And tho it came to be that this day shall ever more be known as Mince Pie Day!
We all have foods we love and for me it is the humble mince pie. The smell of the mincemeat, the stickiness of the substance when it escapes the pastry and caramelises on the outside of the case are all reminders of the most awesome mince pies that my grandmother used to make. They evoke memories of Christmas past and time spent cooking with my Gran.
An abiding memory is the care pack of mince pies that my Grandmother sent to me when I was in my second year of university. A bloody great big box arrived, quite nicely battered by the Royal Mail, and inside where four dozen mince pies. I have to report that there were casualties, of the four dozen at least a dozen had succumbed to “transit torture”. But no matter; with pies these good even the crumbs were delicious! I safely stored then in the student kitchen.
Granny Scregg - The Most Awesome of Students
Now my grandmother was awesome, although she had never been to university herself she seemed to grasp the core essence of the experience. As I bit into my first pie I realised she’d laced it with enough scotch that it was making my mouth feel pickled. It made for the most awesome breakfast treat; screw the American beagle, or the French croissant this was the breakfast of champions; the great British mince pie! - with added fortitude by Granny Scregg.
Just as surely as the sticky insides of a good mince will leak out during the cooking process, news about my seasonal breakfast of alcoholic wonders got out.
The first I heard of this information leak was discovering that supplies of my carefully rationed mince pies had taken an unexpected hit; the box was half full. An inquisition was held and it was discovered that these pies had international appeal! - I knew my granny could cook, but these pies had made it onto the world stage! - Kingsley my Nigerian flat made admitted to enjoying them to.
In the spirit of full international cooperation I did the only logical thing I could, I hid them in my bedroom.
Unfortunately the close proximity of the mince pies to my bed resulted in my careful rationing going out the window and the remaining mince pies lasting just days rather than the projected two weeks.
Do It For the Love of the Pie... Man...
In that particular year, spurred I think by the student competition for the alcoholic mince pies my overall mince pie consumption shot through the roof. After gaining at least 1 stone (14 pounds or roughly 7kg) in weight that Christmas alone I decided that I'd better save the tasty yet modest mince pie for just the Christmas season. From here Mice Pie Day was born. I embarked on a Mince Pie Fast which lasts from the 1st of January right through the year until Mince Pie Day - the 1st of December. Today I'll be enjoying the my first Mince Pie of 2015... and the challenge every year is to find (or make) pies that can pickle your mouth like Granny Scregg's did.
Predict that my twitter feed will feature at least one Mice Pie this lunch time.
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.
We had a brilliant time over the Christmas break, with two trips back to England one for new year, and another to catch up with friends in early Jan. Watching Matthew as he observed other babies, toddlers and assorted infants of all ages was fun. His excitement at wanting to get involved was really reassuring. Since my last post about Matthew we feel he really has made huge progress. However not all has been smooth sailing. During one of our recent ferry crossings I saw Mattie have a spasm, and in total we've seen about 10 individual spasms over the Christmas and New Year period. In fairness Mattie had also picked up some sort of virus over the Christmas period, which I'm sure didn't help. Seeing the spasms made both Emma and I more keen than ever to hear what the doctors have to say about his progress, in particular if anything in his last EEG was concerning or worrying.
On Tuesday Emma took Mattie back into Temple Street hospital for his check-up. Mattie was seen by a new Registrar, the head of neurology, physiotherapy and occupational therapy. While he didn't get another EEG he did get a good checkout, with some interesting feedback.
Neurology & Weight Check
First up was the neurology department where a nurse weighed him and Mattie checked in at 12.7kg. Mattie's weight is important as it defines the dosage of medication he receives.
Meeting the New Registrar
This meeting consisted of explaining Mattie's history to a new doctor. Within Temple Street Hospital the registrars all rotate between departments, our last doctor had moved on and Emma spent a lot of time filling in the new Registrar. Emma recapped all of Matthew's history and touched base on the last EEG, which was performed in Nov, although Emma didn't get the chance to discuss it in detail. Next the reg and Emma went through the process of checking Matthew's developmental progress.
Q. Is Matthew standing yet? A. No... But Mattie is sitting up brilliantly by himself and playing well on his own. The Registrar wasn't concerned by the fact he wasn't walking. The Reg shared statistics that only 25% of kids walk by 12 months, which increases to 97.5% by 18 months. Then of the remaining 2.5% only few have reasons for not walking. Mattie has no reason apart from IS, so he should catch up. Mattie's pincher grasp and eye/hand eye/mouth coordination was excellent. Mattie was really focused on trying to grab his pen whilst he was talking to me - got it in the end and was trying to take the lid off:)
As I mentioned in the past, the drug Mattie is currently taking (Vigabatrin, also known as Sabril) has the potential of causing peripheral vision loss.
The Registrar also checked Mattie's peripheral vision while Mattie was distracted - his response was excellent 🙂 Mattie picked up on the slightest movement out of the corner of his eye - this bodes well considering the medication he is on.
The Registrar also asked about Mattie's verbal skills. Mattie is doing brilliantly here - "Mama", "Dada", "Hiya", and "all gone" (which doc was particularly pleased to hear about - two words together:)). Also Mattie responded brilliantly when the Registrar called his name :)Emma explained the that we had seen two spasms on Jan 28th when he had a virus but nothing since.
Meeting the Neurology Consultant
The Registrar had obviously done all the leg work and filled the Neurology Consultant in. The Consultant welcomed us into the room with a smile and said he heard Mattie is doing brilliantly. Queue Mattie smiling, clapping his hands and banging the table.The Consultant said he wouldn't change anything and plan to see us in 6 months providing Mattie stayed well.
I had some questions though:
Q. Mattie's EEG in Nov showed much improvement, little or no hypsarrythmia but a slow electrical background. I asked the significance of this.
A. Apparently his EEG was much improved but one or two individual electrical spikes where observed along the slow background. This can mean a few things:
1. Vigabatrin is keeping IS under control but might not be suppressing it 100% this could account for spikes and slow background may be present due to medication
2. Spike and slow background may be due to another form of epilepsy being present. This will remain an unknown until we wean meds and observe what EEG and Matthew does going forward - so it's a wait and see
Q. What is the plan for weaning?!
A. The Registrar had said they would wean a year from the last spas`m observed - which would be jan 2015. However the Consultant corrected this, he said we would wean from a year the last significant presence of IS was observed - so that makes it end of September or early October. The plan which was agreed was to return to hospital on September 23rd for an EEG and another appointment with the Consultant, if the EEG is good the Mattie should start to be weaned off his Vigabatrin over an 8 week period. If more IS/seizures are observed then we need to go back in for another EEG to figure out how to treat going forward.
Q. We're planning on a trip to the US later on this year, but the time zone difference and the time spent travelling may mean that we have to reset or alter the set times we currently give Mattie his medication.
A. The Consultant had no concerns here, apparently Vigabatrin is one of the less time sensitive medications. So giving doses 10 or 14 hours apart will cause no issues.
We've also seen Mattie exhibit some odd behaviour, like arching his back and flinging his hands up to his head, particularly when he's tired. It doesn't look like a spasm, but it doesn't look 100% normal either. Emma wanted to check that out too...
Finally I had described some behaviours that Mattie has - which is arching his back/flinging himself back when tired. Fortuitously (or not) Mattie did this at the end of the appointment with the Consultant.The Consultant's response was that is it's an odd behaviour but is most likely just that - a behaviour. But again only time will tell, so we just need to keep an eye on it!
Appointment with Physiotherapy & Occupational Therapy Specialists
The Physiotherapist's first impress of Mattie was that he has come along brilliantly in 3 months. Compared to his last visit Mattie is now sitting up fabulously and balancing himself. He's also reaching, stretching and grabbing well. We've also seen Mattie try to start shuffling on his bum, he's not got the hang of it yet, and when he does do it both Emma and I are pretty sure he doesn't realise that he's actually moved himself. But all in all the Physiotherapist thought this was also brilliant.Mattie still hates his tummy time, but the Physio said need to continue to encourage him to do this. The Physio suggested working on getting him on his knees (because he does hold his weight well) and showing him how to get from knees to bum and vice versa.
Mattie has started taking his weight well on his feet too, but there is room for improvement here too. We need to encourage positive pressure on his feet, we can do this by sitting him on one of our thighs and then putting Mattie's feet flat down on the ground either side of our thigh.
Mattie has all the precursors for walking, we just to work on encouraging him to give it a go.
When we were initially referred to a physio for Mattie we were unable to get a physio close to home. There were none available. So instead we've been driving into the centre of Dublin to visit the physio centre in Temple Street Children's hospital. Things seem to have improved and we are now getting a referral to a closer, more local physiotherapist. Hopefully that will work out. If it doesn't we've a back-up booking back at Temple Street for later on in March.
The Occupational Therapist was absolutely delighted with Mattie :). Matties seems to be doing everything he should be at this age and more :). Mattie's pincher grip is awesome, he's passing from hand to hand well, picking blocks out of a box and putting them back in. We just need to encourage Mattie to move up to the next stage; shape sorting !The next visit with the Occupation Therapist is tentatively scheduled for May / June (waiting for appointment confirmation and final date), that should be our last appointment with the occupational therapist! ... Wohooo go Mattie!
Mattie playing just before bed - check out those motor skills!
The problem with Hypsarrhythmia, the tell tale electrical activity present in an EEG which signifies Infantile Spasms, and can cause brain damage is that it's transient; it comes and goes. It's most frequent during tired periods before or after sleep, but it could happen at any time. The spasms we associate with the condition are just the physical side effect of what is going on in Mattie's brain, they can be subtle and hard to detect. For months now we had been treating Mattie's condition with the only indication of success being the lack of a spasm. Getting some hard data - a physical measurement via the EEG that the drugs are working is what both Emma and I were waiting for. If clear it would give us confidence that the electrical activity had returned to normal, and we'd not just missed another spasm.
In the two weeks following the EEG we'd called the hospital twice to try to get some feedback on what it showed. When we didn't hear anything we assumed that, since Mattie was doing so well, "no news was good news".
It felt like it took forever and a day, but eventually Emma got the call from the hospital and relayed the news to me. The EEG did not show any signs of Hypsarrhythmia! Success! This was fantastic news! But there was a "slow background", which we were told was common in babies with Infantile Spasms.
I think it's fair to say that between Emma and I, Emma is the more optimistic; I immediately focused on the slow background news, and couldn't relax until I found out what it actually means. The truth is overall this was good news, but I just had a nagging feeling, an itch which needed to be scratched, what did this slow background mean?
The EEG's Slow Background
After talking to David if there is one thing that stuck with me, was not to take things at face value. So I started a mini-research project into the EEG's "slow background". What did this really mean?
An initial search turned up an article which suggested that the slow background was a result of a brain injury, or other physical issue - something we've been told Mattie doesn't have:
Following a seizure (ie, during the postictal period) the EEG background may be slow. However, interictal background EEG frequencies that are slower than normal for age usually suggest a symptomatic epilepsy (ie, epilepsy secondary to brain insult). Normal background suggests primary epilepsy (ie, idiopathic or possibly genetic epilepsy). Thus, EEG background offers important prognostic[Christine1] and classification information.
So how could this be? Was there something more significantly wrong? We were about to call the hospital back when Emma read an article from another parent on the Infantile Spasms Facebook group, it was asking roughly the same question. One of the answers offered was that a doctor has informed one of the parents that slow background can be a side effect of the medication. I'd not heard of this before so checked it out.
The only EEG modifications induced by GVG monotherapy were a more pronounced slowing of the background activity at rest with EC [Eyes Closed] and a reduced responsiveness to BR [Blocking Reaction].
This research paper suggested that the medication Mattie is on could produce a slowing of some of the background waves. What did this mean in the real world? - According to the same article it could induce a mild "sedative" action on attentive tasks rather than on cognitive function. I imagine that while the folks in the study could still complete a mental task, it look them a little longer than expected to get it done. - Which is a good approximation to what the doctors had told us to expect.
Mattie is doing well. In the run up to Christmas he started suffering from normal everyday baby problems, conjunctivitis, and a cold. Emma and I would take this rather than more IS. He's now 9 months old and has just about got the sitting up straight bit down... Well for good 10 minutes before he gives up. Mentally we think his doing really well; he's turned into an octopus, hands everywhere, grabbing everything.
Emma's employer organises a children's Christmas party in their staff canteen. We took Mattie and Ben down, Mattie fell in love with the balloon sword he had been given, and with a little help from daddy enjoyed whacking his older brother and mother with it. Enjoyed it so much we couldn't get the balloon of him until, in the middle of the party he fell fast asleep, normally, without any spasms.
We know this is something we'll need to keep an eye on, but for now at least, we've got just what we wanted for Christmas.
Thank you all so much, for your kind words and support, it has made the world of a difference, Merry Christmas everyone - I hope you all have a fantastic Christmas break.
I've got my son, Ben in my arms while in trying and failing to run a bath for him. Ben has other ideas and he's trying to break into my aftershave bottle. The door bell rings and brings someone unexpected, who through a random act of kindness will help us have a good Christmas.
My first thought when the door bell rung was one of frustration - I was so close to getting the little man in the bath. But luckily I hadn't quite managed to get Ben undressed yet.
Like most of the population Emma and I had done a lot of our shopping on line. We had received reminder notes that some of our parcels where ready for collection but being flat out in the run up to Christmas we'd not managed to arrange to collect them.
It is nearly 7pm at night and when I open the door I'm greeted by a guy dressed in a postman's uniform. There is no post van behind him, just a little silver car with the engine still running.
Hi, I'm the guy who normally delivers your post. I noticed that there's were a few parcels waiting for collection, some had been sitting there so long that we had been told by our manager that we have to start sending them back. They look like Amazon boxes and I guessed that they were Christmas presents. I didn't want you guys to miss them - it would make for a terrible Christmas. I'm off work now, on my way home and I volunteered to drop off your parcels. I've got a bunch in the back of the car.
Wow - now these guys are crazy busy this time of the year, I'm sure that this posty was knackered but he'd taken some of his personal time out to drop off our amazon orders.
It was a small random act of kindness which I'm really very grateful for.
Tonight Emma dropped off a big box of Roses chocolates to the post office sorting station as a way of a thank you.
An post - you have some fantastic employees! And a very merry Christmas to you all.
So tonight I got the chance to try some mince pies from Aldi. They had some exceedingly stiff competition from Mr Kipling.
As you can see the decorations on the top of the Aldi mince pies were definitely more appealing than that on the Mr Kipling's. These Aldi specimens held so much promise. Also tipping in Aldi's favour was that all of the mince pies in their pack were undamaged! A feat that Mr Kipling couldn't achieve. The Aldi pies shipping with additional packaging which protected the pies.
Filling In The Details The filling in the Aldi mince pies was delightful, it had a light orange tang to it which I personally really liked. However the filling was very shallow - if only there was more meat! Mr Kipling's on the other hand have a a deep feeling. While not quite as tasty as Aldi's they don't skimp.
Verdict - draw. If Mr Kipling had good packaging - they'd have got this one in the bag!
I love mince pies. So much so that I limit myself to only eating them in December. Today is December 1st so it was with great excitement that I broke my mince pie fast and opened a packed of Mr Kipling's best and was instantly disappointed.
Two of the pies had been smashed on their packet 🙁
Poor job Mr Kipling! poor job.
On the bright side I noted a marked increase in mincemeat volume compared to Mr Kipling's effort last year.