Hi all,
It's been a fair while since I last updated - longer than I'd have liked - but in a way its a good thing as I actually have a fair bit to update on as a result...
The additional heart tests involving tracers, a CT scan and an hour long MRI which really tested my lung capacity with one or two of the hold the breath sections, are now all complete and the transplant team have been in touch to say that they will be reviewing my case at one of their MDTs in the weeks after Easter and will call me before the end of the month with their recommendation.
They didn't say anything about the heart scans so I'm hoping that it means that they didn't find anything untoward or serious otherwise they'd surely already have looked to bring me in for further tests, right? In the meantime, I had a video consultation with the respiratory team at the hospital I'm under to try and get a bit more info about the future and what that might look like and when if I don't pursue the transplant.
Unsurprisingly they were fairly non-committal in terms of timescales but reiterated that based on the current rate of decline which has been fairly steady, I'm looking at about 5 years or so before I'm at less than a third in terms of lung capacity and oxygen transfer rate. At this time they'd expect me to require some form of oxygen therapy but were at pains to say that every case is different and that prognosis beyond that is very difficult to say as some people can - as many on here have and continue to demonstrate - live for a long time once on oxygen therapy whereas others can unfortunately fade quite quickly depending upon how fortunate they are/aren't in regards to infections etc.
While the call didn't tell me anything I didn't already know or give me any concrete answers, it did bring me some unexpected reason for greater encouragement in the form of a new drug that I can try taking called nintedanib. Many of you, I'm sure, will have heard of it already and possibly even been part of the clinical trials. It's an anti-fibrotic which can slow down the disease progression by as much as 50% in some cases.
Nintedanib recently received Nice approval and is now available to ALL IPF and ILD patients provided that they meet the criteria. There are potential side effects - principally diarrhoea and less commonly liver damage - and it's never been used for PPFE but if it has any success at all in slowing down the rate of my lung disease then I think it's got to be worth a shot. They're going to get me started next month and have liaised with my GP surgery to organise monthly blood tests for three months which will then become quarterly blood tests to monitor my liver function to make sure that the drug isn't having an adverse affect.
I'm delighted to have the opportunity to try something that might slow down the disease progression and by me some more time whether that be prior to a lung transplant if that's what I decide to do. Even if I don't go for the transplant, it will hopefully keep my lungs going longer than previously anticipated.
Of course, it might not work so we shouldn't get too carried away but it shows that there is progress being made in terms of research even with limited funding. If Asthma & Lung UK are successful in their efforts to get industry to commit to further funding over the next few years then there's no telling where we could go in the future. I was at a launch event recently where a Professor talked to me about all sorts of therapies they were considering which they believe could be game changers in the future.
However, in the short term I think it's really important to push your treatment team on options that might be available. I only found out about nintedanib and got on the list because I kept pushing on what treatments might be available. If I hadn't asked then I wouldn't have even known it was a possibility. It was the same with doxycycline which I pushed for as a base level protection during the winter months to help reduce my chances of picking up a cold. I only got back on it in December (after two chest infections in as many months) and it helped me get through until the end of March when I have to come off it without any further infections.
Ironically, I've now picked up a chest infection since coming off of the doxycycline but I got on the Co-Amoxiclav quickly and I think I'm already beginning to nip it in the bud which is a good thing.
The other thing that's happened since I was last in touch is that my wife contracted COVID. She's fully over it now and having self-isolated early managed to avoid passing it on to me which is fantastic. Fingers crossed I continue to remain COVID free despite everyone bar those of us who are clinically vulnerably seemingly having given up on testing, wearing masks or taking any kind of precautions. It's as if it never happened now which is scary but not much we can do about it I guess.
That more or less is everything I had to update on. The only other thing I wanted to say is thank you for tagging me in a post from a fellow PPFEr. I've just left a message with them and will look to support in any way that I can.
I really hope everyone is doing ok and I'll update you all once I know what the recommendation(s) is/are from the transplant team. Big decisions are on the horizon but in the meantime I'm going to do my best to enjoy life starting with an Easter break in Devon with the family.
Cheers,
Andy
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