Tag Archives: Featured

Mattie fast asleep in Emma's arms

Mattie’s Spasms Return

It was about a week after Mattie’s last check-up. I was back home, my travelling temporarily suspended. We were in the middle of bath time, and Ben was still splashing about in the water. Mattie had already been been picked up and dried off. I was sheltering from a storm of splashing about when Emma poked her head into the bathroom, she had Mattie in her arms. “I just saw another one” she said. That is when we knew that the spasms had returned.

Spotting Trouble

We’d had such a good check-up, to have them return so soon was upsetting. Just seeing one spasm isn’t so bad – they are hard to spot at the moment, and the guidance we got from the hospital had been to keep an eye out for spasms which cluster, and particularly so around tired times – waking up, or just about to sleep.

We left it a few days, watching and waiting and hoping that we wouldn’t see any more, but we did. Within two days of originally spotting the spasm Emma had called the hospital.

Thinking Pragmatically

Matthew’s medication is calculated based on a formula, he is given x milligrams of Sabril (Vigabatrin) per kilogram of his body weight. Emma and I came to the conclusion that when Mattie grows, which all babies do, his dosage effectively reduces; he gets the same x milligrams of Sabril (Vigabatrin), but his body weight has increased. So it is necessary to keep an eye on the medication and to make adjustments as he grows. Given Mattie’s recent, very successful check-up we believed that it couldn’t be anything too worrying. But still it is hard to tell.

EEG Request

Mattie has not had an EEG in months.

The spasms Mattie suffers from are just a side effect of the increased electrical activity in his brain. The plan, as described to us by the doctor, is to treat the symptom in the hope that should it go away, the underlying condition, the electrical activity will also have reduced. You really wouldn’t think of using this in an engineering world and it seems odd to both Emma and I. It is even more odd when you realised that the spasms can be so subtle that they are hard to determine.

We felt that getting an EEG would help. It would give us some confidence that the medication was working, or if the spasms had returned, some measure of how ineffective the medication had become.

We were told that EEGs are only carried out when it is felt that it will answer a question. The original plan had been to not do another EEG until next year. But given our concern, we really wanted one. So when Emma called the hospital she explained our request, and the reasons why. Consequently, we were able to get Mattie booked in for one in just a couple of days time. At the same time we all agreed to up Matthew’s dosage of Sabril (Vigabatrin).

That evening we increased Mattie’s dosage from 800mg of Vigabatrin to 1000mg per day. It normally takes a couple of days for the impact of any changes in dosage to be visible, and sure enough, after a couple of days Mattie’s spasms vanished. As a precaution we planned to up his dosage to 1400mg per day.

News from the Crèche

A couple of days after we initially increased his dosage, Emma arrived home with the two boys from crèche and said “The carers in the crèche seem really happy with Mattie. I even got a comment that ‘he really seems to have come alive’”. It was great to have a third party confirm what we had noticed at home. With the increase in dosage Mattie really seemed to be a bit more alert, and he was into everything, picking things up and throwing them about. It really seemed like the increase dosage had taken effect in such a great way.

Business Trip

I really haven’t had that many work trips this year, however two seem to come almost back to back. As a result, I was out of the country during Mattie’s appointment.

The Appointment

In addition to the hastily arranged EEG Mattie had an appointment with the physiotherapy and occupational therapy nurses. Starting at 8am it was going to be a very busy day.

Mattie was up early. Emma woke him at 5 am for the trip into hospital. The EEG is conducted when Mattie is asleep. The hospital is about 40 minutes from our house and Mattie was bound to sleep in the car, then wake all ‘bright eyed and bushy tailed’. But Emma had a most cunning plan; to arrive in early and wake him in the car, give him a bottle and play with him, basically try to knacker him out before his appointment. The plan worked. One very tired baby fell fast asleep during his EEG. Unusually towards the end of the EEG, when Mattie was awake and still hooked up to the sensors the nurse administering it started shining lights into Mattie’s eyes. It didn’t seem to phase him. We didn’t hear why they were doing this, but I assume it wa s because for some children the spasms can be triggered by flashing lights.

Unfortunately we wouldn’t hear more for another two to three weeks, until the results of the EEG were shared with us.

Occupational Therapist & Physiotherapist Update

In contrast with the wait for the EEG results, we got the results from the Physiotherapist and the Occupational Therapist instantly: Great Progress.

In stark contrast to the last time Matthew visited he was now almost sitting up by himself – he was able to do it for a couple of minutes before getting tired. His “tummy time” work had also improved considerable.

The Occupational Therapist was impressed by his new found motor skills – he picked up a Cheerio with no trouble, he was passing things between hands, pinching and grasping everything within sight, all the things they were looking for at his first appointment.

Community Health Worker Check-up

I’m sure many parents who have two children do the same thing; compare how the youngest is doing with how the eldest developed. Of course you try not to, because every baby is different, but it is almost impossible not to and particularly when the youngest baby has a condition which might affect their development. So it was extremely reassuring when the community health worker came to visit.

In Ireland a community health worker will frequently check up on new born children and their families, they help provide some initial contact for parents. The community health workers also measure and track babies development, and during our latest visit Mattie was weighed, measured and recorded. At 80 cm in length (about 31 & 1/2 inches) Mattie charted at over the 100 percentile for babies his age. I think he’s going to be a tall chap. The community health worker also provided us with some feedback on how Mattie is doing. She told us not to worry too much about the fact that Mattie wasn’t sitting up by himself all the time, many babies his age can’t do that.

Mattie at the Disney Store for the first time

Mattie’s Check Up Results

Tuesday 5th November

I’d just sat down at the meeting, the first speaker had stood and was about to start talking when my phone buzzed.

I’m away from home, in Israel attending a meeting for work while Emma and Mattie are back in hospital in Dublin for Mattie’s check up. Waiting for news, when the phone buzzed I grabbed it eagerly.

The Check Up Results are In

Emma had just arrived at hospital and had texted to give me an update:

"His genetic screening came back clear - it's official :)"

This was great news. Infantile spasms can be caused by a genetic disorder. The hospital had taken a blood sample from Mattie when he was first admitted to check for this. It takes a long time to run the relevant tests. We had only just got the results back – and it was great news! It means that Mattie is officially categorised as “idiopathic”; subset of all infantile spasm suffers who happen to have a great prognosis.

30 minutes later, after Emma and Mattie had seen the Doctor, the news was even better:

"Our boy is amazing! :) they are delighted with him :) aren't concerned about the eye flutters only said if we see it in clusters or out of waking that we should be worried :) see them in 3 months and an EEG in the middle :)"

It speaks for itself really! We are delighted. He’s not out of the woods, he’s still on his medication and we have to watch him. But it does mean that the medication seems to be working! – we’ve still got to watch him just in case, but it does give us so much reassurance that his development is ok.

… Now I’m pretty sure there is a meeting going on ….

The Disney Expression

After the appointment at the hospital Emma took Mattie to the Disney store in Dublin for the very first time, his expression is brilliant:

Mattie at the Disney Store for the first time

Mattie at the Disney Store for the first time

Mattie’s Checkup is Coming

Saturday November 2nd

I should be packing bags for a work trip which starts tomorrow (Sunday) morning. Next week, when I’m away, Mattie will be returning to the hospital for a check up. Thoughts of this have trumped my ponderous packing.

Continue reading

Mattie and Emma

Mattie’s Progress – Returning Spasms & Physical Development Report

Last Night

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 Results

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

Chart of Mattie's Medication and Spasm Count (Oct 10)

Chart of Mattie’s Medication and Spasm Count (Oct 10)

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.

Learning about Learning: Myelin, Infantile Spasms

Infantile Spasms and Why…?

The interesting thing about infantile spasms (west syndrome) is that it happens to infants, not to adults (the clue was in the name). Infants do grow out of it. We also know that Infantile Spasms can have a huge impact on a child’s development. The obvious questions all revolve around “why?”:

  • Why do children grow out of it?
  • Why does it have such a huge impact on a child’s development?

Recent Research

While not covering Infantile Spasms directly, this article from the BBC details the results of research, published in the Journal of Neuroscience into language development in Toddlers.

The study which was lead by Dr Jonathan O’Muircheartaigh, from King’s College London, looked into brain formations and language development. The research showed that “myelin”, a kind of insulation that forms in the brain develops over time and by the age of 4 the myelin has effectively locked into place.

Myelin is an electrical insulator, as we grow, myelin develops around the neurons which form the brain. It prevents electrical activity from escaping from a neuron, and helps make a neuron faster at propagating an electrical signal within it.

The study indicates myelin formation stops when we reach the age of four. It also suggests that a lack of myelin, as we grow, helps us to learn and pick up new skills and abilities quicker. Without myelin new connections between neurons can form quickly, effectively signals will jump from one neuron to another. After myelin coats a neuron it will insulate it, preventing these connections from being made.

The study also suggests that there may be a link between myelin and slow language development, a key symptom of Autism.

The interruption of this process of forming new connections, with conditions like West Syndrome will have a large impact on a child’s development, since the connections which form between neurons when we are under four appear to be more difficult to change as we age. This causes learning difficulties. It also explains why Infantile Spasms can have such a large impact on development; it interrupts our neurons, during a crucial window of development, as they try to make connections between each other and form our brain.

Myelin and West Syndrome ( Infantile Spasms )

In 2007 research by Shiga University of Medical Science in Japan looked at a link between delayed development of Myelin and Crytogenic Infantile Spasms. The team in Japan concluded that while they did find evidence of delayed myelin development in 55% of the cases they looked at, they did not see a clear positive correlation between delayed myelin development and Infantile Spasms.

Ticking off the “Why-s”

From what I read it looks like myelin could be a factor, but it’s not the root cause (if a single root cause exists). While I think I’ve answered why Infantile Spasms has such a large impact, I still haven’t completely understood why babies can grow out of it. – I guess that’s a job for more reading at another time.

Mattie feeling better. With out Spasms and Keeping his head up

Mattie – Is he back to normal?

Tuesday 2nd October 2013

“Is this baby ours?” – It’s a question Emma and I asked ourselves as we lay in bed listening to Mattie in the cot beside us. It was 3am, and Mattie had been playing and giggling from 1:30am, and has no sign of slowing down… Maybe he’s hungry?

First Week on Vigabatrin 600mg / day | (54.54 mg/kg)

Mattie has made some good steady progress over the last couple of weeks. Over the first week we saw Mattie’s spasms drop off. It became very hard to tell if he was having them. Mattie’s demeanour changed, he became more excitable and interested in everything around him. We got even more giggles and laughs, it was a joy to see. I hadn’t seen any spasms at all, but Emma, who is still on maternity leave with him, was convinced that he was still having mini spasms.

Slight Spasms

The spasms have changed in nature, they’ve gone from a full on sequence of jerky movements, like we’ve recorded in our initial blog post, to something much more subtle. A head jerk, or an odd eye role, twitchy movements of hands, movements repeated for a long time. The repeated movements reminded me of watching people high on ecstasy dancing in a night club, the repeated dance steps, were like Matthew’s repeated head movements.

But eventually over time even these movements have become less frequent, and ness noticeable. To the point where we end up asking ourselves, “Was that a spasm, or has he just startled himself – like a normal baby?”.

Check-up Monday September 23rd 800mg | (72.72 mg/kg)

The plan we agreed with the doctors had always been to increase the dose of Vigabatrin on a weekly basis until it reached the point where Mattie’s spasms had disappeared. This is something both Emma and I are careful to watch, as I mentioned in a previous post the side effects of Vigabatrin include possible permanent vision loss. We used Mattie’s weekly check-ups as the opportunities to assess the need to increase the dosage. As I hadn’t seen any spasms I was reluctant to increase the dosage, but right there during his check-up he had two spasms, and they were witnessed by Emma and the nurse. This was ultimately bad news, but it was great to have someone else there to see them happen. They had become so slight that it was getting increasingly difficult to confidently spot.

Check-up on Monday September 30th (dosage / kg)

Over the course of the week since the last check-up Mattie has continued to improve. He became more alert and more crazy! – Giggling laughing, and physically a lot more active. It’s as if he’s a super charged car, which has been driven with the handbrake on, now it’s been released he’s zooming away. At his check-up on the 30th he got the all clear from the nurses and it was agreed that there was no need to increase his dosage.

It is still very hard to be sure that he is not having any more spasms. He’s so very active, we had thought his older brother Ben was active, but this is on a different scale all together. That it has made Emma and I wonder if the over activity is related to the Vigabatrin, or the Infantile Spasms themselves?

I would like to have another EEG, just to prove that the hypsarrhythmia has gone and that Mattie’s back on full form, but we had agreed with the consultant that it would not be necessary as the consultant said at the time it wouldn’t provide him with any additional information. But the more I think about it, now, the more I find that hard to understand. If the original idea had been to treat the EEG (the hypsarrhythmia) and that the removal of spasms would be an indicator that the EEG is back to normal, well then, now… when we’re not sure that the spasms have completely passed, and we’re struggling to tell, – isn’t it now a great time to check with the EEG? It is something I feel I need to raise with the consultant, just to be sure.

Wednesday 2nd October

Emma had searched the internet and learnt that the jittering and excited-ness is also a side effect of Vigabatrin. Perhaps with would explain Mattie’s excited-ness during the nights?

The Bug Strikes…

We’d just sat down in front of the TV, both boys where in bed and all was quiet in the house. When I heard it – a cough. “The cough” which changed what was to be a relaxing evening, complete with some down time to update this blog, into another all night sit up. Except this sit up was to be one most parents are familiar with. The winter vomiting bug had arrived and poor Mattie was the first in the family to come down with it.

I headed up stairs to find Mattie awake, and smiling in his cot, but all around him was a halo of vomit. Poor guy.

We’d been told that Mattie’s immune system would be compromised after taking the Prednisone, and it would take some time to recover. We’d also been told that if he gets anything like a vomiting bug that we were to not wait around like we would if it was Ben, but to get in touch straight away. So after cleaning Mattie up and bringing him downstairs I called Temple Street and spoke to the on call medical registrar. After explaining Mattie’s background she told me that the intake of fluid was important, and to try Mattie on some dioralyte. Assuming he could keep that down he would be doing well. If he was having any trouble with taking fluids that we should seek help straight away.

We started the Dioralyte that evening, giving him small drops every 10 minutes. Within an hour we had a full “exorcist” baby moment and everything came back up. However the next hour went much better and eventually Mattie fell fast asleep.

Thursday 2nd October – Docs check-up all ok, you can relax now.

The next morning Emma took Mattie in to see the GP. He got the once over and we got some reassurance that he was OK. The doctor was telling Emma that now the treatment is starting to work for his IS (touch wood) we can start to look forward to treating Mattie just like any other baby. It was great to hear this, and I guess after all of the stress of the last month it still seems a little odd to treat him normally.

Friday 3rd October – Is this the return of Infantile Spasms?

Mattie woke early after a restless night. He was still tired from the day before and his little system is probably recovering from the bug.
Emma was with Mattie, when the spasms returned. Five in a row, slight, but definitely there.

In the morning Emma called and spoke to the Neurology Registrar at Temple Street. Normally a spasm would trigger an increase the Vigabatrin dose, however the Reg suggested that we wait. See how it goes over the next day or two, was this a once off? Or is it a trend? – Only time will tell. If Mattie appears to be developing normally than there isn’t anything to worry about, yet.

Saturday 4th October

Today was a great day, Emma and I cleared out the nursery and got Matthew’s gear moved in. Tonight Mattie spent his first night in his own room. To achieve this Emma and I both needed to get stuck into all of the junk that had been deposited in the nursery. Emma’s sister had offered to look after Mattie, while Ben got to hang out with his Grandfather. It was great to get a second pair of eyes on Mattie, to get a different perspective, and apart from Mattie still recovering from his tummy bug, and his appetite being less than before there really wasn’t anything to report. . . a normal baby once more.

From speaking to other parents we’ve met via Facebook Emma discovered that often an illness will bring back some of the spasms, but this is normally due to the baby being under some increased stress because of the illness.

It was also a huge milestone for us as a family, Mattie got his first night sleep in his own room, and we got the first night in our room alone.

Sunday 6th October – More Spasms?

Matthew was just sitting in his new bath chair, in the bath. Holding him tight I reach up to wash him with a sponge, when he jumps, back straight. — Was that a spasm? The rest of the day had been normal. Nothing unusual. Mattie had slept well in his own room, and the only news to report was that his appetite still isn’t 100% – but then perhaps we’re just seeing it return to normal after the Prednisone has worn off.

But after his bath, as Emma was getting him ready for Bed Emma thought she saw two more spasms. We think we did, but it is just so hard to tell.

Researching Medical Conditions – Advice from an Expert

I recently got some advice on how to research medical conditions from a parent who has a wealth of experience in health care systems and researching illnesses.

David Prendergast is a senior researcher at Intel, although we work in different teams we share the same office. David has, unfortunately extensive knowledge of dealing with a child who has a serious medical condition. His daughter, Beth, suffered from a rare form of leukaemia. Unfortunately Beth passed away, but in the 9 months of Beth’s illness David learnt an awful lot about researching medical conditions, and how to interact with doctors, he took the time out to share his tips with me, and I wanted to write them down and share them with everyone else here. I hope these few ideas and concepts will help other parents who, like us are facing the steep learning curve required to get up to speed on infantile spasms.

 

Tips on Research

When we first heard of Matties diagnosis Emma and I panic researched. We searched the internet for Infantile Spasms and randomly read articles we found. It is something that the doctors and nurses in the hospital had told us to avoid. It is easy to get confused or to read articles of questionable reliability, and Emma and I quickly found ourselves facing two questions:
1. Why isn’t the information we’re reading matching what the doctors are telling us?
2. How do I know what articles to trust and what to avoid?

Tip: Asymmetric Data Exchange

As you’ve probably noticed doctors generally don’t appear to give you the whole story. Depending on the doctor in question it can often take us as parents, many questions before we get a sensible answer. This doesn’t happen by accident. Medical professionals are often trained to behave in this way. This is called “Asymmetric Data Exchange” where we, as parents provide the doctor with all the information we know, in return the doctors provide us with only the little bit of information that they believe that we need to know. For a parent it can be incredibly frustrating. The doctors don’t do this to be mean or to annoy us, they do it for a reason. Doctors have to speak to a wide range of people, for some people facts and figures help and make sense, but for others the same information can simply be very confusing, scary, and frightening. So doctors tend to err on the side of caution and only tell us what we need to know.

Warning: Not for everyone

These tips and tricks will help you discover all the facts and figures about infantile spasms, but first ask yourself if you really want to know? – some of the details and statistics can be concerning.

Tip: Keep notes

When researching you’ll come across lots of information, so much so that you can easily confuse multiple points. Keeping good structured notes is the best way to help prevent this confusion. It also allows you to quickly look back and remind yourself of what you’ve found or to double check a point. David mentioned that it was very easy to think that you’ve read a fact, when in reality you’re confusing two reports. This can cause all sorts of confusing when your speaking to doctors. Keeping good notes is important and will help you avoid this.

Tip: Understand the Condition

It is important not to skip the basics. Doctors apply the asymmetric data exchange principle. They may have explained the condition to you, but it is important to do your own independent reading on it too. Think of the following key questions when doing your own research:
1. How does the condition show its self?
2. What happens if the condition is not treated?
3. How is the condition diagnosed?
4. What causes the condition?
5. Are there any related conditions?

Tip: Knowing your Doctors

It is important to understand your doctors area of expertise, is this an area that they are good at, or have an interest in? Doing some reading about your doctor will help. Searching Google or Bing for your doctor’s name and the name of the hospital will usually bring up some good information. When researching your doctor keep the following questions in mind:
1. Who is the doctor in charge of your child’s treatment?
2. What is this doctor’s background?
3. Have they published any papers on the topic themselves?
4. Where did they work previously?

Tip: Understand and Map the Treatment Plans

Treatment plans are structured methods of treating a patient for a condition. They provide doctors with a rough guide on how to address a condition. Treatment plans exist for a range of conditions, including Infantile Spasms.

The plans themselves are based on known research, and national guidance into the differing treatment options. They group the differing treatments into stages, a stage is often referred to as a “line”. The first stage, or “first line” will contain treatments which have been shown to be the most effective at controlling infantile spasms, with the least severe side effects. The second line will contain treatments which have not been as successful, or have worse side effects.

Research and national guidance differs around the world, consequently treatment plans differ from country to country. Understanding what treatment plans exist and what drugs are suggested will help when speaking to the doctor. It provides us as parents with information on alternative treatments which we can suggest. It also prepares you for the treatments which might come next, assuming the current treatment doesn’t work. This is important as it ensures that we parents are prepared when the doctor starts suggesting changes to the medication.

I’ve started the process of mapping treatments for Infantile Spasms on a page on my personal blog. It’s very rough at the moment, if you could provide information on what the treatment plan in your country is like, please do, and I’ll add it to the treatment plan page.

Tip: Research Papers – Search for RCTs

By searching for research papers you may find treatments which are not listed on the treatment plans, or additional background information on the treatments which are listed. But how do you know what research is good, and what is bad? Randomised Controlled Trials (RCTs) are experiments which are run by medical researchers. In a typical RCT a patient will be randomly assigned to one of two groups, the first group will be a group receiving treatment, the second group will be a group who doesn’t receive treatment. Typically, until the end of the trail the patient will not know which group they are in. If the treatment is a new drug, then both groups could receive a tablet. The tablet’s will look identical, except only the patients in the first group will receive a tablet with a drug in it. The others, in the second group will receive a tablet with no drugs in it.

Medical researchers use RCTs because the human body has the ability to trick it’s self into getting better. Patients who receive a tablet can believe that the tablet is helping them and can even make progress, when in reality the tablet is a dummy. What’s happening is that the patient will believe that the tablet will make them better and it is actually this belief which is giving them the boost, not the tablet at all. This is often referred to as the placebo effect, RCTs account for this in their experimental structure.

Searching Google for “Infantile Spasms RCT” and “West syndrome RCT” turns up a lot of research papers.

Identifying Centres of Excellence

Researchers in any profession will pick niche areas to focus on. This will be reflected in the RCTs that are available, you’ll find a lot associated with the same university or medical establishment.

Citation Loops

How do you tell good research from poor? – look for citations. Good research papers will get “cited” (listed) in other research papers. As a very rough rule of thumb, the more citations the better the research.

Pick up the phone

If you find a bit of research which you think might help, but you’ve got a few questions about it – then pick up the phone. Each of the research papers will list the individuals who worked on it, and the organisation for which they work. David explained that most are happy to receive phone calls about their work. So pick up the phone and ring, David said that they often walk through their research again and can go through in a little more detail, or can explain in plain English what they discovered. I asked David “Don’t they get upset receiving a lot of phone calls about this stuff?”. David’s response was sobering and enlightening, he simply said “Do you really care?”.

Tip: Be wary of Wikipedia

This was counter intuitive for me, because when I’m looking for details on an IT question Wikipedia is fantastic (perhaps that is because it is mainly updated by geeks).  Remember, that Wikipedia is a crowd sourced site and particularly when dealing with media information you should not to use it as your sole source of information, but should double check what you find using other sources.

Matthew's Spasms and Medication Charted

Big-Data and Infantile Spasms

All the doctors we’ve spoken to about Mattie and his condition have commented on how little information is available. When we ask about treatments we get the same response – “we just don’t know”.

It appears to be a common refrain from doctors when speaking about Infantile Spasms. I found another blog about a family also coping with Infantile Spasms and the last entry concluded with the following lines:

The truth is I’m tired. I’m tired of hearing “we don’t know” from doctors. I’m tired of all the appointments. I’m tired of all the tests and procedures. I’m just plain exhausted in every sense of the word.

Emma found a fantastic Facebook group of parents who are all dealing with children who have infantile spasms – there are over 500 members, coupled with the blog posts and other data shared by parents which emerges from a quick Google search. There seems to be a wealth of information available from some very knowledgeable parents.

It reminded me of some of the big data discussions I had earlier on this year. I think by combing all this already existing knowledge we can produce a fairly large collection of data…. with all these cases, and all these parents, how can there possibly be “no data” ?

Before going further with this I’d need to verify the cause of the lack of data, it would be good to speak to a researcher in this field first. My ex-boss told me that an old health related project had, the company had in the past made contact with Trinity College, and with Ian Robertson. It would be great if I could follow up with him. – That’s another task to put on the to do list.

Matthew is delighted to be home.. complete with his new tooth!

Getting Matthew Home Again

Tuesday 17th September

Matthew and Ben are both sound asleep upstairs. Emma is recovering from a night spent in chair by drinking gallons of tea and catching up on the Great British Bake Off… I’ve just spent the last 5 minutes making baby bottles, and shaking them in the style of Tom Cruise from cocktail – no, there is no video of this, sorry. But next time…

…All seems right with the world. It’s great to have Matthew home….

Under pressure – pushing down on me…

It was a very long night, but Matthew is now on an additional medication for his blood pressure, which appears to be under control. Emma spent the night camping in a hospital chair beside Matthew, she didn’t get much sleep. It would be fair to say that Emma is currently doing a great Zombie impression. But it all seems to have paid off. Matthew is doing much better.

I got text messages from Emma at every blood pressure reading this morning. It was a series of steadily decreasing figures – all great news. His blood pressure continued to decrease to a relatively normal figure today.

Like wise the Doppler exam on his heart came back perfect! – Everything is spot on.

Medication Changes

The neurology team kept Mattie in until this afternoon to ensure that his blood pressure had stabilised. To get there though his whole prescription has been modified.

Accelerated Weaning

In addition to giving Mattie the blood pressure drug, the doctors have also accelerated his Prednisolone (steroid) weaning process. A side effect of Prednisolone is increased blood pressure, and Matthew has been on a lot of it. To give you some context, as an adult if we were prescribed a steroid it would be about 15mg per day. Matthew has been taking 40mg per day!

Hospitals are full of sick people – who knew?!

Another side effect of Prednisolone is a weakened immune system. Matthew will be able to catch bugs much more easily while he’s taking the drug. As one of the doctors outlined to Emma today, being in a room full of sick people while on this drug is not a good thing – we’re delighted to have Mattie back home.

Accelerate the Spasm Treatment

The goal of the steroids had been to try to address the Infantile Spasms. They haven’t completely worked – we have seen a reduction on both the severity and frequency, but the spasms are still there. With their withdrawal the neurology team have increased Mattie’s dose of Vigabatrin, the anti-epilepsy drug.

Happy Baby

Wow the “Cranky Baby Week” really has come to an end! – Emma reported that she couldn’t get Matthew to relax and sleep last night he was all smiles and giggles, playing and using his hands. – That’s something Emma and I are watching very closely. Infantile Spasms can produce developmental issues so we’re constantly watching and noting his progress. This is why both Emma and I were delighted to have a playful non-sleeping baby.

Mattie’s good mood seems to have carried through to today – he has been great craic today. Long may this continue!

Possible Spasm Reduction ???

In addition to all of this; the types of spasms that Mattie has been having have also changed over the last day. Instead of the full body jerk he originally presented with, we now have slight twitches, small jumps, and the odd weird eye movement. Over all these are less aggressive form of spasm – we’re recording them all same though – just in case.

A tooth! – A tooth! My Kingdom for a tooth on steroids!

Matthew is delighted to be home.. complete with his new tooth!

Matthew is delighted to be home.. complete with his new tooth!

It arrived! The tooth fairy has delivered. We have a tooth! – Just a small one at the front, but it is here. Wow did Mattie earn that tooth. He really seems relieved to see it arrive too..

All in all

It’s been a good day so far (touch wood).. let’s hope this continues through to the weekend….

Mattie back in hospital and not sleeping for Mummy - it's time to play!

Matthew’s Back in Hospital

Back to Hospital

Monday 16th of September

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.

Matthew's Spasms and Medication Charted

Matthew’s Spasms and Medication Charted

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

Mattie back in hospital and not sleeping for Mummy - it's time to play!

Mattie back in hospital and not sleeping for Mummy – it’s time to play!

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.

3pm (ish)

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

Mattie back in hospital and not sleeping for Mummy - it's time to play!

Mattie back in hospital and not sleeping for Mummy – it’s time to play!

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.

Mattie’s admitted

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…