The “Mummy Card”, Mattie and Infantile Spasms (Part 3)

Wednesday 28th August 2013 - 10:25pm, Home.

Emma is once again with Mattie. We got news today that his MRI is scheduled for tomorrow morning. As a young baby this will mean sedating him, to do this he needs an empty tummy.

Apparently the low quantity of the sedative they use has one of two affects, it will either put him to sleep, or it will have the opposite affect and make him go hyper and a little wacky. To give the sedative its best shot at working Mattie should be tired too. So the plan for the morning is to wake Mattie at around 3:40am, so he can have a bottle before the four hour cut off before sedatives. He will then have to be up for the day 5:30 / 6am. His MRI is around 8, but the first, preliminary and verbal results won't be available until tomorrow afternoon. The final results should come through after that.

Mattie was in a much better mood today, we got smiles and giggles from him, and at least the nurses were not taking blood samples from him today, they did that yesterday.

I had agreed with Emma that I would do a night time hospital stay with Matthew, but when we got the news that the MRI would be first thing tomorrow morning Emma, in her own words “played the Mummy card” and decided to stay. I honestly can’t blame her.

Infantile Spasms

Mattie has "Infantile Spasms". It is labelled a catastrophic neurological condition. In the majority of cases it causes mental impairment. Most children will not develop at the expected rate, and will go on to have issues in later life. Catching it early, treating it before any regression in development makes a huge difference to the eventual outlook. What Mattie has going for him is that:

  • It was caught early (6 months is relatively early)
  • His development appears to be more or less on track; Emma says the last few weeks appear to be a bit slower
  • The dermatology scan was normal and for all intents and purposes he is a healthy 6 month old

The Growing Neurology Team

The Neurology team at Temple Street is bigger than we’d realised. Along with the doctors we’ve met so far this week there are also social workers and nurses. Today we were introduced to both.

Neurological Social Worker

We met with one of the social workers; it is their job to help us to adjust to the new reality of Mattie's condition. As Mattie has a long term condition he qualifies for some additional state funding for his treatment, we've bunch of paperwork to complete for this. The social worker also took some time out to ask us how we were feeling, and coping with it all. I appreciated this; it was nice to be honest and frank with someone, and just explain what Emma and I were feeling at the time.

Emma and I both realise we ask questions about Mattie’s prognosis or outlook that it’s impossible for the doctors to predict at this stage. But knowing the condition and the data about it would really help us - as non-medical people - in understanding what we are all up against.

Emma said "We're both engineers, we like the facts that the figures, we can deal with the uncertainty, just tell us". The phrase "I'm an engineer" seemed to strike a bit of fear with the social worker replied with "Oh god. OHhh..kay". Emma went on to explain that she works on and builds medical equipment, and while not neurology equipment, getting up to speed with the condition Mattie has certainly isn't in a challenge - just tell us. I’m not sure if it was an approach they see that often. The social worker explained that each doctor has their own style and that some of them can be rather brutally honest. I think the honest truth is that Emma and I actually found that refreshing, and comforting in a weird way.

Finally the social worker recommended that we avoid Google for information as it wasn't always reliable, and tended to be rather pessimistic. – I think, especially for Emma that this advice had arrived a little too late.

Neurology Nurse

Directly after chatting to the social worker we walked from the ward up to the neurology department to talk to one of the nurses. This being the maze of old buildings which is Temple Street we needed a guide to get us there. Even the signage inside the hospital reminds you of the older Irish road signs; 'Neurology is that way' points a sign. Following its direction you reach a junction, with no more signs for Neurology. It's more a general guidance than anything you can actually navigate by.

One of the nurses met us at Neurology and we sat in a private, side room for a chat. Mattie was with us and on the way up to Neurology he started having a spasm. It was a bad one. When they hit, he can cry out. Emma and I have taken to holding him to comfort him and his whole body rocks with the spasms. He'll hold your hand and either through spasm or reflex he grips you so very hard when they come. Afterwards, when they are really bad he'll cry. Yesterday I tried to put him down on his cot when one hit. Immediately after one hit him he looked at me with such fear and panic in his eyes, that the only thing I could do is pick him up and hold him, try to sooth him through it. This was exactly what Emma was doing as we sat today talking to the Neurology Nurse.

Emboldened by our conversation with social worker, Emma's opening gambit was an explanation of our backgrounds. The "I'm an engineer" line had roughly the same effect on the nurse as it did on the social worker. "Ohhh God.". We started out by asking form background information on Mattie’s condition; can you give us some recommended reading?

The nurses’ first response was - Whatever you do, don't Google it. (whoops too late).

My recollected and paraphrased conversation with the nurse follows:

Us: So do you have any recommended reading we could do? - Is there a book?

Nurse: No. There is no book. The research is still evolving.

Us: Do you have anything we could read? - If we were going to start out researching this condition where would I start?

Nurse: Erm, Googling "Infantile Spasms for parents" (giggle).. (pause) Well I do have this, which I got from Google; Understanding Infantile Spasms

The Understanding Infantile Spasms link is one Emma had already found, I've subsequently read it too. It's pretty good, and doesn't take long to fly through. - I'd recommend it if you were interested.

The nurse did tell us that the condition “Infantile Spasms” used to be referred to as "West Syndrome"

What the EEG showed

The nurse mentioned that the EEG gives them so much information, so I asked what did it actually show?

Mattie has Infantile Spasms. The old name for this is West Syndrome. It is diagnosed by an EEG. Not just the physical presence of the spasms. If the EEG shows a "hypsarrhythmia” - then that is Infantile Spasms. Mattie’s EEG on Monday showed a modified hypsarrhythmia pattern. I asked in what way was it modified, was it more aggressive, or less? - She didn't know. Just that it was modified.

Prolonged presence of hypsarrhythmia will cause developmental delays and cognitive development issues. The purpose of the MRI is to ensure that there is no underlying physical issue for Matthew’s hypsarrhythmia. In 30% of cases no underlying cause is identified, this is known as a cryptogenic case. From the EEG the neurological team seem hopeful that the MRI will not show anything, hence boosting the chances of a successful outcome for Matthew.

Side effects of the drugs: Steroids and Immunosuppressants

Mattie is on a very large dose of Steroids. They do have some side effects. He's going to each a bucket load more, he'll put on weight, his tummy will be upset and he'll be grumpy. More importantly it can affect blood pressure and if there is diabetes in the family (which there is, from my side ) then it can trigger issues there too. A more immediate concern is the impact to Mattie’s immune system – in short it will take an absolute hammering. We've to avoid anyone with a cough / cold / dodgy tummy / chicken-pox etc. Basically anything we would have let Ben just deal with, we can't with Mattie. If he develops anything like that we need to take him to the GP straight away or take him to A&E in Temple Street.

The 6 weeks run straight over and into Emma's return from maternity leave, which we may need to extend. It also raised a question for us; will our crèche actually accept a baby with this form of epilepsy?

Matthew trying to sleep in hospital
Matthew trying to sleep in hospital

Mattie will be on the steroids for 6 weeks, 2 weeks of aggressive dosage then 4 weeks to wean him of them. His entire system will be hooked on them and basically stopping the steroids directly will affect his whole metabolism. It wouldn't be a good thing at all.

Assuming that the steroids don't work - there is an internationally agreed list of drugs which are then attempted, one at a time and at varying levels of intensity. With all possible level of intensity attempted before moving to a new drug. - This can be a long process.

Treatment - assuming no root cause

The key to treating infantile spasms is to treat the EEG. If the EEG returns to normal then spasms should also go and he'll return to normal. In the mean time we'll be doing a bucket load of EEGs.

If on the very rare chance that the Steroids do work first time, and if the MRI comes back clear and if his blood tests come back OK, then once the six week course of drugs has completed Mattie could go on almost normally with no additional treatment required. He will have continual monitoring however as a precaution. - But there are a lot of "if"s in that statement.

With all of the above comes such a huge sense of disbelieve. It's hard to believe that Mattie could be so ill - he just looks physically so well. But he's in Temple street for a reason, and while the potent ‘so far so good’ stands, and with the neurology team and everyone writing and reading this rooting for him, the published odds are not so good. It’s hard to imagine, and even harder to articulate, but if you were putting money on him to come out all clear, you'd probably lose it.

Tomorrow is going to be a long, hard tough day. The signs so far are good. But I know Emma and I are trying hard not to count our chickens yet. We'll be waiting for the final MRI results first.

PS: We're no experts; a small reminder

We're not experts in Infantile Spasms, we're learning. If you work in this field, or have been through this before and believe that some of the information in any of these stories is not completely accurate, then please - leave a comment and let me know. I'd only be too happy to correct.

Why are we sharing Mattie’s story?

Emma (@emmawoods) and I (@mcwoods) have learnt a lot about Infantile Spasms over the last week. We are by no means experts - this is just something we’re working through at the moment. What we did learn is that the prognosis for children with Infantile Spasms increases dramatically with early treatment and hence early diagnosis. Unfortunately diagnosing Infantile Spasms can be very hard. To us parents it can look like something innocuous; “he’s sleepy, his head is too heavy” to “it’s colic”. Then even once the diagnosis has been made we had a steep learning curve – what is it? What are its impacts? It was, and still is confusing. About half way through last week Emma and I both realised that if we were struggling with this, then other parents probably will do to.

We asked for some recommended reading from the hospital, and there didn’t seem to be any. The truth is in this day and age – we all google (or occasionally bing / yahoo). Our hope that is that if someone stumbles across these blog posts that they will provide them with comfort, forewarning, and hopefully some help to gain all the new knowledge they’ll need when speaking to their doctor about this.

During the course of last week I started making notes. I sat down in the evening and tried to make sense of the day’s events. With Grandparents, Godparents, family and friends all asking for updates I decided to share my notes. Just writing the notes was a cathartic process for me, but the feedback both Emma and I got from friends and family, and the positive reaction to the notes really blew us away. It was such a comfort.

I’m sharing all of these notes on this blog. I hope to have the blog up to date by the end of the week.

I’m removing the names of the doctors and the nurses, I don’t think I could / should write about them publicly without checking with them first, and of course in all the commotion last week that was the last thing on my mind.

We both hope you all find these posts useful.

Infantile Spasms and Matthew (Part 2)

Tuesday 27th of August 2013 - 8:45pm, Temple Street Children’s Hospital

Mattie is a sleep. At last. It’s great to see him at peace. The last few hours, his normal bed time, have been hard. His last sleep was around 3pm, and the ward is very noisy around his normal bed time. It’s when he is tired that his spasms hit. They are particularly common just as he is waking from a deep sleep, or is restless and can't sleep. To top it all off the steroids he's been prescribed have started to take effect, the first impact has been his increased appetite - he's eaten loads and early. Then he started teething, poor we guy. His cheeks where flying off him the nurses have been fantastic, they are very observant and right to hand whenever you need them - they are so good it’s almost spooky. But they came over and gave him some calpol. That helped a lot.

As I’m sure you all know, Mattie was admitted to hospital following a meeting with a consultant yesterday. It took Emma and I by surprise. Mattie had been experiencing spasms, he’d throw is head forward. At first we thought it was just a muscle development thing, perhaps his neck and upper body had not developed enough to keep him upright for long? But when we started to notice his eye’s rolling in his head, just before the action took place we decided to get it checked out. Emma had recorded some of the spasms in her iPhone and was able to show it to the consultant. Within 3 hours a bed was found on a ward, Mattie was admitted, a letter sent to our family GP, an EEG performed, the results analysed and he’d been started on a course of steroids, with an MRI tentatively scheduled in a few days.

It caught Emma and I on the hop; I think we both half expected the consultant to tell us we were worrying over nothing.

Emma stayed with Matthew last night. Emma's sister collected Ben from crèche and I was home in time to do bedtime with Ben before swapping; Emma's sister baby sat and I was able to visit Emma and Mattie in hospital. Emma did the first night with Matthew and then we swapped this afternoon.

After Emma left Matthew woke almost immediately, typically – just as I’d left his bedside to visit the loo! He was grumpy when I got back and was in the middle of a spasm attack.

The spasms come in waves. We’re keeping a record of them on a log sheet for the ward. This helps to see if the steroids he’s on are having an effect. They normally last about 8 minutes, during which he’d have lurching like behaviour, throwing his head forward if sitting. When lying down he’ll kick is legs into the air. They can upset him and he’ll cry during them.

Trip to Dermatology

Matthew had a trip to the dermatology department. He’s suffering from eczema, always has done. But apparently skin abnormalities can be indicators of what’s wrong with him. We walked through the maze of corridors which is the hospital. They all seemed to be heated to the same temperature as any public swimming pool (roasting), with the exception of one room – the dermatology room.

The dermatology examination, which consisted of shining a UV light (Black light for the American’s – I think) over his skin in a dark cool room, revealed one thing; Mattie loves UV lights and cool dark rooms – he was almost asleep. Nothing untoward was found. He’s a follow on appointment in 6 months’ time just to check up on him and any possible changes.

While walking back the ward we passed the Temple Street School, it’s a full Department of Education school, currently shut for the summer, it opens next week. There is no escape from homework even when you are ill. Well at least Matthew has a couple of years yet before he has to worry about that.

Neurology Visit

One of the doctors from Neurology came down to visit and asked how he was doing. I had a bunch of questions. I wanted to know what the EEG showed, was there anything additional they could infer from it? The doctor said it showed increased electrical activity. That Mattie's brain wasn't returning to a normal rhythm at all, even without the seizures. They were looking to get that 'baseline' down to normal. The steroids would do that. I asked if it was epilepsy? The doctor said 'no' but it was in the same category. I asked if it was like the traditional version, with an electrical storm over the brain which then passes from one side of the brain to the other. The doctor said no. 'It’s very complicated',  - That was a very frustrating answer, I had to try to check my response. I know its complicated, I'm trying my best to understand it. I felt myself wishing there was some recommended reading on this.

The doctor went on to explain that based on what they'd seen in the EEG they didn't expect to see anything in the MRI, but wanted to do it rule anything else out. Finally the doctor tried to reassure me that they had seen a little girl recently who was 9 months old who suffered from the same condition. The little girl had reacted well to the steroids and now was basically normal.

Visit from all of Neurology

The Doctor in charge of Mattie's case came down to visit. Along with this doctor came every other doctor from Neurology I'd ever seen before.. Wow. The group descended on the bed. I'd just put Mattie down, hoping he'd sleep - obviously that wasn't going to happen now.

Matthew asleep in Temple Street Hospital
Matthew asleep in Temple Street Hospital

The Doctor asked if everything had been explained to me. I said yes, I think so, but there is a lot to take it - it was a shock to have Mattie admitted yesterday. I'd appreciate an explanation.

The Doctor explained again what was in the EEG. That this was typical of this type of condition. Almost classic. It's something they watch out for in this age range. The pattern it showed was fairly typical. He's smack bang in the middle of what they would expect for this condition. He's 100% on that. This Doctor said it was a form of epilepsy.

The Doctor said the plan is to try to control the spasms with steroids. They try to nip it in the bud at this early age, It is treated fairly aggressively. They are concerned that continuing to let them go on will have a long term effect on his development. The steroid treatment will last for 2 weeks. No conclusions will be reached before the end of the two week period. If it is controlled within two weeks and the steroids are working then they will look to wean him off them for a following couple of weeks. Then continue to monitor. There are a team of nurses and experts on hand who can help. This includes a telephone help line.

During the conversation The Doctor asked to see the videos we have of Matthew, unfortunately they are only Emma’s phone, not mine.

At this point I had an opportunity to ask a few questions.

Infantile Spasms & Long Term

I first asked about the long term impact. Is this a condition Mattie will have to live with for the rest of his life? - With on going treatment?

The Doctor: It's too early to tell for Mattie. That will become clear over the coming months. There are generalities which I can explain, but obviously you'd like to know what's going to happen with this chap? (Point’s at Mattie).

Me: 'Yes, I would. But the generalities would help - it would be good to understand the ball park - assuming the MRI doesn't show anything.'

The Doctor : Well there are children who go on to have learning difficulties, and need assistance, who we continue to see on a repeated basis as we go forward. We do see those cases.

There are children for whom the steroids don't work, and they have learning difficulties and need additional help.

There are children for whom the steroids work very well. They may have some slow development, and learning difficulties.

We see everything in between.

Infantile Spasms & Short Term

I was keen to understand, as much from a personal planning point of view, as anything else how long Matthew will be in hospital. The Doctor explained that Mattie will be in until Friday, They don't want to keep him in beyond the weekend. The Doctor is keeping him in to ensure an appointment for the MRI. In the past the steroids were taken via injection and we’d have to train the do parents to do the injection. But now the drugs are orally administrated mixture which can be taken at home. But access to in house facilities is quicker as someone on a ward; an MRI for an outpatient takes much longer. So if it means staying in hospital for an extra day to get the MRI then The Doctor recommend we stay.

The Doctor explained that the best hope is that Mattie takes to the steroids really well, the MRI shows nothing and that we get him home soon. The impact on him going forward and developing will only become clear in the coming months.

Our Personal Observations of Mattie

I’m not sure if the steroids are helping, maybe it’s too early to say, maybe I’m seeing more because now I know what to look for...

Infantile Spasms and Matthew (Part 1)

This time last week our little boy, Matthew who is not yet six months old was admitted to hospital and Emma (my wife) and I were trying to come to terms with what had happened.

Background

I remember it like yesterday, Mattie was in my hands - almost Lion King style, and the whole family was around welcoming the new arrival to the world. Mattie’s older brother was playing in the corner of the room. As I showed my new son off to the world it happened. Holding him in my hands, his smile vanished and his head dropped forward witch such force that I clutched him to my chest to prevent him from landing on the floor. Scary, and odd. I mentioned it to my wife Emma, after discussing it we decided that at his age, about 4 months it was to be expected. After all he was only a few weeks old, perhaps his little body wasn't capable of holding his head up?

Dismissing the Fear

"Maybe it's in my head" Emma said, "but I don't think Mattie is doing as well, developmentally as Ben. I think I recall Ben doing more at this age."

Mattie was now 5 months old, and perfect, how could there be anything wrong? I thought. Mattie’s head bobs had continued and Emma had become increasingly worried about them. "I've been Googling the head bobs, they could be some form of spasms." Emma said, "The good news is that they are benign and should pass in a couple of months" .. Nothing to worry about I thought. Nothing at all, Emma was probably just getting worried about nothing.

"Chris, I've got that appointment with the doctor on Monday, can you look after Mattie that day, I'd love to go alone, it would be so much quicker." asked Emma.

Unfortunately I had a bunch of meetings on that day and couldn't take the time off. Instead Emma took Mattie with her to her doctor. It was after her check-up that she mentioned Mattie's head drops to her doctor. Emma's doctor gave us a referral to the Children's Hospital in Temple street in Dublin, we had the appointment booked with the paediatric consultant booked for one week away.

The week did not pass normally. Emma became increasingly worried about Mattie, and what he was doing. We decided to video Mattie’s behaviour and his head bobs. Sod's law we thought, we'd get the consultants appointment and Mattie will be perfect, no head bobs - at least this way we'd have one on record to show.

The Consultant

I'd let work know I would work from home on the Monday of the appointment with the consultant. I had a lot to do with a work conference scheduled in London the following week, working from home made sense. I had imagined that for Mattie's appointment that I'd only be out of the office for a couple of hours, returning directly afterwards.

Sitting in the consultants office, with Mattie on my knee we described the head bobs to him. He didn't seem really concerned. Then Emma offered him the video on her phone.

 

"They look like spasms" he said.

The consultant advised us that we needed to get an EEG to check him out. He made a phone call from his desk and found space on a ward for Matthew. He filled in the admission slip, collected together all of Matthew's notes and made asked us to wait outside while he dictated a letter to our family GP. He came out and met us, asked us to walk around to the hospital, Mattie's notes in our hands and go straight to the admissions office.

The Hospital

Temple street is like a maze. Walking from the admission office to the ward was not straight forward. Honestly if you've ever been in Temple Street you'll know it makes Hogwarts look like a walk in the park. As we pondered what direction to turn, the consultant appeared. "Hello" he shouted, "This way". He led Emma, Matthew and I to the ward. We'd nothing with us, as we were not expecting to have a hospital visit, so once we'd packed the buggy up, got the hospital tags on Matthew's arms we were off - into the Neurology department.

Neurology

With little waiting around we were whisked into a room, Matthew and Emma sat on a bed facing a camera hooked up to a PC / work station. Leads were attached to Mattie’s head and the EEG was started. Just as the EEG kicked off Matthew began spasming.

Baby Matthew having an EEG with Emma at Temple Street Children's Hospital
Baby Matthew having an EEG with Emma at Temple Street Children's Hospital

Infantile Spasms

"I'm sure you'll get the results soon." Once the EEG was complete the operator noted that "It's very unusual, but the consultant came up in person and handed me the paper work for the EEG. They don't normally do that."

The speed at which everything had happened was frightening and reassuring at the same time. We still had no idea what was really wrong with Mattie.

We moved from the EEG room into a waiting room in the Neurology department. The consultant came in to see us. He explained that Matthew had "Infantile Spasms", the EEG had shown that. He then explained that the Neurology department would be looking after Matthew from here on.

Two members of the Neurology department came in to see us and as they were explaining what was going on, Matthew had another spasm attack. It was a confusing conversation. Looking back I can't really remember what was said - just that it was cut short with the spasms, and that they were going to start Matthew on a very high dose of steroids and would return him to the ward. He'd be staying in hospital until they could arrange an MRI.

I called Emma's sister to ask her to collect our eldest little boy, Ben (not yet two himself) from his crèche, while Emma and I worked out who was staying with Matthew and was needed for a hospital stay.

This was to be the start of a very confusing week.

Getting ready for the night ahead

We decided that Emma would stay with Matthew that first night and that I would return home, get our other little boy ready for bed before heading back into the hospital with all the gear needed.

Ben knew something was up the minute I collected him. They say kids have a sixth sense and I do think that's true. We got home and the house simply felt empty.. dead quiet.. and a little weird to be honest. It was hard not to feel scared when I walked into our bedroom and saw Matthew's empty cot beside the bed.

As soon as Ben walked into the house he said "Mammy! ... Baabaa?" and walked around looking for them.