HLH

So we’ve been given a diagnosis.

Kind of.

It’s a diagnosis for what is happening in Scooby right now. Just before Christmas he was due his fourth chemo infusion but he couldn’t have it because his platelets were slightly low. We went back the next week and they were still low which put the doctors in a quandary about what to do. The plan was to try again the week after but on the 1^st January (I know – what a great way to start the year) his hand became red and swollen. We took him into the local hospital and he was treated for cellulitis. He came home on Jan 4^th (Turtle’s birthday) but a few hours later had a soaring temperature.

Richard got one of his feelings that things weren’t quite right, and as his intuition on Daniel’s condition has been 100% right so far, we decided to skip the local hospital and take him straight down to Manchester. Within a few days more soaring temperatures, even weirder blood results and an increase in seizures told us he was getting worse again. The decision was made to stop the chemo, and we were gutted. We’d pinned our hopes on it and now we were back to square one. On the two year anniversary of his first hospital trip, exactly the same investigations were being repeated yet again. Blood tests, organ scans, lumbar puncture, MRI, bone marrow sample – he was given the works. But this time they found something that wasn’t there before. All the symptoms added up to something.

Richard rang me on Thursday – the day we were due to swap over anyway and told me to be down in Manchester for a meeting for a possible diagnosis. I couldn’t get down there fast enough but I didn’t dare hope just yet. As much as we’d wanted a diagnosis, there had always been an element of hope in the fact that nobody had ever said the words ‘terminal’, ‘incurable’ or ‘degenerative’ to us yet, and although this time (as well as the previous time in September) I was preparing myself for the worst, I still didn’t want to hear it said by someone as fact.

Our consultant was able to tell us what he knew that evening, but we had to wait until the next day with the other specialist to get the full picture – which gave us time to Google the condition (is that a bad thing or a good thing?).

And this is what we know now. Scooby has a rare condition called HLH - hemophagocytic lymphohistiocytosis. It’s a 1 in 1.2 million people disease, but even then Scooby is showing an unusual form of it so I guess he’s even rarer than that! It’s a blood disorder where the immune system attacks cells it’s not supposed to:

 ‘HLH results when critical regulatory pathways responsible for the natural termination of immune/inflammatory responses are disrupted or overwhelmed. In HLH, pathologic genetic defects alter normal crosstalk between innate and adaptive immune responses in a manner that compromises homeostatic removal of cells that are superfluous or dangerous to the organism. The result is excessive and persistent activation of antigen-presenting cells (histiocytes) and T lymphocytes’.

 

It’s usually genetic but as Scooby was not a baby when it started, he must have the acquired form of it. What is not clear is whether this has been the problem all along or whether some other disease, or even treatment, has caused this to develop. So we could be treating the mystery disease that’s plagued him for the last two years, or we could just have a new problem on top of the old one.

The treatment for HLH is aggressive. Once it’s found, it needs to be treated immediately as it’s usually fatal. For the next eight weeks he’ll be on new steroids, immuno-suppressants and chemotherapy – the ‘real’ stuff this time, not the very mild form that he’s already had half of. He’s now on the oncology ward and we’ve been told to prepare ourselves for a tough time. He’ll be in for at least four weeks, then the treatment halves and I think there might be the opportunity to bring him home while he has the rest. Also by the halfway stage we should know whether or not it’s working. If it is, then we will go through the process of finding a blood marrow donor, so that once the treatment is finished and the disease is eradicated, he would start from scratch with someone else’s immunity.

We asked what would happen if it wasn’t working. There is no plan B.

 

So we are starting a whole new phase of this thing. We’ve watched our boy be poorly in varying degrees for the last two years, but we are now into unfamiliar territory. A new ward, a new group of doctors, new medicine that for the first time we haven’t even asked the name of or wanted to know the long-term side effects of because we just want to get it into him before it’s too late. He’s had a central line put into his shoulder which, instead of being traumatic like it would have been a year ago, is a welcome relief from the many many cannulas and individual needle pricks that have riddled his body. No more bruises, just instant access in and out all the time. He’s also started with blood transfusions as he needed more platelets and plasma before he could even go into surgery to have the line put in, and he will be needing more transfusions as he goes along, so to anyone who has ever donated blood – THANKYOU!

 

I am so so grateful that our amazing doctors found this. I am so so grateful that he is being treated  eight years after the case studies and the agreed treatment for HLH were completed and were made known to medical practitioners everywhere. I am so so grateful that Manchester Children’s Hospital has one of the top haematologists in the country who has had involvement with 60% of all known cases of HLH in the country. And I am so so grateful that we had a fantastic Christmas at home all together before facing this next giant.

 

And I am so so afraid. I am afraid that after two years of disease working its way through his brain and slowly wearing down the rest of his body, that two months of aggressive treatment might be too much for him to handle. He’s starting this battle at a point where he can no longer walk or use his hands, and is even struggling to hold his own head up now. He is sleeping nearly twenty hours every day and has very little appetite. His speech is slower and his eyesight is weaker than it was just two weeks ago. I want to be strong and to say firmly that we will handle anything the next few months throw at us, but after two years I am battle-weary too and my coping capacity is wearing thin. Fear seems to have displaced hope and is taking over all my internal organs.

 

But I know I am not doing this alone. I don’t know how all this will turn out but God has been talking to me through Hebrews chapter 11, how faith is not knowing what will happen, but trusting in the One who will make it happen. It’s also about people who didn’t see what they hoped they would see in their lifetime, but who were part of a bigger plan. Some saw miracles and some didn’t. Some saw the fruits of their labour and some didn’t. I am hoping and asking to see our miracle but trying to keep my eyes on Him who is bigger than the miracle.

 

And as always, we are so amazed by all the people who are taking time to think, hope and pray with us. After two years people should be sick of the ups and downs and the continual requests, but still they are contacting us and wanting to know what to pray. I am blown away by the amount of people who let us interrupt their prayer life, their church meetings and their daily thoughts.

 

My friend Jacqui has set up a group to pray and fast for forty days for us. Obviously we’re not asking anyone to give up food for forty days, but maybe if enough people feel called to do it, there could be a different person fasting food each day. Or something else – TV, chocolate, etc. It’s here at http://www.facebook.com/events/278897825505045/ if you would like to join or get more information.

 

2 Corinthians 5v7: For we live by faith, not by sight.