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.
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
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.
25 days post transplant already. How did that happen?!?!
So we now have confirmation that the immune cells we have been watching rise each day on the bloods ARE Nik’s, so the transplant has found the marrow ands grafted. So that is one hurdle overcome. This means that he has cells and is producing cells that are all donor. With regards to problems caused by the new cells we still have to wait on that one.
At the moment things are going really well. His skin is great and he is not needing any additional oxygen.
His hands are also looking amazing (in comparison to what they were). We are hoping that when his dressings next need changing (Friday), he won’t need the bandages anymore. It is likely his wrists will need a dressing, but hopefully he can have his fingers back!
We have been able to get him out everyday, going to the park for a walk. Before he returned to hospital this time, Seth hated being outside, he would complain he did not like the sun, would not want to be outside at all. Now he loves it and is saying he cannot wait to ride his scooter outside. So for us that is brilliant to see. Our little boy just wants to do normal everyday things.
Immunology wise the doctors are still happy with him. Sunday and Monday this week his levels dropped and it sent me into a mild panic. A chat with the doctor and a text to a friend who is also a parent on the ward soon eased my fears. Their cell count goes up and down often and twice daily bloods are necessary to monitor Seth’s red blood cells and platelet counts. But they also mean his immune system is under close monitoring, sometimes it might be too much information.
Seth is still needing regular platelet infusions, almost daily. This is again common at this stage of transplant so at the moment it is not a major concern and is being managed. The minimum level they allow his platelets to drop before they transfuse is 50.
I found this sheet from January – that is how long Seth has been without any immune system, amazing that he made it this far so well!
Above i mentioned speaking to another parent about Seth’s neutrophils. When your on a BMT ward you get to know other parents, you are all there a long time, and you are all going through a similar process. However, the reasons we are there are different and even if the diagnosis is the same, the child is different. So i would never advise people to compare their child with another, no matter how similar they seem. Speak to your doctors, use the information from other people’s experiences to help you have expectations, but be very prepared for your child’s journey to be different.
Seth’s energy levels are getting back to normal and we have had a lot of playing this week. Including some time using his bed as a mountain to run away from Daddy – dressed as a superhero – as you do!
A few days since my last update, and have there been any changes.
Well only this little thing…..
Yes – Seth has been dropped from RED isolation to Purple.
As you can see the rules on cleanliness are still strict. But we can give him kisses and don’t have to curb our arms as much. He has not been allowed outside yet because he is still on oxygen and still on pain relief 24 hours a day.
It’s good, the Neutrophils are still rising but they have slowed down a lot. We are now at 1.87. Which is 1.86 more than he has had this year!!! On Saturday blood was taken to send away to test the new cells and see if the graft is Nik’s – we hope that it is. If the cells are Seth’s or the previous graft then his marrow has rejected this transplant. However, the way things are going at the moment that is looking highly unlikely.
They are starting to get better. Now he has an anaesthetist come up when he is having his dressing changed who basically gives him Ketamine and Midazolam as needed. Last time he had quite a large amount. But it meant we could clean his hands and get some of the dead skin off lowering the risk of infection. Afterwards he was a bit out of it and at one point was laughing at me because i had 3 eyes and looked like an Alien. The hands are definitely beginning to heel. New skin is present, so its just time.
Lungs: Seth is still needing Oxygen. However, we are now weaning him off it a little each day. At his highest requirement he was on 2l of Oxygen. Now he is on 0.2 during the day and 0.4 when asleep. This is good news as it suggests that the new immune system is not currently causing problems in his lungs, and it is another step closer to getting through this.
Everything at the moment appears to be going in the right direction. Doctors are cautiously happy with his progress so far. To date he has exceeded all our expectations with his strength and his little personality is back in full swing. He even started having school lessons again this week!
But, i am sure other BMT parents will know – especially those embarking on a second or third transplant – that when things go well you want to relax but can’t. At the moment we have to wait and see everyday if anything changes. We have another 6 is weeks before we can start to relax a little because until then we worry about the new graft causing problems with his breathing, his liver, or GVHD. Even when that time has past we have until day 120 (or there about) to wait and see if Seth’s thymus is still working. If it is not although his bone marrow will be producing baby t cells, his thymus won’t be educating them so they will not work. As Seth had Graft Versus Host Disease for so long it is a worry that his Thymus may not be working. Which will mean a Thymic transplant.
Then there is the ongoing worry about him picking up an infection. Yes his immunity is better than it was but he is still quite a way below the normal range and what he has will not be working as effective as a normal immune system. These are all things that we just have to wait and see what happens day by day.
WELL NOT FOR US!!!!!!!!
I totally am putting today and yesterday down as AWESOME days. Seth has a LONG and uncertain road ahead of him. By no means is he out of the woods…..
HOWEVER, look at these numbers:
Yeah – that’s right – WE HAVE AN IMMUNE SYSTEM – and because the neutrophils are coming up it is most likely to be daddy’s magic and not the old mean and grumpy immune system from the last transplant.
These numbers are still too low for Seth to leave his bubble. But as you can see – they’re getting bigger. Slow is good as if the cells rush in they are more likely to rush about causing inflammation/Graft Versus Host Disease (bull in china shop springs to mind).
Not only do we have some cells but my boy is BACK! He is cheeky, a little obnoxious at times, sharp as you like and determined to be a normal little boy. Yesterday he was walking to and from the toilet and both days we have been playing games and doing rescues with Fireman Sam and Paw Patrol. The last two days Seth’s strength has knocked me sideways.
He still has incredibly painful hands and ulcers in his mouth, he is weak from spending so long in bed. But he is fed up of being in it. So much so that today and yesterday he didn’t want to get back in and fell asleep on his playmat on the floor (don’t worry the playmate is sterile!!!). I also got to have my first proper cuddle in 3 weeks. He is still in RED isolation so no facial touching, but a cuddle is enough. Seth gives THE BEST cuddles.
So what now? All sunshine and roses ahead? Unfortunately, no one can say. He has got through the chemo and engraftment, so far, is going well. But the next 5 or 6 weeks will be telling as to whether or not the engraftment is going to cause problems. Our main worry is the return of Graft Versus Host Disease. Having battled GVHD for nearly 4 years I kind of consider it to be part of transplant. The fact that is may not happen has not registered with me. But i am scared of it returning. The treatments for GVHD take there toll on Seth, as well as the GVHD.
So the next weeks consist of watching and waiting day by day. Watching Seth looking for any sign of GVHD which could be problems breathing (GVHD of the lungs), acute diarrhea (GVHD in the stomach) and his skin flashing red initially (skin GVHD).
The risk of infection remains, Seth has a new immune system that is not at the same level as a fully functioning immune system. But at least he has a bit now which will help him and the doctors have a better chance of managing infection.
The next positive stage – being dropped from RED isolation to PURPLE and being allowed to go out for an hour or so each day – a big step for us. A big scary step. Mixed emotions at the moment as I am scared to hope because I don’t want to get complacent and then be totally floored by hiccups or problems. However, 4 months ago I was telling myself I would probably end this year as a mother of 1. Now, I might, just might, end it as a mother of 2.
International Nurses Day today, and we as a family appreciate the nurses of the world.
We appreciate them spending 2 hours to change the dressing on Seth’s hands because it is too painful to rush.
We appreciate them taking the time to explain what they are doing and why.
We appreciate them taking the time to make sure we, the parents, are ok. (even though that is not their job).
We appreciate them dealing with traumatic events and then coming to our child and putting on a brave face.
We appreciate that if they are worried, or panicking, we don’t know about it.
We appreciate them making Seth laugh playing with him and building his trust so he is happy for them to do their work. Making this all that bit less stressful for him.
We appreciate them working constantly hard for their 12 hour shift to make sure Seth gets the drugs he needs. Seth is currently require 12 intravenous medicines per day. One of these is 24 hours a day, another 20 hours a day and quite a few are more than once a day. The shortest is half an hour but the rest are all an hour – and that is without a flush. All this with only 2 lines and a child that often needs little extras such at blood, platelets, magnesium, potassium and the like.
We appreciate them answering our seemingly never-ending questions; going away and getting us an answer they’re unsure of, and not telling us what we want to hear because that would give us false hope.
We appreciate them taking a later break because Seth’s in pain
We appreciate them taking the time to learn about a child with complex health needs and adapt their practices to keep them safe.
So thanks to all the nurses in the world. You’re doing alright!
Today we returned to our bubble – as a BMT parent it is the place you feel most safe. But mainly it is nice that we were able to come back as planned. The trial Seth was on is a 4 day trial so was always planned to end today, however, if Seth had needed intensive care he would have stayed there. It’s always nice not to need it.
The numbers in his blood (liver, kidneys, inflammation etc) are all good. They are not perfect and won’t be for a long time, but for Seth on day 11, they’re not getting worse and that is a good thing.
The hair is gone now. It starts falling out slowly and then just falls out in clumps. When it began falling out more it was long and patchy so we asked the play specialist to come and shave it off…..Seth though she had taken all his hair and made him bald! Haha – until I reminded him that the medicine would do that and i got a sigh and “o yeah”. Today we showed him what his head now looks like and he says he quite likes it! Hopefully he will also be happy when it comes back!
His skin is starting to setting, it is quite flaky at the moment and his ankles, toes, heels, armpits and neck are still sore to touch but slowly getting a little less angry each day.
His hands are still so sore and the patches where the skin had become raw have now joined up. However, we had someone from Burns and Plastics come and see him yesterday and they have recommended a non stick dressing which has some silver in it that will help prevent infection. Unfortunately there is nothing more that we can do until the graft starts taking and he starts growing back the skin. In order to help him with the pain when we took the bandages off yesterday he was given some Ketamine and Midazalam. The really helped and he was not screaming in pain like he has been the last few times. But he had to have a bit extra Ketamine, which made me a bit sad because he must have been in complete agony the previous times for him to need so much medication to help him cope. Hopefully it will start healing soon. At the moment all we can do is try to keep it clean and infection free whilst managing his pain. Luckily when it is bandaged the pain is manageable with Fentanyl which runs 24 hourly.
Over the weekend we have been having Lymphocytes showing in his white cell count. This has been analysed and shows that Seth has 1500 T cells (Gamma Delta) in his blood. These could be the cells they gave from Nik as they only gave Gamma Delta cells. Also the Neutrophils have been coming in, but then going down again (0.03 then 0.01 then 0.3 today). Were waiting now with baited breath. Lymphocytes coming in before Neutrophils could be a sign that the previous graft has come back – therefore evidencing rejection of the new cells. Or they could be Nik’s cells floating around in the blood attempting to fix the things that are inflamed in Seth’s body. We just have to wait and see what happens and keep taking it day by day
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.