12 months ago the whole family enjoyed a Christmas steam train journey. It’s a fantastic trip organised by the Irish Train Preservation Society. Importantly at the end of the trip everyone gets to meet Santa. Last year Ben, our eldest son, had an absolute blast but in the middle of all of the excitement myself and Emma took turns holding Mattie as he shook with spasms. At the time he’d only just started his Sabril, was still bloated from the steroids he’d taken and suffering from conjunctivitis. The future felt very worrying. However, this year’s trip was very different!
Over the last couple of weeks Mattie has been weaned off Sabril, and is now medication and spasm free! At his last EEG on September 23rd there was absolutely no sign of hypsarrhythmia (the EEG signature associated with infantile spasms).
The routine of giving him his medication had become so ingrained in both of us that initially it felt very weird not to dig out the syringe and mix up his drug. A mental itch, like we’d forgotten something.
On the train this year both Emma and I were stretched trying to keep tracks on both boys. I think Ben believed he was on the Polar Express - we were just worried he would try to ski down the length of the train! - of course Mattie wanted to do everything his big brother was doing too! … Fingers in this, trying to eat that, stealing toys off his big brother…
Mattie had a blast with the on board carol singers. They walked the length of train bringing Christmas joy, and in between mouthfuls of chocolate and treats Mattie would try to join in, singing in only the way a nearly two year old can!
It was such a change from last year, and so much fun.
Mattie has come a long way this year and we are all delighted - he has achieved all of the things we were unsure he would; walking, talking and such a strong and funny wee personality. It is the best Christmas present we could have asked for.
We’re also hopeful that he will have escaped the worst of the Sabril side effects; vision loss. There is defiantly no sign he has any, but we won’t know until he’s much older but even if there is some the trade-off will have certainly been worth it. In the meantime the four of us have so much to look forward to.
Next year, Mattie will have one more check-up with neurology in April. Assuming he stays spasm and hypsarrhythmia free the hospital will discharge him. So far it’s looking good!
As you can probably tell from the older blog posts when Mattie was first diagnosed both Emma and I were shocked, a bit dazed, confused, and I think it’s fair to say a little scared. During the nights sitting beside Mattie in the hospital Emma and I would do what the neurology nurses recommended we didn’t; we checked the Internet.
“Whatever you do” said the nurses, “don’t google it. The Internet is full of all the bad cases, no one likes to write about the positive outcomes.” They warned.
Perhaps maybe, in April this blog will help counter balance the current weighting on the internet. I can only hope that in the future another parent, sitting beside their little one in the middle of the night, will stumble across this blog. I hope they know that they are not alone and the blog can help allay their worries a little.
Mattie peered at me from his just over Emma’s shoulder as she carried him away from me towards his new bedroom. Just out of the bath he was wrapped in a warm towel and was pleased as punch after splashing in the water. We made eye contact and I got a huge smile, he reached out toward me his arm over Emma’s shoulder. His eyes twinkled mid smile. Then in an instant he was gone. For half a second the Mattie I knew, “My Mattie” vanished, his eyes rolled up into the back of his head. The spasms had returned. Subtle no more - it was clear they were back. It was time to make the call to the hospital and to talk about upping the dose of Vigabatrin.
It was only one spasm but after such a good run of not having any was really disheartening to see them return.
Today - Thursday
Emma took Mattie into Temple Street Children’s hospital again today, it was a scheduled trip and nothing to do with the increase in medication. Rather Mattie was in to be assessed by the physio and occupational nurses. Infantile Spasms (west syndrome) can cause regressions and slow development and this was Mattie’s progress checks by two experts who know exactly what they were looking for.
The physio nurse concentrated on assessing Mattie’s gross motor skills, and the occupational nurse checked for Mattie’s fine motor skills.
The Not so Cheery, Cheerio
At one point the nurse placed some cheerio’s on the plate in front of them. He’s in no way ready to actually pick them up - something he should be heading toward at 7 months.
The tests done, the results came back. Mattie is behind in some of his development. He needs more strength in his arms and his hands, with some additional fine motor skills in his hands in particular.
We’re lucky in that Mattie is very young and we’ve caught this so early, we’ve got the chance to do something about it. We're also lucky that Mattie isn't too far behind, according to the nurses unless you were looking at him with the same critical eye that they have, the areas he's behind on would not be immediately noticeable.
The nurses gave Emma a set of instructions and suggested exercises which can help Mattie. - The doctor has ordered more tummy time as this should help Mattie develop the muscles in his arms which he needs, when these develop more it should also assist him in his fine motor skills.
Increasing the Drugs
During the course of the day Emma noticed Mattie being ‘skittery’, and ‘jittery’ as he was when we first moved onto Vigabatrin. But after our observations yesterday the spasms are definitely back, no second guessing required. As we had planned after observing them last night Emma called the hospital. After speaking with nurses and conferring with the head of neurology, we decided to up Mattie’s dosage to 1000mg of Vigabatrin per day (90.90mg /Kg). Let’s hope this does the trick, I know Mattie could do with the break... The good news is that the neuro team have been very impressed with the way that Matte has responded to treatment overall so far. Even with this increase Mattie is still on a low dosage, and they are pretty confident that this increase should take care of it.
The house feels empty and quite. Ben is in bed sleeping and I'm typing away in the study. No one else is here. Matthew is back in hospital and Emma is by his side. All down to a routine check-up, that wasn't that routine, and a small factor which started at his check-up last week...
Tuesday 10th of September (Check-up Last Week)
During Mattie’s check-up he had his blood pressure taken. It was 118/70, high and the nurse and doctor seemed concerned. It appeared higher than normal in the hospital when he was last admitted however this time it didn't seem much of a concern, so little of a concern in fact that I didn't mention it in my last note.
Spike of Spasms
Confusingly the start of the Vigabatrin seems to have resulted in Mattie having more Spasms, not less.
The Vigabatrin comes in sachets of 500mg, we’ve got to give him two doses of 200mg. Tuesday night we got our dilution calculations wrong and gave him 20% (1 fifth) and not 40% (2 fifths) we should have done. We corrected that for the next dose on Wednesday.
At the same time that we introduced the Vigabatrin we reduced the steroids. The increasing in spasms has gotten Emma and I both quite concerned; the steroid reduction wasn't significant enough to have resulted in the rise of spasms, so why has it happened?
Cranky Baby Week
The change in medication also heralded the start of "Cranky Baby Week". Ohh my word... Mattie would cry and scream almost constantly. As soon as we put him down he would start to cry. The crying sometimes marked the start of spasm cluster. With Ben, as I guess with a lot of babies, once we knew he was OK and that nothing was wrong we'd let him cry it out. But for Mattie with his spasms, we felt that that really wasn't fair.
To top it all off Matthew has continued to teeth like mad, with bright flushed cheeks, and his fingers almost constantly wedged into his mouth. Dummies last about 5 minutes before being spat out and the crying returns.
The crankiness continued from Wednesday all the way through to Saturday morning - none stop. Emma is a rock star and kept a brave face through it all but it really wasn't fun. - This really was Teething on Steroids.
Worried About Spasms
By Thursday afternoon Emma and I had grown very concerned about the increase in spasms, so much so that we decided to get in touch with the hospital.
Spasms Return in Intensity
Up to this point we had seen a drop in the spasms. The strong, intense spasms we saw before going to the hospital had vanished; they had been reduced to a spasm which looked more like a little startle, and we’re getting hard to spot. That was until the new treatment started.
By 10 am on Thursday morning we had noticed several individual (not clusters) of spasms, more than we saw before the change in medication. They were "full on" intense spasms, like the ones we saw before his medication started.
Could this rise in spasms be related to the Vigabatrin? Is it too early to tell?
11:21 AM - We’re worried enough to call Temple Street.
I got through to a registrar who works for another lead doctor, not the head of neurology we had been seeing. I explained what we’ve seen so far and the doctor on the phone explained that, that was not what they would expect to see. She is going to talk to someone on our doctor’s team. The doctor on the phone took down my telephone number, and thanked me for calling to let them know. Now we’ve just got to wait and see.
Got a call from a registrar from our normal doctor’s team in Temple Street. The long and short of it is that the new drug won’t cause the spasms. It seems that the steroids were not as effective as we hoped. The presence of spasms now suggests that the spike is coincidental. The reduction in the steroids so far isn’t enough to have caused the spasms to return either.
Trying to Introduce Normality
The spasms and grumpiness continued right through Friday and only let up a little on Saturday when we did a family trip to the zoo. It was great to get out of the house and try to do something normal as a family.
I think that Independently Emma and I had reached the same point. For me it came while washing and sterilising bottles, I looked down into the sink and realised I wasn't sterilising bottles any more - I was sterilising the syringes we use to give Mattie his medication. It was a very depressing though, I never thought I'd be putting more syringes in the steriliser than baby bottles. I found this upsetting. After feeling sorry for myself for at least a good 30 minutes I decided to give myself a good, mental kick up the arse. Being upset about it wasn't going to help anyone. With Ben's birthday coming up there was plenty to look forward to.
The trip to the zoo was a welcome reminder of normality. The trip also appears to have been a watershed moment for Mattie; his grumpiness seemed to reduce after this.
Small reductions in spasms and grumpiness continued over the weekend and by the time Mattie arrived at the hospital on Monday morning he was all smiles and giggles.
Emma and I were both looking forward to chatting with someone face to face about Mattie's spike in spasms and what has been causing it.
Monday 16th September
When Emma arrived at Template Street for the check-up she discovered that the consultant we are seeing was out on vacation and the registrar that we had been seeing was also out.
Emma and Mattie were only in for a check-up by one of the nurses so that wasn't a big deal. During the check-up the nurse took Mattie's blood pressure 4 times. It was high, very high - at one point 140/90. To put that in context a baby's systolic BP (the top figure) should be (usually) somewhere between 70 - 90. The nurse asked Emma to stay close to the hospital while she got hold of a doctor to check out Mattie’s stats.
Emma had planned to go out shopping for Ben's birthday in town anyway, so this worked well. The nurse said that they may want to see Mattie again after she had spoken to a doctor.
After about a week of a really, extremely cranky baby Mattie seemed to be in a much better mood today. He was playing and “goo-ing”.
The nurse explained to Emma that a side effect of the steroid (Prednisolone) is agitation and restlessness (cranky baby). But this side effect doesn’t hit straight away, it arrives after about two weeks of starting the medication. Apparently it catches a lot of parents by surprise. It’s nice to see that it has eased off a little.
Emma called me over lunch
I've just heard back from the hospital. They want me to bring Mattie back in. The nurse said that we should be prepared - they might need to admit him for observation while his blood pressure is so high, They want to see if his blood pressure as settled since we've been out and about.
After returning to the hospital Mattie had his blood pressure checked again, it was still high, too high. Mattie was admitted and given medication to bring his blood pressure back down to normal. Additionally the doctors are looking and speeding up weaning process to get him off the Prednisolone (steroids) more quickly than we had originally expected. Then, just to confirm everything is OK Mattie is scheduled for a Doppler exam on his heart tomorrow. The Doppler exam should tell us if there has been any damage to his heart from the high blood pressure, of if, there is anything wrong with the blood flow which might be causing the higher blood pressure. The doctor's aren't expecting to find anything. I hope they don't either...