Today: T+49

49 days post transplant and Seth is doing ok really. Better than expected which is great!!! It’s June and we are halfway through 2015.

I have had a bit of time at home the last week as Hugo has had a cough and I had to return to the dentist (joy!). This was hard leaving Seth, but Hugo thrived. It made me realise how much this effects him as well as Seth. He is only a baby but he has loved seeing my best friends little boy this week (who is 2 weeks younger than Hugo). It got me thinking about what this year should have been. I should have been on maternity leave at home, taking my new baby to baby groups, meeting my friends and spending time helping our new baby grow and become part of our family.

Instead, we have spent the first half of this year giving Seth a chance. So I think it is maternity leave time well spent. Hurrah to the first half of 2015.

In himself, Seth is doing great. He is full of energy jumping all over daddy. It is clear he has a bit of cabin fever – in that he is bored of being in his hospital room for most of the day. He has time in the physiotherapy gym for 3 half hour sessions a week which he absolutely loves. We also get out to the park and make boats to race on the water.

Ryder had a hickman line fitted this week. Doctor Seth checked his bloods – all with a sterilise field and alcohol wipes – ‘got to be clean daddy’.


Walking the ‘snake’ in physiotherapy

  

One of Seth’s favourite physiotherapy exercises – racing to move the beanbag animals from one place to another faster than daddy or mummy!

Today we went out for the longest time yet, about 2 and a half hours and we went to walk around the fair ground that has been set up. Seth loves looking at it (he cannot go on!!) and he keeps talking about how when he is better he is going to go on the fair. He has also become interested in a skate park that we pass everyday and is saying he wants to ride his scooter on it.

Greating Grandma speeding ahead on his scooter. He had not seen her since transplant when the chemo was making him feel pretty ill. So he did not speak to her.

It is so great to see him how he is at the moment. So full of life. He has always been a happy, smiling boy, but he has never quite been a cheeky child of his age. Until now. I sometimes find my self watching him and smiling that finally I am seeing a child emerge.

Hugo learning about Seth’s face – through a protective rain cover…

Infections:

At the moment Seth is testing positive for Noro Virus,  Adeno Virus and the Human Herpes Virus 6. He has been on treatment for the HHV6 infection for 2 weeks, initially the virus went from 10 to the 5 in the blood to 10 to the 3, but it has now gone back up to 10 to the 6. There is an alternative treatment which also treats Adeno virus which he is now on. This is a once a week treatment to start with and will then drop to once a fortnight. The positive thing is that at the moment he is not showing any signs of illness. No temperature’s or feeling unwell! – yey!! He is vomiting every day, this is usually just once a day and we are not sure which is causing it. It could be the mentioned virus’ or low tolerance for milk feeds. But it is not significant so at the moment we are just monitoring it.

Hands:

Look at these bad boys! No more bandages. He has better movement in his fingers that he had before because the skin is not thickened from GVHD. At the moment his old damaged nails are growing out and new nails are coming through. This is common as when you are really ill the body stops doing things that are not totally necessary – like growing nails.


       At the moment Seth is still needing platelet and blood transfusions, but these are at the moment getting less frequent. I don’t want to get ahead of myself, but, I would love for a time to come where he does not need them again. He has been having 3 or 4 weekly blood transfusions since September 2014 and platelet transfusions since October/November at a similar rate.

So, things have changed so much in the last 6 months – surely as a BMT parent 49 days after transplant I can start to relax right?! Well, maybe not quite – I have graphs so I can see any trends or changes in his stool output, Red Blood Cell level, Platelet Level and Neutrophil levels. This is probably not necessary – but as I have said before you are sometimes armed with too much information. I have really found the graphs helpful for me to see the overall picture, rather than panicking because his neutrophils have dropped two days in a row, or he is still needing blood transfusions.

At the minute I feel a bit like we are sat waiting for a train that we don’t know where it’s going. We have a few virus’, we have a little bit go GVHD, we don’t know if the Thymus is working or not. Only time will tell on these. Today, these problems look like they are being managed….but, what will tomorrow bring?

Don’t get complacent and take it day by day

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Today: T+9

So since my post yesterday there have been a few changes…….

Seth was on the CPAP machine again for 12 hours over night as part of the SCARF research.

He had the most settled night he has had in a while, and today has been maintaining his oxygen level WITHOUT the need for additional oxygen! He has been breathing a lot easier and his chest sounds better. Hooray! Towards the end of the day he did start to breathe a bit harder and needed additional oxygen when asleep. But its an improvement.

His cheeky little character is also coming back a bit and we sat on the floor to play paw patrol again today, although he again tired very quickly. He is just so determined not to be sick!

His hands remain sore but we are now only going to change the dressing every other day, and they have doubled the amount of pain relief he gets when he has a bolus. So that should help him a little with the pain. In addition he has been referred back to the tissue viability team who will know how best to dress the hands and ensure he is getting pain relief.

Fluid wise Seth is still retaining fluids, his genitals are less swollen, but he is puffy on his face and legs, and his belly is very distended at the moment. The doctors are continuing to monitor this closely and treating it with diuretics when necessary as well as giving him the minimal amount of fluid.

Being on the intensive care ward is stressful for Nik and I, and is creating some frustration and conflicting feeling (it may be that we are crazy BMT parents scared at leaving our safe little bubble!). Seth is doing well with the CPAP support and it seems to be helping him to improve. But we also feel like we may be compromising his safety in terms of infection. The nursing staff on intensive care are not BMT nurses and so work differently and have a different understanding to isolation nursing. So having Seth on the ward is a learning curve for them, but he is still not receiving the protective care he would receive on his normal ward. The strict isolation rules seem to not apply as much here, but if he needs it he needs it right? This may be fine and not an issue, but 9 days post transplant Seth is as vulnerable as it gets and we are worried.

Tomorrow we are going to speak with the relevant people to confirm what is absolutely necessary to protect Seth so that we can ensure that is happening. It may be that we are more cautious than needs be and he is already getting the level of protective nursing that he requires. At the minute Nik and I are feeling more stressed than we have throughout this process so we need to clarify and understand and then consider our options. As removing him from the research and back to his ward may be detrimental to his breathing and could lead him to need to be in intensive care, which would not change anything anyway.

Now….about that magic…….

It looks like engraftment has begun, he has some lymphocyte numbers coming up in his blood. At the moment that means nothing as they need to check further to see what is going on. But hopefully it is the sign that the magic is home and beginning to settle. This is good – it what he needs, but it may cause him to go backwards again before he starts improving. The new cells might go to all the areas we have problems at the moment and cause further inflammation trying to fix things.

So today was a good day…..tomorrow who knows were still taking it day by day.

Today: T+8

So a few more days have passed and I don’t know if I am saying things are better or worse. It’s such a confusing time at the moment. Somethings are looking better, but then again he seems to be struggling in other ways.

His hair has started to fall out. We’ve talked to him about that and he really doesn’t care. Which to be honest neither do we. Obviously you don’t want your child’s hair to fall out. But we know why it is and it is the least of our worries. It will grow back!!

Seth’s skin is a bit better, a Treosulfan rash is like REALLY BAD sunburn, the skin swells, goes bright red and is hot to touch and incredibly sore, then as it gets better it starts peeling. Ignoring his hands of the moment we appear to have gone through the worst of it and although his legs are stills red and swollen, they are starting to peel and are looking a lot better than they were.

However his hands are still agonizingly painful. He is able to rest when they are bandaged up. However, the minute we take off the bandage to change it the air hits them and he screams in pain. I have started giving him bolus’ of Fentanyl every 20 minutes for an hour before we change his dressing, but it does not seem to help him when the air hits his hands. The sores appear to have got bigger, but they are dry and not weeping. It is the chemo attacking his already damaged hands and unfortunately we have to wait for the effects to go away. We just have to try to keep him as comfortable as possible and infection free until then.

His mouth and tongue are covered in ulcers, but they are bleeding a lot less and 4 times a day mouth cares appears to be enough to keep his mouth clean and free of mucus build up.

As his skin and mouth appear to be getting better, his breathing has slowly been getting worse. His oxygen requirement has been increasing and he is clearly working harder to breathe. Chest X-rays have shown some changes, but only very slight. However, this was enough for him to be suitable for SCARF research being conducted here and at Great Ormond Street Hospital. It is fairly common for children with immune problems to develop breathing difficulties. Sometimes these get so severe that the child needs assistance via intrusive ventilation. There has been some research recently that has evidenced that non intrusive early intervention reduces the likelihood of the patient going on to need intrusive breathing support later on (i think about 60% of the time). The non intrusive ventilation is via CPAP (Continuous Positive Airway Pressure). We were asked if we wanted to take part in the study, which we thought about and decided to go ahead as the CPAP will give Seth rest to be able to recover and not have to put as much effort into every breath. Once our consent Seth’s name was put into a computer which chose whether he would be part of the control or test group. Seth was in the test group. This means that he has been transferred to the Paediatric Intensive Care Unit and for 12 hours a day is on CPAP ventilation. It’s a four-day trial so as long as during that time he does not get to the point where he needs intensive care he will return to his normal room.

Infection wise we still do not know what is causing the high inflammation markers or the high temperatures. However, the marked in his blood have been going down for the last couple of days – fingers crossed they keep going!

In himself Seth is coming back slowly. Yesterday he insisted he was playing Paw Patrol and that he was getting down off his bed to do so. He managed to make it down there and get all the pups in the lookout, however, then was tired and I suggested he got back in bed. It is heart breaking watching him desperate to just play and it be such a struggle for him.

Still no sign of the new cells, but that’s not expected just yet. We could see them anytime from today onwards so – BLOOD RESULT WATCHING is on!!!!

The past two days have been better – starting to go in the right direction. But things could change back very quickly, and might do so when the new cells really start to engraft. We just have to wait and see and take it day by day.

having a nap during time off the CPAP. this is just a normal mask with hudrated oxygen going through it.

Today: T-1

The day before transplant and Nik’s has been to the Freeman Hospital to have his bone marrow cells harvested. This meant that Nik was hooked up to a machine for about 3 hours. The machine took out his blood, then separated out what it needed, and put the blood and unwanted parts back into Nik. Wowzers!!! It’s come along way from poking a needle through the bone! Doing it this way actually results in more cells being harvested. As soon as Nik came off the machine he felt much better, the pain and flu-like symptoms of the last 5 days have gone now he is not carrying increased bone marrow cells. Nik’s main complaint was that he could not eat the chocolate biscuits I had sent him with because it occluded the machine…… such a shame!

Nik Strapped to the machine

Nik Strapped to the machine

The machine

The machine

ready to transport to the lab for manipulation

ready to transport to the lab for manipulation

Looking good Nicholas.... ;P

Looking good Nicholas…. ;P

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Seth has had no vomits today and so far no temperatures. The doctors are concerned about the effect of the drugs he is having on his kidneys and liver, and concerned about fluid retention. So he had an ultrasound which, thankfully, showed no changes, fluid or infection. Seth is feeling unwell and so got upset when he had to have the ultrasound. He was crying, but when she went over his lower abdomen he started saying “ouch that hurts”. She said that every looked fine, so we think his skin is even more sensitive where he had his gallbladder out due to the scar and the chemo. As he was having the ultra sound all of a sudden i thought, they are going to tell me he is in kidney failure, I need to prepare myself for this. It is a total defence mechanism, because i guess at the moment, they might say that to me one day. But thankfully…..not today.

Today he has been in pain in his shoulder and left side and this seems to have been causing him breathing difficulties and a high heart rate. He has been asleep most of the day exhausted. He did not want to talk to anyone or be bothered, which when you are in pain and have been feeling rubbish for a few days I think is fair enough! He was dealing with it more admirably than I would anyway!

As such he is now being given morphine, and can have bolus’ if needed. Within half an hour of having this Seth perked up clearly happier now the pain is being managed. He stood up shortly after the morphine kicked in and shook his arm about declaring “Look Mummy! My shoulder is better!”. Nothing like a bit of pain relief to make things easier! After that he spent time playing with his fire engines and paw patrol toys and did some colouring. He got tired quickly but again I think that is acceptable considering the circumstances!!!

No matter what happens Seth continues to humble me with his strength and positive attitude. He has rough times, as I am sure he feels pretty pants. But the moment he feels a bit better he grabs it with both hands and makes the most of it. I could not feel more privileged than being able to say I am Seth’s mummy.

Seth asked about Daddy a few times today, he understood that Daddy was going to have the magic removed so that Seth could have it tomorrow and told me that before he could have it the Nurses needed to clean it. That is a close enough understanding for me! We had a conversation about Daddy’s magic needing to find a home inside Seth so it might not make him better straight away, and that the medicine that has been making him feel poorly this week might make him feel more poorly over the next week. I am not sure how much he understood that but he mentioned later that he won’t get better straight away. So hopefully he understands enough.

So as the day comes to and end, Seth’s pain is managed and he is happier. its transplant tomorrow. Lets just get it in…. we still have a long way to go and its day by day which has been proven this week where we have had something different to deal with every day. But for now, its OK.

History: Part 3 – To transplant and getting home!!!

It’s been a little while since I last posted about Seth’s history and the things that have happened up until his readmission to hospital at the beginning of February. You can read the first part here and the second part here.  So much happened during this time it is difficult to remember, but the basic over view is here – i hope!

We left off with Seth being deemed well enough to be moved from Leicester Royal Infirmary to Great Ormond Street Hospital in London. This is approximately a 3 hour drive. Because he was still on oxygen and still really quite a poorly lad we had to blue light there with two nurses in the back just incase Seth had any problems. Arriving at Great Ormond Street I was overwhelmed. This was the children’s hospital that we have all heard about (in England at least!). They fix the sickest of children. So they had a reputation to live up to. This was my first time seeing Great Ormond Street and my first time being involved with tertiary care (tertiary care means that they largely provide third-level specialist care. Primary care generally consists of frontline services such as GPs and dentists, while secondary care is offered by local hospitals. Therefore they address more specialist issues). We were on Robin ward, a ward for infectious diseases and immune deficiency. You may be thinking woah woah woah – immune compromised kiddies on the SAME ward as children with infectious disease?!?! Yes its true. But the ward is a corridor with a bunch of rooms, all with filtered air that takes out any bacteria. Prior to a patient going into a room the room is deep cleaned. The nursing staff follow strict guidelines to prevent infection between patients. So actually it makes sense that both are on such as ward as they both need isolation. Also children like Seth often carry infectious disease because of the nature of their illness.

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Robin Ward at Great Ormond Street Hospital.

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The ward is a corridor of rooms. As you can see in this picture the children have a large glass window to look out of.

Seth arrived at Great Ormond Street Hospital at the beginning of October 2010. Within a few days of arrival we had met Seth’s allocated Immunology Specialist Nurse, she would be his main contact throughout transplant organising his care alongside his consultant, Dr Wasim Qasim.  The first thing was to discuss donor options. They were approaching the bone marrow and stem cell registers world-wide to identify any potential matches. Within 11 days we were told there were 4 matches, each being a 100% match to Seth. The doctors decided to go with a cord transplant from a cord donated in Germany in 2006. The reason they chose this was because Seth was carrying virus’ in his stomach and chest and research had indicated that a cord transplant was more likely to be able to address these issues quicker than a donation from an adult marrow.

In addition to this we were told that because of the damage caused to Seth’s chest, and because he was carrying these virus’ chemotherapy would put his life at risk. Normally you have chemotherapy to prepare for a bone marrow transplant. The chemo empties the bone marrow of all cells and the new bone marrow goes in without there being any cells to fight.

Seth’s condition (x linked Severe Combined Immuno Difficiancy) means that he does not have any T cells (T cells are white blood cells, but the most important white blood cell as none of the other cells work without them). So as he has no working immune system and is missing “the management” he is in the best position to have a successful transplant without conditioning (chemotherapy).  However, the risk would be Graft Versus Host Disease which can cause significant complications and in some cases be unmanageable causing death.

In an ideal situation we would go for the conditioning and get rid of anything that Seth may have had in his bone marrow. But, unfortunately, we had no choice. He wouldn’t survive chemo and gene therapy at the time was new and thought to be causing leukaemia in some patients. So we agreed and plans were made.

Whilst this was happening Seth’s chest was getting stronger and he was becoming himself again. Because of the virus’ in his stomach he was nil by mouth and being given TPN (Total Parenteral Nutrition). So he was getting all the nutrients he needed, but was still hungry. He would go crazy when the nurses brought in his oral medicines and suck them out of the syringe despite them not tasting very well. He would also suck the water of the sponges we used to keep his mouth clean. It was hard to watch your baby being so hungry and you not being able to explain why he could not have food. However, as usual, Seth dealt with it and got on with thing most of the time  ( way better than i would have done – i get ANGRY when i have missed one meal never mind several!!).

As the days and weeks went by there were a few hiccups with Seth spiking a temperature and having kidney problems. This delayed the transplant on one occasion.

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Sucking the water out of a mouth cleansing sponge

Seth received his transplant on 5th November 2010. When you get to transplant your child goes into script isolation, where gowns have to be worn at all times, clothing and bedding boil washed daily, very strict hand washing, no drinking or eating in the room, everything Seth touched had to be sterile and only 3 named carers allowed in his room until he has neutrophils. Our named people were me, Seth’s dad and my mum. In the few weeks before the transplant we asked friends and family to wear yellow (like we have this time, however, it did not get so big last time!!!). This was really good for us as parents isolated from the people we know. It’s a way of making you feel like everyone is closer. I mentioned in my previous post (an aside) where the idea came from but i will re post here in case you did not see it.

An old friend of mine was also battling leukemia. She was a big supporter of Seth and spoke to me about the significance of the colour yellow in relation to immunity. Apparently is signifies a strong immune system.

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A few of the pictures we received on the previous wear yellow for Seth day 5th November 2010

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Below – Seth and I on transplant day in our yellow tops!

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The cord had arrived frozen and was defrosted in the lab before being brought up. Seth had three bags of cells! The process is less exciting than you expect it to be. It happens very similar to how a blood transfusion happens. However, Seth was on so many medications he was attached to his line pretty much 24 hours a day.

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The new immune system going in!!!!

The actual transplant days (he had one bag of cells a day for 3 days) we uneventful. Although it came with an overwhelming smell of canned sweetcorn! The substance used to freeze the cord blood after harvest smells of sweetcorn! So as it is given to Seth he starts to smell of sweetcorn! I swear I loved sweetcorn until that! It was overpowering at times!!!!! Seth didn’t seem to mind though.

4 days after the transplant Seth grew his first 2 teeth! All of a sudden out of no where! It was quite a surprise! On the 5th day i noticed Seth flash red, his skin went a patchy red for a few seconds and then back to normal. I called the doctor as they had stated that this was a sign of Graft Versus Host Disease, but they said it would be longer before there would be any signs of it.

Following transplant it was all a bit of a blur. The days were very much the same routine. Normally volunteers and play specialists spend time with the child playing with them helping them develop, and giving them another face for an hour. Because Seth had virus’ he was not allowed such visits. So Seth and I spent all day everyday together, which wasn’t too bad actually and i was able to get out of the room when he fell asleep for a nap or in the evening. There was an intercom system that the nurses could switch on when i left so they could hear if Seth woke up. Nik, Seth’s dad had to work so during the week he would stay at home and at the weekend he would come to visit us. He would spend the nights with Seth and I would take a break and stay on the mother’s unit (accommodation for mothers provided by GOSH). Because i had been unable to leave the room all week I would often spend time during the days walking round London ( I can now navigate our great capital pretty well on foot!). This meant that Nik and I did not spend much time together. It mattered, but at the same time it didn’t, because this point in our lives wasn’t about us. It was something we had to get through. I think because we both accepted that we got through it.

After 10 days I went out as i did everyday to check Seth’s blood results and…. FANTASTIC… he had neutrophils. They were still very low, but had started to come up – this means that the transplant had worked…to some extent at least.

Also as time went by it became apparent that Seth did have Graft Versus Host Disease in his skin. So, although he was already on IV steroids (prednisolone) this dose was increased. In addition it was time for him to start eating again. As he had been nil by mouth for 4 months his stomach was extremely sensitive. He didn’t tolerate the smallest amounts of milk for the most sensitive stomach and refused to drink milk himself. So we had to feed him slowly through a pump and a NG tube (a tube from his stomach coming out of his nose). He tolerated very slow levels (5ml of milk per hour) and even his medicines if given too quickly would cause him to vomit.

Eventually he was off TPN, tolerating enough milk and was being managed on oral medication. This means the plan was home. Hurray! After 5 months and 17 days (170 days) we were going home!!!! YES!!! Although….this is Seth…. nothing runs smooth!!

5 days before we were due to go home i looked up and he was sat in his cot shaking, so much I thought it was the start of a fit. I pressed the buzzer and the nurse came in. He was spiking a temperature. His body was shaking to get itself hot. Phew, not a fit. But, a temperature, damn it so close to going home. The doctor was sure it was an infection in his Hickman line (the line that gives them access to a large vein in his neck so he can have large volumes of medicine) so they removed it and he had no more temperatures… we got to go home!!!

Going home wasn’t straight forward. Nik had to deep clean the house to make it as clean as possible for him. As although he was going home  he still needed to live in isolation for a while. So no visitors, especially no contact with children, no going out to crowded places and when out where there may be people to be in his pram with the rain cover over him.

The next step of his journey had begun though and we were home. With the boy who doctors weren’t sure he would make it out of intensive care. The next few months were all about weekly visits to GOSH (3 hour drive from home) for check ups and monitoring, and learning to live as a family again. Nearly 6 months apart is a long time.

In mid April I had to return to work as my maternity leave ended, so we had to think about what on earth we were going to with Seth. I had to work as we needed the money, and if i did not return I would have to repay my maternity pay. We were very lucky that a close friend was able to look after Seth when I had to go into the office. This made a huge difference to us as a family. 14 months later in June 2012 he was allowed to go to a local child-minder who had only a few children. This did Seth the world of good. Up until then he had only had contact with one child and as such he did not know how to interact with children. He had been  raised around adults and nurses and so children made him anxious. They are more unpredictable than adults! An adult doesn’t take a toy off you, or push you over so this was a whole new experience to Seth. He did not know how to communicate with children and so he took to being passive. If a child took a toy from him he would let them and there would be no response from him. As a parent it was difficult to see him being completely indifferent to situations like this. He did not get angry, or upset. He just sat there.

In addition to his lack of peer-to-peer social skills Seth’s development was delayed. At the age of one he was not yet crawling. At 18 months he had begun to crawl, but on all fours (like MowglI from the Jungle Book!), and did not start walking properly until he was 3 years old. He had support in trying to walk. The main reason for his struggle was that the steroids were preventing his muscles from growing properly and his ligaments were too loose making him hyper flexible. Although he now walks confidently he still cannot run or jump, and just prior to returning to hospital continued to lack confidence outside because of his lack of stability.

The graft versus host disease continued to get stronger causing extremely dry and flaky skin, inflammation and redness to the extent that his skin begin to crack and he would have sores, his lips at times were just scabs. It was not until his skin healed recently that we realised how much this affected his smile. He now has a great big smile that was not there before.

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Showing the GVHD in his face and hands

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The skin being very dry and thickening as a result of the GVHD

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GVHD on his belly that is very distended due to steroids

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Showing the GVHD in his lips, preventing him from smiling properly – but he still does it!

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Since transplant and until January 2014 we tried to manage this with steroids, for this time he has been on a very high does of prednisolone (2mg per kilo at times) which has caused its own problems. Steroids are a necessary evil i have learned. As a result of the prolonged steroid use Seth has osteoporosis. He is very small (the size of a small 2-year-old), and has poor muscle development. The steroids reduce the effectiveness of the immune system, therefore preventing it from attacking Seth and reducing the GVHD. However, this also makes him more prone to picking up infections.

Despite this reduction of his immune system he managed quite well, although we did have hospital visits regularly (if Seth has a temperature of 37.8 or above he has to go into hospital for antibiotics and blood cultures). These hospital visits also seemed to coincide with weddings! In 2013 we had 4 weddings to visit and we only made it to one as a family, luckily that one was ours!!

Although Seth’s health has never been perfect, we have managed it along with getting him to experience things. He had missed out on so much we want him to experience what he can. He has travelled to Europe – because he cannot fly due to the extensive damaged caused to his lungs when he was in intensive care.

However, we were failing to control the GVHD and there were growing concerns about him being on steroids at a high dose for so long. They were now becoming the problem. Luckily, the Great North Children’s Hospital had begun a trial using ECP (extracorporeal photopheresis or light therapy). To have the treatment you are connected to a machine by a central line. Your blood then goes through the machine, which separates off some of your white blood cells. You then have your blood, minus the white blood cells, given back into your vein. The separated white blood cells are treated with a special drug and then exposed to ultraviolet light. This light activates the drug so that it is able to destroy the abnormal white blood cells. After this process your nurse gives back the treated white blood cells into your vein. Seth had this treatment at the Great North Children’s Hospital once a fortnight for 3 months, then once a month. Although at the time Seth’s management was at Great Ormond Street Hospital. The Great North Children’s Hospital was the only place in the country able to offer this treatment to someone Seth’s size. So once a fortnight we would travel up to Newcastle Upon Tyne (4 hour drive) and stay over night to have the treatment over two days. Seth’s skin did improve a lot, it meant that we were able to reduce his steroid from 20mg per day to 7.5 and 5mg alternate days.

This was looking BRILLIANT! Finally there might be an end in sight……….. however, in September 2014 Seth and the rest of us attended a friend’s wedding. Seth was doing amazing. For the first time ever he was confident in public like a normal boy. He got up with the band playing a toy guitar. It was amazing to see. However, at about 4am he woke up and came into our room complaining his leg was sore, we thought he had slept on it funny so allowed him to get in our bed and went back to sleep. At 7am he woke complaining it was painful again. Seth doesn’t complain. He broke his arm in 2013 (as a result of the osteoporosis) and we only knew because it was swollen and he wasn’t using it. So we knew something was wrong.

I don’t know why but i suggested deep vein thrombosis to the doctors who thought he had broken his leg. I just couldn’t see how he’d broken his bone, he had not fallen, or twisted it or anything. He had been fine. Seth was getting more and more pain in his leg which swelled to an unbelievable size. A wee man who doesn’t complain was screaming in agony when he was lifted to go to the toilet. So much so that he stopped going. Refusing to wee because it was just too painful. Eventually a ultrasound was completed and a large clot was found in his left thigh. It was a DVT. This meant that they could start him immediately on blood thinners and his leg should go down within a few days. However, when doing the CT scan and moving his leg it seemed that some of the blood clot had moved which caused Seth to have a turn. He started shaking, his temperature shot right up and he went unconscious. I felt sick, immediately i thought we were going back to intensive care. I genuinely thought that was it for him. Luckily, it wasn’t he was stabilised and then transferred from our local hospital up to Newcastle to the Great North Children’s Hospital.

For the first time ever Nik, Seth’s dad was the one going instead of me. I was 33 weeks pregnant and so not allowed to travel in the emergency ambulance with him. I have never had to watch him speed away before. I am always the one holding his hand. It was a horrible feeling not to be there for him.

That time we stayed in Newcastle for 3 weeks before returning home, luckily I stayed pregnant and had Hugo as planned in the hospital near our home. Hugo’s cord was collected at birth and frozen incase Seth would ever need another transplant. Hugo was also tested for SCID. The likelihood was that he would not have it because I am not a carrier, and he doesn’t.

In addition since August 2014 Seth has been unable to maintain his HB (red blood cell) level, so he has been having 2 to 3 blood transfusions per week, in December he also stopped maintaining his platelets (the things that make our blood clot) so he has been having transfusions of these ever two to three days. Initially it was thought that this was related to Haemolytic Anaemia (an auto immune disease were that attacks blood cells)…. however, we now know that it wasn’t this but that the Graft Versus Host Disease had begun to attack his bone marrow….leading to where we are today.