Feeding time in Neurology - Mattie grabs a quick bite to eat between examinations

The EEG & Difficult Choices – Matthew and Infantile Spasms

Tuesday September 10th

The Neurology Check In - Temple Street Hosptial

The Neurology Check In – Temple Street Hosptial

Its Tuesday morning, this is a big morning. Mattie is due for his 2 week EEG. We’re hoping for good news but suspecting Mattie will need some more help to kick his Infantile Spasms to touch. It’s a disappointing thought considering all the great progress Mattie has made during his first week back home from hospital. We are worried too as from what we’ve read the next line of drugs can be particularly nasty.

The first two to three days back from hospital were hard, we had a teething and tired baby, Mattie didn’t seem much better. But towards the end of the week we saw a dramatic improvement – we suspect that it’s not quite enough to prevent further treatment.

Chart of the total number of Infantile Spasms Mattie has had since hospital. The last two days are highlighted red and green. - Is there a pattern forming.

Chart of the total number of Infantile Spasms Mattie has had since hospital. The last two days are highlighted red and green. – Is there a pattern forming.

We’ve been recording and charting Mattie’s spasms, noting everything from a lurch through to a definitive “jerk”. It was towards the end of the week, from Wednesday onwards as the spasms seemed to slow, that we saw the best improvement and the biggest leap.

Mattie's drugs for the day - Steroids and a Tummy stabiliser

Mattie’s drugs for the day – Steroids and a Tummy stabiliser

It was harder than expected to get Mattie’s dosage right. On two occasions Matthew spat up part of his steroids. The first time he did this we thought we’d let it lie – there was no way to know how much had had taken, and how much was still left to go. However when on Tuesday night, for the second night in a row he spat up we thought we should do something about it. Mattie had managed to spit up at least half of his dose; it covered his bib, so we called the hospital and asked for some advice.

We got through to the doctor on call for Neurology. The doctor advised us that we could give him another half dose. The doc also suggested mixing the diluted steroid tablets with a little flat 7-UP to take the bitterness away. The doc explained “Don’t you know, flat 7-UP is an Irish cure for everything!” Unfortunately we didn’t have 7-UP to hand, so we substituted dilatable juice. It worked wonders! – Medication delivered!

If Wednesday saw the first noticeable drop in spasms and clusters, then it was Thursday when we saw Mattie’s first big leap.

Looking back to Thursday 5th September

What a day, I worked from home today while Emma and Mattie hung out down stairs. Emma has in good spirits and with good reason too. Although Mattie had still had some spasms today he, it was as if he had a complete miracle week in a day! He was alert and bright and giggling with fun. About mid-morning I came down to the kitchen for a coffee to find Emma and Mattie both laughing away. Mattie was in his jumperoo and was bouncing! A first!

In the past we’d pop him in and he’d last 60 seconds max – before the crying would start. Now as I acted like a Muppet and was jumped up and down myself Mattie would giggle at me, then kicked his legs and start to copy my actions. It was so very good to see.

He didn’t seem to have many spasms at all today – this looks good.

Emma: Thursday was the day that I noticed the biggest change in Matthew. LOTS of smiles and giggles! He found his voice….oh my goodness the ?oohs? and the ?aahhs? and the cooing coming from him was absolutely unbelievable! In addition he actually played in his jumperoo!!! Something he has NEVER really done, he happily played in it for about 30 minutes!! He also sat very sturdily and robustly in his bumbo – again something he had never done.

Looking back to Friday 6th September

Mattie was tired and cranky today, and I think he had more spasms today than he had yesterday. Yesterday Mattie appeared to make such a leap, but today his progress seemed to have been slowed a little, and depressingly the spasms are still with him. Emma and I are pretty much living in a state of constant hope; perhaps this will be “the day” – the day when the spasms stop and we don’t see any at all. Then there is the crushing feeling when they do hit, especially after the good bit of progress he appeared to make yesterday. Objectively it is perhaps not that Mattie had a bad day, it was still better than when in the hospital, but that he didn’t make it throughout the day without a spasm – that was something I think Emma and I were both hoping for after yesterday.

There is still cause for more hope. Emma’s father came back down to visit today. It was great to see him, and it was good to get his opinion of Mattie after his break away from him. Emma’s dad commented on how much more alert and with it Mattie seemed, and he still wasn’t jumping at the sound of his voice, something he had done before being admitted to hospital – all good news.

After the disappointment of more spasms, today did manage to end on a really fantastic note. Emma has taken to reading Mattie a bed time story (when he’s able for it). Tonight the story was Love Monster. He loves this book! – This story combined with his newly found voice, made for so much noise that Emma actually recorded it on her phone. I’d highly recommend a listen.

Tuesday September 10th – The EEG

Emma giving Mattie a bottle during his 2nd EEG

Emma giving Mattie a bottle during his 2nd EEG

I sit, watching Emma give Mattie a bottle. The room is cool and dark, all the ingredients for a good nap, which is exactly what Mattie needs right now. He’s been all go this morning and the room is a welcome relief. The Neurology department is brimming with children and parents; a far cry from last week when we arrived to find a deserted department.

Preparing for today

Last night we started to prep for the EEG, just trying to read up on stuff before talking to the consultant about any further treatment, and wanting to quiz the doctors on the off chance that the EEG is OK, but the spasms remain – which is something that has been known to happen[1].

Emma has done a bucket load of fantastic research which she has started to catalogue and share. We’ve noticed a couple of things, first off each country appears to treat infantile spasms differently.

US v UK and Ireland

The treatment in both the US and the UK is to give steroids initially. However the types differ. In the USA they use ACTH which is injected into the child. In the UK and Ireland they use an orally administered synthetic steroid prednisolone. The latest research seems to show that the UK and Irish method is just as effective, if not more so, without the risks associated with injecting something. Emma’s recent research indicates that the US is progressing toward the UK / Irish norm for first line treatment.

Ireland (or maybe just Mattie) v UK

There are differences between the treatment Mattie is receiving at the treatment recommended in the British Medical Journal. Basically the BMJ suggests that after 7 days the steroid dosage is reviewed; if the spasms are continuing or an EEG shows no marked improvement, then the steroid dose is increased to its maximum for the remaining week, before tapering it off. – Mattie didn’t get this; instead he had a single flat level of steroids.

Japan

In Japan they often prescribe Pyridoxine (aka Vitamin B6). Which is interesting, because there is another blog Emma and I are following Baby Bradley and his Fight with Infantile Spasms and he too is taking B6, and has been in addition to ACTH.

Second Line Drugs

We’ve also started looking at potential second line drugs. Wow are those things strong, and wow to they have some potentially nasty side effects. One of the things Emma and I are keen to explore is, if the EEG isn’t back to 100% normal (which it shouldn’t be – given our observations) can we revisit the hormonal treatment, rather than moving on to the more aggressive and harder second line drugs?

Tuesday September 10th – EEG Progress

Removing Mathews "Digital Dreadlocks" after his EEG

Removing Mathews “Digital Dreadlocks” after his EEG

Back at the EEG, Mattie has still fast asleep when the EEG Operator who had been silent speaks:

The EEG Operator: How many spasms did he have yesterday?

Emma: Seven.

The EEG Operator: Is that the most he’s had since starting his medication?

Emma: No, the least.

The EEG operator opens the blinds, letting the mid-morning sun shine into the room:

The EEG Operator: Let’s wake up him, it will be good to see if anything unusual happens then.

Looking back to Monday 9th of September

I was in a meeting at work when the text arrived:

Bad News - More Infantile Spasms on Monday

Bad News – More Infantile Spasms on Monday

I had been hoping, so hoping that today would be the day. That he’d have one day without a spasm. But it doesn’t look like it – and that’s hard. Mattie got so lucky during his stay in hospital, he’s in the rare 30% bucket with good prognosis, there is nothing else wrong with him, we appear to have caught it early. On top of this he’s made such fantastic progress today.. everything was pointing in the right direction. It’s pretty upsetting.

Mattie has been teething like mad today too. With some tiredness and crankiness which is probably a triple combination of teething, drugs and his Infantile Spasms.

Over all during the week he’s made some amazing progress:
Emma: Something else that is VERY noticeable. We now have regular nap times again!! 🙂 Mattie will take about a 2-2.5hr nap in the mornings and a 1hr nap in the afternoon. But in between these naps he is awake and interacting! (also sometimes grouchy but thats ok). This is a HUGE difference, prior to his diagnosis even when up from naps he was dosing in his chair and his level of interaction was low. This is massively heartening to see 🙂

Tuesday September 10th – The EEG Results

After the EEG we get taken in to see the head of neurology. Sitting in his office we get the EEG results. If it was an exam we needed an “A”; we got a “C”.

Emma and I were worried that the EEG would be all clear. We’ve seen and felt Mattie continue to have spasms and a clean EEG would mean that the doctors were not getting the same picture as us. The EEG wasn’t clear.

The EEG showed a good improvement, perhaps as much as 60%/70%. But the hypsarrhythmia pattern remained present. This aligns with the spasm pattern Emma and I have witnessed.

The Risks

Infantile Spasms, by very definition, only happens in babies. The problem is that small changes to the brain now have huge impacts later on in life. The continuing hypsarrhythmia can have a huge impact. In terms of Mattie that impact can range from a slight learning problem – through to inability to sit unaided, the inability to read or do basic mathematics.

Today Emma and I have the chance to review Matthew’s progress. He had tracked the developmental milestones for his age group up until 2 weeks before his hospital admission. During these two weeks his development slowed. Last week he made huge leaps and we feel he has caught up, but just not all the way.

The Head of Neurology’s Office

We asked the head of Neurology what the outlook was for his Infantile Spasms. The conclusion was, without doing something it will not get better. It may plateau at this point, or it may reoccur. However all the time the spasms and hypsarrhythmia continues – and continues to alter his brain.

Sitting in the doctor’s office, and working with the head of Neurology we reviewed the options available to us. Importantly in the field of Infantile Spasms there is not enough data to answer many of the questions we had; in particular Emma and I had wanted to know if the steroids would work completely if we increased the dosage?
The head of neurology: There is no evidence that they would. The head of neurology recalled being an conferences where, at the same conference one person had stood on stage and said that the steroid dosage should be upped after the first week, and another had stood up to say if the steroids don’t work after the first week – forget them and move on, don’t waste the time “testing” theories.

The head of Neurology told us about Vigabatrin (also known by the brand name – Sabril), a second line drug which is used to treat Infantile Spasms. This drug has some long term, non-reversible side effects – it can be pretty nasty. In 30%-40% it causes permanent vision loss. The doctor explained that he had seen some cases without vision loss, and some who were unable to drive a car because of the vision loss. The risks to vision loss increase along with the dosage of the drug.

If Vigabatrin is prescribed and if it works Mattie will be on it for a year. Initially the dosage of Vigabatrin is low, and increases each week for four weeks until one dosage level is found which works, stopping the spasms. If no dosage level works, then we’ll drop the drug and reassess the best way forward.

I asked about Vitamin B6, “What’s with the Japanese prescribing B6?”
Well according to the doctor there are a wide range of epilepsy syndromes which benefit hugely from the introduction of B6. There however is no data to suggest that Infantile Spasms are one of them. As a vitamin supplement B6 has no significant side effects.

We distilled the options available to us:

  1. Increase the dosage of steroids
  2. Move to a second line drug Vigabatrin (Sabril)
  3. Introduce Vitamin B6

What we know:

  • Time is ticking and with every delay Mattie’s outlook deteriorates.
  • Staying on the steroids is unlikely to help.
  • Vigabatrin has the next best chance of helping – but with a significant risk.
  • Vitamin B6 has no known side effects.

This left us evaluating the outcome of Infantile Spasms with and without Vigabatrin . – With and without the benefits, and the risks. In the end we decided, along with the doctor that having Mattie on Vigabatrin – stopping the spasms was the better way to go. The potential benefits – spasm free, and reduced learning difficulties outweighed the risk to his eye sight.

It was, and still feels like a very difficult decision to make. It makes it worse knowing that we’re doing so in a field which is largely unknown, with limited concrete data to go on.

We also decided that since there is no risk to using Vitamin B6, that we should go head and use it.

Mattie’s Drug Cocktail

Cocktail of Drugs: Matthew's new perscription

Cocktail of Drugs: Matthew’s new perscription

Mattie is now ramping down on his steroids, ramping up on Vigabatrin, starting B6 and continuing his other drug to control the impact of his steroids on his tummy. The cocktail of drugs he’s on appears to be pretty hard to come by. We ended up trying 5 pharmacies before eventually getting a pharmacy which didn’t have what we needed, but could get it couriered to them before Mattie’s next scheduled dose of drugs.

Physical Care

Emma asked about physical exercises we could do to help Mattie. There is a physiotherapy clinic in Temple Street. The head of neurology explained that they don’t normally refer at this stage, but he’d be happy to – and effectively start Mattie early on this.

Keeping Positive

There are lots to be positive about. Mattie has responded well, just not well enough to the steroids. He was in a good place developmentally to start with, and we appear to have caught it early. As the consultant said, Mattie is a member of a group of people who suffer from Infantile Spasms. As such the average prognosis is not good. There is a subset of this group which do better – Mattie is in that group, but at the same time it is still a high risk group.

Fingers crossed the new drugs will work early at a low dose…

[1] “A standard EEG to evaluate interictal activity may miss the hypsarhythmia pattern, which can be variably present in an awake child, but is detected more sensitively in sleep.” – http://www.uptodate.com/contents/management-and-prognosis-of-infantile-spasms | Back.

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