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One Year on and we are STILL WAITING for Orkambi!!!

Posted by Lorraine Barnes on June 6, 2017 at 9:45 AM

Can't believe it's been nearly a year since my last Blog ..... and we are STILL WAITING to get our hands on Orkambi!  Every step towards getting it approved seems excrusiatingly slow for families like mine desperate to grab a hold of something which might make a massive difference to the lives of our children ..... time is ticking away and Cystic Fibrosis being degenerative by nature means it's not waiting for anyone.

Since my last Blog a year ago both of my sons healths have deteriorated considerably and it's very scary to think of a future for eiher of them.  Joseph had Surgery for a Fundoplication procedure in October 2016 (pictures from this admission below) and since then his life has just become one round of treatments and even more horrible complications - he can no longer be sick, but he can retch when he feels sick, and he does it a lot.  He suffers from terrible wind problems now and eats very little - the Overnight Feed was too much for him to begin with, but that is slowly getting better.  He has feelings of fullness when he's eaten barely a mouth full of food and meal times are a real battle to encourage him to eat just the tiniest amounts of food - most days he eats nothing at all and the Overnight Feed is what is keeping him going.  His weight gain since Surgery has been none existant - we take one tiny step forward and then 10 steps backwards as soon as he picks up a bug like a cold, which he did a couple of months ago.  His lung  function now averages 43 - 49%.  Imagine living a life aged just 14 years old with half lung capacity.  He gets breathless often just by walking up stairs on really bad days - he has poor quality of life for a child his age who should be out enjoying himself on his bike, walking, running and eating properly.  He has one round of treatments after another before and after School which can last up to 2 hours.  He has a ventilator now which blows air in to his lungs to open up the airways to make mucus clearance easier.  He nebulised 3 drugs twice a day - DNase to thin the mucus, Hypertonic Saline to help shift the mucus, and nebulised antibiotics.  He has Insulin due to having CF related Diabetes.  Ursodeoxycholic acid for  -liver disease.  Vitamins for bone density - Vitamin D3, Vitamin D, Vitamin E.  Creon with all food and Overnight Feed.  Probiotics.  Domperidone for sickness.  Various forms of Physio from Percussion, Acapella, PEP.  He has Nasal Polyps so needs spray up each nostril once a day.  He has IV antibiotics every 3 months for 2  - 3 weeks at a time.  His growth is poor and he is noticeably small for his age.  And yet he never complains - but I know he hates CF and all it entails - he just wants to live a normal, happy, pain free life.

Daniel, aged 22, has been undergoing treatment for Mycobacterium infection since September 2016 which began with 3 weeks of intensive IV antibiotics followed by 18 months of 3 different oral antibitics a day along with various Vitamins and has just this week finished another 3 week course of IV antibiotics after picking up a cold a few months ago which settled on his chest - he lost 10% of his lung function which dropped to 66%, which is unusual for him as his lung function has always been mid 70's since transferring to Adult Care when he ws 16.  This worrying slide in health is concerning.

Both my sons are handsome, intelligent and popular young men - as their mother I want them to live a long, happy and healthy life.  I fear for their futures so much now - the older they get the more care they need, the more medication they need, the bigger the worry. 

Cystic Fibrosis causes so much stress not only to the sufferer, but to their family and friends who look after them and every infection my sons pick up these days leads to less and less lung function.

Orkambi to us would mean less worry, less stress, a better securer future - better health would mean so much.  I know Orkambi doesn't work for everyone, but quite frankly when your children are as ill as mine now are, I'm happy to grab hold of even the tiniest glimmer of improvement - we just get deterioration these days - blows down every visit, weight less.

I'd like to personally challenge any of the decision makers for approval for funding for Orkambi to come live with me and my family for 1 while week.  Experience what it's like to juggle all the medication my sons need daily - and their Prescription lists are vast now.  Try drawing up IV antibiotics for 3 weeks and administering them 3 times a day knowing how sick they are going to feel, but that their lungs are priority.  Try listening out for the Overnight Feed pump alarming all night long even on nights it's not alarming because your brain is tuned in to listening out.  Try changing soaked sheets when the Overnight Feed line disconnects.  Try watching your 14 year old retching because he can't be sick, but he can feel sick - watching mucus and spit coming out of his nose, which sometimes ruptures a vessel in his nose which makes his nose bleed.  Try cleaning up blood after a serious coughing fit after a lung bleed.  Think how you'd feel watching your sons suffer every single day knowing you can't do anything to stop the decline no matter how many drugs you pump in to them, how many hours of treatments you do with them - because Cystic Fibrosis by nature is degenerative.  Cystic Fibrosis makes you feel useless.

I'm joining the Cystic Fibrosis Trust in London on 26th June to Protest for Orkambi for my sons, for all those living with Cystic Fibrosis and for all the friends I've lost to this dreadful condition - Cystic Fibrosis is NOT just about Physio - it's time more was known about it and all it entails because it is still robbing us of our young friends and I've lost countless to it over the past few years.  Time to do battle and to fight for the right to have access to these drugs - my sons lives are priceless to me and I'd gladly give my life for them both if it mean't them having a long, happy life - to see 50 and beyond.

I'll finish this Blog with a few harsh reality images from my youngest sons Surgery in October from which he is still slowly recovering from - more body image problems for him to live with.  Do support the Orkambi Protest if you can on 26th June either by attending one of the marches in London, Cardiff, Edinburgh or Belfast, or you can support on-line during the day.  For more information long on HERE




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