Tuesday, 22 March 2011

Dear TOF, I'm beginning to loathe you.

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.

Friday, 18 March 2011

I'm not superwoman.

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.

Tuesday, 8 March 2011

There's something about Sophie...

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

Sunday, 6 March 2011

Your help is NEEDED!!

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.


Wednesday, 2 March 2011

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

***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.


Tuesday, 1 March 2011

Clinic and button.

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!

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