Friday, 14 November 2014

Four little letters.


For anyone that has hidden under a rock for the last week I'd like to draw your attention to this.


For those of you who don't feel inclined to read it allow me to summarise.

Jon Snow is a reporter for channel 4. He broke the above news story via his blog and followed up with a report on channel 4 news and it sent the majority of the CF community into a spin.

Okay, I hear you say but why has it caused such a frenzy?

The frenzy induced madness was caused because the report was about some research that has been jointly done by Researchers in Dundee, France and Italy.

Well that's perfectly normal and happens all the time right?

In this case no. The attention grabbing headline was something to make even the most dubious of folk a little curious.

"Eureka moment offers hope of Cystic Fibrosis cure"

Cure?!

Currently we are in a very exciting time in CF land. So many studies, trials and research are in progress for medications that treat the underlying defect/cause of CF. However they are exactly what they say on the tin. Treatments, not cures.

So to hear about results of a trial that no one has really heard of before be declared as a major game changer for CF got people slightly dizzy.

I'm a sceptic at heart and always like to see evidence, stats etc so with the help of a few friends we did a little 'research' of our own.

It turns out the research, which appears very promising is at a very early stage in its development. The trial was completed with only 10 patients and they each had double DF508. It also appears that this treatment may only work on adolescents and young children only. This is something to do with the lining of the lungs becoming tougher the older you get, but as its so early in trials there aren't masses of results to chew over.

So back to earth we fall. Well not entirely, the research looks very promising but it needs a larger scale study to really see if the results are as good as they have seen in the small group used in the trial.

Another point i'd like to raise is this particular study is using two drugs that are already in use for other conditions so I'm not sure whether safety trials will be as lengthy as others are. It is however a long way off becoming a potential treatment for people with CF.

Back to the magic word. Cure.

To me a cure is a one off treatment that you receive which gets rid of the illness/condition. This particular drug combo, to me is not a cure as I'm assuming the medications would be taken for life?

The media have a lot to answer for by throwing about these types of words in a world where people live in hope and often desperation for something, anything, that will nuke Cystic Fibrosis once and for all. 

I watched a lot of the CF community get excited in a way I haven't witnessed since vertex initially announced the early results of VX770 (kalydeco) quite a few years ago.

It was very sad to then see that hope almost deflated as the reality of the headline began to unravel for those who took it as literal.

People that have lived with CF for a long time are seasoned professionals at being very sceptical when headlines like this are announced, but for those with newly diagnosed children or people who get baffled by science, it raises false hope in a cruel way.

Cautiously optimistic is probably the way I feel but it definitely pays not to stack all of ones eggs in one lonely basket. 

The day my daughter has a treatment prescribed for her that can change the game dramatically is the day I'll celebrate. 

Until then, we will work our backsides off to keep her as healthy as we possibly can.

X

Wednesday, 12 November 2014

Hide and seek.




You know how you can become comfortable with something being there whether you like it or not?

That's sort of how I feel with some of Sophies lung invaders.

I know that sounds utterly ridiculous and I kind of agree sort of, but let me explain.

Sophie has grown Mr P (pseudomonas) since she was about 4 months old and we know how to deal with it. She has daily nebs to keep it calm and IVs every year to proper sedate it. If she becomes unwell and it exacerbates we use oral antibiotics. This works.

I can pretty much tell by sputum colour, amount and changes in Sophs cough as to when it's having a strop and she needs extra treatment.

Over the last year or two, things have changed. She grew MRSA and Mr P pretty much went into hiding. Our new nemesis became MRSA and we threw everything we possibly could at getting it eradicated.

The purpose of the bronchoscopy was to compare to the last and to also get some nice deep samples of secretions to culture. A big deal really as this was to give us the answer we needed.

Have we eradicated MRSA?

Sophies consultant told us Sophies lungs  looked great, no inflammation or signs of infection. There were some secretions as expected (she does have CF after all) but they were pleased. He also saw the narrowing on her right side from her TOF surgery and he said that looked okay too.

Home IVs were started and we were sent away to wait for phone calls as stuff was cultured.

We now have the results.

The only thing that has grown from all of her sputum cultures is Strep A again.

Nothing else.

I should be celebrating but it's left me wildly confused.

Over the moon MRSA hasn't shown up and there is a glimmer we eradicated it with the onslaught earlier this year, but where on earth is Mr P?

I don't for one minute believe it has gone. The theory we are working on is that her current regime is suppressing it so well it's dormant.

I guess time will tell. She hasn't cultured pseudo for over a year now.

We are to plod on with these IVs and they've added in some oral antibiotics to try and kill off the strep A.

It's just news we haven't really heard before and it's left me speechless for now. Possibly a new chapter, who knows but I'll definitely take it.




This is the copy of the proof pic for current school pic. She's growing up way too quickly!






This is Sophie showing me how to stop her IV pump.






And this is my 7 year old flushing her own port line like a pro. Seriously she has the correct speed and will make me redundant very soon ha!






She's also still absolutely loving ice skating and is getting really good! 




And that's all folks! I'm gonna leave it here for now.

Take care all
X

Friday, 7 November 2014

Bronchoscopy and IVs

Hello!

Today we are en route to hospital for Sophie to have a bronchoscopy and start her IVs. 

Praying to the science gods for clear lungs and very very little lung invaders to be found. We'll have some results next week so depending on what is cultured will depend whether her IV meds get switched or not.

All being well we will do her IVs at home after an overnight stay.

Will keep you all posted.

Massively hoping the staph bitch from hell aka MRSA doesn't make an appearance.

Sophies last lung function was great 

Fev1 104%
FVC 114% 

She had been poorly and grew strep A and staph. Just happy, sensitive staph so has had a course of oral augmentin duo and oral clindamycin.
We had to stop the clindamycin a week before the bronch so we don't know if the oral antibiotics have cleared these two bugs or not.

We just have to get the bronch out the way and see what delights grow over the coming days.

On that note we are off!

X

Tuesday, 5 August 2014

Annual Review.




We were a little late sorting Sophies annual review as I had to reschedule her appointment.

All in all things are looking great!

Height and weight are steadily increasing and her chest sounds good.

Lung function left a little to the imagination because Sophie appears to have forgotten how to actually blow into the machine lol

We've worked so hard on getting her to take a big deep breath before actually blowing, that today, she took a huge breath and held it-bless her haha!

By the time she remembered to blow out it was really affecting her FEV1 result (the amount of air she can blow out in a second)

Consequently her FEV1 was 88%

Now this is still within normal range but we know it's a lot less for Sophie but aren't concerned as we know why it was down.

Her FVC (the amount of air her lungs hold) has gone up to 121%! Super happy with that!!!!

Cough swab and a tiny bit of sputum have been sent for culture so we will wait and hope nothing grows.
Interestingly, Sophie hasn't grown pseudomonas since last October. I fear I may have jinxed that now but I had to mention it.

All her bloods are normal except her vitamin D which is a little on the low side. Partly due to diet, partly due to her being fair skinned and having to wear loads of sun cream. We are gonna add a vitamin D supplement in to see if that helps.

We ran round to X-ray for her to have a quick chest X-ray done. She wasn't best pleased at having to wear a gown today!
But she was a star having the X-ray done.

She had a physio session and learnt a few techniques and we now have a new regime to try. So we will see how that goes and if it help her.

Only other things to note are a CT scan and a bronchoscopy in the near future.
CT scan because her last wasn't a great result as it was done under GA and the pics were rubbish & the bronch is to get a true picture of her current lung gunk and what delights are living down there.

I assume these may be done near her next IVs which will probably be done before the cold and virus season arrive. Watch this space.

It's currently the summer holidays so we have been busy on my days off having lots of fun and getting lots of exercise in.

Swimming continues and Sophie is starting her iceskating lessons in September-exciting!

We had a small bit of drama while Sophie was riding a roller coaster. We were just coming down a huge hill as a wasp flew in front of Sophie and stung her on the forehead! What are the chances eh?! Luckily she didn't react and a lovely first aid lady stuck something on it and covered it with a plaster. Didn't stop soph feeling sorry for herself for a little while though!





She was soon back smiling once the plaster came off. Couldn't even see the sting after a couple of hours thankfully.




I will leave things here for now. Just an small update today.

Take care

X
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