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!

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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+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.

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.

Today: The process has begun….

Before I really start talking about Seth’s progress I wanted to thank everyone for their kind words and support and for sharing Seth’s video. It means a lot to Seth’s dad and I. I have had a few questions about donations and the wear yellow day. I will continue to respond to all of your questions as quickly as i can,  but I thought it would be helpful to post some links here for easy reference.

First of all i would like to highlight that our aim with #wearyellowforseth is to raise awareness and show Seth he is not alone in this.

Links I have been asked to share:

http://www.bubblefoundation.org.uk.

http://gofundme.com/SupportSeth

I have also had a number of friend requests. Unfortunately I am not in a position to add them as my Facebook is my personal page. If you would like to add yourself to this event:https://www.facebook.com/pages/Wearyellowforseth/946709205361894?skip_nax_wizard=true&ref_type=pages_feed. Updates will be posted here and you can also share you yellow pics on here too!

Now to the boy in question!

This week we have been told that Nik, Seth’s dad, will be the donor. We are both an equal match. However, I carry a virus called CMV (most healthy people don’t even know they have had it or may suffer a sore throat). Which stays in your system after you’ve had it like chicken pox. About 80% of the population is assumed to carry it, but for someone like Seth it can be quite dangerous.

ATG started this week and as it will wipe the T cells and put him more at risk.

 We were allowed to take him into the interview room on the ward to be able to have a family photo. We did not have a picture of all 4 of us (other than one taken at halloween where we were all zombies covered in face paint!) and I felt like I needed to get one incase we don’t get the chance again. Some people may consider this to be pessimistic. But I consider it to be realistic. Seth is not guaranteed to get through the next 4 months. A bone marrow transplant is a dangerous risky procedure when you don’t have all the complications Seth has. Seth’s consultant has been very careful to remind us of this. So i just wanted to make sure it was done!

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not the most fabulous family photo…..but we have one!!!

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and a cheeky cuddle with his brother

I came home on mothers day as I have to have a root canal (o joy to the WORLD!) so we’d planned to do it on the Saturday. Unfortunately, it seems i have been a bit tired and run down as Saturday morning i develop a cold sore. This means I can’t have direct contact with Seth. I was allowed into the entrance part of his room to see him through the window. But he was sat crying. So i went to get nik. The doctors saw we were a bit upset so allowed me to go in and see him if i wore a face mask and was only in there briefly. So he got to give me the things he’d been making with the play specialist and have a quick cuddle.

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This last week,  Seth has remained well in himself. However, he doesn’t like things to run entirely smooth! So he started the ATG drug on Monday of this week, which kills all off the T cells which have been causing all the problems and killing his immune system (making him have less immunity than he did before the initial transplant). When having this drug it is common for people to have a response, usually a temperature spike so it is given with Piriton and hydrocortisone cover. Seth managed it fine though with no temperatures! He did has a slight temperature increase on the Thursday evening but it came back down on its own within half an hour. However, on two occasions I went to pick him up and he had a wet right armpit…just one which i found strange. The first time i had picked him up and took him of the toilet so i thought i must have had a wet hand from washing them. The second time there was no water in sight and i realised his Apheresis line must be leaking medicine.

The Apheresis line, where it is placed and how it works.

The Apheresis line, where it is placed and how it works.

This is a problem for two reasons. 1. If it has a hole or has been displaced by being pulled then it is a site where infection can get into his body. 2. It is needed for the antibiotics Seth needs to help keep him well and protect him from infection, the blood he needs as he cannot maintain his red blood cells or platelets and so they can give him neutrophils. Because they could not risk infection they had to stop losing the line immediately and do an x ray using dye through the line. This could not be done straight away. So a cannula had to be fitted. Seth hates this. They use numbing cream on the skin, but his veins are weak from years of having to have them so it always takes a few attempts to get them in. He cries so much, but never moves. He’s really so good with it. Once fitted he will not use the limb it is in. This time it was the foot (after 3 attempts in other places), so he would not walk with it in.

So far the ATG has done what it should and he has no lymphocytes (T cells or B cells). There is a chance that his own neutrophils may come back, but this would be a surprise and so far (4 days after ATG has finished he still has none. So at present he had no immunity of his own at all and is entirely reliant on the buffy coat (neutrophils given by infusion that die quickly) and the anti biotic/anti fungal/anti viral medication he is on.

All the medication that Seth is on takes it toll on his body and he now has very loose diahorrea (sorry if you’re eating!). He manages it well, getting to the toilet and he doesn’t appear to get any stomach cramps so that’s good!

Seth has been on Prednisolone IV or Oral since his original transplant in Nov 2010. (see previous blog entry here).

Seth’s getting a little bored in his room and is become more and more like a coiled spring waiting to pounce….I’m not sure how he will cope with another 3 or 4 months in here!!!

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This is the entrance to Seth’s room. Before anyone enters we have to scrub our hands and wear a gown plastic apron.

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School and Seth’s art work on the wall

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He’s asked to go home a couple of times this week, but accepts it when he’s told he can’t. He already knows but I think just likes to mention it every so often.  This week however he has discovered Teenage Mutant Ninja Turtles….so we get a break from Toy Story 3 and Paw Patrol!!!!

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The history part 2: it all gets a bit grim….or does it?!

At the end of the last history we had just been given the news that Seth had SCID. How do you respond to that? Well if I’m honest I didn’t know what to think. I’d never heard of immune problems, nevermind not having an immune system at all, and the consultant said it just needs a bone marrow transplant….easy right?!

They will fix the infection, and send him off to Great Ormond Street who will give him the bone marrow and we’re sorted right. Oh wait a minute…this is what I’ve heard about time an again since being a child – no match for bone marrow, and people doing massive world-wide campaigns looking for a match?

But whilst all this is going through my mind, there’s a feeling of relief – I’m not a rubbish mum who can’t cope with a simple baby! However selfish it may seem, I felt it. Now we know what it is we can do something. Hurrah! So first things first, he needs to get better.

At the time he was on an oscillator. This is a huge frequency type of ventilation, so effectively it uses little puffs to breathe for the patient, keeping their lungs open (give it a try quick short breaths you will feel that your lungs don’t fully close). More conventional ventilation requires the lung to open and close, therefore requiring more work. Seth wasn’t coping with normal ventilation, so oscillation it was.

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Nappies on his hands and feet to get them warm, all the medicines he was on, and the oscillator

They wheeled out what can only be described as a washing machine from the 70s, and they confirmed that in fact it was from the 70s. It sounded like a steam train sat in a station….but it worked! Within 24 hours of being on it Seth was coping, he was maintaining an acceptable oxygen level and his heart rate and pulse had improved – he was more relaxed. After this first week my mum went home as she had to return to work, and Nik also had to go back to work, He visited straight from work until 11pm but the rest of the time I was on my own, watching Seth’s monitor, doing his cares (wiping the inside of his mouth with a wet sponge, putting his eye drops in), and waiting for daily bloods to see what his infection marker was doing. He was improving.

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Every morning the consultant did their rounds and parents had to leave, but they let me stay and be part of the discussion about Seth’s progress. They’d always ask me how he’d been over he last 24 hours. On the Monday morning he was moved back onto conventional ventilation. But he struggled, he had to have very high pressures to keep his oxygen levels up. But in the afternoon the next day (1pm- it’s funny what seemingly irrelevant details you remember!) they felt he was well enough to be taken off ventilation. They warned me that it may not work and he will need assistance from other measures. He did need other measures, he couldn’t manage on his own at all and was put on a type of ventilation CPAP (continuous positive airway pressure) which uses low pressure oxygen to keep open the airways making breathing easier. I hated it. He hated it. The docs and nurses were saying it can take time for him to get used to it but I struggled to watch him, he was struggling, he wouldn’t settle, he was making this whimpering noise. I hated it.

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Off the ventilation, struggling to breathe and the bottom pic is cpap (ugh)

I got to hold him, but only for 2 minutes as he struggled. Nik came as usual that evening and he got to hold him, but for longer. I felt so jealous! It’s ridiculous but I was desperate to hold him. I had to leave and come back later. The nurse had noticed and let me hold him again, so I felt bad… It was silly really I’d been with him the whole time.

I made Nik stay with me that night, he wanted to go home – it was fair he did have work in the morning, but I made him stay. I’m glad I did, at 3am there was a knock at the door. Our room was across from intensive care. It was a nurse. When your partner opens the door at that time to a nurse with a sad-looking face when your baby is in intensive care, you don’t tend to think all is well…and it wasn’t. He’d failed and had to be resuscitated again, they’d struggled to get him stable, again, and he was back on ventilation. We went to see him and the faithful chugging of the oscillator had returned. It was comforting. In my mind he was safe. The next day was another difficult one, he had to have three chest drains fitted throughout the day because his lungs kept puncturing, and when the evening came, he had two fits. This was harder than I thought. I’d coped up until now. But I was alone again, and he wasn’t getting better. I struggled this week and withdrew. Seth went for a CT scan on his brain which showed he’d had a stroke and was left we two small bleeds on the brain, and his lungs kept puncturing so he now has 5 chest drains. It got to the weekend and he was still struggling, Nik was there and I didn’t want to go and see him. I thought he was dying and I didn’t know what to do. The ward sister came to see me and said a few things but I remember she said “we don’t know where we’re going with Seth right now, but he’s still here and we still have options”. I cried. I slept. and then I was ok again.

Obviously I wasn’t happy or joyous…but I was back coping. It was as if Seth knew…he then started to improve everyday. The nurses would send me out for a walk for an hour a day. It helped, but sometimes i would become a bit of what i refer to as a ‘pram kicker’ (internally of course – i didn’t go round sticking peoples prams, I’m sure that would be at the very least frowned upon! it means that i would momentarily resent people wandering round the shops with their healthy children) Small steps and sometimes he took a few back wards steps, but the overall trend was in the right direction. Another 2 weeks later they took him off the ventilator again and brought him out of the coma. He coped this time – and didn’t need CPAP! He needed oxygen of course but not the horrible CPAP.

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04-09-2010 (6 months old) Tube out for the second time….still poorly but managing

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As he started to recover his personality came out. He charmed every one of the nurses- who incidentally were brilliant the whole time we were there.

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cuddles

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kisses for the cow whilst practicing sitting again

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he’s getting better…really!!!

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peekaboo

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playtime

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bathtime in intensive care

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ugh this is hard!

They learned about SCID so they could answer our questions. One day I was ill so had to stay away from Seth. He was on his own. The next day they showed me LOADS of pictures of them playing with him all day!!!

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Having cuddles with the nurse

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20-09-2010 (7 months old) Learning to sit up again

Also everyday that he was there they filled in a diary about his day (see pics below) it was amazing and although it was an awful time we really like having it. It is written to him so he can understand when he is older.

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The book

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First entry

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6 weeks after first being admitted he was ready to be transferred to Great Ormond Street. He was still very poorly so we had to have an emergency ambulance there. But we were going to the mighty great ormond street! They fix the unfixable. It’s all going to be fine!

Getting ready to go to GOSH, saying goodbye to daddy

Getting ready to go to GOSH, saying goodbye to daddy

The history Part 1: A baby was born!

How did we get here…..i’m not going to harp on about the background to our family and Seth’s illness. I will keep it as short and interesting as I can….but its complicated…..so bare with me!

Seth was born in Feb 2010 on the day before his due date. The labour was short for a first labour (7 hours) and unremarkable. He was here. The baby that had been a surprise to me and his daddy had arrived. This perfect little bundle weighing 6lb 14oz. The first night he fed like a trooper keeping me awake whilst daddy went home. We were allowed to go home the next day after he had been through the standard newborn checks. All was well.

Just a few days old. All running smoothly!

Just a few days old. All running smoothly!

For the first 2 weeks everything went smoothly. I was breast feeding and he was doing well with it. He didn’t lose weight just started piling on the ounces straight away. Easy!!!…..until the crying….o my god the crying! Colic. Any parent who has had a child with colic will attempt to tell you how the constant crying makes your ears, brain and heart hurt. This coupled with lack of sleep makes for an unforgettable experience. Probably not the ones all the baby books are describing…but unforgettable all the same!

At around the same time Seth began to vomit his milk, The health visitor suggested a milk intolerance but as he was breast fed it was unlikely that there was a lot of lactose in the milk and instead considered it to be reflux. So we started with the infant gaviscon. This vomit was projectile, and generally all down me (and on one occasion all over my laptop!). It flew out of his mouth at great speed and in large amounts.

He was also constipated. Something most new mothers worry about as their baby may not poo every day. But then normally you will experience what is known to me as a ‘poonami’.  A flow of yellow mousse which often spill out of every possibly gap in the nappy exploring up the babies back and down their legs, staining their perfectly cute brand new (and probably pale coloured) vest and sleep suit. O the joy. However, this is not constipation, this is just a baby. Seth was actually consitpated. Not pooing, and being really uncomfortable with it, pulling his legs up, crying, trying to go and nothing coming out. When he did eventually go a hard yellow pellet would pass. The first doctor was not convinced and advised us to try baby massage. However, the second doctor soon prescribed lactulose when he managed to go through the motions in her office and she saw the extent of his discomfort.

At this stage Seth is a mighty 5 weeks old, the doctors had prescribed him lactose free formula and i had to stop feeding him myself as he was too sensitive for even breast milk. So much for breast is best!!! The next couple of weeks he began to poo, with a large amount of lactulose, but continued to vomit. His milk was changed again, this time to a protein free milk prescribed by the GP. His weight gain started to slow and he was beginning to fall down the centiles, and by 10 weeks old his poo had changed to be very loose and extremely smelly. I don’t think my husband or i will ever be able to eat hollandaise sauce again!

Because of all of this Seth was admitted to the local hospital at which he was tested for various things, including his immune system (just not all of it). I remember being really worried he had leukaemia. They had tested his neutrophils (white blood cells) which he had. But didn’t test lymphocytes, which he had none of (lymphocytes are made up of T and B cells. T cells are like the management – they tell the immune system what to attack. B cells are your anti bodies, they fight the infection but only if T cells tell them too). Their main concern was his lack of weight gain and they wanted to force feed him through an NG tube. But he had not poo’d in 4 days. They gave him a suppository, which worked and then he ate. So was allowed to go home. 

So this went on. We also noticed that his belly button had failed to heal properly, it looked like raw meat (ugh). But again doctors weren’t concerned. I was told to stop taking him to be weighed every week and that there was nothing wrong with my baby. By this stage I think everyone thought I was an over worried first time mum, and too be honest I thought it too. Where was the magic that everyone goes on about?!!! I thought. I was just not cut out for it.

When he was 5 months old he coughed up a bit of what I thought looked like phlegm, so i took him to the GP who listened to his chest and said there was nothing wrong with him. 3 days later i noticed he had swollen genitals so we took him to A&E (as it was midnight, and i had, had enough of being fobbed of that there was nothing wrong). We were left waiting for 5 hours in the waiting room before he was seen. The doctor said he was worried about him, but did not admit him. The nurse took his vitals, but couldn’t find a O2 monitor for a child so looked at him and said he looked fine to her (at the time i had no clue as to the significance of a SATs monitor and O2 levels).

One week later, after a particularly hard week of winging and crying and being difficult Seth had a temperature of 37.4. We took him to the out of hours doctor (it was a Sunday) and upon arrival the doctor was concerned and checked his O2 level which was 72. He immediately put him on oxygen and then phoned an ambulance. On the way to the ambulance we had to take the O2 mask off him, he went blue.

Then its all a bit of a blur time wise, but over the next few hours Seth struggled to breathe more and more and the hospital struggled to maintain his SATs. At one point his level went down to 22, and he went blue. Alarms went off and doctors came running in from god knows where. They revived him and took my baby away to be intubated. He was being transferred to intensive care at the children hospital 50 minutes away. We were allowed to see him a few hours later when the intensive care team arrived to transfer him. He was in a forced coma. We were told not to follow the ambulance and that we would be able to see him when we arrived.

When we arrived, Seth was not stable enough for us to see him until 2 hours later. We got to see him for a minute before we had to leave again as they were struggling to get a line in to take bloods and give medicine. The consultant said to me “you have a very sick boy, he is just holding on”. He made me feel like it was my fault. That he had a chest infection that I had not tried to get treated. 4 days later, Seth was still fighting (i will talk about this in my next post), but we were told tests were being done on his immunity. The nurse told me “you don’t want it to be that”. The next day we were pulled into a meeting with a consultant, an immunology consultant who had tears in his eyes when he told us that our boy had Severe Combined Immune Difficiancy and would not live to his 2nd birthday if he did not have a bone marrow transplant.

Just 4 days before he was admitted to intensive care and had to be resuscitated.

4 days before he was admitted to intensive care and had to be resuscitated.