It's pants
My journey with Myelodysplastic Syndrome and Bone Marrow Transplantation
What's going on? The graft versus host disease (GVHD) is still rumbling but less than it was, although the initial response to a higher dose of cyclosporine didn't boost energy or mouth much so I was started on some steroids (prednisolone 30mg OD) just over a week ago which were .... amazing! The day after the first dose I was able to get out of bed with a spring, less aching, more positivity, got my work brain on and started emailing and catching up on projects. Mid-January I had no desire to leave the house, but in the last few days I have started walking around the park again. My gums are back to pink, but my palate is still sore, inflamed and covered in red spots and tiny cysts, and swallowing medicines is uncomfortable. Rashes are settling but everything still tastes pretty gross, a bit like chewing sandpaper and mud, so I survive on yoghurt, porridge, tinned fruit, energy drinks and pink milk. My mouth also gets pretty dry so I've been given some saliva in a squirt bottle. They tell me it's not donated. I've got some hair on my head, makes life much warmer. New hair is baby soft so I spend plenty of time gently stroking it. Don't judge me, you would too if you could. I had thought January was going to be relatively straightforward and the big news would be the repeat bone marrow biopsy looking for the extent of any residual disease ... but this is going to be topsy turvy so that biopsy is 'on hold' for now. My oral GVHD meant I dropped out of lots of zooms and other meetings I had wanted to do. A friend had given me great advice "to be kind to myself" so I dropped out without feeling my usual guilt of letting people down. But I have been able to join a few meetings and speak, especially on the Butterfly course we released a few months ago. I might blog on that another time but if you want to do the course it's completely free, just lick the link. It means a lot to me that people see it. If you've been paying attention, you'll remember that the BMT gave me a whole new blood AND a whole new (but naive) immune system. All your white cells (neutrophils, lymphocytes etc.) and the antibodies you produce ultimately start in your BM so a transplant resets your immune system back to the beginning. Whilst I'm making red blood cells and platelets and white cells fine, the white cells are troublesome. These new lymphocytes see my body as 'foreign' and try to reject me. Stupid really, if they just stopped to think they'd realise that was an act of mutually assured destruction. The cyclosporine keeps my 'new' immune system under control whilst we learn to be friends. But a consequence of this is that I am "immunosuppressed" - and my usual ability to keep infections under control is limited. It's basically walking a tightrope between infection and getting rejected. Bit like what Amanda did to me. Whilst many of these infections are just lurking around the environment or on passers by, we all carry viruses that hang around in our body forever. These are particularly the herpes family of viruses that reactivate, usually after a bit of stress. Cold sores are herpes that lurk in your nerves and reactivate when you're feeling sad, have to write an essay or stay 10 minutes late after work. Shingles is your chickenpox virus coming back years later. CMV can very occasionally reactivate during pregnancy with devastating consequences for the foetus. Epstein Barr Virus (EBV) gives you glandular fever as a teenager but almost never reactivates in later life, although traces of EBV hang around in the cells at the back of your mouth and throat and your B lymphocytes forever. Risk of viral reactivation post-BMT is well known, so some drugs I take (acyclovir, letermovir) are used prophylactically to prevent some of these, but there isn't an anti-viral for EBV in this way. The level of EBV can be measured on bloods and you could find low numbers in lots of people but 10-20,000 copies is high and 60,000 is getting pretty high. Mine has gone from 6,000 to 9,000 to 32,000 in just 10 days, so I was admitted as a day case a couple of days ago for treatment using a monoclonal antibody (artificially synthesised) which you would expect would directly target the EBV, but in fact targets the B lymphocyte cells that carry it. This is because the real risk here is not infection as such, but what the virus does to B cells. The EBV in the B cells makes them all frisky and they go on a mad one. Un-checked this can lead to a lymphoproliferative disorder, a bit like a lymphoma which is really not good news. The monoclonal antibody is called Ritoximab which has a list of side effects which includes basically anything you can think of, including other virus reactivation (because you're killing off some of your immune system.) Trying to understand what is going on without a medical degree would be pretty complex. Anyhow, it's all been fine so far and the £££ cost, like Stella Artois, is reassuringly expensive so it must be good. Someone needs to take responsibility here. Unlike the MDS which I am happy to put down to bad luck, I most certainly know who is responsible for this clusterfuck of immunological complexity and she's called Amanda. It was the heady summer of 1979. Sony had produced the first Walkman, the 3-mile nuclear accident threatened disaster, and Maggie came to power but I was fully distracted by getting a girl friend. Stuart Hutchinson had gone out with Amanda previously and told me she was 'playful' so I set off on my mission. There was going to be a 'pyjama party' at a friends house in a couple of weeks (life was pretty racey back then) so I needed to make a pre-emptive move fearing I wouldn't be able to compete for her attentions when there were more players on the market. In the late 1970s, cinema's largely existed for teenagers to snog in, and James Bond's Moonraker film was just released (the one with jaws in it). 90 pence each for a ticket was quite steep, especially as you hope you're not going to see much of the film, but she agreed to come along, and we sat in the back row. I'm pleased to report Stuart's inside info was as accurate as Sue Gray's report will be. Party time indeed.
Sadly, a few days later I started feeling quite unwell, nasty tonsillitis and a trip to the GP confirmed I had glandular fever caused by EBV. Amanda had taken the playfulness too far but there was no going back. Not only was I poorly and feeling crap, I couldn't go the pyjama party. I was gutted. She didn't even ring to apologise. She went to the pyjama party where she copped off with Thomas Alcock who bloody fancied himself but I thought he was a twat. He was from the year above and bigger than me, so any thoughts of revenge were put to one side. Bollocks to both of them. Suffering the double rejection of my new graft and Amanda is making this a particularly difficult time for me. I've literally no idea what is going to happen next. The plan is to try and wean both the cyclosporine and steroids bit by bit, spend some more of your taxes on expensive drugs, and do more blood tests. Right, enough chatting, pass the saliva.
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Not going to lie, the start of 2022 has been crap. Things had all been going well, and I was slowly reducing my cyclosporine (CyA). Remember, this is a commonly used immunosuppressive drug that keeps lymphocytes (a type of white cell) under control and is used in most people following a transplant. However, its use following a BMT is unlike use in 'solid organ transplants'. After any organ transplant, your body's immune system will recognise that donor organ as 'foreign' and try and destroy it. This results in inflammation that if left untreated, would slowly result in damage and failure of the new organ, be it kidney or heart etc. That is because your lymphocytes will always recognise it as foreign, so you need to stay on CyA (or similar) forever. If you start to get rejection, then it can be treated by increasing the dose of CyA or adding in new drugs like steroids. This is why getting a well 'matched' donor is important whether it is bone marrow or kidney. Better matches result in less rejection or inflammation. The situation following BMT is almost the opposite of this. In this case, the new donor bone marrow (graft) starts to produce lymphocytes that are initially kept under control by the CyA. This keeps your lymphocyte numbers low, but it also means you are at slightly higher risk of infection (because you are suppressing your immune system). Infections that are particularly problematic are viruses like herpes or CMV, but also more unusual infections like fungus or weird things that used to be seen in people who are HIV+. When you are taking lots of CyA you also won't respond to vaccines very well, so you have to wait to get your COVID vaccine until you are ideally off CyA or on low dose. Over time, the CyA dose is decreased - that started early December and was going OK. However, just after Boxing Day, I first started feeling tired, a bit achey like flu, and then my lips started tingling before becoming a bit painful. I also developed some mild liver inflammation and my CyA dose was increased. Unfortunately, there's a lag time between increasing the CyA and suppressing the lymphocyte activity and within 2-3 days I had developed a really sore mouth. My lips lost their outer layer, turned bright red and swelled. It looked like I'd had collagen fillers. Then the whole of my mouth developed white plaques that slowly coalesced and my gums turned white. They're still white now although the ulcers are settling. Basically, the new donor lymphocytes went on a mad one and turned my mouth into a sewer. They've had a massive party in my mouth without permission AND failed to tidy the mess. The graft was attacking me (host) hence the term GVHD. I was going to take a photo, but even I was grossed out looking at my mouth. My taste buds that had been recovering have taken a beating, and just about every food except for milk and yoghurt tastes disgusting again. I also developed a widespread itchy rash over chest and back, and a very painful purple rash with tiny cysts on my palms. Fortunately I don't have it on the soles of my feet - if I did it would make walking extremely painful. Steroid creams and steroid mouthwashes +++ and good mouthwash to keep it all clean. The tiredness and exhaustion is crippling, not sure why it's so bad, but at its worse, I could hardly sleep; consequently I have done nothing useful for 2 weeks now. That's really irritating me. Not even played the piano.
What next? Not really sure. Important to get this episode of GVHD under control - whilst I can cope with rashes, and my mouth will settle, I don't like the idea of something important like my liver getting hit. We've increased the CyA twice now so I am on the same dose as when first discharged but will need to try and decrease again when things are under control. I've gone from loathing CyA to thinking of it as my temporary saviour! Chronic GVHD is not uncommon, but hopefully if that happens it won't be as severe as this episode. I'm optimistic it will get sorted out, but GVHD can obviously be pretty serious. I'm always looking for silver linings otherwise all of this would be relentlessly depressing, but there is some good news. It means the graft is working well - thanks Mx Donor, I still love you even though you're trying to kill me. I'm sure we can reach a compromise. Also, a strong graft has a slightly better chance of being a strong cuckoo and ousting those malignant host bone marrow stem cells. Slightly less likely for the graft to fail. Still, long way to go, slowly slowly. Rather than share a picture of my mouth, here's a random photo of me biting my brother. |
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May 2022
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