National Transplant Week 2015 - A Day in the Life....

Hi everyone. As it's National Transplant Week 2015 here in the UK right now I thought I'd dust off the blog to try and help to raise some much needed awareness of organ donation and the transplant process. As some of you may know I am now active on the transplant waiting list so I thought I'd try and get across an average day in my life and exactly what it entails. If anyone has any questions about Cystic Fibrosis, organ donation, or the transplant process then please feel free to ask in the comments. I hope it helps people to understand things a little better.

09.00am - I wake up exhausted no matter how much sleep I have, which is usually a lot less since going on the list. Bedtime brings thoughts about being on the list, if you'll get a call, about your friends who have already died and just how long you will have left if you do get a transplant. As soon as you wake up you have to clear your chest of the sputum which has built up overnight so there's lots of coughing and hacking which adds to the tiredness. I'm usually in some kind of pain too, if you sleep in an awkward position or slip down your pillows (I use quite a few now to prop me up in bed and assist my breathing) whilst asleep then you'll wake up aching so you'll need to take some pain relief which leaves you doped up so already a great start to the day. 

09.50am - the carer comes to help get my nebulisers, tablets and anything else together whilst checking that I'm ok. Whilst I'm doing my nebulisers which have to be spaced out at 30 minute intervals she makes me breakfast which I have to eat no matter how sick and exhausted I may feel. It's massively important that you keep a good weight on when you're on the transplant list as it is vital to getting through the operation and recovering well afterwards. She helps get my clothes ready whilst I'm eating and then I get showered. 

10.45am - Showering isn't as simple as it used to be. I have to wear my oxygen whilst getting showered now as it makes me very tired and breathless. I know that sounds ridiculous and it makes me feel like an invalid at times and I now have a shower stool so I can sit down. The worst part is that I have to rest for 15 minutes after a shower too to let my oxygen saturation levels return to normal and I literally don't have the energy to do anything else. I haven't lost my dignity yet by being showered by my carer but that will come, eventually. 

11.05 - I'm exhausted and desperate for a sleep. I can't have one though as I need to complete my morning treatment regime by taking my steroid inhalers which help keep my damaged airways open. 

11.15 - I still can't rest. I have to do a morning physiotherapy session. 30 - 45 minutes of exhausting breathing and coughing using a PeP mask. It looks a little bit like an old fighter pilot mask and helps open your airways so you can cough up more of the thick sputum filling your lungs. 

12.00 - I finish my morning physiotherapy session and I'm shattered. I now have a little free time. I may nap. I may read. I may watch something or play video games. I'm limited as to what I can do now. I'll usually have calls to make to oxygen companies, chemists, arrange appointments and such like to make sure that I'm fully stocked up with all of the things that I need to keep stable. 

14.00 - my carer returns for my afternoon call. I only have one nebuliser to take which is a slight relief. Whilst I'm doing it she'll make me an afternoon snack and drink, you gotta' keep those calories going in! Then I have my pulmonary rehab exercises to do. These are designed to keep your strength up whilst you're on the transplant list so it's stuff like standing press-ups, weights, walking up and down a step for 5 minutes, core strengthening. All to keep your muscles strong so you can recover from the transplant procedure. At this point however, it adds to your exhaustion so is very, very difficult to maintain. 

15.15 - I'll try and sleep, or watch something but I'm usually overtired and don't have much success, or my mind will be wandering so I usually come on here or Twitter and piss people off. 

16.00 - 18.00 - I'll try and get up and about the house to do some chores, sitting on your arse because you're ill is not an excuse and I need to stay mobile too but again, this is now very difficult for me and going upstairs makes me breathless. This is usually the time that the family are due home as well so I like to help out and still have Dad duties to attend to. It's hard because you want to support your family but you're exhausted from your daily schedule. If I'm relatively stable then I will make the family meal but more often these days Helen has to do it after a long day at work which upsets me as I just want to support her and provide for my family because I love them. I couldn't ask for a better family though as we all pull together and support each other so everything gets done, with teamwork, and love. 

18.00 - time for my early evening nebuliser. Just the one to help keep my airways open along with my early evening oral antibiotics. 

20.00 - 21.00 - the nursing team will come and see me. They come in a pair, one healthcare assistant and one nurse. They'll help me get ready for bed and get me anything that I need plus make up and set up my NG feed which I have to insert down my nose and into my stomach. The calorie rich liquid slowly drips through the tube overnight and gives me 1,600 calories whilst I sleep. I usually have it 3-4 times a week but sometimes more if my weight has dropped. This is because CF also affects your digestion making it incredibly difficult to put on and maintain weight. It often leaves you feeling bloated and full though which isn't great when you're attempting breakfast the next morning. The nurses will also get my evening nebulisers, inhalers and tablets ready too and thus, the cycle continues. 

21.00 - 21.45 - evening physio. By this time I generally really can't be arsed but have to force myself to do it, or Helen has to bollock me. It feels like an endurance test as I just want to rest. I want a break from the daily regime of treatment, medication and exhaustion. It pisses me off but I know that once it is done then I will finally have peace. I'll be in pain again so will require more pain meds as you're constantly battering your already weakened lungs every day, desperate to keep them clear and free of infection. 

21.45 - 22.45 - now I have free time to spend with Helen. We'll usually curl up together in bed, with a brew and watch something to laugh at on tv to give us some respite before passing out to sleep, or she'll fall asleep before me so I'll try and read to reclaim something for me in the battle for normalcy, but not before I set my feed off, hooked up to two machines and two sets of tubes coming out of me. One for oxygen, and one for feeding. I'll usually fall asleep at midnight. 

This is a snapshot of my daily life and it's often much busier than this. I don't know how long this will continue for. I do know that it will get worse and much more complicated the longer that I'm on the transplant list. I'm tired of this now. I need a transplant. 

Please sign up to the organ donor register and inform your family of your decision.

#‎CysticFibrosis‬ ‪#‎OrganDonation‬ ‪#‎Transplant

No Future?

So, yesterday was a pretty significant day in the Cystic Fibrosis community. Vertex pharmaceuticals in the USA announced very promising data from their phase three trials of a combination drug that will treat the genetic cause of Cystic Fibrosis directly. The data claims that if and when the drug should become available in the US and EU markets that it *could* offer suitable patients with the double delta DF508 gene mutation anywhere between a 6-10% (or maybe more) increase in their lung function and see a decrease in the amount of time spent in hospital as it seems to also reduce the amount of exacerbations of CF too.


People with the double DF508 gene mutation make up about 50% of the CF community.


What does this mean? Well, it's a bona fide scientific breakthrough as, since now, all medicines and treatments have been tailored towards treating the underlying symptoms of Cystic Fibrosis - this would be the first drug ever to attack the cause directly. The real world implications of this could be staggering indeed, young children born with CF could now take this drug and live a whole and normal life, requiring much less treatments and time in hospital and experiencing much less serious long term damage to their lungs, perhaps for some, none at all.


And yet, I'm not jumping around with happiness... I don't really feel anything about it. Should I?


Undoubtedly this is a major scientific breakthrough the likes of which have never been seen before and I'm glad that many people won't have to go through many of the horrible things that I did growing up. However, part of me feels angry too. There's a lot of people throwing the words 'miracle cure' around right now, people are elated with the news of the breakthrough, which is completely understandable. There's lots of hope and there's lots of optimism that this drug will lead to other drugs and new treatments and that this whole process could potentially be the first baby step towards an eventual cure for Cystic Fibrosis.


I guess, being the super dooper realist that I am I'm just deeply entrenched in cold, hard reality. If the FDA approves the drug in the US it will probably take between 8-12 months to do, then subsequent approval has to be sought in Europe too which will most likely take another year. Then there's the question of whether the already under strain budgets of the NHS can afford to purchase it. There's all kinds of cost quotes flying round right now, some say in the US it will cost anywhere between $160-250,000 dollars per course of treatment for each patient so it is likely to cost a shitload in the UK.


Another issue is one of what about the people who don't have the double DF508 gene mutation? They continue to wait and hope for a similar breakthrough as that's the problem with genetic diseases like CF - any breakthrough in treatments invariably denies another group within the community as it is just that complex to gain any ground on. Irritating bastard that it is.


My anger is for those of us who the drug offers very little for, the older people with CF who have struggled and fought their way into adulthood. Those of us close, or already on the transplant list, and yes, those of us who have already lost the battle.


Time is always against you when you have Cystic Fibrosis, and discoveries like this are too late for many. I can't help being angry about that. I'm not 100% sure if I qualify for this new treatment *should* it become available, my CF unit are busy attempting to categorise people's secondary mutations if they have them so I'm sure they will tell me once they have assessed the information. I'm more concerned that If I am viable then by the time the drug is approved and if the NHS can fund it what state will my health be in by then? 2-3 years is a lifetime away right now, despite me doing really well and busting my balls to stay that way with another transplant assessment coming up.


Don't get me wrong, I'm happy and recognize the massive importance of yesterday's breakthrough. I just wanted to share some thoughts about the harsh reality of how it will affect the CF community as a whole.


*throws gang sign with hands and struts away from laptop*