Late-acute Graft versus Host Disease of the Liver (and breathe).

When you've been stuck in hospital and haven't seen your boys in over a week this just has to be done.

It’s been quite a while since my last blog post, but with good reason. When I initially started this blog my instinct was to go back to the beginning and tell the story until the present moment, but I have realised that that just isn’t realistic and I would be missing out on the right now. It’s also kind of tedious and time-consuming which made it seem like a bit of a chore and that’s not what I want this blog to be about. I want to enjoy the process of blogging and treat it as both a cathartic and an informative process. So I have decided to just write about whatever I feel writing about with no particular timeline. But just for a bit of context, the last couple of months my health hasn’t been on point and I was readmitted to the Queen Elizabeth Hospital in Birmingham with a diagnosis of late-actue graft versus host disease of the liver.

In a nutshell, graft versus host disease (GvHD) is a condition whereby the stem cells that I received from my sister during my transplant (i.e. the graft) are recognizing me (i.e. the host) as foreign and launching an immune response to attack my “foreign” cells. In my case, the graft is attacking my liver but GvHD can affect many parts of the body, particularly the skin and gut. 

There are two types of GvHD, acute and chronic. Historically, “acute” GvHD was considered to occur within the first 100 days post transplant, and “chronic” GvHD was considered to occur any time after this. However, medical professionals now tend to base their diagnosis on the clinical manifestations of the disease rather than the timescale. Thus I was given the almost chewable diagnosis of “late-acute GvHD of the liver”. It all started towards the middle of August. No, strike that. It must’ve started in June. That’s when I had another inpatient admittance due to a severe case of shingles which extended from my lower back, down my sciatic nerve to my foot. It was the second-worse pain I have ever experienced. But that’s another story for another time. As a result of the shingles I was put on a pretty high dose of a drug called Gabapentin for nerve pain relief. At the same time, I was coming to the end of the taper and eventual end of my Ciclosporin medication, an anti-rejection drug given to suppress my immune system to prevent my body from rejecting my sisters donated stem cells. 

At the start of July my liver function tests started to grumble. The Gabapentin can cause liver dysfunction, as can a lot of the other drugs that I was on, but there was nothing particularly concerning so things just carried on as normal. However, by mid August I started experiencing itching all over my body which would keep me up all night. I mentioned it at my clinic appointment but my doctor dismissed it and said to use an “emollient”. The following week I began to get symptoms of jaundice, mainly the yellowing of the pigmentation of my eyes and skin. My liver function tests all showed abnormally increased levels, particularly my bilirubin level, and so my doctor decided to send me for a liver scan to make sure there was no blockages or damage to my liver. They also decided at this point to wean me off the Gabapentin medication.

At this point I was convinced it was the Gabapentin causing the problem because my dosage had been significantly increased over the last few weeks because I wasn’t coping with the residual shingles pain. The liver scan was organised for the following day, and showed no obvious problems but before I had even left the hospital, as me and Matt were sat in Costa having lunch, I received a call from one of my doctors saying that they wanted to admit me to perform a liver biopsy. There was no bed available right away so I went home and then travelled back later that evening.

Now all of this was happening at the most inconvenient of times; Matt was going away for ten days the following day with his scout group on an abroad holiday to Spain which they organise every other year and he was the main leader responsible for about fifty kids. It was typical. The last time Matt went away was in January when he went skiing and I developed a hideous infection in hospital which was so bad it required a specialist infusion of donated white blood cells because it was between my final chemo and my stem cell transplant at a time when I had absolutely no immune system of my own. I've told him he's not allowed to go away without me any more because it's bad for my health.

So I was admitted to the oncology ward as there were no available beds on the haematology ward later that Thursday night and the biopsy was performed on the Friday morning. I didn’t expect to be in for long because, like I said, I was still convinced that this was due to the Gabapentin. Unfortunately, later that day the doctor told me that it was indeed graft versus host disease and that they were going to commence a first line treatment of high dose IV steroids (Methylprednisolone) right away. What the steroids would do is basically suppress the body’s immune responses so that they would stop attacking my liver. 

Everyone’s liver function fluctuates on a day to day basis, and it takes a while for the doctors to see if there is a particular pattern in the results or if it’s just a natural peak or trough for that day. Each day the doctors would do their rounds, tell me it was too early to judge whether there was an identifiable trend, and tell me to sit tight and wait and see.

After a week of this I was going out of my mind. My peak bilirubin level was 217 (I think it should be below 20, but don’t hold me to that). A week later and it was still raised, at which point one of my doctors came to see me on the ward, coat in hand as she was on her way home, to tell me that in all likelihood a second line of antibody treatment called Campath would be started the next day because it looked like I wasn’t responding to the steroid treatment.

That night my mind was in a whirr. Things had been slowly and steadily getting back to normal and I felt like I had taken ten steps backwards. Throughout my treatment I have done my best to avoid googling anything about treatments or survival rates or prognosis because the internet can be a very scary, a very outdated and a very dangerous place to look when you don’t really know what you’re looking for. But that night I googled, and boy did I google, and I drove myself half insane. I diagnosed myself with stage IV steroid-refractory GvHD of the liver with a poor prognosis. I didn’t sleep that night.

Campath sounded terrifying. It is a monoclonal antibody treatment used to target t-cells more directly and is used in some cases as part of chemotherapy regimes. Here we go again, I thought. More infections, one of which will likely see me off, I thought. But the next day my levels had dropped, so they didn’t start the Campath. The day after they had gone up again so they were still going to wait and see. The day after they went down, and again the day after that. That week my bilirubin hit 150 and stayed down and by the Saturday, I was allowed to go home. The relief was immense.

Since then, I have been going back to the weekly haematology clinic and my results have been up and down, but they have not risen so much to cause too much worry just yet and my doctors are slowly tapering my massive steroid dose accordingly. So it looks like, for now, fingers crossed, that things are going in the right direction. Apparently GvHD of the liver is a particularly stubborn one and can take months to completely normalise. If I’d had GvHD of the skin or gut which hadn’t responded quickly, it is likely that a second line treatment would have been considered sooner.

A word on steroids: fuck those motherfuckers. If there is one drug that can give you every conceivable side effect with the explicit purpose of demolishing your self esteem, then steroids are it. MY GOD it makes me wish I was bald again. They give you “moon face” which makes your cheeks swell up like you’re a hamster storing food; they make you put on weight but only on your belly, so you look like an orange with matchstick arms and legs; they give you cankles; they give you dry old-lady looking skin; they give you folliculitis all over your face which looks like acne; they give you insomnia; they give you anxiety; they give you high blood sugars; and in the long run they give you osteoperosis. 

But right now my energy levels and mood are in a good place and last week at clinic my haemoglobin level had actually risen by itself for the first time since my transplant! I have been reliant on blood transfusions every three weeks or so since my transplant because there is a mismatch between my sister’s A positive blood type and my original O positive blood type. Although my bone marrow is apparently now producing A positive blood, I have lots of leftover O positive antibodies that are killing them off, so I need O positive blood transfusions to keep the anaemia at bay. 

So that’s where I am right now, I am feeling positive about now and for the future. Let’s hope those pesky steroids don’t have other plans for me.