Today: That boy just gets more amazing…..

So my last post detailed my concerns about the return of some of Seth’s immune system (t cells). We have now spoken with Seth’s consultant who agrees with us – this graft is unstable. It is going to be better for Seth to get rid of it and start again. Even though it is dangerous the years of steroids and problems with this graft are taking its toll on his body. So its time for that graft to get out!!!

This week the plan was to remove Seth’s gallbladder because of a large gallstone that has been causing problems. This happened on Tuesday and whilst he was under he had his PEG changed to a button. The PEG is a tube going from the inside of his stomach, through the stomach wall and out of his belly that milk and medicine can be put down. Seth has a very poor appetite so we need this to help him put weight on. The button changes the tube into a shorter tube that stops at his skin. It is more hygienic and doesn’t dangle down so won’t get caught on things.

The gallbladder removal went well and they were able to do it via keyhole surgery. Afterwards he was in a lot of pain (which is always difficult to judge with Seth as he doesn’t complain, but when he tried to move he cried so we know it hurts a lot) as such he had morphine to help him manage the pain and get some sleep. The day after his surgery we were surprised at how Ok he was. He was his normal self, sore and needed a morphine bolus when we carried him to the toilet, but he was sat up in bed playing paw patrol and fireman sam. The day after however, he was very sore and did not want to sit up or move at all, he slept a lot and they had to increase his morphine. By the Friday, however, he had perked up again. He had a wound at the bottom of his belly (similar place to a c section scar) which started oozing. This made Nik and I worry as our first thought was INFECTION. However, the surgeon came to see him and was very pleased with the wound. Leaking is expected apparently because it is a high pressure wound and as long as it is dressed properly and kept clean it is fine.

Seth has now been moved into the room he will be transplanted in this room his smaller than his previous rooms (no reason – just how it is) and it has a blue line on the floor. Inside the blue line is the “clean” area. Above Seth’s bed is an air system. It pushes air down onto Seth’s bed. Vents in the corner of the room suck air out. So the air inside the line is filtered and clean. Outside the line is where the dirty air is pushed to.  So this means that he can have visitors – as long as they are well and do not enter the area within the line.

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The blue line that keeps Seth safe, and the rules of RED isolation.

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As a result of the move we are now in RED management and will be so until he has neutrophils post transplant (approximately 10 days to 3 weeks after transplant we should see him having neutrophils). At that point he will go down to PURPLE management and be allowed out (wearing a mask).

This week two other families on the ward have children that have now got neutrophils after transplant. This is brilliant You spend a lot of time on the ward and although you never meet the children, you feel like you know them. You spend a lot of time with their parents and asking after their welfare. It is brilliant to see things going well. But at the same time you have a pang of sadness wishing to be in their position. So much could happen in between now and then, we may never get there.

I had a brief conversation with Seth’s consultant on Sunday and he is very happy with Seth’s progress so far. A CT scan last week showed the infection we were worried about has gone – so what the doctors are doing so far is working. Ideally they would like to transplant tomorrow – as soon as possible. But it is not safe to do so whilst he is still healing from the operation. Chemotherapy could cause the wounds to re-open, which could be devastating. So it is a fine balance rather than best case. Were hoping he will heal well and we will be able to start chemo in 3 weeks. But that is entirely dependent on Seth’s recovery. We have had so many twists and turns over the years we take everything day by day. Never look to tomorrow – things could be entirely different and that leads to disappointment.

When it is time to transplant Nik and I have to give consent, at which time we will be told in the ins and outs of chemotherapy and the risks. We know that these will be scary. But we have no choice. We do this or we definitely lose our boy. This gives him a chance. We know that Seth has extensive damage to his lungs from the lung infection i spoke about here. Chemotherapy may cause issues here, this may be a problem, he may struggle and need assistance to breathe. We have to be ready for that. We hope it doesn’t, but we have to be prepared.

On a lighter note during this conversation we spoke about Graft Versus Host Disease, which we have been battling for 4 and a half years and lead to where we are today. Research indicates that a T cell depleted transplant carries a low risk of Graft Versus Host Disease. Seth’s chances are higher because he has had it before, but even so they are still very low.

My mum and step-dad came to visit this weekend, they spent time with Seth (only my mum was allowed to touch him as she is the 4th carer. My step-dad was allowed in the room but had to sit in the dirty area and was not allowed to touch anything.). They also took Hugo for a while so that Nik and I could be in Seth’s room at the same time – which was weird AND BRILLIANT! I really can’t wait for the day that Hugo can be there too.

New bedding sent to him. He LOVES it!!!

New bedding sent to him. He LOVES it!!!

Doing school work - learning to write

Doing school work – learning to write

Crafts! (he thinks it is fantastic that he is now allowed to use scissors)

Crafts! (he thinks it is fantastic that he is now allowed to use scissors)

Easter Eggs! All donated to the ward by kind people....Seth does not like chocolate though so........

Easter Eggs! All donated to the ward by kind people….Seth does not like chocolate though so……..

Playing with Paw Patrol pups and showing off his new hair cut!

Playing with Paw Patrol pups and showing off his new hair cut!

the certificates he is given when he has to have something done that isn't nice, or he does something that we want him to do like eat.

the certificates he is given when he has to have something done that isn’t nice, or he does something that we want him to do like eat.

We have started a timetable (awful pic because i decided to put it on the window). This really works for Seth, he loves it and at snack time he actually ate (it was one carrot - but thats the most he has eaten in weeks!)

We have started a timetable (awful pic because i decided to put it on the window). This really works for Seth, he loves it and at snack time he actually ate (it was one carrot – but thats the most he has eaten in weeks!)

After the overwhelming success of #wearyellowforseth we have started to decorate his room….

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So that is where we are – no we wait and take each day as it comes. We wait.

Today: This will be quick…it’s only been a week…although this is Seth….

Seth is continuing to improve and it totally back to his normal cheeky self! He’s current phrase is if he is asked to do something to shrug his shoulders and say “alright bossy!” sigh…

Here’s how he’s been spending his time:

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Being a Ninja with the play specialist

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Using the suction as his ninja weapon!

Taking time out to watch some TV after a busy day

Taking time out to watch some TV after a busy day

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A bit of golf to pass the time

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A sunday roast….with gravy in a mug for dipping purposes (thats the northerner in him!)

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Talking to Hugo via Facetime (what would we do without technology!)

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Doing his own obs – taking his own temperature

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Concentrating!

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Check out the guns!

A lot of people ask Nik and I how we get through this, and its difficult to answer. We’re no different to any other parent out there I don’t think. You just get on with things. We’re helped by having family and friends who help us every step of the way. 

I’m not naturally someone who relies on wishes, prayers and other things that aren’t tangible. For me thats a risk, it’s not tangible or measurable. 

My gods are numbers. Numbers in the form of Seth’s daily blood results. As a parent in this situation you become knowledgable about the meaning of the blood that is taken from your child at 6am every morning. By 10am you wait for the nurse to come in and fill in the sheet you keep on the wall to look for patterns and changes.

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The 2 ‘gods’ of this page are the ALTs and Bilirubin. These levels are related to liver function and the issues we’ve been having with Seth’s gallbladder. Were looking for these numbers to come down. As you can see on the 17th Feb when Seth had become quite unwell on the afternoon of 16th Feb his levels have jumped up high. He was also very yellow that day.

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On this sheet you can see that Seth’s Neut (Neutrophils – white blood cells) are below 1. The numbers our looking at here indicate that he has virtually no white blood cells. CRP (C-Reactive Protein) – This is a infection marker in the blood. So when you have an infection this level will rise, although it tends to work 12+ hours behind the patients clinical signs. As you can see on 17th Feb this number also significantly increased.

Knowing about this and being a major geek about immunology helps me cope. It gives me control back in a situation that we didn’t plan for. It would be easy for me to feel sorry for myself – why do i get a sick kid?….but this isn’t about me. I’m not sick. I made a choice to be a parent and that means my job is to do what i can to be there for Seth.

 We have two children, Seth and his baby brother Hugo who is 4 months old. Being at the hospital is hard on Hugo as he ends up spending far too much time in his pram or being held whilst in a hospital waiting room. So this week I have come home to give him a break. It’s obviously nice sleeping in my own bed (when Hugo lets me sleep that is!).

 Seth continues to be really well and I get videos of him making the most of his time (see below for some examples). 

 Stealing the HCA’s water (as a ninja obviously!!): http://youtu.be/WCp7NZ4kEuw

Moving rooms – Seth had to move into a new room this week because he was in a room with a special filter system for babies in cots, a baby was coming in so his room was needed for them. Seth decided he should move into his room on his scooter! http://youtu.be/zPv45aQvI7Q 

 So here we are ploddin along waiting to get to transplant…..or are we. Of course you don’t all know Seth but those that do are aware that he doesn’t like to conform to expectation!!! Why should he be any different in this situation?! 

The consultants have been having a few confusing, head scratching conversations about our boy recently.

 They had previously sent of a sample of Seth’s current (post transplant) bone marrow to GOSH to compare it with his bone marrow prior to his original transplant. The aim of this was to identify which parts of his current bone marrow is his and which parts are from the donor. 

 A bit of history (i will try and keep it simple and provide links to terms you may not be familiar with): Seth had no T cells prior to transplant but had his own Neutrophils and B Cells (although without T cells his B cells wouldn’t work). Normally the patient has chemotherapy which wipes the entire bone marrow, and then the donor’s immune system comes in and takes its place. So in a normal transplant the new bone marrow would be entirely donor cells. 

Seth, however, had a transplant without chemotherapy because he was too ill to survive chemo. Because he had no T cells he was in the best position to receive this type of transplant as he had nothing to fight the donor cells. 

 So it was expected that when they look at his bone marrow now he would have donor T cells, but his own Neutrophils.

HOWEVER….of course nothing goes as plans…and it appears that Seth’s bone marrow is 96% donor (the split is different but without confusing things its WAAAAAAY higher than expected). 

They’re not sure what’s going on but have two possible explanations:

1. The donor T cells are still rogue and have gone to the bone marrow and been attacking that. This would explain his recent inability to maintain, neutrophils, platelets or red blood cells. 

2. The previous stem cell transplant has started to take over his bone marrow and make it it’s own (basically sorting itself out and begin in to behave). 

So the plan now?!

They will give a drug called ATG everYday for 5 days starting Monday 9th March. This drug wipes out all the T cells. Then we wait. There is a possibility that Seth’s own neutrophils will come back, but they may not. He may be left with an empty bone marrow if the T cells have been able to cause enough damage. So them coming back and giving him a little bit of protection is best case. 

Or the bone marrow may come back as donor cells…this will cause more head scratching and frowning from the consultants!! Let’s be honest Seth really likes to confuse things so let’s not rule it out even if it’s thought to be unlikely!! 

Whilst his bone marrow is doing all this, or not. They will continue to prepare for transplant. In 3 weeks it’s a CT under general anaesthetic with a bone marrow sample being taken at the same time. The CT scan is to check the infection in his chest to make sure they are controlling it. Then a week or so later he will have his gallbladder removed- the anaesthetist will need the CT results to ensure that Seth is at as little risk as possible during the op.

Then all being well we go to transplant 4-6 weeks later. But hopefully more like 4 weeks.

Transplant is incredibly risky. But if we want Seth to have any chance this is what we have to do.

Keep an eye out in the next few days for a #wearyellowforseth announcement.