did!!

When we picked her up, she was happy and so excited, she'd had a blast and cannot wait to go back tomorrow.

The crazy thing is I didn't cry, does that make me bad?

Before you all think I'm some evil mummy, let me explain.

I had wound myself up so much over this. Whether she would like it there, whether the school could and would cope with her, how the other kids would react to her crazy, loud cough.

All I felt was utter relief and calm. I actually feel amazing and I'm so glad she loves it. Once Sophie decides she hates something it is very hard to get her to change her mind. This is non negotiable and she has to go (well September is mandatory) but if this had gone bad today, we would have had a huge battle to conquer.

Luckily she has accepted it and hasn't stopped talking about it.

As requested, the pictures...

This first one was before she went, as you can see she isn't impressed at all and it is the only one of her not crying at the camera..

Here are the pictures that now melt my heart but in a good way. She still needs to grow into her uniform as this is the smallest we could get. All taken after her first day there. Her cardigan is actually purple but looks blue on here...

Thank you all for your messages of support, so so appreciated and needed :)

Much love xxx

So quick?

2011-01-13T21:09:00.001Z

Ian and I met with the head teacher and deputy yesterday.

The school we want Sophie to attend is actually my old primary school. It was a strange experience walking the corridors again.
Everything seemed so much smaller than I remember, but the same feeling was still there. You see I loved school and had so much fun there.

We were escorted around the whole building going from class to class. Every room was filled with enthusiastic children who were busily going about their current activities. Some were colouring, some were working hard on computers and others were doing something very creative with paint, glue and newspaper. Honestly, I wanted to join in and then I remembered I'm now a grown up, I refuse to say old!

We all sat down after a surreal trip down memory lane and got down to business.

A small amount of anxiety oozed from Ian and I as we knew the school had every right to not offer Sophie a place due to her extra needs. Obviously they couldn't discriminate but they could decide that the environment wouldn't be 'beneficial' to Sophie (code word for too much hassle for our school).

Luckily after an hour or two of discussion, they felt that they could meet Sophies needs and were happy to offer her a place in September.

Woohoo and phew!

They are really keen to learn more about both of her conditions and would like her CF team to visit them, which I am really pleased about. They also wish to speak with her feeding psychologist so they can ensure they are doing all of the right things regarding her eating issues.

We were very impressed and I'm feeling much more stable over the whole situation. Well that was until they asked us to start her ASAP.

We have agreed that it would be more beneficial to Sophie and her teachers if she could start at the school nursery. To give everyone a chance to get to know each other before she starts full-time school in September.

So...

On Monday, Sophie will start proper pre-school!

We have had to dash out and buy her, her first school uniform-emotionally don't even get me started on that!!

We also had to go and tell her present nursery the news. Friday will be her last day there.

I cried, they cried.

They have been a huge source of support to all of us and I can only praise them for all they have done with Sophie.

So...

This is it, the issue I thought would occur in September is going to happen on Monday and I actually feel okay about it currently. To warn you all I will be a bubbling mess on Monday, that's normal for all parents right?

I will try and snap a few pictures of little miss in her sparkly new uniform if I'm stable enough to even work a camera and providing we can actually get her to wear it!!

Thank you all again for your lovely comments.

Take care and wish Sophie (and us) luck. I hope she likes it there...

xxx

Mixed emotions.

2011-01-11T14:32:00.002Z

We have not long got back from the hospital, Sophie has had her port flush done this morning.

Huge improvements made and next month we will attempt it without sedation. We all feel she is ready to at least give it a try. I cant say I will be sad to see the sedation stopped as although it has been a huge help to Sophie in reducing her fears, the after affects are always very tiring.

Since she has gotten older and bigger, she has become harder to keep safe when 'under the influence'. All she wants to do is try and run around and when her legs are like jelly and not wanting to do what her brain is telling her, it makes for a traumatic couple of hours.

However cute and funny 'drunken' Sophie is, the anger and violent side is somewhat unpleasant :)

She didn't want to leave the ward at all today. We eventually got away after lots of bribery with half a dozen stickers, a piece of toast, her Christmas present from the CF team, 3 pictures of Thomas characters that Sophie made her CF nurse print off the computer and a small NHS bed sheet!

Here is what she wanted the sheet for (really blurred, sorry! and stood by a very dirty hospital door :D)

Super Soph!!

It took me over an hour to get her back to the car. She was just able to walk and wanted to explore EVERYTHING. She managed to lead me into an area of the hospital where the staff have their breaks. She stood for 10 minutes pulling faces at them through a window and swaying like a mad woman.

This would have been fine if it was staff that knew her and knew she was 'under the influence' but this group of people were obviously unsure of why she had such strange behaviour. I'm surprised someone didn't come out to us in the end!

I was able to navigate her back to the car eventually and we made it home in one piece where she has 'sobered up' well now.

Tomorrow is the day we meet her (hopefully) new headmaster. Thank you to everyone who responded to my previous post. I'm a little calmer and excited for Sophie now. Sophs team have said if the school want them, they will happily go to the school and talk to all the teaching staff and answer any extra questions they have. I'm really impressed that they would go to the trouble for us. Her CF nurse, dietician and physio would go. We'll see what the school say tomorrow.

Cough swab results are back and nothing extra grown other than her usual resident, Pseudomonas so I'm content with that also.

So all in all, everything is going good for Sophie.

However...

We've had some very sad news in the CF community. 10 year old Matthew lost his fight to CF on Thursday 6Th January following a massive lung bleed. I feel so numb for his family. He was just entering the evaluation process for lung transplant.

10 years old, it's no age and it brought back all of the emotions I felt when Conner lost his battle at age 7. As much as treatments have moved on, CF still takes no prisoners and still takes children. I just want to send my sincerest condolences to Matthew's family and friends at this very very sad time.

Breathe easy now.

xxx

A Date with the Headmaster.

2011-01-07T17:13:00.002Z

We finally got round to calling the school we want Sophie to attend in September. We had been given some wrong information a couple of years back and the secretary was very keen to get us in to meet with the headmaster ASAP.

So next Wednesday, Ian and I will be meeting with the head and deputy. I was always good at school so have never been summoned to the headmasters office before. I'm already nervous!

The deputy head is in charge of Senco which deals with the special needs affairs. Although I do not see Sophie as having special needs, she does have extra needs so we are keen to see how they can accommodate her in the education system whilst ensuring her health needs are always at the forefront of their minds. I have already produced an 18 page document (with the help of a wonderful CF mum) outlining both of Sophs conditions, her extra needs and what the school needs to do to ensure her health is monitored while in their care.

Sounds a little obsessive but we did similar for nursery and they were so grateful we had as they could refer to it and also ask any questions that popped up along the way.

I cant believe Sophie will start school in 9 months time, she's way too little in my eyes but I know it will do her the world of good. It's a hard thing to deal with as a mum at the best of times, but add into the mix, not one but two serious health conditions and you can imagine how I am feeling about the whole situation.

Learning to trust a whole group of strangers with your child's health is hard, I don't do it well even when we have known the people a long time. See my multiple questioning of her doctors for evidence :)

I guess we will see how Wednesday progresses and go from there.

This is one mum who is definitely apprehensive about cutting the apron strings so soon.

On the other hand I cannot wait for Sophie to experience the joys of living life to the full, learning new things and having many wonderful experiences and adventures. She's a very intelligent girl, we realised this at a young age and school will challenge her in so many ways I hope.

Does anyone have any experiences or words of advice or encouragement?

Nine months from now I feel I'm going to be a bubbling mess at the school gate after dressing my tiny little girl in her school uniform!

The only place Sophie isn't wearing her cycle helmet is bed!

Take care all and thanks for the lovely comments.

xx

Bronch report. Sophie 1 - 0 CF.

2011-01-04T16:12:00.002Z

CF clinic has been rather bizarre today. Weight up a tiny amount from the last admission and height has gone up too. Chest sounded clear on examination and tummy was soft and no signs of issues.

Sophie was so well behaved after waking from her slumber like a teenager and initially refusing to come out from under her duvet. Once at clinic, we met her psychologist who has made her a Thomas the Tank jigsaw puzzle. For every procedure Sophie completes at clinic, she gets to add a piece of the puzzle. Once completed we get to buy her a treat.

Sounds so simple but it works really effectively.

Cough swab was obtained with complete ease, we'll have the results possibly Friday or Monday.

Here comes the really striking news...

Her consultant showed us her bronchoscopy report and looking at all areas examined the results were as follows.

Normal.
Normal.
Normal.
Normal.
Normal.

One area, right upper had a small amount of mucus, not excessive at all, just the only thing the doctor could find to report apparently.

Sophs consultant actually expressed how shocked he was at the results. He expected there to be inflammation, sticky mucus and general nastiness, considering all that she has been through since birth and also due to her cultures.

This was not the case, there was NOTHING. He said he was most surprised about the lack of mucus, he said that most patients have an area suctioned and as the team re-introduce the suction catheter, that area has filled back up with mucus again.

He said he was more than happy with how things are despite the chronic pseudomonas. It was found on both sides, not just the right side as we had originally been told but it isn't an excessive growth and currently he isn't worried. He'd prefer it wasn't there but as long as it isn't causing any bother he isn't concerned. The situation will be monitored regularly and she will stay on her colomycin nebs indefinitely.

Now here comes the surreal bit.

We can stop all other nebulised treatments if we choose to.

Eh? Come again?

Yes you heard right.

He said he doesn't get to say this very often, but as Sophie's lungs are so very healthy, much healthier than anticipated we could stop the saline and her DNAse if we wanted to. The decision was ours.

We have decided to stick with the DNAse as without it, Sophs nose gets really bunged up and I don't want to cause any sinus issues and the saline will be kept just for when she starts with a cold or cough as an extra boost.

I couldn't be happier at the moment although a little shocked.

I think he may think I am slightly insane as I kept saying, 'yeah but what if...'.
I was just thrown right off guard with this and I prefer to not become too complacent with Sophs CF as it already has proved how quickly things can change.

But for now, I am going to try and not worry. We will continue all preventative treatments and physio and throw in loads of exercise. Basically carry on as we are as clearly we are doing all the right things for Sophie currently.

We bought her this new bike as her old one wasn't particularly great or safe..

It's freezing cold, dark and miserable but she insisted on going for a spin...

She's soon got the hang of it and I was breaking into a sprint just to keep up with her, luckily she was heading straight at daddy :)

Can you see why I need a new camera!!? ;)

Take care all

xxx

2011

2011-01-03T15:00:00.001Z

Happy New Year!

Okay so I'm a few days late but you'll forgive that minor detail I hope.

2010 flew by so fast, where do the years go? I'm not going to do a month by month review of last year as I think it may turn into an epic blog!

Significant events of last year include,

-our first family holiday in years which was lovely.
-Sophie got her first port fitted-her beloved Bertie, and our first home IVs.
-Too many IV courses for my liking which failed to eradicate Pseudomonas in the end.
-First culture of Staph, which proved to be a little bit of a tinker to Sophie. Luckily he has left the building for now.
-First bronchoscopy which gave us a clear picture of Sophie's windbags and very promising results.
-Sophie has started to improve with the hospital environment and now clinic visits are much more calmer for all involved.
-Tube wean has finally started. Here's hoping 2011 is the year Sophie becomes an eater!

Poignant memories include, the many losses in the CF community this last year has brought. Many who leave a lasting legacy in all of our hearts and the wider community. Also love to all who have lost a loved one.

Acceptance came with the realisation that Sophie is colonised with Pseudomonas and this bad beast is here to stay. Luckily for her, currently doing no more than just showing up at the party when a culture is needed.

Shock at finding out I was pregnant and then sadness when it ended in miscarriage.

2011 is bringing us a couple of milestones. Sophie will turn 4 and will start school in September, to say I am apprehensive would be an outrageous understatement :)
I will be turning 30. This just sounds absolutely absurd as I still think I'm 16 in my head!
Sophie will be starting swimming this year, which I hope will do wonders for her health and I will be pulling my finger out and getting back into running to start Marathon training again. I will be counting on you all to kick me up the backside if you aren't reading about my progress on here.

Just wanted to say a huge thank you for all of the camera recommendations, we have a few in mind and hopefully some decent pictures will start to appear in the near future.

I also want to express my hopes for the future and the exciting drugs in the pipeline, which may come to fruition this year. The Vertex trials in the states and the gene therapy here in the UK. I don't care which comes first as long as something will help all with CF!

I hope you all have a very happy and healthy 2011 and thank you all in blog land for the support you have given us since I started this blog. It means more than I can articulate.

Much love.

xxx

Quick update!

2010-12-31T14:15:00.001Z

Well time seems to have ran away with us lately. I'd love to say our MIA status was due to us flying around the world or finding the answer to world peace, but we have just been busy with day to day things i'm afraid.

We had a lovely Christmas though. I have had to work quite a lot over the holiday period so another reason i've not been blogging. All available time off has been spent spending time with Sophie and Ian and family.

Sophie was spoilt rotten and we have had to have a complete early spring clean of the house to find room for everything. Our house was beginning to look like a Toy warehouse and we have had a good clear out now. I can see my floor, it's fantastic!!!!

Update from the previous post.

Sophie's consultant has agreed to increase her saline strength further which I am totally surprised about, but ecstatic all the same. We have a re-scheduled CF clinic appointment this coming Tuesday so hopefully will be able to have a good look at the bronch report. I am also pleased to say that Sophie has managed to fight off this last cough/cold on her own with NO ANTIBIOTICS.

This is the first time she has managed this in a very long time and we are so pleased for her. She's managed to stay off anything since her IVs finished and long may this continue. She's doing really well at the moment and is growing up so fast, where does the time go!?

We also have her port flush in two weeks also.

I will leave you with a few pictures of Sophie from when we visited her grandad and nans over Christmas. Of all the presents she received, she loved her jar of marbles and another marble run. The old toys always seem to be the best don't they? The marble jar did attack her slightly later on though as it somehow managed to split itself in half and cut her finger, but one plaster later and a magic cuddle, all was well in the world again.

Quick question for all you budding photographers out there. I want to invest in a decent camera. It's only for taking good pictures of Sophie as the one I use isn't the best quality. Any recommendations? I couldn't handle anything too technical and don't want to spend an absolute fortune, just something that has a better result and able to take multiple shots to catch a very energetic toddler in action. Any suggestions?

Hope everyone is doing okay and having a lovely holiday. I will be catching up on all of my favourite blogs later today.

Take care xxx

Everything has gone green!

2010-12-20T13:25:00.003Z

Well Soph had a busy time with nursery last week.

She had her nursery play on her first day back to nursery after having 2 weeks of IV's. To say she wasn't keen would be a slight understatement. She happily walked out with the small group she was with, but as soon as she clocked her nan and I she came running over to us and spent the whole performance, snuggled into my shoulder.

She did enjoy being back at nursery though, I think she missed bullying the boys if I'm honest. She also had the chance to go with a small group of nursery children to watch 'jack and the bean stalk' at a local theatre. I thought she would love it.

How wrong was I!?

According to her nursery teachers, she cried throughout the whole performance and only stopped when they started walking back to nursery. I asked Sophie why she didn't enjoy it and she told me it was because of the girl with the green face. I assume she meant the witch? It makes sense as Sophie hates Halloween and all the creepy masks etc

All normal experiences for her though and whether she enjoyed them or not, her CF wasn't even considered. This is something I am going to blog about in more detail at some point so I wont elaborate anymore at the moment.

Onto the health side.

Hmmmmmm, a double whammy of illness has occurred.
Problem one.
Sophie has been suffering from a tummy bug. Due to her nissen fundo op, she cannot vomit, she can 'dry retch' and bring up a little spittle but nothing else. This proves particularly problematic when she gets a tummy bug. Everything has to go south so a typical 24-48 hour bug can last weeks sometimes as it travels through her body.

I'll spare you the graphic details but put it this way, we started off with yellow poop which has now changed to a greenish colour and has a very striking aroma. The poor mite has had a few unsettled nights where she has been trying in vain to vomit all the nasty stuff out and the only way we could help her was to vent her mic-key button and allow some of her tummy contents to drain out.
It gave her some relief.

Problem two.

Sophie has a cold with lots of green, slimey boogers. I have to smile as she is only a week off of her IV's and picks up a cold. Typical! No need to panic just yet as she seems to be holding her own and fighting this bad beast quite effectively on her own steam.

We should be at CF clinic tomorrow but cancelled it due to her tummy bug, not the greatest of gifts to bestow on someone a few days before Christmas is it?

I have spoken to her CF nurse today and filled her in. We have oral antibiotics on standby just in case they are needed later this week or over Christmas, so I can stop 'what if-ing' about that now.

I am a little disappointed we cant make clinic as I wanted to see the full bronch report and also discuss increasing her saline strength. Sophs Cf nurse said they will still discuss this and she'll phone or text me later tomorrow with the outcome. I have a slight amount of dread that her consultant may suggest stopping it as her bronch was so good. My argument would be we don't know if her lungs were as clear as they were due to the help from her treatments. Problem is, we wont be there to fight our side if a battle does ensue so I have to wait to hear the outcome.

I'll let you all know.

On a brighter note, Sophie's mouth is clearing up a little and thanks for all of the tips and suggestions.

Take care and hope everyone is doing okay with all the cold weather. Thank you to all the votes sent our way. You can still vote by clicking on the previous blog post or the union jack banner over on the right of this blog. Thank you to This Mid 30s Life for nominating us. Go check out her blog as it is hilarious and maybe send some bloggy love her way and vote for her too.

xxx

Please vote and advice needed.

2010-12-15T12:20:00.000Z

I had quite the shock this morning, I was reading a blogging friends post about how she is a finalist in the British Mummy bloggers -bloggers in brilliance awards for 2010. I went to have a look at how to vote and I was almost thrown off of my chair when I saw that our blog is also a finalist! This a huge deal as we're amongst the creme de la creme of mummy bloggers and I couldn't be more surprised if I tried!

We are in the Inspirational blogger category and actually up against two of our blog favourites, Mum in the mad house and crystal jigsaw, both blogs can be found on my blog list. I have no expectations of winning as we are up against some very strong competition and I feel a fraud us being there if I'm honest. I hope that this will help raise some huge awareness about CF and TOF so if you are reading this and haven't voted for anyone, please consider voting for us.

Vote here by scrolling down about half way and find the inspirational blogger category and vote for us!!

A big thank you to those who have nominated us, I am so very very grateful as you will have helped our main goal by raising much needed awareness for us and that is invaluable.

Right back to business, Sophie is still doing really well since IV's have finished. She does appear to have a small sniffle though but very well with it so just keeping a close eye in case she turns into the not so lovable snot monster!

Since her IV's started she appeared to be suffering with sore lips and small cold sores. I attributed this to her being run down and really not being well, however these all cleared up.

The past few days, her poor mouth has started to become really sore and now looks like this...

She doesn't appear to be run down at all,

Is it the weather?
Is it a CF thing?
Is she going to be a cold sore sufferer?
Are her nebs irritating or causing this?

All of the above?

Does anyone else or your child suffer like this? I cant use cold sore remedies as they are pointless unless you catch them at the tingle stage and Soph isn't really old enough to make that connection yet. We use a Vaseline lip balm to try and help the cracked lips but it doesn't seem to be preventing it.

Any thought or tips would be greatly received, she wont let me near her mouth which is entirely understandable.

Take care all

xx

Guess what?

2010-12-10T18:30:00.001Z

We're freeeee!

IV's finished a day early due to a pharmacy mishap with the amount of drugs supplied, but 13 days was enough for Soph and Bertie had his hair cut yesterday.

Iv's are over :)

Although they have been fairly problem free they are still tiring on all of us and we are all glad to see the back of them for hopefully a long time now.

Sophie is firing on all cylinders and I actually think she has developed a few extra ones as her energy levels are unbelievable and it is wonderful to see.

I am in the process (with a lot of help from my dad) in creating a video montage of how we prepared all of Sophie's meds. I had hoped to just do a straight video from start to finish but the whole process took 29 minutes and that wasn't including the pump time. Obviously that would be one huge thing to watch so we have taken stills from the video and we will produce something that should give you an idea of what it all entails to 'do it old school stylee' with home IV's.

After giving Sophie a well deserved and needed bath (we couldn't bath her as she splashes too much and water would have gotten under her port needle dressing and risked infection) we visited another indoor play centre to burn off some of her extra energy and get some good exercise as we all know how important exercise is on the old wind bags AKA lungs.

Sorry some of these are a little blurred, it was hard capturing stuff as Soph was so so active. Bonus was Ian and I were both able to run round in there with her. We are such big kids!

This girl has no fear of heights.

Soph even worked up a little appetite and tried a little beans on toast.

If any of you don't follow Worth his salt, firstly why not? It's a great read and the gorgeous Jackson's momma Amy writes so very eloquently. Secondly please check the above link out as Amy has helped create an eye shadow called SixtyFiveRoses and all proceeds are going to the cystic fibrosis foundation.

Great colour, great cause, make a great little present. What you waiting for? :)

Thanks and take care all!

xxx

Proud of my little girl.

2010-12-07T17:30:00.001Z

IV's end on Friday and Sophie is 100% back to her normal self. Yay.

On Saturday we went to get her blood levels checked (tobramycin levels and kidney function etc) as it was day 8 of IV's.

The plan was to sedate Sophie due to how 'animated' she had been on the previous levels check. On the way to the hospital Ian and I discussed this and decided that we wanted to try without sedation. We explained to Sophie that the nurses needed to get some blood from her finger and fill a very small bottle with her blood. All they would do was gently prick her finger to do this. Once this was done, we could go back home.

Sophie seemed to accept this so when we arrived we explained to one of CF nurses what we had planned. Sophie happily ran onto the ward and hugged and kissed many of the nurses. This is a huge improvement in her behaviour and shows how far she has come in reducing her paediatric medical traumatic stress.

Only a few months ago we wouldn't have even got Sophie to walk onto the ward without a huge, physical fight. Picture wrestling a lion into a cage and you are half way there!

Her nurse was a little apprehensive about our decision but was happy to go with our plan.

Sophie sat on Ians knee, and the nurse pricked her finger and took the blood. Two small whinges and that was it!!

No wrestling.

No scratches.

No kicks.

No punches.

And no tears from Sophie. Awesome!

She even happily showed me her finger and told me the nurse had 'took blood from this finger, aren't I brave'.

I'm so proud of her and how much she has matured. The past two weeks, although tiring have been an absolute breeze in comparison to previous IV courses and it is all down to Sophie.

Thank you to everyone who has shared the CF video link I posted about. It's a great video and needs to be seen by all.

Sophie engrossed in watching Tom and Jerry at her nan and grandads.

I think the IVs have done something to Soph, she thinks she is a cat!!

Oh my...

Take care all.

xxx

Animated Cf video for children!

2010-12-03T23:46:00.008Z

Please share this video as it is brilliant for educating children and adults. It's fab!! Thank you to everyone at the CF trust and to Oli who created it.

View here

Spot of bother.

2010-12-01T15:19:00.001Z

You know how I said how wonderful and straight forward home IV's were going?

Can I retract that statement? :)

Minus the dramatics they are still going really well, but we ran into a spot of bother over night and especially this morning. Last nights dose seemed really stiff to push in and Sophie was complaining it felt cold.
Both Ian and I inspected the site as much as Soph would allow and we couldn't see any leaking, which would be a sign that the needle was dislodged and it was tissuing. We were due to change the bio-connector (posh end on the IV line that helps prevent infection and the line leaking blood all over the place if the clamp became loose)the following morning and sometimes they can cause port stiffness when due for a change.
This morning, we changed it and tried to flush the line...

It wouldn't budge, not even a drop would go through.

Hmmmmm. Is it the line or is Bertie on his last legs??

No need to panic, phone the CF team and arrange to get up to the hospital and get it re-accessed. Simple. Be there and back in a couple of hours.

That was until we looked outside and realised that we had had several inches of snow dumped around us. Grand!!

After a few frantic phone calls and Sophs wonderful CF nurse eventually able to make into work, we were able to navigate ourselves over to the hospital, Sophie was sedated and Bertie has had a new hair extension which works beautifully.

We've gone for a gripper needle as the one that was put in last week was much smaller and Sophs port is quite deep inside her and we think it probably wasn't long enough. This needle makes Sophie's port quite animated. It clicks every time she moves (this is the tip of the needle scratching against the base of the port and quite normal) and it sounds like she has a pod of dolphins in her chest :)

Either way it flushes grand and should now last the rest of the course!

Sophie managed to arrive in style at the hospital.

Take care all!!

xx

Results part 2...

2010-11-30T13:33:00.000Z

The Bronch cultures are back.

Well it would seem that Mr and Mrs Pseudo have had a seperation. I'll call it a trial for now as it doesn't pay to get too optimistic too soon.

Mrs Pseudo has vacated the building and gained custody of the Staph baby as they have both disappeared. If you remember, Mrs Pseudo was the strain that was resistant to Cipro. 'The bitch' as I lovingly referred to her as has for now, gone.

Mr Pseudo is still present though but we knew that he would be. He is sitting in the middle and lower sections on the right side of where the cultures were taken. No trace of him on the left at all. Oddly, the 'gunge' that was hoovered from the right upper cultured nothing?

Obviously we will know more when we see the full Bronch report but that will be in a few weeks time in the next CF clinic just before Christmas.

What I can take from this is Sophie has cultured pseudomonas on and off since she was approximately 6 months old. Going from these Bronch results, it would appear that he isn't causing much bother at all currently which is great news.
I would love to have reported that he'd gone but I'm sensible enough to know that it would take a small miracle for that to have happened.

When you get the first culture of pseudo it makes you feel sick. The emotions you feel as a parent are overwhelming and you genuinely fear for your childs life, but I hope that Sophie shows it isn't entirely the end of the world and she is now 3 years and 7 months and it hasn't affected her yet.

The plan now is to keep things this way.

Her recent episode of sickness I am putting down to Staph. Since she first cultured this she has been poorly and it is only now that it seems to have gone she is back to her baseline.

Just another example of how CF is so unpredictable and each persons journey is so very very different.

We're very pleased with the overall results and really glad the bronch was done. Sophie's IV's are staying as they are as they are her pseudo weapons. Ceftazidime would be the drug of choice but little miss is severely allergic so we will stick with Aztreonam.

Take care all and thank you for the bloggy love :)

xxx

Bronchoscopy results part 1.

2010-11-28T19:20:00.000Z

We're home!!!

Got back late last night and it's wonderful. Sophie is so much more relaxed and is hardly bothering when having her IV's.

Back to business. As mentioned on the previous post, Soph was given a large dose of midazolam prior to her anaesthetic where as normally she would just go cold turkey. This caused her to come round from the anaesthetic aggressive and very very wild. She didn't open her eyes for nearly 2 hours and we had to put her on a giant beanbag on the floor for safety until she had come round properly.

When she was slightly more back on the planet we were able to put her into the bed.

The doctor came to speak to us about what he had seen down in her airways.
The news was very positive.

Hardly any mucous, no inflammation!! Awesome.

She did have a very small amount of 'gunge' in her right upper side which he got a good sample of and then hoovered out.

Five other samples were taken and we should hopefully have some results tomorrow or Tuesday. This is the nervous part as it will help determine what she is growing down there. As soon as we have news I will let you all know.

Home IV's have started and going really well. As I mentioned, we are mixing the meds ourselves. This is some of what we came home with!

Here are a few pics of Sophie while still in hospital. She was constantly asking for toast to eat and drinking cans of coke and cups of tea.

This was the view we woke up to yesterday. Not as much as the rest of England but it's still snow, which Sophie adores!!

And Sophie sitting on her bed while daddy gets some well needed sleep behind her, as we had been up at 2 am doing her IV's as she wouldn't let the nurses anywhere near her.

Here are a few of Sophie's meds.

This is the pump used to give Sophie Tobramycin. This is the first time we have given this once a day through a pump and Sophie is unimpressed with it.

You cant really see here but Sophie is connected up to the pump while sat on her nannys lap.

This is me giving Sophie her Aztreonam. We don't use gloves at home as apparently we all share the same bugs. We just have to make sure everything is clean, the tray, the vials, our hands etc and we use the wipes to hold the line so we don't actually make contact with it.

That's alot of photos! I plan to show you all how we make the iv's up at some point so keep an eye out for it. We have to go back up to the ward on Saturday for Sophie to have blood taken to check her Tobramycin blood levels. This is really important as if they are too high, her kidneys could be damaged and also her hearing. She has already had some bloods taken and everything is within normal limits so far.

Most importantly, Sophie is back to her normal bouncy self and it's lovely to see. Now I realise how poorly she had been as her energy levels are crazy now!!

Any questions please ask. Thank you all for your lovely comments again and I hope everyone is doing okay.

Take care.

xxx

First Bronchoscopy.

2010-11-27T13:00:00.000Z

Well yesterday, Sophie had her first bronchoscopy and all went really well. The only problem we had was that the doctor had given her a pre-med and when she came round from the anaesthetic she was quite violent and very disorientated. I don't think we'd opt for it again before an anaesthetic!

Her iv's have started, Tobramycin and Aztreonam and we are going to be mixing them ourselves at home old school style, so watch this space for our IV experience. Currently Sophie is howling the ward down during every dose as she still thinks it is going to hurt like her old long lines used to. Hopefully after a few days at home she'll be as good as she was in June after having the port fitted. She has adjusted really well to Bertie suddenly 'growing hair' but hating any of the nurses near her, so we have been giving her iv's from the first dose to minimise any further psychological trauma.

We are still in hospital but hopefully will be getting discharged home later tonight if all continues to go to plan.
I will do a more detailed blog about the bronch and what it showed once home as the Internet connection here is a little hit and miss!

Hope everyone is well and will catch up on all the blogs very soon.

Take care all.

xxx

Last few days at home..

2010-11-23T15:30:00.001Z

If the hospital phone to say there are no beds available, Sophie has a solution!!

Who needs a bed eh???

Friday will soon be upon us, been trying to mentally prepare for the hospital stay all week and I think I'm just about there. Does anyone else find hospital stays just so emotionally draining??

All being well, I'll blog about the bronch sometime on Friday and then hopefully the next one will be when we are back home doing the iv's!

Sophie is nearly back to her normal self now, which I'm putting completely down to the steroids this time. She's back at nursery and generally just destroying the house again with her toys :)
She'll still have the iv's though as part of a tune up.

Think of her like a car...

Occasionally the car totally breaks down and needs seeing to immediately so goes in for repair, but every now and again the car just needs servicing to ensure it runs effectively for longer.
This last month Sophie nearly 'broke down' however she has somehow managed to recover rather well, so instead of needing instant 'repair', she just needs to go in for a 'service' now.

If daddy had to choose what type of car she was, I guess he'd choose a Shelby GT500. She's strong, robust, pretty and very very LOUD :)

Thank you all so much for your lovely lovely comments and well wishes.

Take care all.

xxx

Improvement.

2010-11-19T14:15:00.001Z

I'm pleased to say that Sophie has picked up a little.

Her energy levels are much improved and although her cough is still evident, I think this 5th or maybe 6th course of antibiotics (I've lost track) has started to help.
It may be the steroids masking some of her symptoms but I'm not going to complain. She's started to sleep better at night and isn't collapsing in a heap at random times in the day now.

I think she may be able to hold out until the bronchoscopy a week today which is good news. She will still have two weeks of IV antibiotics though as clearly something has been causing all this bother. I'm guessing back to back viral infections have stirred up 'The Pseudo family' and clearly they are in need of a good kicking.

We have also had to increase her movicol again as her bowels went into slow motion again and we didn't want her to get backed up like last time. Due to the amount of mucous she is producing at the moment, inevitably she has been swallowing a lot of it and I think it may be contributing to the slow mo bowels.

If anyone is interested in how home iv's work, I may do a short video or at least lots of pictures of the regime Sophie will be having.
We don't usually have pre-made syringes and bags etc as we don't mind mixing them ourselves as we are aware there are a small number of slots for ready made, pre-mixed home iv's and we'd rather someone else gets our slot who may need it more.
If the demand isn't big currently though, we may get the ready mixed ones so we shall see. Either way I will try and blog as much as I can about the experience.

I also want to mention cystic life. I was recently introduced to it via some of the other parent blogs. It's actually a really great CF community set up by Ronnie and his wife from run, sickboy, run and a few of their friends. They are currently on a mass recruiting drive so please go check it out and sign up if you haven't already.

If possible can you also pop by and visit Gem as she is having a really crappy time at the moment and despite all her own problems, always finds time to support other people. Please go send her some positive vibes etc

Also great to hear of how well some of the other little ones are doing right now, really really pleased for everyone :)

Take care all.
xx

Small change in proceedings.

2010-11-17T10:19:00.002Z

Okay so the plan that we arranged for Sophie has changed just a little bit.

Well it's more down to Ian and I. Instead of the chloramphenicol (have you noticed I spell it differently every time!!), we have decided to go with Augmentin duo.

Reason being?

There are some rather unsettling side effects with chloramphenicol, which we would rather not risk with Sophie. The risks are minimal and usually with more longer courses of the antibiotic however, seeing as Sophie doesn't have much luck with odds when it comes to most things we don't want to risk it.

We will save this drug for a time when Sophie may really need it.

So she will have Augmentin Duo until she goes into hospital at the end of next week and continue with the prednisolone which is actually a 10 day course, not 5.

She has made some improvement and has more energy than last week but is still coughing a lot, especially at night. When doing her chest physio I can feel lots of fizzing and bubbling on both sides of her lungs at the moment which is not great at all.

We are still set for home iv's after the bronch though. So hopefully only a couple of nights in providing everything goes straight forward. Should have some culture results on the Monday and her iv meds may be changed depending on what is grown.

I'm getting slightly nervous about the bronch. Not the actual procedure per say but what they may find down there. I guess it is just pre-emptive nerves or something.

To top everything else off I have now got a streaming cold which I am trying in vain not to pass onto Sophie!

I wish I had something more positive to blog about today :)

Leaving you with a picture of Sophie nebbing her saline while playing on Ian's new toy (birthday present)! Her cheeks are glowing but it's all down to her infection currently.

Take care, thank you for all your comments and a big hello to all the new followers, your support is really appreciated!

xxx

Cysty roses.

2010-11-14T16:49:00.000Z

Sophie and I popped to visit a friend of mine and her children who we hadn't seen in a while.

I was explaining to Sophie the day before who we were going to see. She turned to me and asked of my friends daughter, "does she have cysty roses like me"?

Eh? Come again?

I asked her if she knew what that was and she said, "it makes my cough bad and my chest poorly".

Hmmmm.

How little credit I give her, I honestly thought it would be much longer before she started to notice that not everyone deals with what she does. When on earth did she grow up so flipping fast?

No time left to sugar coat things anymore.

Don't get me wrong, obviously Sophie is very aware of hospital, doctors, tests, procedures, mic-key buttons, ports... The list goes on.

But, this is the first time Sophie has actually put a name to it and I was very taken a back if I'm honest.
How long she has known, I mean really known I don't know, but now there is no going back.

We try and make her life as 'normal' as possible so that she lives a life with CF, and not a life full of CF.

Having her first proper CF exacerbation at the moment has just reinforced that the beast we have tried so hard to put out of the forefront of our minds, is still very much there in the background.

Steroids have started and already she appears to have a little more energy but she is still poorly. Ian and I have forgotten what sleep feels like as the poor mite is up all night coughing and in the depths of the dark, all we hear is that gut churning, toe curling, sickening cough that makes any parent shudder to the core.

I just hope that the latest treatment and ultimately the bronchoscopy and IVs will give us our bouncy, full of life 3 year old back.

Take care and thank you for all your lovely comments.

xx

Time, ladies and gentlemen.

2010-11-10T14:20:00.001Z

After looking back over the last couple of months, I have actually realised how long Sophie has not been right. She's been on antibiotics since the beginning of October with no real improvement. She's not been her usual rubber self and bounced back to her baseline.
This has proved to be uncharted territory for us as Sophie has never really been particularly poorly.

We have a plan.

Tomorrow, Sophie will start 2 weeks of oral chlorenphenicol and 5 days of steroids. Her port flush will be postponed for 1 week as on the 26th November, she will go into hospital for a bronchoscopy and then start 2 weeks of IV antibiotics.
Any deterioration between now and then and she will be instantly admitted for IVs and the bronch done at a later date.

Hopefully the hospital stay will only be a day or two as we plan to do the course at home now Ian and I are both happy doing them.

Cant say I am surprised as Sophie has been coughing none stop day and night, which is completely off her normal.

Lets hope we get some answers from the bronch, and the IVs ultimately see her through the rest of the winter.

We consider ourselves fortunate knowing that she could have chest complications due to her TOF scarring, that she hasn't been admitted for ill health before now.

I will leave you all with a couple of pictures of Sophie's new 'pet'.
Meet Lulu, a very life-like cat that meows, purrs, moves, and feels like a real cat!

Sophie loves her.

Take care everyone.

x

Dear Mr & Mrs Pseudomonas

2010-11-08T23:15:00.000Z

I appreciate that you have been incredibly satisfied with your lengthy stay, so much so that you have moved your family into the property, but I now feel that the time has come for you to find alternative accommodation.

You are hereby informed that you have 7 days to vacate the premises that you are currently residing in which is the legal property of Miss Sophie. You have been living in her lungs as a tenant without causing any real unrest or discomfort to the property owner for the past year or more. However, for the last month or so, you have not been sincere in adhering to the advice given by the owner about taking proper care of the property. You are requested to leave the premises and remove all of your possessions.

I hope you will take action to evict the premises within the stipulated time, else the hospital will have to take immediate action against you.

Yours faithfully

Angry Mum.

Lets get physical

2010-11-06T15:40:00.001Z

These are some pictures I took of Sophie on the day she out ran a 'healthy' child.
It was hard getting any decent pictures as she wouldn't stay still for more than two seconds!!

This is what she was up to...

Take care all.

xx

Sophie always has a cough.

2010-11-05T11:50:00.002Z

This is what one of Sophie's friends from nursery said to his mum when we had bumped into them at a local indoor play area at a farm.

It didn't upset me, it made me realise how kids accept things as 'normal'. This is what he had noticed about Sophie but wasn't phased by it, didn't ask anymore. Just that was Sophie.

The one problem I do see is I don't want her to be defined by her cough and ultimately her CF. I have no idea how to change that.

Sophie will always have a very prominent cough, partly CF, partly TOF. It's loud.
I just want her to be recognised for her and her own accomplishments, not by some disease. I don't want it to define her, she is already an amazing person in her own right but people do not always see past 'the cough'.

Luckily her little friend didn't care, they played for hours. Lots of running, giggling and cuddles, it was lovely to see. What was even more fantastic was Sophie was able to out run him and her stamina level is very very high. He was sat, out of breathe while Sophie continued to climb, run and bounce.

That's my girl.

Sophie is still 16 kgs so no weight loss still, grand! We also tried the garlic however we are switching brands as the odourless one we chose made Sophie smell like she had been rolling round in a whole bag of garlic. It kicked a cold into touch but no one dared go within 50 paces of her.

We are getting the correct brand of Kyolic garlic by quest, this is the one that is constantly talked about and wont leave Sophie looking like a cartoon character where they show the garlic smell, green, steaming out of them! It was bad!!

I'm waiting on the CF team to contact us to organise the next port flush and also the increase in her nebulised saline. I'm very excited about increasing as Sophie is already coughing well during these treatments.

Just over a week in of the antibiotics and very nervous about how the bugs have reacted. Only time will tell. One clear swab means nothing, you have to take a full picture over a period of months to be sure.

Take care all and lots of positivity to all those with their own battles to fight at the moment.

xxx

Thank goodness for the extra hour!

2010-11-01T17:45:00.007Z

I know some people tend to complain about British summer time ending and our clocks being turned back an hour, but this year I was very very grateful.
I used to love this time of year, always had until we had Sophie and winter became a time of dread. Most people dislike the eternal dark days, others dislike the reality of cold winters. I dislike it due to it being a sign winter is really here and with it lots of bugs and nasties.

However this year I was very glad to gain the extra hour. Why I hear you ask?

Well, after giving Sophie her final feed and medication on Friday night her Mic-key button decided to block in spectacular style. Nothing was budging this build up.
In an ideal world we would have just changed it, but Sophie decided she was going to scream the house down.

Was she in pain?
Was she just being mardy as it was late?
Was she hating getting wet from the ever increasing spurts from the blocked button?
Was she just scared of the numerous syringes Ian and I kept producing like we were some king of magic act?

We still don't know now to be honest, maybe a combination of all of the above.

It became ever so apparent that we were not going to be able to sort this out without her being sedated. So on the phone we get and after speaking to some lovely ward nurses and the doctor on call we set of on our 45 minute journey to her CF hospital at 12.30 am!

On arrival and after being checked in, cue a lot of puzzling over how we would get the sedation into Sophie.

Hmmmm.

Couldn't give her the normal dose orally as she wouldn't take it due to her oral aversion.

Couldn't give it IV as she has the worst venous access going and she needed sedation to access her port.

Couldn't give it via the button as it was totally blocked.

Step up a very clever doctor. We were able to give a minute sized amount of solution orally (talking 1 ml) but it was the strength she would normally have.

Result!

After thirty minutes of giggles and I love yous from Sophie it had worked, and button was changed in less than a few minutes.

We had to hang around for longer than normal as the team on call didn't know Sophie and wanted to make sure she wasn't going to react in any way to the sedation.

On the way home, Sophie was going through the emotions of her sedation.
I call it her drunk-induced-emotional-outburst phase.
She covers every form of drunk person, from happy drunk to angry drunk to emotional drunk to violent drunk.

Well in this state, sat in her car seat in the back of the car, clutching on to an empty can of red bull she had found in the car, I happened to glance at her in the mirror. I looked at Ian who had clocked the same. If a police car had pulled along side us they would have thought we'd just picked her up after a night on the town. In the wrong light, the energy drinks can looked like beer and her face just screamed, look at me I'm sozzled.

Oh my. Another glimpse into the future me thinks.

At 5.30 am we finally arrived home, tired and I was due to start work at 8 for a 12 hour shift!!

Don't worry, I rang work and went in at 2pm after some sleep. Sophie finally fell asleep at about 8am so not much I may add!

That night was the dreaded turn back of the clocks. Days get shorter, nights get longer, however it meant we gained an extra hour of sleep so I was able to catch up before my next 12 hour shift on the Sunday.

Saturday night after I had finished work, we went to my dads house for fireworks. Sophie adores fireworks and isn't so keen on Halloween at the moment so we thought we'd let off some fireworks instead of the Halloween stuff.

We started off with this many...

Which increased to this many...

We all had some difficulty snapping the fireworks and this is the best effort we have.

Sophie loved it but we still have some left as she was totally exhausted from her adventure the previous night. It's bonfire night this week anyway so I'm sure we'll make good use of them!

That's all for now folks so take care and thank you for all the advice and comments. Will keep you all updated on the garlic.

x

Garlic!

2010-10-29T15:57:00.004+01:00

We had to delay starting Sophie's clarithromycin (antibiotic) due to the MMR jab. This was because it is a live vaccine and the antibiotics would have done something funky to it and possibly stopped it taking affect. Meaning, she would have had to have a blood test to test levels and if low, a repeat injection.

Obviously we decided to wait a few days after checking with the CF team.

Well now we are on day 2 of 14 of operation kick Staph up the arse. Time to see how this little monster wants to play the game and what it's next move will be.

Sophie has another cold. Seems to be too coincidental not to be linked to her flu jab. Obviously she was brewing a new cold or the old one hadn't fully shifted before the jab. She's okay in herself except for tiredness periodically.

The nebulised saline is continuing to work. The amount of crap Sophie is shifting at times is alarming.

It is great, but I say alarming because all of this gunk was sitting down in her lungs causing mayhem, and it is no wonder, Mr and Mrs Pseudo had found themselves a nice, warm, hospitable habitat.

I cant wait for the strength to be increased to see how this couple react to it.

I've also ordered some garlic supplements for Sophie. The UK CF forum are constantly talking about how great it is at stopping colds in their tracks. It's a natural product so I thought we'd give it a go. I did struggle to find a suitable form to give Sophie as it needs to be given via her Mic-key button and eventually found a tablet form that could be crushed. Also went for odourless too!

Is anyone out there taking garlic and seen an improvement in cold prevention?
I'm interested to know.

Sophie spent her time with nanny this week at a farm and also visited a pet shop, so really sorry but no Friday food fun this week.

Sophie's nanny managed to get this picture of Sophie at the pet shop with a rabbit. The kind lady said it was nearly feeding time so allowed Sophie to feed the rabbit a carrot. Cute.

Almost forgot to mention that Sophie has started to allow us to give her chest percussion!

This is a huge turn around and although we have kept trying for 3 years, we fail miserably but our little miss happily grabs a pillow and lies across my lap and lets me do her pats. She's got a long way to go before she tolerates a full session but the relief I feel is huge. Anything Sophie will allow to beat the crap out of her lungs is priceless. I just hope we can help her out a bit and give her a break from some of the nasties in her lungs.

Will leave you with another picture of Sophie from her nanny. She wanted to show me her new PJ's. You can also clearly see the scar from her port(Bertie) operation.

Take care all.

xx

It's a new day.

2010-10-25T17:00:00.000+01:00

Firstly, I wish to express my heartfelt thanks to the overwhelming response I received on the last blog post.

It was unexpected but well needed and everyone of you in blog land rock!

Thank you.

On to business.

Plan of action is two weeks of clarithromycin to try and get baby Staph monster to vacate the party. I'm not going to get over excited, I've learnt the hard way it doesn't pay off to be over hopeful.

Don't get me wrong, my fight alongside Sophie is as strong as ever, more so in fact, but I have to stay level headed and realise that not all battles can be won with this disease. You do have to look at the bigger picture though, that's what counts.

It's about finding the balance. I've noticed I get burnt out emotionally after a while as I put all my energy into fighting whichever obstacle gets in our way at any given time.

This isn't healthy for me or Sophie.

So I have now realised I cannot control EVERYTHING. This is very hard for me to admit, I am somewhat of a control freak by nature, but admitting this finally may help us all out in the long run.

No longer am I going to waste emotional and physical energy on the uncontrollable, it would be better well spent going to Sophie and Ian, and used to enjoy all the brighter moments in life.

What is the point of fighting for life if it is never enjoyed?

Sophie has started on the saline but a very low dose, her team have decided to gradually increase the strength over a period of weeks. So far she loves it and it is also making her cough more up, which was the whole point of it.

Looking at how her body has responded to a very low dose, I am very excited as to the results we may see as the strength is increased.

Sophie will also be having a bronchoscopy finally.

We had been asking for well over a year if it would be beneficial and finally the team are on our page. They'd like to know what her mucous is like and the state of her lungs in general. Also good for taking lots of samples and getting a true picture of what lies beneath. The dates will be decided but it isn't an imminent procedure.

I am grateful that Sophie has always had a good cough and the ability to get stuff up and out when we need it for cultures.

We aren't treating her blindly, we don't have to wait for her to become ill to get treatment, and the bugs aren't generally allowed too much space to build up a whole colony as we give them a full on beating down because we know they are there.

This is something I am thankful for.

Sophie had her pre-school boosters and flu jab today, she was awful, fighting the nurses and I hadn't seen her this bad for some time, but as soon as we got out of the surgery she was fine. Thank goodness!

Weekly weigh in shows Sophie is still 16 kgs, so no weight loss at all since starting her tube wean!
Sophie is still trying a range of foods and is starting to bite into more and more things. All still going really well, which is unbelievable.

And,for all those parents out there who are having difficulty with their children and treatments here is a picture of Sophie doing her colomycin nebuliser.

It used to take both Ian and I to give her this. She used to fight and kick and scream. After just persevering, this is how she does this neb, every day.
Peaceful and calm, so don't give up, it DOES get easier :)

I'll leave you with a picture Sophie's nanny sent me, of Sophie in a tunnel at a park near where she lives. She loves being outside :)

Take care everyone!

xx

What lies beneath.

2010-10-22T11:40:00.004+01:00

Invisible.

In the dark, deep depths of my daughters lungs.

I text Sophie's CF nurse today to find out when to pick up the prescription for the HTS and also to find out if the sputum sample had been more than just saliva.

Chest Xray had been reported and the great news is that the upper lobe collapse had resolved and had completely re inflated. Everything else looked normal but will be waiting on a full comparison of previous films to find out if there have been any significant changes.

CT scan may be considered.

The sputum culture was mostly saliva as expected, however some sputum had found its way out as we have results.

Mr pseudo was still enjoying his environment as he was his usual punctual self and still fully sensitive to all antibiotics.
Clearly he is so impressed with his habitat that he has now moved his wife in.

Mrs pseudo.

This is one lady who clearly wears the trousers as she is one tough cookie. Excuse my expletives but she is actually a bit of a bitch.

She is resistant to ciprofloxacin.

On reflection this isn't the first time we have met Mrs pseudo as she single handedly was responsible for the start of Sophie's triple whammy of IV antibiotics at the end of last year and beginning of this year.

This however, is the first time the two have put on a united front on the culture plate.

Now we are left with the realisation that Sophie cultures not one but two strains of pseudomonas.
I say strains lightly as they are both pseudomonas aeruginosa but are still two seperate entities.

You'd think they'd be content with taking over Sophie's lungs but clearly not as they have decided to adopt.

They are now the proud parents of a Staph baby.

You may remember Mr Staph making his debut a few blogs back and we thought he'd left quietly.

How wrong were we?

He's still very present and now this leads us to the question, what the hell do we do now?

Sophie's CF nurse is currently consulting with the pharmacist on dosing and will be in touch shortly.

Current options are..

Pseudomonas 1 and 2 will be left for the time being as not causing any bother and already covered with nebbed colomycin.

Staph will be hit with oral antibiotics. Either clarithromycin for 2 weeks or clindamycin for 4-6 weeks to try and eradicate it.

Or option 3.

2 weeks of clarithromycin, followed by a cough swab/sputum sample and if still present then 4-6 weeks of clindamycin.
If this all fails then 2 weeks of IV's will be on the cards.

So now we wait...

To see Sophie you would have no idea she has a family of bugs playing house inside her. She is symptom free. A great clinic appointment means nothing and all it does is teach you not to let your guard down for a minute.

So frustrating.

I'm tired of CF today. It's making me angry as I cannot do anything about it.

I hate that Ian and I have both got excited about starting a new nebulised treatment on our 3 year old daughter. In any other life it would be horrifying but in a CF life it's different.

It's a chance of improvement.

It's a chance of damage limitation.

It's a chance of prolonging Sophie's life by maybe a few years or more.

This is the reality of CF. This is living a life alongside CF.

This is our life with CF.

On the menu this week, fruit skewers.

Take care everyone.
xx

Clinic visit.

2010-10-19T19:00:00.003+01:00

Today has been very interesting!

Ian and I got Sophie ready and off to clinic we went.
First was outpatients to see the whole CF team.

Height good.
Weight stable.

Sophie allowed the doctor to listen to her chest and that was nice and clear. On the whole, they have no concerns with Sophie as she looked really well. Brilliant.

Obviously we have the issue with the chronic pseudomonas but there isn't much that can be done about it as we have all bases covered. Just a case of hope and wait for someone to create something that will blast it away. Currently causing no issues that we can see.

Sophie's psychologist had spoken to all of the team just before our visit and had explained all of my concerns and feelings etc.

It worked, and worked well.

All of the doctors kept asking if we had anything to ask and when we enquired about trying Sophie on hypertonic saline (HTS), her consultant didn't even hesitate.

He said absolutely!

Yes you heard right, we will be able to try HTS as soon as the prescription is sorted and we can arrange to test it in a controlled environment to check for any reactions. Unsure of which strength she will start on as I think they will start on a lower strength and work up as needed and tolerated.

So happy. Just want to give Sophie every chance of easing her pseudo problem.

We were also handed an Xray card to try and get one done when she was sedated.
We were shown the Xray Sophie had done just after her port insertion. She had some upper lobe collapse which we were never told about.
The thought is the ivs she had would have corrected it and as she is clinically well then they aren't concerned but wanted this one done to absolutely rule it as reversed.

Off to the ward we went with Sophie's crew in tow (CF nurse and psychologist)and sedation was given on arrival, 20 minutes of play time with the nurses and we had a very drunken Sophie. Port flush was done with ease and instead of using emla cream they used the coolant spray, which we all feel will probably be better for Sophie instead of emla.

We then whizzed up to the xray department and were able to get a chest xray done however it was in surpine position (lying down) as there is absolutely no way Soph will sit up for one currently.

We weren't going to argue as at least we got a good one done!

So all in all a fantastic visit. Just need to wait on the sputum culture we took in but it may be bounced back as it was only a very tiny amount. If that is the case then we will need to get a cough swab sorted.

Hoping she grows nothing new and that her xray is clear.

As soon as we start the HTS will let you all know. We are also keeping her on her DNAse and although this means an extra two nebs per day, we don't mind if we get the results we are expecting.

We visited my dad and step mum and they had bought Sophie a small project. It was a felt reindeer hand puppet that Sophie had to stitch together and glue on the face pieces. She only needed help with the stitching and was left alone to glue the detail on. This was the end result!

How well did she do!!

Very proud...

Playing with some of the string..

Just wanted to say hello to Cheryl and Katey who are new to our blog and hope you both come back soon and also thanks to Gem and Stacey for all of your lovely comments. I really appreciate them and all comments from everyone else!

Ooooh nearly forgot to say, Sophie's weight is still 16kgs! Yay.
Sophie ate half of a pancake and a pot of yogurt at nursery yesterday. She's doing amazing and we are all really proud of how well she is doing, learning to eat.

Take care everyone.

xx

Light at the end of the tunnel.

2010-10-15T15:19:00.006+01:00

Today we spent the morning at the outpatients department with Sophie's CF psychologist. Again to work on her anxiety with all things medical.

She did great!

We got her height done, 95cm and her weight was 16.22kgs (dressed and on hospital scales).

Sophie was able to navigate round the department and tell us exactly where certain procedures happen to her and which were her rooms. I'm so proud of how far she come and how much calmer she is.

She isn't completely there yet, still loads of work to do but the improvement is amazing.

We took a (long) walk down to the other hospital building to spend a little while on her CF ward and to meet up with her CF nurses.

Sophie spent the whole time playing in the outdoor area where everyone that came out to us, ended up riding round on bikes and scooters.

I wish I could have photographed them all but couldn't, I did manage to snap a few of Sophie though.

She managed to have a good run about and obviously some physio as she did have a good coughing session.
She was much more relaxed on the ward too so all in all, great visit today.

Sophie was also given a near complete version of her book that her psychology team have been working hard on. I must say it's great and Sophie is really interested in it.

Again I am limited as to what I can publish on here but in addition to some of the pictures I have blogged before, here are a few more. I highly recommend doing something like this for any little ones that are struggling with hospital visits.

Sophie happily getting onto the scales, all of the pages have some sort of caption on them.

This is Sophie having her cough swab done, had to cut out lots of faces out of this one!!

This is what Sophie's port looks like when accessed, here she is having her monthly flush done (Bertie's drink). The redness was caused by removing the sticky, clear dressing and isn't a reaction btw.

Off to Clinic on Tuesday and also port flush due. Will let you know how we get on.

Again thank you so much for your responses to my previous posts. I have taken everything said on board and am just going to be myself when it comes to asking questions. Lets see if we get anywhere with the hypertonic saline. I only want to trial it with her, just to see if it has the same effect as the sea air does.

Sophie's nearly completely back to normal after this virus and her antibiotics finish in a few days.

Fridays food fun.

Fruit bread.

Smiley cook.

Concentration is key you know :)

TaDa!

Take care all and have a lovely weekend.

xx

Weight update

2010-10-12T20:45:00.003+01:00

Very short update from earlier (see previous blog post)

Sophie does indeed weigh 16 kgs!

Woohoo! My little porker has actually got some calories out of her tasters. Still very early days and a very long way to go but it's still fanbloodytastic :)

Even more so fab as she has been poorly.

Go Sophie.

x

Frustration or anxiety?

2010-10-12T17:00:00.001+01:00

We are due at CF clinic on Tuesday. This time, Ian is coming with us so he can ask all the questions instead of me.

I still feel like I cant ask anything at the moment, despite the reassurance I can and wont be judged solely over my profession.

The main subject is the possibility of trying hypertonic saline. There are one or two other things but I will blog about those after clinic.

Again I must add that I hold all of Sophie's team in the highest regard, they are amazing.

I had a call from Sophie's CF psychologist today. We are meeting up on Friday to just have a fun day in outpatients and on the main site where her ward is. This is again to work on her hospital anxiety.

I honestly didn't think I was still feeling as bad about the whole nurse vs mum thing.

I am.

Soph's wonderful psychologist will be speaking to the whole team before clinic to try and explain my side of things and how I am feeling. Hopefully this will work and we can all move on and lower our defensive walls once and for all.

I cant decide where it all stemmed from. I guess it has been an accumulation of things, possibly misinterpretation from all parties too.
I also cant decide whether I am just totally frustrated that I cant control Sophie's CF or whether I am just completely anxious about the whole thing and that I probably know way too much.

Anybody have any ideas or thoughts?

Anybody feeling or felt like this?

I guess I'm just having a mummy wobble at the moment, doesn't help because both Sophie and I have been poorly. I'm finally on some antibiotics as I was actually coughing up stuff way more colourful than Sophie!

Nice ;)

Anyways, we are both feeling much better and thank you to everyone for your well wishes, really sweet and actually needed. You guys rock.

On the eating front, Sophie is still doing really well, I am so proud of her.

She's trying many different types of foods now and actually asking for things she will at least lick and she has also been biting into things.

Yes you heard right, BITING.

She ate nearly a whole cracker at nursery, her chewing technique needs some work but this girl is learning!

I did her weekly weight this morning but I am going to repeat it tonight only because that is the time I normally weigh her, so I want as true of a reading as I can get.

I will do a very small blog later to compare it to the one I got earlier.

Last week as you may remember she weighed 15.5kgs, this morning she weighed 16kgs!
I am not doing cartwheels just yet though, as I want to check as I cannot believe she has put on 0.5kgs in a week!

Random photo.

Another picture from Esme's christening. Sophie has no issue with dirty floors it would seem!

There will also be some interesting news over on our feeding problems blog later in the week so keep an eye out for that.

I have added a new page to the top of my blog about Sophie's daily treatment regime for anyone who may be interested in knowing what she does everyday.

So Friday we meet with the psychologist, and Tuesday it's CF clinic AND port flush.

Take care all.

xx

Feeling rough.

2010-10-08T15:25:00.009+01:00

This Autumn has met us with one almighty bang, in fact more like a huge kick up the backside if I'm honest.

Sophie's cold has sort of shifted. I say 'sort of' as now her nose seems blocked and she's still producing an impressive sputum fountain off of her chest.

So hoping that these little lovelies will do the trick.

She's actually about five days into a two week course now but although she's not cough, cough, cough all the time, she's much more productive than I have seen her in a long time. Guess this viral bug has stirred up Mr pseudo from his slumber.

I'm still suffering from it too and I have to say I feel horrible, really tired and it's hit my sinuses like a ton of bricks. Thought we'd both recovered but it's come back with a vengeance.

Poor Sophie.

At least I can understand why she's being so grumpy and sleepy. Just hope it buggers off soon!

She's so pale at the moment except for when her cheeks are flushing due to this bug.
She managed to get to nursery today although more interested in the green bus that drove past as I took this picture!

She's Still taking small tasters of food and no major problems on the wean front still so nothing really to report, which is great news.

Enjoying what probably is the last of any decent weather for a few months!

Sophie's nanny has been spoiling her this week, she's bought her her own chefs outfit complete with hat, so cute.

I'll leave you with a few pictures of Sophie busy in her new outfit at her nanny's. Sorry for a short blog but I just want to go and sleep!

She made some cakes and biscuits in the shape of people this week, delicious!

Take care everyone and speedy recoveries to all who need it.

xxx

Mmmmm!

2010-10-04T16:35:00.005+01:00

Baked Beans and toast for breakfast.

This is what Sophie is constantly asking for at the moment. She doesn't chew the beans because she has absolutely no idea about the concept of chew and swallow when it comes to food.

This is something that we are going to have to teach her.

She likes to dip the toast in the sauce and suck it off the toast. On occasion she will put a few beans into her mouth and suck them and then spit them out.

All progress though and I cannot believe how well she is coping without nearly a quarter of her daily intake. No major tantrums yet, just asking for lots of things to try. She has also taken to sipping actimel yogurt drinks, some days she tries more than others but, hey, it all adds up and calories are good!!

Sophie's weight on our scales last week when she was having her full tube intake was 15.5kgs, and this week, her weight is...

15.5kgs!

So she has not lost anything which is awesome!!

This means that the little bits of things she has tried have actually added up!

Still a very long way to go though and weight loss is going to happen but for this first week, her weight has stayed stable and I am so happy :)

Go Sophie!

On the cold front, Sophie has started oral antibiotics today. She seems brighter in herself but her cough is wet and nasty sounding so hopefully 2 weeks of orals will help. We are at CF clinic the day after her antibiotics finish anyway and her port flush is also due.

I'm not worrying about a thing this time though, as Ian is coming with us to ask all the questions I want to, but as it will come from him I cant be accused of asking too much etc

I really want Sophie to try hypertonic saline.

When we were last on holiday right by the sea, for the first two days she coughed so much crap up off her chest it was almost worrying, but it soon settled and her chest sounded crystal clear. We initially thought she was having a flare up but then quickly realised that the sea air had actually given her a well needed clear out.

Two possible problems though,

1) Her consultant isn't a huge fan of hypertonic saline.

2) She is already on Pulmozyme/DNAse, which we had to fight for and it really has done a grand job of clearing out her nose/sinus's and we don't want it stopped. It just doesn't seem to work as well as the sea air clearly did (which was partly what made them develop HTS).

Her consultant doesn't want to add too many treaments but Ian and I would much rather put the time in now to prevent lung damage occuring sooner rather than later.

It's so hard, does anyone have any thoughts, experiences etc with hypertonic saline vs pulmozyme or use both?

Fore-warned is fore-armed.

Thanks for all of the well wishes, our boiler broke over the weekend and left us with no hot water or central heating! Timing is everything isn't it? but luckily Ians brother is a plumber and was able to do a patch up job for the time-being as he is going away so now we have a nice warm house again!

Sophie's injections have been postponed for 3 weeks to make sure she is well over this virus and off her antibiotics. She'll be getting her flu jab at the same time.

Take care and lots of mendy thoughts to all of you struggling at the moment.

Love to you all.

xx