A life less ordinary

Recovery was going so well. Bloody viruses. We are back to surround sound coughing (Anouk and Isaac right now), and in response, the live-in bacteria camping in his lungs are throwing a mean and nasty party. 

His lung function is down significantly, his oxygen sats low, his infection markers, high. Five days of fevers have at least broken now, which indicates he is over the virus but is left with the pseudomonas party to fight. He was to be admitted, upon his consultants advice, but he refused. A few years ago, hearing this news from others I would have been insistent (there is no choice! If the doctors recommend it, this is a must!) but eighteen years of experience, knowing his own body, and his own response to infection, we have to respect his choice. Of course, this means extreme vigilance at home (checking his O2 sats often) and IV antibiotics (just two weeks after the last course, which even for us, is a record I think). If anything changes, we rush him in. And to be fair, he has been stepping up well and doing his own IV’s. 

As a parent, I can’t tell you how emotionally challenging this is. It being his choice only. But as a mum of a smart, risk informed and lovely kid; of course we want him home, we understand, and we have to respect his decision. For the first time ever, he told me that being on the ward depressed him. That hit hard. Frustrated? Yes, of course, always, but depressed? That has never been part of his lexicon. Listening to him coughing right now, I’m in tears. His poor lungs. His poor throat. His poor head. His poor muscles. His poor back. His poor tummy, which often rejects meals due to excessive coughing. It’s all just so shitty. But he’s right, the only thing he would have on the ward is access to immediate medical care and O2. Being at home he is happier, more rested, and free to make his own choices, even when limited to just the house.  

We do have huge amounts of hope, but right now, his reality is just crappy. I can’t say anything about clinical trials at all now (NDA) but we do have near hope. For now, I’ll cling on to that, and Isaac’s amazing ability to see the best in every situation. I think I lost that somewhere down the line x x x

Puppy therapy 

Recovery

Relieved to report that the surgery went ahead. One week later, he is headache free, can smell (hurrah) and breathe through his nose once again. I was told the op would be about an hour. Four hours of frantically pacing the familiar corridors, we were finally reunited on the ward. This is the first time that I’ve not been with him in recovery, as he is 18 now, that will only happen in an emergency. Directed to the ward bay, I found it empty and with a strangers belongings on the floor, I’m trying to suppress my mania by this point, and finally find him in the next room. Despite this being his sixth or seventh sinus surgery (we have lost count) we always forget just how bad the first hours are.

The good news is that he was retested with Fentanyl, and had no reaction, so hopefully that was a one off, and we can continue use when needed. He was first on the list (always a very good sign) and by evening he was eating, drinking and getting about just fine. Despite the pain in his whole face, head, and throat from intubation, it’s actually the dry mouth post anaesthesia that he hates the most. After the twelfth jug of water I had to stage an intervention about his drinking issues. 

We are now home and while he should still be on IV antibiotics, he is not. His liver is struggling with the toxicity of one of the two drugs he was on, and it was deemed safer to stop. The hope was to carry on with the course as post sinus surgery, stirring up all those infections, it would give him extra cover (his cough is actually much better). Despite a low level fever for most of the week, his lungs continue to do OK, so we’re happy. Free from all the extra treatments, feels very good - not sure it’s ever much fun to have an inch long needle in ones chest. 

I’ll share what little I can on the gene therapy clinical trials when I can (will be under NDA, so cannot share details on social media) but I can say is that news is patchy, but mostly promising, and we are very, very excited for the NY. 

This week brought news of the NICE decision on the groundbreaking new CFTR modulators drugs (or HEMT as they are often named as now ‘highly effective modulator therapies’ - note the highly effective there!) which work for the majority of CF patients (Isaac is in the 10% who need a different kind of fix). While NICE recognise these drugs as effective, they do not approve the licensing of them in the UK based on cost. I recognise that this is a process, and no child or adult on these drugs now will be denied them going forward, but this is brutal, especially for those under 6 who have not yet started on the drugs and may now not get the chance. Please, if you can, read more here and then register your disgust at the decision which could have knock on effects on research trials and future licensing of effective treatments for the whole CF community. 

Cystic Fibrosis is a life limiting, genetic condition, affecting some 10,000 people in the UK alone. CF sufferers often, as a last resort, need to trade in their damaged CF lungs for the lungs of a generous stranger. To sign up to support organ transplantation, please visit https://www.organdonation.nhs.uk/ and tell your loved ones of your wishes, as even when signed up, the decision becomes theirs after death. Thank you. 

Peace and love x 

Gerhard Richter ❤️