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+5

So I would like to start with my opinion on chemotherapy…..It’s a BITCH (sorry mum).

I should probably expand on that statement a little. Chemo in our situation is a necessary evil, and it is EVIL. Looking back at Seth’s first transplant we made the decision we did because, well we were told that he would not survive chemotherapy. It got us this far. But the reality is that if Seth would have been well enough for chemotherapy in the first place, we may not be here now. So we are here and we have used chemotherapy this time to try to get a better graft and a cure. It’s necessary, absolutely and I do not regret the choices we have made.

But,

My baby boy is so unwell with it. His mouth is full of bleeding ulcers, he’s violently wrenching and vomiting bringing up bile as he cannot tolerate anything in his stomach other than 6ml an hour of Dioralyte (and he has to have 2 hour breaks every 4 hours).

They are also concerned that he has VOD (Veno-Occlusive Disease) another chemo side effect. It can be life threatening. However, Seth was on the treatment from the first day of transplant because of worries about his liver, so hopefully it will not develop into a severe form.

Then there is his skin. His poor skin. It looks like sunburn and apparently also feels like sunburn, so its painful, especially when he is touched. In parts the skin is a bit worse and sores have developed. His hands are the worst part, which we knew was likely to happen because of the previous damage to them as a result of the Graft Versus Host Disease. They are completely raw in places and oozing a bit. We have special none stick dressing on them and then they are bandages to stop him picking. But when we change the bandages just the air hitting them makes him cry in pain. We also have to soak them in warm water with antiseptic lotion in and he is so scared of putting his hands in I have to force them in the water. He just looks at me sobbing, it makes me feel so bad but we can not risk his hands getting any worse.

my baby’s poor hands

In addition to this he is still retaining fluid and it seems to be going to his genitals so they are very swollen, this in addition to the sore skin makes it a very tender area.

So we have all that going on with the chemo, and he is also still spiking temperatures and has a high CRP (inflammation marker in the blood) so the doctors are concerns about a fungal chest infection. He is also needing more and more oxygen each day to maintain an acceptable O2 level. This may be partially down to the chemo (which is likely to have gone to the already damaged area in his lungs), but also likely to be related to an infection. As a result his antibiotics have been changed and his oxygen requirement is being closely monitored.

He is now getting Buffy Coats again (neutrophils that go in, attack and then die) so hopefully this will help him deal with any infection alongside the antibiotics until the graft takes and he starts to get his own protection. Hopefully this will be the beginning of next week, but it may take longer. There is no way of telling we just have to wait and see the blood results each day waiting for the 0 to change to 0.01 or higher and then watch is hopefully keep climbing. As that number climbs we should see the side effects of the chemo start to reduce and we should start to get our little man back.

His Kidney’s continue to be a concern and are being very closely monitored. He has had an ultrasound today to check his liver, kidneys and stomach and although there are changes they are not significantly concerning at the moment, but they need to be closely monitored.

In himself he is tired, and lethargic, which is understandable considering everything that is going on. He is so good with everything that has to happen, dressing changes, creams, baths,all sorts of things keeping him awake and that are, currently, very painful. We keep saying how brave he is. But I don’t like saying it. I don’t want him to think he has to be brave, or do anything that we want just because we want him to. I want him to be pain-free and a normal 5-year-old. I want him to have a tantrum because he has to eat his greens or because he can’t watch his programme all day long, I want him to run around and have to be told off for running into his brother, I just want him to be able to go home.

So right now, we do what we can to make that happen. We bathe him, put his creams on, keep his hands clean, change his bedding, give him medicines, make sure any changes are discussed with the doctors and nurses and we just keep going. We make sure we are there so that when he wants to read a book, or play a game he can, and we keep taking it day by day.

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.

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.

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.

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.

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

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.

cuddles

kisses for the cow whilst practicing sitting again

he’s getting better…really!!!

peekaboo

playtime

bathtime in intensive care

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!!!

Having cuddles with the nurse

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.

The book

First entry

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