Today: Taking it day by day….

Since my last post Seth has started to go in the right direction. His liver enzymes have come down a little – they were over 600 now they are at around 400. So still high but better. The level of HHV6 in his blood has reduced from 10 to the 6 to 10 to the 4. This won’t mean a lot to many reading this blog (as it doesn’t me) but its gone from 1,000,000 parts in one ml of his blood to 10,000 parts in his blood. Again this is still very high but a good drop.

squirting water at the nurses

supporting primary immune deficiency week

 

taking bloods (complete with sterile field) from the ward doll – to help Seth deal with everything happening to him

making daddy hit himself in the face

beating daddy at guess who

playing the guitar and singing to fireman sam

At the same time, his T cell and neutrophil numbers had returned, but were clearly donor cells as his Graft Versus Host Disease had returned. So his skin is red and dry and his has broken skin and sores on his hands, and he is not able to maintain his HB (red blood cell) level so was needing at least one bag of blood per day.

When he was very ill last weekend and the doctors were worried about him they added a second anti viral drug. It is rare to use them together, but they did. These drugs are Foscarnet and Ganciclovir. Also on Saturday 19th April Seth started the pre conditioning for his transplant with a drug called Campath. This drugs wipes out T cells, B cells and NK cells (basically all your immune cells).

Since this drug has started Seth has not required blood as often, and the virus in his blood has improved. Last week and the weekend he was getting temperatures of 39.4 and above and paracetamol was not bringing it down significantly, also his respiratory rate had increased. So since Sunday he has been a lot brighter and Tuesday and Wednesday was back to his normal cheeky self keeping us busy having ‘parties’ in his room (which involves playing the guitar whilst fireman sam songs are played on daddies phone) – even the nurse have to get involved!

Tonight, however, he has spiked his first temperature in nearly 5 days. He has been given IVIG (antibodies) today which does make him spike a temperature, but he has also vomited. So we will see what the bloods bring tomorrow and remember the importance of taking it day by day. It’s a high risk transplant the journey is not going to be smooth.

On the consent forms for the transplant the hospital has to put a chance of failure in Seth’s case it is around 50% so 1 in 2 chance of success. It may seem good – we have  50% chance of Seth getting through this. But at the end of this the percentage will not matter. If one of the things that the doctors are worried about happens it doesn’t matter what the chance of it happening was, just as if we come through the other side successfully, it won’t matter what the chance of success was.

Does Seth get what is going on?

I have explained it to him and he understands that he needs to stay in hospital. I said to him that Mummy and Daddy and Hugo all have magic inside them that make them get better when they are ill. I said that Seth was born without this magic which is why he has been poorly so much and now Daddy is going to give Seth some of his magic. It may not be the best description, but he has since told this to his dad and appears to understand that he has something missing that means he gets ill and daddy is going to help to try to fix it. He accepts that he needs to be in hospital.

In fact the two things he gets most upset about are – having his bath and brushing his teeth!!!

So what’s the plan now?

Transplant day is day 0 today we are on -8

-7 we start Ondansetron (IV anti fungal medication to protecting him from difficult to manage fungal infections particularly in his chest); defibrotide (to protect his already inflamed liver) and Treosulfan (chemotherapy drug)

-6 he has the above plus Fludarabine and they stop the buffy coat (neutrophils that they give that go in and fight infection then die) so he will have even less protection. Nik starts GCSF injections which stimulate his bone marrow so he over produces bone marrow cells. He will be given this injection everyday until harvesting. The side effects may cause him to have bone pain as his marrow is working harder.

-5 Treosulfan and Fludarabine again

-4 Fludarabine

-3 Fludarabine

-2 Fludarabine

-1 Nik goes to the Freeman Hospital to have his bone marrow cells harvested.

0 – Transplant day! The official description is infusion of CD3 TCR alpha/beta/CD19 Depleted PBSC. He will also start MMF (an immune suppressant so they can slow the speed at which the graft takes over the marrow) and Buffy Coat will be restarted.

What are we concerned about?

We will Seth everything, you never know how he is going to respond, but the main areas we know may cause a problem are:

Liver – the liver already has some damage, chemo may cause further problems, and the T cells from the graft when it is given may go to the liver and cause problems there. This is a worry.

Lungs – Seth has extensive damage to his lungs from the infection he had at 6 months old. The chemo could cause problems there, and the T cells from the new graft may also go her and cause problems.

Human Herpes Virus 6 – we know this virus is currently live in Seth, and, although the levels have reduced dramatically, it is still very high. We could lose control of this virus and the only real way it can be resolved is with Seth having his own immune system to fight it.

Once the transplant has been done we wait, we wait for the bloods every day, waiting for his bloods to change. We want to see his neutrophils coming up within 10 to 20 days post transplant. Other cells will follow. Once he has these cells bloods will be taken to identify who’s they are – we want them to be Nik’s.

So that’s it. Tomorrow it begins and we have 6ish weeks of anxiety, not knowing what the next day will bring.

So.

We take it day by day.