T+161

160 days since transplant day!!!! How has that happened?!!?! I am very sorry it has been so long since I have last provided an update. It wasn’t my intention to leave things so long, but being at home is just total brilliant chaos!!

After coming home everything was nice and settled for the first week. We went back to Newcastle for a check up and again they were happy with him. He was booked in to have his apheresis line taken out on the Friday of the following week. However, the line was not causing any problems and Seth was still requiring blood tests regularly. Also although his need for blood and platelet transfusions had reduced we were still not confident he would not start dropping again. So, the decision was made to postpone the operation to have the line out for a bit longer just to be sure….

However, on the Monday of the very next week his line was accessed for blood tests and shortly after Seth approached his dad with his t-shirt up saying “my line is leaking”. He was totally unfazed by the blood coming out of his line and going everywhere. Upon inspection the line had split near the top where it entered Seth’s body. We knew that this would be unrepairable so prepared ourselves for a trip to Newcastle to have it out.

First stop though is always the local hospital so that they can do initial checks and liaise with Seth’s consultant in Newcastle with regards to a plan. Also he was put on antibiotics immediately in case an infection had got into the line through the break. 24 hours later we were back on the road to Newcastle. The next day the line was taken out and luckily because Seth had not had a temperature he was able to return home the next afternoon. For the first time in a long time we had a fairly straight forward trip to a hospital, no unexpected long-term stays. BRILLIANT!!!

back in newcastle waiting for the op

school work !

the line- the blue clamp next to his skin is stopping the line from bleeding.

ready for the op!

All gone!!!

Being at home is fantastic for all of us. However, Seth is now more of an energetic, curious 5-year-old than he ever has been. He wants new challenges and new experiences like any young child, which can sometimes be difficult when he still has to remain isolated from other children and busy places. We have been pursuing home schooling via the school he is registered with and whilst waiting for that I have been trying to home school him so that he does not fall behind his peers. I think he will have enough to deal with socially when he finally goes to school so we like to try to reduce any other sources of stress for him.

Learning numbers playing Bingo

making bread

fine motor skills practice poking pipe cleaners through a sieve

learning about dinosaurs

collecting leaves , learning about autumn

  

making trees with a straw and water paints

Health wise he has been great. No illnesses or temperatures so far. So we are really pleased with that! Immunology wise we are still waiting for confirmation as to whether or not the Thymus is working. It is quite a source of stress for me at the moment. My understanding is that there is no definitive way to confirm whether or not anyone has a functioning Thymus, you just have to wait for ‘educated’ T cells to appear. This can be monitored via 3 things – which I cannot now remember the specifics. However, one is increased T cell numbers. This last week Seth’s T cell numbers have doubled – but this can happen also if he has in infection that he is fighting – then the uneducated T cells would multiply. Also there is a marker in the blood that is monitored in percentage – this has decreased significantly over the past few weeks which does indicate a working thymus, but again it is not conclusive at this time. The third way is by detecting the educated T cells in the blood. At present there is very little evidence to suggest educated T cells. So we continue to wait. Seth having GVHD for so long prior to this transplant may simply delay the Thymus sending out new educated T cells. Or there may be irreparable damage. Wait and See – rubbish!

He has managed to get rid of Rhinovirus which is promising. He is still positive for Adeno virus but is keeping it low level, so again that is positive. His skin is fantastic! Really clear. We have managed to be able to reduce the steroids right down again and we have not seen the GVHD rash in a few weeks!

IMG_9015

As a parent in this situation you have to manage your own emotions and stresses, trying to stay realistic and not to allow optimism to overcome you. With Seth being better in himself than he has ever been it is difficult to prevent myself from getting too comfortable, forgetting that this is not over yet. Also you find yourself trying to manage other people’s expectations as well. Everyone historically knows Seth as a child with physical struggles because of his health. They knew him as a child that did not run about, lacked confidence and tired easily. Now they see this normal (albeit small) 5-year-old energetic cheeky boy running around, so its difficult for us to highlight that we still do not know if he will be ok.

When we arrived in Newcastle in January this year, Hugo was only just 3 months old……he turns 1 in 3 days!!! What a year!



  

Today: T+112!!

It’s all a bit strange at the moment, time seems to be going so quick, Hugo is 10 months old! He was only 3 months when we first embarked on our Newcastle leg of Seth’s journey. My 12 months of maternity leave is due to end in a months time. Luckily I have been able to apply for addition time off, so i am not returning to work until January. That will give us time to return home and start living as a proper family again!

Over the past few weeks Seth has really settled into living with us all again. However, he does ask about going home quite a lot. He misses his home and his friends. I am at home at the moment getting the house ready for his return and when we speak on FaceTime he asks for a tour of the house and to look at his favourite toys!

With regards to his eating we are seeing an improvement! He is now eating reasonably well. Most days he eats lunch and dinner. It is still only small amounts however its regular eating. He still struggles psychologically though. He will say that his tummy is rumbling. But then when sat with the food in front of him he uses a number of different distractions techniques to delay putting the food in his mouth. Once he does have that first bite he eats well. We just keep eating our food and let him go through this process. It does seem to be getting a little shorter.

Immunology wise we are pleased that his bone marrow is still showing 100% donor. This means that all the cells in his bone marrow are Nik’s. They test this every month to see the strength of the graft. So far so good!!!
This week we have noticed that his cheeks are a little red and a patch on his arm. This looks like a bit of skin Graft Versus Host disease again. He has been given some topical steroid cream to put on the effect areas and we hope that will be enough. Over the past few weeks they have weaned him off the majority of his anti-rejection (immune suppressant) medication. He was on Tacrolimus, Mycophenolate Mofetil and Prednisolone. He is now only on 2.5mg of Prednisolone on alternate days. This is the smallest amount of Prednisolone he has been one ever!!!! As a restful we are continuing to see he face appear as his cheeks reduce and hopefully his belly will continue to reduce so he gets a much more normal shape.

Virus wise things are looking good. He has had 2 weeks of negative results and he is not longer on medication to treat them. We are just waiting for the all important 3rd negative! This is important as 3 negatives is what they want for Seth to be considered free of the virus – although it may come back later. Although the hope is now that he is not on any significant anti-rejection drugs his immune system will be able to fight these virus’ itself – another excellent out come of the 3rd negative result! FINGERS CROSSED!!

Were getting closer and closer to the time where we will find out whether or not Seth’s Thymus is educating the new T cells. Its something I am thinking about daily. Seth is doing so well at the moment I just want to enjoy it. All his life he has been tired and moaned a lot. Since this transplant he is like a normal boy. No moaning, no winging, loads of energy. It has made us realise that for the past 5 years he has probably felt rubbish all day, everyday. It makes me even more proud of him, as despite that he has had a go at everything put before him. I just don’t want to get too comfortable and then find out we have to get him through a Thymus transplant. If it comes back that the Thymus IS working I can start to relax! FINALLY!!!!!

Seth has been booked in to have his Apheresis line removed on 18th September, this is a big thing for us as this normally happens just before you are allowed to go home! Fingers crossed things continue to go well and we can be home in just a few weeks!!! PLEASE!!!!


  
  

T+89

A little while since my last post. Apologies for that.

However, we can now say Seth is no longer an in patient at The Great North Children’s Hospital!!! He was discharged on Thursday last week so is permanently living at the halfway house near the hospital with Daddy, Hugo and me!!!!


  
  
Seth is settling back into family life well and has FINALLY started to eat. It is very small amounts and not everyday, but he is eating and has a good relationship with food. He is no longer getting anxious about sitting at the table or being around food. Last week we did homemade Pizza which he loved making! This was the start of him eating more, he did not eat his slice, but did eat the ham off the top. A couple of days later he ate the meat and gravy part of Shepherd’s Pie, and today he has had some yorkshire pudding, vegetables and gravy! We are really pleased with his progress. Its hard not to pay attention to him eating, we have to turn away to hide our big grins! He seems to have started eating more as we have stopped the intravenous nutrition, as he is now feeling a bit more hungry. He still has a 14 hour feed of high calorie milk over night, as we cannot risk him losing weight.


  
Being at the halfway house more and school finishing for the summer holidays means that we have had a bit more time to do things. We have been to a local centre for disabled children called the Alan Shearer Centre (founded by a local footballer/soccer player). Here we can hire out sensory rooms or their hydrotherapy pool, and they have an outside play area which, so far, has not had any children playing on it so Seth has been able to use it. We are not at the stage where he can use the hydrotherapy pool just yet. But we used the sensory room which was great for both boys. They had a brilliant time.


  
  
  
  
  
Medically Seth is doing really well, hence the discharge! He is still positive for the HHV6 virus but the level is still low so it’s not a major concern at the moment. He is no longer positive for the Adeno virus, although that has come back once. He is still on the drug that treats both of these so hopefully they will remain under control until his immune system can deal with them itself.

Seth’s skin is amazing still. The GVHD rash appears to have gone and we have been able to stop one of the anti-rejection drugs. He is still on steroids as a management but only at 2.5mg on alternate days – the lowest he has ever been on! He is on a third anti rejection drug called Tacerolimus but he will stay on that for a while after transplant. The next step is to ‘stress’ test him (medically) to see if he is able to produce his own steroid. Because he has been on steroids for so long (about 4 years) his body may have stopped producing it – which means he would need to remain on oral steroids at the current level to match what the body naturally produces.

These next pictures I found this week. They are of Seth 1 year ago! What an amazing transformation!  
 All in all his progress has been brilliant so far. It is hard not to get over excited about his future, but we are certainly more relaxed about things. The next major worry is whether or not his thymus will educate the T cells his bone marrow is now producing. If it does that will be a MASSIVE relief for Nik and I. If not we have to look at a Thymus transplant at Great Ormond Street Hospital. I don’t know what that entails at the moment as I am hoping it will not be necessary. They will test his T cells around 120 days post transplant so see what is happening. So another 34 days to wait!

Today: T+68

Sorry it has been a little while! I started writing this post, and then my laptop had a falling out with the wifi. However, they are now communicating again so i can continue!!

In the last couple of weeks we have had one brilliant change……we have been allocated a halfway house. Hugo and I are now living at the house. Seth is allowed to be at the house all day, unless he needs medication or a transfusion. However, he must stay at the hospital at night because he still has to have TPN (intravenous nutrition). He is still not eating any food. He is showing interest in food which is an improvement so hopefully as each day passes we will get another day closer to him eating again. He is tolerating milk better now so we are able to increase it 1 ml every other day which means he is getting better nutrition through his gut.


  
In himself Seth is OK. His mood is good, however, it is subdued. When we first got the halfway house, Seth lost a bit of his sparkle. It was a little like it was a bit too much for him. He also found Hugo difficult to fathom. He isnot the peaceful little newborn Seth remembers. He now takes Seth’s toys, and does not understand the rules of sharing. Hugo also demands a lot of attention, he has to be watched. Seth isn’t used to that. He has not had the opportunity to grown with Hugo. So we make a big effort everyday to make sure we spend quality time giving Seth attention alone and with Hugo.


His skin is brilliant at the moment!!! The GVHD rash seems under control, we have been able to reduce the steroids so he is on 5mg one day and 0mg the next. This is still IV at this time, but will change to oral when we drop next time which will be to 2.5mg one day 0mg the next.

The Adeno virus is back, but only at a low level. The HHV6 is still at the same level. But neither are affecting him. So at the moment we are not very worried about them – they are managed and hopefully as we reduce the suppression his immune system should come up more and be able to deal with the virus’.

So things seem to be going well at the moment. Things are just taking time. We have worries, but they could be worse.

Today: T+54

As time goes on and Seth still receives gifts and letters in the post and messages through various media wishing him well. We feel grateful for all the support he has received. This week has brought a new surprise. A letter from our local Member of Parliament, and a second letter from the Prime Minister, David Cameron. It is nice to know that Seth’s story has been heard by so many and will possibly help others dealing with Primary Immune Deficiencies and/or Bone Marrow Transplants.

As I have previously said, the Bone Marrow Transplant Unit at the Great North Children’s Hospital is supported by The Bubble Foundation, an incredibly small but amazing non-profit set up to support children with Seth’s illness, Severe Combined Immune Deficiency. However, the unit transplants children who suffer not only with immune problems but any other difficulty that can be cured or helped by a bone marrow transplant, such as leukaemia and childhood arthritis. These children also benefit from the great work of this charity. Speaking to other families on the unit also opens your eyes as to how different every journey through transplant can be. I have discovered that with leukaemia a small amount of Graft Versus Host Disease can be a good thing as it can prevent the leukaemia from returning, which is the exact opposite of what we hope for with our transplant! As there has been so much interest in the charities supporting Seth I will do a blog entry (separate to this one) highlighting the support we have had so far. Also I have been contacted by quite a number of families who have themselves been through the bone marrow transplant journey and I thought it would be nice to have a blog post dedicated to helping families prepare for transplant that everyone can add their experiences to. Again this will be added in a separate blog.

Seth in himself is continuing to do really well.

Eating – He is still not eating consistently but on Monday he had half a yorkshire pudding (i am not sure if our readers from outside of the UK will know what that is – but we have them with a roast diner …. and they are amazing haha) and a floret and a half of broccoli. Today he had 2 plain pasta shells.

It doesn’t sound much, but his attitude towards eating is improving. We know he does not have a great relationship with food, so we have made a sign for his room door asking people not to come in if he is eating, and if they need to, not to mention the food. If people talk to him about it even if it is just “o you are having something to eat!” you can see him shut down and become anxious. He will then refuse to touch the food. Today, however, when his pasta arrived he gave a big “Whoop!’. So it is slow progress but if we can make food for him like it is the rest of us we will have won the battle i think.

He is still having milk feeds over night to give him nutrition alongside intravenous nutrition. The milk feed is now running at 23ml per hour, 4 hours on 2 hours off from 5pm to 7am. They have reduced his intravenous nutrition to 10 hours, so he has all day off his lines now other than the odd short medicine or blood/platelets if he needs them. He is still vomiting daily, and this increases when we increase his feed, but seems to settle down 3 or 4 days after.

This means that we are now waiting for a halfway house! Seth won’t be able to stay overnight but can be there during the day – which I really think will help his relationship with food – especially as Hugo LOVES eating!!!

Also I think having a bit more normality will help Seth psychologically. Over the past few weeks his behaviour has been getting a bit difficult to handle. He is not a naughty child, never has been. But he has so much energy he is bouncing round the room, switching from one activity to the next, not concentrating and pushing boundaries. It does not help that British Summertime is not to be seen and its raining everyday at the moment. We did manage to get him out on Monday to the museum again though. Which he walked round on his own showing me everything he loved.


    

Unfortunately, our family dog, who Seth loves SO much, has a tumour behind his liver. They don’t know if it is cancerous or not at the moment so we have no idea what will happen in the future. But Seth has been so interested in what is happening. He asks questions about blood tests, and needles and has made Stevie (the dog) 2 get well soon cards. Hopefully Seth will be able to see Stevie again.


Health wise, this week things seem to have improved slightly. The HHV6 virus is showing less in his blood and the Adeno virus that he was positive for last week is now negative. So the drug he is on for it appears to be working.

His skin looks great this week. The GVHD does seem to be flashing up now and then but I think the frequency is less, and it only flashes up for a minute or so and responds to the cream. Fingers crossed that this is as bad as it gets. I really hope so anyway.

Graphs

I thought I would post pictures of the graphs I have been keeping so I can monitor his progress.

Platelets. These are still dripping quite quick, but he is not needing transfusions as much as he was.

Neutrophils. these have been up and down but remain in an ok range. i would like to see them go higher though.

Red Blood Cells. These are maintaining much better than they were as you can see (the  rise is when he has had a transfusion) he is not needing these anywhere near as much as he was.

So our journey continues… i would love to live a week or a month at a time…..and go home…..i really miss home… but we are still a journey away from that…and still taking it day by day.

Today: T+42

The past 10 days have flown … and i am pleased to say its because we have been having a great time!! The Graft Versus Host Disease, so far, is responding to high dose steroid treatment and the rash has gone. It comes back  a little bit now and then but applying steroid cream seems to calm it down. The treatment started at 2mg per kilo (seth’s weight) of Methalpred. He is now on 1.5mg per kilo which will be reviewed again in the consultant meeting on Tuesday. HHV6 Seth has been on treatment for Human Herpes Virus 6, which is a drug called Foscarnate. He tested positive for this and the level was 10 to the 5 in his blood. A week later he is now 10 to the 3. So it is improving. Another positive thing is that he has had no temperatures and he has not been symptomatic with it. Immunology The doctors are happy with his immune system so far. He still needs to be kept away from busy places and children. But we are able to take him to the park everyday and he went to the local museum during the week. Which he loved as there were Dinosaurs and fish like Nemo.

a hour at the museum

holding on to daddy

Today: T+32

So where do i begin? Its been a little while since my last post, and i apologise for that. But we have had a few developments and I needed to process one in particular and find out more information.

So the good news first!

Look at these hands!

        

An amazing improvement and finally he has his fingers back (well most of them anyway!) He still has a couple of bits to heel but they are generally really good. The first thing he did with his hand (before they had finished dressing them) was touch his head.

 

We hadn’t realised he had never felt his head. He was surprised at how it felt but says that he likes it. Losing his hair isn’t a problem for Seth. His foot is also getting better and he is walking on it confidently.

The infection level in Seth’s blood is

However,

The Human Herpes Virus 6 (HHV6) is back, low levels in his blood at the moment and we are waiting for the results of a re-test to know whether or not he will need treatment for it.

Also, on Friday we got a devastating blow. The Graft Versus Host Disease has started again in his skin. It’s obviously not caused by the same cells as it was last time as he no longer has those cells (they were from the previous graft which the chemo killed off). GVHD is when the T cells attack the recipients cells. In Seth’s case the T cells from the transplant are attacking his skin – thinking it is an infection.

At the moment it is presenting as a red rash quite angry looking around his groin and then he has patches of the rash on his arms, legs and chest.

The rash to me looks different than it did last time. Last time is was less ‘rash like’ and instead angry red skin that was dry and peeled. I don’t really remember what it looked like in the beginning. However, last time the GVHD was predominantly on his hands and feet and, at the moment these areas appear to be pretty clear. The treatment at the moment is IV steroids at a very high dose (2mg per kilo) for 5 days, clobavate ointment and tacrolimus ointment Today he was the 3rd day and doctors feel that he is responding but its a long road – we have been here before.

At the moment Nik and i just feel deflated. All of the fighting for the past 4 and a half years a second transplant, and we feel like history is just repeating itself. It is early days we know. we are just gutted.

But all this happening has made me feel a little bit like I got complacent. I am really worried about it. Things seemed to be going so well. I guess being aware of what might happen doesn’t necessarily mean that it won’t affect you when it does. That’s the nature of the bone marrow transplant it seems. I guess that is why we take it #daybyday.

In himself Seth is brilliant.

He is still tiring easily but he is motivated to do things and get out.

The physiotherapy department at the hospital has a ‘gym’ that Seth is now booked into on a Monday, Wednesday and Friday for half hour sessions. At the moment the physio is hiding animal bean bags (which Seth loves) and getting him to find them, moving about the room. We encourage him to race Daddy to really get him moving.

The increased movement makes him breathe harder so it is good exercise for his chest. He is still having his daily school sessions and is learning so much.

He is now mentioning going home a lot more. We have told him that before he goes home he has to go to a house here in Newcastle with Nik, Me and Hugo. He is happy about that, as long as he can teach Hugo how to play he is happy! However, he is still a while away from moving on.  We don’t know exactly what he needs to achieve to get there as things change daily and every child is different. We do know though that he needs to be off intravenous nutrition (TPN). This is an infusion that runs for 18 hours giving Seth all the calories and nutrition he needs. We need to replace this with milk feeds via his button and/or normal food. At the moment he is not eating and only tolerating 20 mL per hour of milk (split over 16 hours a day). To be able to come off the TPN he needs to be on 67ml per hour (this is without any food consumption). Seth has been told this and today decided to have two crisps and a mouth full of noodles. He says that tomorrow he is going to have 3 crisps! Although we will see. We are not going to pressure him. We want to encourage him to have a positive relationship with food.