Monday 3rd – Thursday 6th October
It’s been a week now since Josh’s rapid decline. Whilst every day and night has dragged, it’s still hard to believe so much time has passed. My back’s aching and the last thing I want to do today is cycle to school, but Joseph’s keen and it’s looking like another scorcher out there. I don’t quite know where the morning went but I end up with the usual mad rush to school to pick him up at 3pm. While I’m waiting for him to come out a mum gives me a huge carrier bag containing Claire’s cake stand, two large jugs and 3 even bigger cake boxes that she gave them for the Church Autumn Festival at the weekend. I take them gratefully, forgetting I’m on a bike and have to somehow transport them home. I take the scenic route back, so nobody can see me teetering along. After swimming I catch up with Claire who’s on the phone in the bathroom – so nobody else can hear her. She’s spoken to one of the doctors today and they’re very worried about Josh. Given that they’re normally ludicrously optimistic, it sounds ominous. Whilst the state he’s in at the moment is similar to when he slipped into a coma last year, it’s very different in that there were things they could do to reduce his immuno- suppression and increase his lymphocytes back then. This time there’s nothing they can do for him. The doctor asked Claire how we would feel about taking him home. It’s a no brainer at the moment as Joseph couldn’t possibly see him like this. If he was in the shape he was last week then maybe, but now ? It’s a ludicrous question. The second thing they asked was if I could come up tomorrow, so we’re both there for a meeting. That sounds equally ominous and we both think we know what’s coming... In the meantime, Josh’s enzymes are creeping up and we suspect another pancreatitis bout is on the way. He’s been more settled and slept from 6.30am until 3-30pm. The ophthalmologist checked his eye and the haemorrhage is still there but no worse, the optic nerve is no different and there’s no sign of any increased pressure. Josh has stopped lashing out and started to reach out for big hugs from Claire. He’s sleeping with his eyes open for long periods and may need drops to stop them drying out. He had an Albumin infusion during the day to try and stop him retaining fluids. When I come off the phone I sit downstairs and just sob for 20 minutes before going up to bed and watching TV in the hope that I drift off. I don’t. We’re pretty sure we know what the doctors will say tomorrow, I’m just not sure what we will...
It’s Tuesday and I’m up at the hospital before noon. Claire’s in the room with a visiting friend. Josh is asleep and I sit in with him while they get some fresh air. Ward round takes place just before 1pm when Claire’s back. It’s more about going through the motions than anything else with comments on how good his skin’s looking and equally superficial stuff. The play specialist sits in with Josh and we follow the BMT team into the seminar room as the room they booked out still has a meeting going on. There are no surprises in what they say. The steroids blasts they gave Josh haven’t made any difference to his condition, so it’s unlikely the encephalitis is being caused by the new lymphocytes he got in Italy overpopulating his brain. That leads them to suspect it’s the JC virus slowly spreading - as it does. They tell us for the first time that the recovery rate of children with JC after a bone marrow transplant is only 5% and he’s done well to fight it as he has. There is nothing more they can do for him and they don’t expect him to recover. Given that they are normally far too optimistic if anything, it’s a pretty damning diagnosis and we have to assume that barring a miracle Josh won’t survive. Last year they had things to play with to boost his immune system - steroids they could reduce, new drugs they could try - but right now they’re out of options. I ask why they think the Italian cells didn’t work and they don’t know. I can’t understand why they haven’t spoken to the doctor in Italy in charge of the treatment, but with a couple of notable exceptions the team aren’t good at involving people from outside their department. Maybe it’s because they know it won’t help, maybe it’s a touch of arrogance. It doesn’t really matter either way. If it is JC, the steroids they gave him will have fed the virus. They mention going home again and suggest it might improve the quality of Josh’s life and ours, but we can’t see how it would help either and categorically say no. Joseph can’t see Josh like this. It would destroy him. We don’t want to move Josh to a hospice either at this point, as it would bring no extra comfort to him or us. We’re familiar with GOSH and ask to stay put for the time being. The plan is to make him as comfortable as possible here and the Symptoms Care team have been notified. We’ve requested that if something happens to Josh that would mean him going to PICU, we’d rather he didn’t go. There would be little or no chance of him recovering from anything serious enough to end up down there and we don’t want our last memories of him to be with a tube shoved down his throat on a ventilator. The team have nothing more to tell us but are reluctant to leave. I guess they feel as helpless as we do. Eventually, I have to ask them to leave so we can have some privacy. I know we share so much of what we go through with you all on the blog, but the hospital is the real goldfish bowl where all our most private emotions are constantly on display and it’s difficult at the best of times. We sob and hold each other, we talk and cry some more. We’re as close to being inconsolable as we’re ever likely to be. The idea of losing Josh is so much harder having lost Alex. For him to have fought so hard for so long and end up in this position just 4 days before coming home is truly heartbreaking. When we get back to the room Josh is asleep but wakes shortly afterwards and is in real pain. His enzymes are still climbing which suggests it is pancreatitis on top of everything else. Worst of all, in a way given the conversation we’ve just had, Josh seems a little more like Josh again. Whilst he still can’t communicate, his scream is now one of pain rather than agitation and all too familiar. He senses us there and reaches out to hug us and pull him towards him as he unleashes a real sob. It’s so much harder seeing him like this, but at the same time it does give us hope. Josh is a real fighter and he’ll do everything he can to prove the team wrong. Claire stays as long as she can before heading home. The pain team don’t arrive for 3 hours but we save a bit of time when I point out we still have their PTA machine in the room and they set it up there and then. He’s back on Fentanyl. The next 12 hours are the longest and toughest night ever in here given what was said in today’s meeting. Josh doesn’t settle and it’s very difficult to tell pain from cerebral irritation. A nurse watches over him with me and we spend the whole night moving him this way and that as he thrashes around, pulling the covers up and down, on and off with his one good arm – which is still tremendously strong. The crying out and screaming rarely stops during that time and when we do settle him down it’s only for short periods. He reaches out to me every few minutes for a big hug and at one point we think he asks to sit up. We somehow manoeuvre him into a position where he can drape his arms around me and snuggle in. He goes quiet immediately and falls asleep in seconds, only to wake crying again when we lower him back down. He’s had all his prescribed drugs so we call the night doctor for advice. She goes away and can only offer up more midazolam. It doesn’t work. The care team are called and offer a few equally predictable ideas that don’t work either. In the end, he’s so exhausted he screams himself to sleep and settles for a few hours around 5am. I wish I could too, but the full repercussions of today’s meeting go round and round in my head until it’s daylight.
It’s Wednesday 5th October. Sixteenth months since Josh was admitted this time around. The pain team have put him on a background of Fentanyl as well as bolus pushes and he’s a little more settled. The Symptom Care team have been up to discuss what we do to keep make his life more comfortable. They don’t have any new drugs to add to the mix, but are recommending larger doses of Midazolam along with his pain relief. I’ve asked that we only use heavier sedation in the evening, so Josh can hopefully distinguish between night and day and we’ll be able to see any changes – either positive or negative – in his condition. Everyone agrees that Josh shouldn’t have to suffer like he did last night or last year. Claire’s spoken to her parents and brought them up to date. My family know what’s happening too. Dr Robert, who remains by far the best thing about this whole terrible experience at GOSH comes in to see me after lunch. He’s no longer on the ward but wanted to see how things are going. He’s able to fill me in on what the other doctors couldn’t in a sensible, logical manner. There have been so many bizarre theories bandied about over the years that we often haven’t known what to believe – from Josh having a mysterious problem with his immune system to the HHV6 virus being behind all his problems and the Holy Grail we’d been looking for. Basically Robert’s saying that it often takes a while for older children like Josh to produce new t-cells or lymphocytes in their thymus after a bone marrow transplant - sometimes as long as 8 months. During that time the donor’s t-cells expand and proliferate to protect the child. Josh had GvHD after 4 months and the steroids used to suppress it lowered his lymphocyte count to such a degree that the JC virus appeared in his brain – hence the two month coma. They lowered his steroids and other immuno-suppressant drugs during that time and his lymphocytes increased – hence his recovery, but the thymus remained damaged ( probably due to the GvHD) and his immune system never really reconstituted. This is why JC kept disappearing and reappearing. The targeted cells from Italy that were meant to destroy the virus may never have reached his brain and there may not have been enough of them to make a difference. JC has come back, but there’s now nothing they can do to boost his immune system enough to fight it off – and even if there was, it wouldn’t prevent the virus coming back stronger as soon as his lymphocytes dropped again. Robert’s in tears and so am I as we talk about the future. He saw how distressed Josh was for 15 minutes last week and doesn’t think it’s fair on any of us, most of all Joshie, to be in that state. It’s been a shocking day as Claire and I try to come to terms with the inevitable without really being there for each other. As time goes by, we’ll accept it because we have no other choice. Everyone who comes into the room, from the physios to the teachers, knows the situation and it feels like the whole hospital is in mourning even though Josh is still alive and undoubtedly kicking. He spends most of the day asleep and is far more settled now he’s on pain relief. The ophthalmologist somehow manages to examine his eye without even waking him up this time and things are looking better from her point of view. The optic nerve is back to normal and there’s no sign of any pressure from the brain. When I talk to Claire in the evening, I start feeling a bit more positive. There’s no time scale on how long Josh has and there’s nothing to say he can’t recover in the short term or even recover some movement on his left side. We will always be here for him and we’re not prepared to give up. We’d be mad to be optimistic, but we won’t be pessimistic either. It’s a relatively quiet night and I’m only up a few times to change Josh. He settles back to sleep quickly each time without any extra sedation other than the pain relief he normally has and at one point the nurse thought he called out ‘Dad’. There’s so much of Josh still in there, in the way he pulls his sheets up or scratches his head, that you just think he’s asleep and could wake up at any time and be back to normal – even though we know it’s unlikely. The whole situation feels like a bad dream...
Thursday sees Claire arrive early so we have some much needed time together to talk about the events of the last few days. Josh is a little less settled today, but his enzymes have dropped significantly so he’s hopefully over the worst of his pancreatitis and could be pain free again soon. This will give us a better chance to gauge whether or not his cerebral irritation has decreased and if he’s any more aware of what’s going on around him. Claire and I manage to get out for lunch and sit in Giraffe eating steaks, sipping cider and talking about things that would reduce any diners eavesdropping nearby to tears. I leave around 1.30pm to pick Joseph up from school and take him to tennis. Claire calls to tell me that physio managed to sit Josh up for a while and there was a little bit of movement in his left leg. He said ‘mummy’ twice too which is fantastic. He also said ‘ Can I have my blanket ?’ at one point. It’s great to hear he’s making progress, but it’s important we don’t get too carried away as the bigger picture remains the same and it’s pretty bleak. There’s nothing worse than losing a child, but when Alex died I always said we were lucky in that it was over quickly and he didn’t suffer too much. I always said there were far worse ways to lose a child, but never dreamt that we might experience one. It’s hard to believe anything could be worse than what we’ve all been through over the last few years and it’s far from over. As time goes by we may well consider bringing Josh closer to home to be looked after in a local hospice. It would allow us to step back a little, which is something we will have to do at some point in order to protect ourselves and Joseph in particular. For now though, every day is about adjusting and finding some way to get through to the next. It won’t be easy as everything we see around us brings such deep sadness. Every week there are football matches being played I can’t tell Josh about, books sitting around on shelves that he never got round to reading, movies hitting DVD that he’d wanted to see at the cinema and PS3 games being launched that he was longing to play. We will take such good care of him for as long as he needs us, but it will be so much harder now. Joseph doesn’t know anything yet other than Josh isn’t well enough to see him. We will tell him more little by little, but please don’t discuss any of this in front of your children just in case it gets back to him from someone other than us. There may still be time or room for a miracle and we’ll never give up on Joshie and would give anything to have him back as he was - even if only for another day, another week, another month. That said, our prayers have remained unanswered for so long and Josh has suffered so much that perhaps the only miracle we can truly hope for now is that he doesn’t suffer.
We have decided to stop the blog from today as what we are going through is very difficult for us. We don’t really know what the future holds but our thoughts, hopes, dreams and tears are probably best kept private. We will email you of any significant turns of events and kindly request that you don’t ask about Josh in the meantime. It’s just too raw. You have all been fantastic and we know you are there for us if we need you, and we’ll call you when we do.
Thank you for all your support. Jon, Claire, Joseph, and of course, Joshie xxxxxx
