Schools out & a question inside...

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.

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!!

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.

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