Isaac is doing really well. One week into IVs and his lung function which was down 20% on his baseline (already not great) had increased 13%, which for one week on is fabulous. So for now, we continue home IVs, and all going well (symptomatically) they will end next week - perfect timing for Christmas.
Good news for us, but sad news from the wider CF community this week. This is a unique community in that we cannot meet in person without high risk of cross infection (...pretty sure this is unique, as I’ve not heard of it for any other chronic illness? But I could be wrong, t’is not rare!). The bugs which inhabit CF lungs so merrily come in many strains, but from just a few species. They realised back in the eighties that certain strains were prevalent in certain CF centres leading in one case (the Liverpool strain of Pseudomonas) to many deaths. And so came about the end of CF holiday camps, sitting in waiting rooms with other patients, and the start of segregated clinics and beds on the ward so far apart you would never see the other patient. The closest we come to another CF patient in person these days is a knowing smile and a wave across the hospital concourse. As a result, the internet provides those affected by CF our only means in which share knowledge and learn from others who really understand life with CF and what that entails. In those early years, getting over diagnosis, the CF parents forum was my lifeline (shout out to Softie, a kind a generous soul who had CF himself, but spent much of his life advising us parents of newly diagnosed kids from the risks of sandpits, to how to sterilise neb parts, to how to cope with questions from others such as the common ‘that’s the one where you bash them, right?’).
And as in any community, there are some faces that stand out, the social media ⭐️’s of CF so to speak, and this week we face yet another death of a young activist, advocate, and generally lovely person. In my time as part of this community, I have known far too many die (far too soon) and while CF affects sufferers in very different ways, these losses have broadly fitted into three kinds;
1) Those who decline in health, usually in their teens or twenties, and are listed for a lung transplant.
Of these, there are the in the 1 in 3 patients who tragically die waiting for lungs due to a shortage of willing donors (please sign up here ).
Or those who do get a chance at a new life with new lungs, but die following complications post transplant (as with my friend Jayne. Who I miss so much).
Or those who initially do well with new lungs, but who later suffer from rejection (as was the case for beautiful Eva, who inspired so many).
2) Then there are those who decline in health, usually in their teens or twenties, for whom transplant may later become an option, but who tragically die from an infection very suddenly. I am not sure of the details, but in Lea’s last vlog she casually mentions she is starting a new IV drug for her current infection, pretty normal CF stuff, and yet a week or so later, she has passed.
3) Those who decline in health more gently, and may survive their twenties, thirties, even forties before being listed for transplant (if they even opt for transplant). Or have a transplant earlier, and avoid rejection for many good years.
Now, it makes perfect sense that when you are at your sickest, you’re much more likely to be home, alone more, and online. Whereas when you are well, you’re more likely to be out and enjoying life too much to be online, and so the internet can skew perceptions. I get this. But one thing is for sure, I know of many, many more in the first two categories than I do the last; and this can be pretty terrifying (‘sudden decline’ especially after our stint on intensive care last year. Too close. Too real).
While the internet gives us an amazing forum in which to talk about our experiences, and share fears and triumphs with the only people who really understand, it also creates bonds between people who have not, and cannot ever meet. I would not be without my CF community, but with this comes pain and incredible heartache too, despite us never having met.
I shall miss your smile Lea. Sleep well x
To support our fundraising for the Cystic Fibrosis Trust who fund seriously groundbreaking research, please click here. CF is actually one of the areas of medicine where a cure is a realistic idea, but they lack the funding to achieve this potential. As such, myself and a bunch of my amazing friends will walking the Great Strides walk in the Surrey Hills in June 19. For me, a bonkers 67km in a single day 😬 x
