I’m no longer on the transplant list. It seems odd. For nineteen months, the wait for new lungs has been at the centre of my life. Not one day has passed when I haven’t been affected by being on the list, thought about it, or dreamed of the endless possibilities afterwards. So it seems strange, really strange, to have been suspended. When my Pulmonary Hypertension consultant initially mentioned -very briefly- that she was thinking of suspending me, I was in shock. Completely terrified. I just didn’t understand. Even more confusingly, during my routine appointment at Harefield a week later, I discovered that my transplant team knew nothing about it. At that point, my Brompton doctors hadn’t mentioned it to them, so Harefield were happy for me to remain on the active list. I didn’t know what was happening or why. However, a 7pm phone call the next day, from my lovely PH consultant soon clarified things. She apologised for the confusion, explained her thinking, talked through the decision to be made, addressed my misgivings. She was now certain that suspension was the correct choice. I listened quietly, my head whirring, my thoughts swirling. Still unsure of whether I wanted to stay on or come off. Undecided. But what I remember most clearly about that conversation was that she kept asking me to trust her. Confidently, assuredly. Please trust me on this Sarah.
So it was with great trepidation that I went to the Brompton for a few days of tests. Alone. These results would decide my immediate future… stay on the active list, or come off it for a while. Afterwards, a meeting was arranged with that same PH doctor, and thankfully Phil travelled down to attend. During the brief fifteen minute discussion we agreed to remove me temporarily from the list. She was even more firm in her decision, even more confident that it was the best option. I suspect I’d have had the deciding vote if we disagreed… but actually by then I had come to understand her reasoning. By then I supported her choice. By then I was in complete agreement to suspend me. So I’m off the active transplant list. For now. I’m just sitting on the subs bench, postponed until I need to rejoin again. 🙂
My doctors want to keep me alive for as long as possible. Their decision on whether to keep me on the list or suspend me, has been based solely on this. They want me to have the most number of kisses with Phil, cuppas with my Mum, cuddles with Lottie dog. Inevitably both of my options will still lead to an early and premature death… but they’re now guessing (and hoping) that suspension will give me the longest time.
Having a transplant is not a safe and risk-free choice. Far from. It’s dangerous. Sadly lung transplants have the lowest survival rates, sadly PH patients have the lowest survival rates. Being wheeled into the operating theatre will not guarantee that I’ll come out alive. Surviving the operation does not assure that I’ll make it successfully through the recovery. Leaving hospital afterwards does not mean I’ll make it to the one year mark. There is a 10% chance of death during the initial operation, a 10% chance during the immediate recovery, a 50% chance before my 5 year lung-aversary. Bleeds and heart issues and low immunity and infection. Pneumonia and kidney failure and cancer and rejection. Many potential complications and problems. Having a transplant operation will be the start of a ticking time bomb. A countdown to the end. An alarm clock waiting to ring. Transplant is not the easy safe option.
But nineteen months ago, we believed it was my best choice. My sole choice really. New lungs were thought to be the only way I’d survive to 2018. My Pulmonary Hypertension pressures were moderate, affecting my heart’s workings. My PVOD was severe, so my lungs only functioned at 24%. And both diseases were worsening; I was in a spiral of decline and deterioration. With 90% of PVOD patients dying within a year of diagnosis, and no medicines… we assumed I had mere months left with my childhood lungs. Life without a transplant would be a spiral to the grave. Although transplant was risky, although I’d get only a little extra time… it gave me better odds of surviving the year. A 90% chance of death without a transplant -versus- a 20% chance of death with. Nineteen months ago, going on the active list was an easy choice.
But amazingly, wonderfully, thankfully I survived to 2018 on my own lungs! A shock, surprise, unexpected. I’m not only stable, but slightly improved… and that changes everything. 🙂 My PH pressures have reduced from moderate to very mild. My six minute walk test has increased from 180m to 460m. My heart has improved from enlarged and pumping badly to normal size and working well. My lung functioning has increased from 24% to 33%. Miraculous changes, very unusual, completely unexpected. “Rarely do people stabilise with PVOD, never do they improve”. Therefore as my health has improved and is steady, my doctors are wondering if I can survive a little longer before I have my transplant. They don’t want me facing a risky operation this month, when I’m well and could stay alive a little longer with my own lungs. We hope that suspending me from the active list until I start to decline, will give me a slightly greater chance of making it to 2019. A 0% chance of death without a transplant (if I remain stable) -versus- a 20% chance of death with. Delaying the transplant would also delay starting the countdown to the end. So extra time. Extra kisses, cuppas and cuddles. Win win.
So is it the right decision? No one knows. Only time will tell. Maybe I’ll start declining next month, maybe I’ll get really ill, maybe I won’t get new lungs in time. Without a crystal ball, we can only guess the best path. Yes it’s a gamble, Russian roulette, High Stakes. But with my condition there is no obvious option or definite answer. Pulmonary Venous Occlusive Disease is rare. Ridiculously rare. Estimations of incidence range from 1-in-10 million to 1-in-100 million. Few others have trod this path. With more common conditions, doctors can make accurate predictions, based on previous outcomes of many people, and their deeper understanding of the disease and its likely course. However with limited PVOD patients, and limited research into the condition, we can’t. Furthermore my PVOD is acting abnormally; behaving differently to the few other UK PVOD-ers. Fabulous for me but also confusing. So there is no simple and easy choice… just a best guess. But I’m fully in support of my doctor. I’m willing to take the gamble. Now we have to hope and pray.So I’m off the active transplant list for now. However I’m still being regularly monitored by Harefield Hospital. When the decline comes again (and it WILL come), they will immediately reinstate me on the active transplant list with just one phone call. No assessment again. No wondering if I will be accepted. One phone call and I’ll be straight back on. If luck is on my side, I’ll get an extra year with my family, before I need new lungs and start my countdown.
There are wonderful benefits to not being actively listed- I no longer have to answer the phone when I’m sleeping, or have a packed suitcase under the bed. I no longer need to email my whereabouts each weekend, or make complicated plans every time Phil goes away. And best of all, I no longer need to stay close to Harefield Hospital! We’ve just booked our ferry tickets to the Isle of Man. 🙂