Sunday 15th May
The morning revolves around Johnny Depp. No, he’s not handed another £1million to Great Ormond Street or popped in to see how Josh is, he’s being given away with the News of the World as a Lego figure at W H Smiths. Before we queue up there, Joseph and I head to PC World to take my laptop in. Tom’s off for the weekend but the lad on duty today is just as good, if not better. After 15 minutes of trying various things, he’s convinced it’s the lead and not the laptop and phones for a new one to be delivered straight to our door next week. Let’s hope he’s right. And so we sail off down Bromley high street in search of our free Lego toy. Smith’s has only been open 10 minutes, but there’s already a long queue of people clutching several editions of today’s paper, even though it’s meant to be one voucher per customer. The woman in front of us has 12 copies and I’m prepared to start a mutiny if we miss out. Luckily there are still a few left, so I don’t have to clap her in irons or throw her into the briny. Josh wakes for a few minutes when we arrive at GOSH but goes straight back to sleep, leaving the rest of us to eat together again – a rare treat. He’s more lively when we return and plays FIFA Street. He’s alert enough to beat both Joseph and me. Too tired for a bath, we settle for a quick wash and cover his skin with Steroid cream. An hour later he’s awake again and stays up watching TV with me for a good couple of hours - a vast improvement on the last few days. Just as I’m texting Claire to say he’s going to sleep and we’ll catch up tomorrow, she calls me. We’re both too tired to talk properly and just moan and grunt monosyllabically at each other for 5 minutes. You normally get charged a premium for a phone call like that.
Monday 16th May
It’s light at 5.30am and Josh decides to sit in his wheelchair and read for a few hours. He also drinks several gallons of cold water which I have to drain out of his stomach as we’re keeping his fluids down in case they leak into his lungs. He’s tired by 9am and goes back to sleep, so I head for WH Smiths in Holborn for more Lego booty. Today it’s Captain Jack Sparrow’s ship they’re giving away, but Alas the shelves have already been plundered and there are no copies of The Sun left. I return empty handed to discuss Josh coming off Fentanyl with the pain team, then suddenly remember there’s a Smiths on Oxford Street that doesn’t open until 10am. Setting sail once again in search of treasure and more pirate puns, I push my way past the motley crew of tourists and office workers assembled outside the mall to the front of the queue and emerge triumphant. Josh is far less excited than I am, but is up and about soon after I return and stays awake most of the day. Gastro reiterate that they’ll remove his gall bladder if he has another attack, but I’m not sure that’s the way to go if the stones are a direct result of being on TPN. Surely if we can get him eating or on elemental feed the problem would go away without surgery ? Josh doesn’t seem to tire at all today and is keen to eat. I’m told it’s okay and bung his customary pizza slice in the sick children’s trust house oven while a play-specialist sits in with him. After a reluctant bath, we settle down to watch Roald Dahl’s BFG only to be interrupted by a request for him to have his Pantamadine nebulizer, as the room is free. It seems a little late for such an unpleasant experience ( he’s already had 6 MRSI swabs taken today which aren’t nice either) so we manage to put it off until tomorrow morning. We’ll both invariably end up with headaches and feeling nauseous afterwards, so it’s hardly ideal just before bed. As it turns out Josh ends the day as he started it, sitting up and reading. Once he’s asleep I settle down to watch TV but only last about 30 seconds before nodding off myself...
Tuesday 17th May
We manage to delay Josh’s nebulizer from 9am until 11am. It takes place in an empty room next door, so we don’t have to traipse round the hospital for a change. Inhaling the drug over a 20 minute period isn’t pleasant but he copes well, as always, and recovers quickly - albeit with a lots of cold water to soothe his dry, rasping throat. The time we have together afterwards seems to fly today and I’m almost reluctant to leave him when his teacher comes. Having said that I’m out of the door like a shot once he’s happy and spot a table outside a restaurant in Chinatown with my name on it - not literally unless my name’s Tsingtao. Szechuan chilli beef and rice hits the spot while I sit and watch the world go by for half an hour. It’s physio time when I return and Josh performs better than he has in weeks, although anyone who knew him before any of this would be shocked by how little he can manage. The doctors don’t catch up with us until late afternoon but there’s little to discuss regarding his health. He’s had 3 clear norovirus results, so he can officially leave the ward now to visit the gym. His fluid balance remains confusing, with hugely contrasting interpretations coming from different doctors and departments. His skin has improved, although levels of immuno-suppression from the topical creams he’s on have been picked up in his blood, so we’ll have to monitor that closely. The biggest issue is Thursday’s MDM ( multi-disciplinary meeting) which we thought was going to involve representatives from every department, but is actually just us and BMT, with a second meeting scheduled an hour later which includes Tadworth. I can understand why it’s just BMT, even though Claire’s disappointed, as it’s hard to imagine how much progress we’d make with everyone all together in one room and whether we’d even find one big enough. You’d hope that any plans will have been discussed with all the disciplines already and any potential side effects and pitfalls flagged up. I’ve been reading the riot act to everyone today. I’m sick of all the department rivalry and unscheduled corridor conversations that leave us so confused we have no idea who or what to believe. Gastro seem to think Josh is ready to come home once we’re trained on TPN, which is so far off the mark it’s insulting. They’re basing this solely on how his gut’s looking and ignoring all his immune issues problems which remain critical. I despair. Overall, Josh had another good day today, although he remains neurologically slow and a little confused at times. He’s exhausted and back in bed by 6.30pm but wakes an hour later to start exploring Hogwarts again on Playstation for a short spell. No pun intended...
Wednesday 18th May
Claire’s up at the hospital by 9.30am which means I get more of a day at home. This is just as well as I’m back tomorrow for the MDM meeting. I feel like a mayfly, enjoying such a brief existence, only they get to mate during their 24 hours rather than flutter round John Lewis looking for a replacement DVD recorder. A second-hand Panasonic is the only one that fits the bill, so I go for that. Joseph’s at a friend’s house until 6pm, so I have time to pop to the gym once I’ve sorted out my SCART sockets. Josh hasn’t had a good day. He woke an hour after I left and was fine for the rest of the morning. Ophthalmology came to see him just after midday and discovered several new haemorrhages in his good eye that worry them enough to want to see him again on Friday. Josh was shattered after his examination and slept for the afternoon, as did Claire. He refused to go to physio, which generally means something’s up, and developed a terrible headache soon after. He started vomiting and sobbing as the pain got worse and only settled down after he’d had morphine. There’s only one meeting tomorrow now, as there seemed little point in involving Tadworth. All they need to know is how long it will be before they can expect him and that’s a question I don’t think anyone can answer...
Thursday 19th May
The meeting starts at 2.30pm, but I’m up in London an hour before and Claire and I manage to have lunch together first. When we walk into the room there are more people than we expected, most likely because we kicked up a fuss. There are 3 BMT doctors, a BMT co-ordinator, 2 representatives from Gastro including the main consultant, one of the physiotherapists and someone else that I don't recognise. BMT kick off, but I ask if gastro can be given a chance to speak early as they only have 30 minutes. The main issues to be covered in the meeting are head and gut. The gut situation is as we thought. It looked normal on the last scope, which given that his stomach was the most ulcerated they’d ever seen, is nothing short of miraculous. The biopsy from his pancreatic scope isn’t back, but the micro-stones they found embedded around his gall bladder were flushed out and there’s a slim chance we’ve seen the last of his pancreatitis. The best chance of avoiding further attacks is to get him off TPN and onto Elemental feed as soon as we can. For those of you unfamiliar with the difference, TPN is liquid nutrition that bypasses the digestive system and goes straight into his bloodstream via his Hickman line. Elemental feed is a milk-like alternative that goes straight into his stomach via a gastric peg and is absorbed in the gut. Without either of these, Josh wouldn’t survive. Every time we’ve tried to restart Josh on Elemental since he was re-admitted at GOSH in 2010, he’s vomited - even on a rate of just 20ml/hr as opposed to the 140 ml/hr he was tolerating at home. We’ve long suspected the vomiting is completely unrelated to the feed, as it often happens when there’s nothing in his stomach except water, and most likely due to the encephalitis in his brain so he should be fine. If need be we will bypass his stomach with the feed through a second gastric (GJ) tube that goes straight into his small intestine or jejunum - hence the 'J'. Onto the head, and one of the BMT doctors has drawn a graph that clearly shows the co-relation between his low lymphocyte count since transplant and his encephalitis. As we’ve always believed, the increase in his steroids last May, and the subsequent effect on his immune system, is most likely what let the JC virus thrive in his brain and led to his coma. In defence of the BMT team, they couldn’t have foreseen it. In 1500 transplants, they’ve only ever seen the JC virus once and that was in a child they knew could be prone. The chances of what happened to Josh happening would have been millions to one. Josh’s immune system picked up at the end of August as they lowered his steroids during his coma, which is what you’d expect and why he recovered enough to fight the virus. It crashed back down again around Christmas though and is still dangerously low. Nobody has any real explanation as to why this happened. And that is the biggest problem that we face. The only way to kick the JC virus into touch is for his immune system to build itself back up again, but we’re limited in how we can do this because of his GVHD which would thrive on many of the solutions and give us even more serious problems. Claire and I ask quite a few questions as the meeting goes on, but there are no ready-made answers as they’re entering uncharted territory with Josh. Basically there are three possible ways to go at this time. Four, if you count just giving up and taking our chances at home with live-in medical support which we’re not ready to do. Now, hopefully you’re still reading and haven’t switched to ocado.com to order your groceries because here comes the science bit, and it isn’t easy to understand or explain. Basically Josh’s immune system hasn’t recovered following his transplant. Initially this was put down to immuno-suppression but his steroids and other immuno-suppressive drugs have been reduced and there’s still no sign of it picking up. Tests have revealed that he has no new or ‘naive’ T-cell lymphocytes. That is pretty much unheard of 18 months after transplant and is most likely down to one thing - his Thymus gland. I’ve mentioned this before and it’s a massive problem, as it’s the army of ‘naive’ T-cells that are produced in there that run around fighting any sign of infection in our bodies. Josh has none of these T-cells whatsoever. The only lymphocytes he has are the donor’s following his transplant. These are mature T-cells that have been there, done that, bought the t-shirt and are less effective against new viruses. They increase in numbers or ‘expand’ when they need to fight, but they’ll make hard work of it and they won’t last forever. So what can we do ? The first option is called CTL which stands for Cycotoxic T-Lymphocytes. A scientist in Italy has developed a way of manipulating cells so patients can be given lymphocytes that are specific to the virus they have to fight. This has been done in other places around the world including GOSH, but they are the only ones who have managed to create’ cells that will fight the JC virus causing Josh’s encephalitis. The BMT centre is in Pavia in Italy but the hope is that the cells can be transported to GOSH and given here. The Italian Government aren’t keen on this for some reason, so the more likely outcome is that they will ship them 30km to Milan and Josh will be given them there. He would be there 7-10 days and would probably travel by ambulance. One of us would go with him, I suspect the other would fly there with Joseph and we make it some kind of family break. Claire’s probably Googling the shopping malls even as I type. Whilst this treatment is extremely experimental ( only one patient, a young Italian boy, has been treated so far and the results haven’t even been published yet ) it is the safest option and the side-effects and risks are minimal. The second option isn’t really an option until Josh’s GVHD has gone as the risks are too great. It is called DLI – Donor Lymphocyte Infusion. Basically this means going back to Josh’s original donor then isolating and giving just his T-cell lymphocytes to Josh, rather than his stem cells. Whilst they could be introduced slowly and would give his immune system a real boost, the danger is that these mature lymphocytes could run riot and attack Josh’s body. This is, in essence, what Graft vs Host Disease is and the result could be catastrophic. GOSH have carried out DLI many times on BMT patients, but generally as a boost soon after transplant so it’s impossible to say how effective it would be in Josh’s case. It’s one for when we’re further down the line. That leaves us with option 3 to explain. This is called IL-7. Claire and I were convinced it was spelt ‘Aisle 7’ which made us think it was something you’d find on a supermarket shelf next to the coco-pops and the crunchy nut cornflakes. IL-7 is Interleukin 7 which is a cytokine found in the Thymus which has very recently been used as a drug. Together with KGF (Keratynocyte Growth Factor ), it can potentially stimulate the Thymus into producing naive T-cells again. Whilst we don’t know if Josh’s Thymus is simply not recovering fast enough or if it’s been damaged by drugs or viruses, there’s a chance IL-7 could kick start it. IL-7 is very experimental and has only been used on 130 adult patients. Many of these have had no immune system whatsoever, as they have HIV, but the results have been good and it’s well worth trying in conjunction with CTL. The treatment would also help to expand the existing ‘mature’ donor lymphocytes he already has on board. With all three routes on the table, we discuss the risks involved. The first and third options can be put in hand relatively quickly in theory, but it still means we’re looking at another 3 months here at the every least unless we try to get Josh home in the meantime, which is an option they put to us. I’m not sure why, as it just wouldn’t work – either medically, practically or emotionally. Only when they’ve managed to cure Josh or run out of options, do we want to look at bringing him home. One of the BMT doctors sits with us after the meeting to explain the plans in more depth, which he does far better than I have here. Josh is on good form when we rejoin him, but passes on having a bath today. Claire leaves just after 5pm and Josh and I read, play Harry Potter and watch two episodes of The Apprentice. It’s gone 9.30pm before he’s ready for bed, by which time he’s so itchy he’s crying out for Piriton. It settles him eventually and he only wakes once in the night for a wee. As I’m kneeling down holding his bottle he reaches into my pocket and pulls out a tissue, waving it in the air while he sings ‘ I’ve got to pick a pocket or two’ at the top of his voice. Crazy, mad...beautiful.
Friday 20th May
I spend the morning trying to get my head round everything discussed yesterday. Writing the blog helps and some of the pieces start to fall into place. Claire’s checked our passports and both Josh and Joseph’s have expired in the three years we’ve been at GOSH. There’s no protocol here for queue jumping here, although they can call the Passport Office to explain it’s a medical emergency once we’ve submitted the forms. Medical Illustration will take passport photos of Josh for us. We’ve decided not to explain any of the proposed plans to Josh until they’re in place. He prefers to operate on a need to know basis and it’s stood him in good stead so far. Gastro pop up to run through their plans again, although we decide to wait until Monday before starting his feed as I’m concerned the weekend doctors will panic and pull the plug on the whole thing the moment he throws up again. Josh sleeps all morning and complains of feeling tired, even though he had a good’s night sleep. Something’s not right. An ophthalmologist walks in unannounced at midday to examine Josh’s eye, but how she proposes to do that when he hasn’t had drops put in is a mystery to me. I wake him to do it and she returns an hour later. Josh gets up briefly but is back in bed when his teacher arrives at 2pm. The play specialist sits in with him for an hour instead and while he sleeps I nip out to eat. He doesn’t wake all afternoon and when I eventually get him to the bathroom for his bath, he starts screaming the moment his feet are in the water – even though It’s deliberately tepid. There is something up at the moment, I’m just not sure what. I give him a good wash and fold down the handles of his wheelchair so I can wash his hair in the sink with him leaning back. Back in our room, a nurse pops her head round to say that norovirus has been found in Josh’s blood. I’m concerned as it’s never been there before and I want someone to explain what it means. Twenty minutes later a doctor tells me it was a wrongly conveyed message and it’s only in his stools, where it normally is. It’s not great news, but I’m relieved nevertheless. We finish Josh’s Harry Potter PS2 game, but only just before a headache comes. He sleeps the rest of the afternoon and it’s late evening before he wakes up screaming in pain and asking for water. It feels like his pancreatitis is back again, but it’s hard to see how that’s possible...
Saturday 21st May
Not a great night for Josh. Another headache, itching and several calls for the loo mean he gets very little sleep before 5am which is when he wakes up screaming again. We give him oral morph for the pain and Buscapan just in case it’s tummy cramps, but I ask for the pain team to be put on standby as I think it will get worse rather than better. We wait and wait but nobody comes. The day shift arrives without any response other than to say a doctor needs to see him first and they’re busy elsewhere. At 10am he’s able to have more oral morph but it isn’t given even though I request it several times and he’s screaming blue murder. Again, I’m told it’s because a doctor needs to see him. Eventually one comes but does nothing other than say they’ll contact the pain-team. I check that oral morph won’t prevent the Fentanyl being given and it won’t, but it still never arrives. Even after the pain team come, we could have given it but there’s nobody’s around. It’s embarrassing how slow hospitals are to react at the weekend. Josh’s face when he was handed his green button to press was one of pure unadulterated joy and before long he’s drifting in and out of sleep between pushes. Another doctor visits us once he’s settled but there’s little they can say or do. Interestingly enough, he agrees that it would be highly unusual for Josh to get pancreatitis again so soon after his sludge and stones have been washed away. I’m convinced this means it’s an auto-immune problem and nothing to do with his gall bladder whatsoever, although I’m happy to be proved wrong as I don’t even know which is the lesser of two evils anymore. While that little saga was unfolding, so was another. One of Josh’s oral drugs always blocks his gastronomy tube. Claire and I tend to give it most of the time because we’re generally the only ones who know how to unblock it. Today it’s been mixed particularly badly and won’t budge. In unblocking it, the pressure causes a small tear in his tube – luckily it’s as far away from where it enters his body as possible. It’s easy enough to fix by cutting a few millimetres off the tube and putting a new end on, only nobody knows where the ends are. I’ve done it before, but without the parts there’s not a lot we can do and he can’t have any of his oral drugs. It’s several hours before someone finds one, by which time the tube has blocked again and it’s only after a further 30 minutes of careful manipulation that we have a through thoroughfare again. The nurse immediately tries to give his remaining orals, even though they’re due twice a day and six hours late. I politely suggest that we write them off instead. It’s a beautiful sunny day out there, but I daren’t leave Josh for a minute as the staff just aren’t good enough today and the last thing Josh needs is a repeat of the time Claire came back to hear him screaming and found him sitting on his commode covered in sick because someone had ‘ forgotten’ about him. It’s Joseph’s birthday on Tuesday, but it’s his sports party today and I’d give anything for us to all be there together. Claire and her team of willing volunteers manage the proceedings well, but we’re both sick and tired of being a one-parent family. Here, things go from bad to worse. Having waited so long for pain relief, Josh maxes out within a few hours causing the machine to suspend his Fentanyl and emit an ear-splitting screech. A nurse does come eventually, but only to ask what the terrible noise is. By this time I’ve had enough and tell her that unless she can help she should just get someone who can. Several nurses arrive over the course of the next 10 minutes, but they all appear incapable of either turning the sound off or contacting the pain team to override it. Half an hour later, with Josh bravely trying not to cry but wimpering like a wounded animal, I’m told the pain team have gone home for the day even though it’s 4pm. Ten minutes later, one of them surfaces and resets the machine so he can get some relief. He’s retching all the time for some reason and constantly asks for the toilet even though he can’t go. It takes two hours to settle him back down with a cold water compress on his head. During this period only one nurse, who isn’t even looking after him, opens the door to see if she can help. She can’t, but I later go out of my way to find her and thank her as she was the only one that even offered. No nurses come in to do Josh’s observations even though they’re meant to be done every hour when he’s on pain relief. Whether they just think he should be left to sleep or I’ve scared them all off is anyone’s guess. The rest of the day continues in the same vein. There’s a visit from a woman who claims to be a gastro doctor, but when I ask her a couple of questions she can’t answer, she confesses that she’s not a gastro entomologist and is just filling in over the weekend, Quite what she is, I never find out. Lollipop lady ? Vet ? Olympic Shot Putter ? Afternoon becomes evening, evening turns to night with no change other than in the nursing team. I breathe a huge sigh of relief when I see who’s looking after Josh tonight as they’re everything you could ask for and don’t just do, they think. He’s stable as far as pain’s concerned most of the time but he’s up every few minutes and sitting on the toilet for long periods without success. We don’t sleep. By 4am he’s screaming like I’ve never heard him scream before, as the pain is so bad. We try him on IV Paracetamol as well as Fentanyl and I climb into bed to cuddle him, which he doesn’t normally like when he’s feeling unwell, but welcomes this time. He manages to sleep for a few hours and I climb back into my bed just as it’s getting light. It’s been a shocker of a night, but I think he’s over the worst of it.
Sunday 22nd May
Morning brings some relief. Whatever was stopping Josh pooing seems to have passed and some of the pain and frustration has passed with it. He vomited every single time he drank water last night, but it didn’t pour out like it usually does. It only came up after long periods of deep, violent dry retching that left him exhausted, tearful and with a cricked neck. At one point I told him not to drink, but when he told me through his tears and retching that it was his ‘favourite thing in the whole world’, I relented. If he’s willing to go through this, then who am I to argue ? I manage to nip out to the newsagents to buy wrapping paper for Joseph’s birthday presents from Josh. I have to use surgical tape and hope he won’t care. It’s been a long week and I’ll be glad to be home, except of course, I’ll really miss Joshie. You feel so helpless when you’re not here, but so many things are beginning to grate - from the spring loaded commode that nearly splatters you with poo every time you change bedpans, to the shoddy cleaners who are an absolute disgrace. It’s not helped by the fact that several parents treat the BMT kitchen like a pigsty and just leave dirty plates and mugs dumped in the sink. On the same day a note went round saying that the kitchens could be closed for health reasons if it continues, someone dumped a tray of unwashed dinner plates in there along with a badly worded, barely English note requesting they’re put in the dishwasher. It’s beyond belief. Josh is still struggling, so when the anaesthetist pops in, we up his background pain relief. There’s talk of him having an ultrasound of his abdomen before the end of the day. Joseph and Claire arrive around 1.30pm, but Josh is barely able to acknowledge them. We light candles on a birthday cake for Joseph, but only give him one of Joshie’s presents to open. I head home early with Joseph so we have time to play football in the garden while it’s still sunny. There’s been so much to think about this week. A few things have given us hope and there’s a plan in place now. Deep down though, we’re worried. The doctors have admitted that they have no idea what’s behind Josh’s underlying condition and that it may prove to be incurable. It’s what we’ve long suspected, but that doesn’t make it any easier to hear...
