This morning I did something that a while ago I thought I’d never do. I thought I wouldn’t need to do it. Today I wrote our Parent’s Profile about Samuel as part of his special educational needs assessment prior to starting school this autumn. School. SCHOOL.

You may not quite understand the huge significance of this. But let me tell you there was a time that we his parents, his therapists, doctors and professionals honestly didn’t think Samuel would still be here to talk about School.

Samuel starting school.

Bring. It. On.

So after a year of plugging away at the Ketogenic diet and getting increasingly frustrated that despite our efforts and making what Samuel’s BUPA carers call ‘a chemistry experiment’ four times a day for his feeds, it didn’t seem to be having any effect at all. But I was so keen to give it as much chance as possible, I would regularly be saying to the paediatrician Dr H ‘could we just give it another 3 months’.

But then we talked about it and I came to realise it wasn’t making any difference and his feeds were a lot of hassle for no result. Plus I wanted to go back to giving Samuel the odd sensory taster without worrying it would ruin the diet. So I emailed Dr H with some urgency saying we wanted to put the diet behind us & move forward & ‘can we start the plan to wean Sam off the diet’ – which he had been suggesting for a while.

Then we had a really good weekend seizure wise. I mean REALLY GOOD. Then we had a really good week, then an almost seizure free weekend (may I remind you we are talking about Samuel. The boy who has multiple seizures every day – could range from anything from 5 to 20 seizures a day). We are now coming to the end of another good week (not seizure free but still bloody good).

Is it the diet? Is it because he’s put on more weight again and he’s outgrown his current recipe? Is it a very delayed reaction to the last diet tweak?

Or is it just a very good patch? We have very bad patches for no apparent reason, so could this just be a good one?

This might all change in a couple of hours and tomorrow could be an awful day. But I’ll take this good patch for as long as it lasts. Samuel has never been one to follow the rules, ever, and in fact that has what has kept him doing so well for so long. It’s what’s kept him here with us much longer than expected.

And as for the diet, think we might just stick with it for a while longer. Who would want to rock the boat when the going has been so good?!!

I realised today that I’ve been terribly slack with my blog posts lately. I use to be quite prolific but I’ve just been so focused on other things that I’ve neglected the blog. And you. So I’m sorry Mr Blog and our lovely readers and Sam fans.

So, what have we been up to?

Well Samuel got a new buggy a couple of months ago. I had been keen to get a new set of wheels for ages as he’d really grown out of his old one and we felt like we were having to cram him into it every time we took him out. Samuel’s new buggy is called Stingray and is made by R82 and is two-way facing, so while he’s still in the chair we can swish him around to either make him front facing or mum and dad facing. An added bonus of having this type of buggy and being able to make it front facing means he can go into wheelchair accessible vehicles while sitting in it. We are really pleased with it and he looks so comfy in it!

Samuel went to his first every ice skating party recently. It was his special friend George’s birthday party and all the children sat in their chairs and were whizzed around the ice rink. It was really good fun!

We also looked around a school recently. Now there was a time that we never mentioned the S word. Ever. Samuel wasn’t really expected to still be here for us to be considering schools, so the fact that we are discussing when he might start is an exciting feeling. We are totally in love with the school we looked around. It will be wonderful for Samuel. Perfect in fact. It currently has 24 children and has a real village school atmosphere. He will have physio there regularly and hydrotherapy once a week (their hydro pool looked amazing). Samuel would start off by going in for the mornings initially and I can sit in for as long as I feel necessary. We aren’t entirely sure when he will start, it will depend on the paperwork side of things – it might be a bit ambitious for him to be able to start in September, but hopefully we can start going by the October half-term.

We held our first fundraising event last month for Samuel and it was really successful. We were really touched by how many people came along to support us and Samuel.

Samuel finally met a very special friend for the first time last month. Ethan is a lovely little boy who also has ARX, the same condition as Samuel. It was wonderful to meet Ethan and his mum Naomi. We got the chance to have a good chat and I like to think we have become good friends.

As far as the diet goes, we are now looking at putting a plan together to wean Samuel off the Ketogenic diet. I’ve been the one delaying coming off the diet for quite a while now, but we gave it one last shot and I’ve finally acknowledged that it isn’t making much of a difference (if at all). Samuel started the diet a year ago and although we thought it all started extremely well and I was talking about diet dreams coming true, it didn’t last very long and has been a headache ever since. While I would make up the most complicated, time confusing feeds for Samuel if it helped him, since moving on to the MCT version of the diet, feeds are quite complicated to make up and is frustrating when you put all the time and effort in but not making any difference for him. Not sure when we will start the wean off, I will need to give Samuel’s doctor a nudge to coordinate with the dietician, but we will wean him off and move on to a different feed. We may also look at introducing another drug, possibly Topiramate (he has been on it before). Samuel needs something that has more of a consistent effect to help him. Coming off the diet will also mean we can resume sensory tasters which I must admit I’m really looking forward to. I’m keen now to move forward and put the diet behind us. A new chapter awaits.

Seizure wise, Samuel has been OK. Of course still having seizures everyday, but they have been managable and only occassionally needed a dose of Midazolam. This might also be because we’ve been using Chloral Hydrate (which is Samuel’s sleepy drug at bedtime) now and again during the day when he is very unsettled and twitchy, but not necessarily fitting into the protocol for Midazolam, his emergency medicine. One of Samuel’s medicines is Clobazam which is part of the same family as Midazolam. When we are having bad patches and using Midazolam frequently, it could be effecting the same receptors in the brain that Clobazam uses which means Clobazam might not be as effective as it should  be. So the idea is that if we nip things in the bud early with a 3ml dose of Chloral (there is also thought that it contains anti-epileptic properties which is an added bonus), it may stop the need to give Midazolam later.

Looking ahead we also have a few things to look forward to towards the end of the year.

We are still hoping to have the garage converted into a bedroom and wetroom for Samuel. We are being given a grant from the council who will be overseeing all the work for us. Unfortunately this project has been very slow at getting off the ground, but the technical officer is coming out this week and I’m hoping we will get a better idea of when work will start – but it will probably be around 6 months before anything starts! I must admit that I am finding it increasingly difficult to carry Samuel around the house. I can still manage at the moment, but as he is getting bigger it will become very difficult before too long!

We are also hoping to be able to get a grant to make some changes to the back of the house to make it easier to take Samuel into the garden and for access to go in and out of the house with him.

Another big thing which will dramatically improve things for us is getting a wheelchair accessible vehicle. We have to wait until Samuel is 3 (which isn’t until November) and then we will qualify for the mobility part of his Disability Living Allowance (for those not in the know, the mobility DLA payments will go towards paying for the car). Being able to just wheel Samuel in and out of the car rather than having to lift out the very heavy buggy and a heavy Samuel will make such a difference! But we have to wait until his birthday, so need to be patient!

Life feels good at the moment.

An easy way to show that you are an official member of Team Sam is to wear a Team Sam badge.

They come in two sizes and we ask for a minimum donation of £1 for the small badge and £2 for the larger badge – just to cover materials & postage.

To order please send your payment either via Paypal to email address welovechip@yahoo.co.uk (if you donate this way, Samuel’s Fund get all of the donation – just make sure you include your postal address and which badges you’d like).

Or you can click the donate button below to make your order & donation – please make sure you include your postal address and which badges you’d like (Paypal take approximately 12% from donations made this way)

Thank you.

Team Sam x

Samuel now has a new standing frame. He’s only been in it a couple of times so far but seemed to cope with it very well. It must be a very strange and different sensation for him as he’s never been so upright before.

We put Samuel in it in while he is in a laying down position, then gradually tilt the frame until it is as upright as we’d like and think he would tolerate.

It will be good for him in a number of ways, but I’m excited for the very new sensory experience it will give him.

If you are a family affected by ARX like us, please pop over to Facebook and search for a group called ‘ARX families‘. I’ve started this group with the aim of bringing together other families affected by ARX.

If you know anyone that you think would benefit from the group, please do let them know.

Thank you. x

Sorry I’ve not been updating the blog much. There always feel so much to tell you, but sometimes I struggle with getting it out.

So, the Ketogenic diet. Samuel has been on it a year now (hasn’t that flown by quickly?!). If you’ve been following our journey for a while, you’ll know that we did have success with the diet in the very early days. In fact Samuel had a couple of seizure-free days, which is simply amazing given the poor boy has multiple seizures every single day.

But then it all went downhill. In September last year we realised he had not just stopped gaining weight, but was starting to lose weight. It seemed that he wasn’t absorbing the fats from the diet, they were coming out in his nappies instead.

So we moved to the MCT version of the diet which saw him start to put on weight very nicely. Seizure wise though, it’s been the usual rollercoaster. Some weeks good (but never seizure free) and some weeks very bad. We’ve also had to increase his medicines recently as he just needed some extra help.

We’ve decided to give the diet another 3 months. If we feel that it isn’t helping Samuel then we will start the process of weaning him off. The diet will then be replaced with a drug, possibly Topiramate (he has been on that before and it was probably what got us home from NICU in the early days).

But, we are going to give it one last shot. He was weighed last week and is now an even cuddlier 11.8kg, so we have room to drop down the calories again. So this will be the test. We will see if this change helps or whether we need to move him on to another medicine which hopefully will have more of a consistent effect for him.

Although I’ve been the person to start the conversations about coming off the diet, it does break my heart. I so wanted it to work for Samuel. There is a very lovely young girl I know who is a few years older than Samuel and has been on the diet for some years. She is an amazing advertisement for what the Ketogenic diet can do. She is almost seizure free. I wanted that for Samuel, at the very least to reduce the amount of seizures he does have. I wanted to give his little brain and body a break. But it seems that for us, the Ketogenic diet just wasn’t meant to be.

For those who have been asking, this is our recipe (each feed now has 183kcals and ratio 4 x fat : 1 of CHO+ protein)  for making up a feed for Samuel (he has 4 feeds a day):

• 54 mls liquid Ketocal
• 13 mls Liquigen
• 2 mls of Maxijul (1.3g/1 blue scoop of Maxijul, add 4mls water then give 2mls)
• 6g Carbohydrate-free mix (add enough water to mix into a smooth paste)
• Add water to make a total feed of 220mls

Only time will tell if this new recipe will make enough of a difference. But it’s worth a shot, isn’t it?

Other news: At a recent clinic meeting with Dr H, he suggested we start to use Chloral Hydrate (which is Samuel’s sleepy drug at bedtime) during the day when he is very unsettled and twitchy, but not necessarily fitting into the protocol for Midazolam, his emergency medicine. One of Samuel’s medicines is Clobazam which is part of the same family as Midazolam. When we are having bad patches and using Midazolam frequently, it could be effecting the same receptors in the brain that Clobazam uses which means Clobazam might not be as effective as it should  be. So the idea is that if we nip things in the bud early with a 3ml dose of Chloral (there is also thought that it contains anti-epileptic properties which is an added bonus), it may stop the need to give Midazolam later.

Fundraising for Samuel.

Last week we learned of some devastating news. A young boy called Harry Churchill (almost 4) who went to Julia’s House had passed away. Then we heard two days later that his younger brother Cody (nearly 2) had also passed away. Both the boys had pneumonia.

Harry and Cody both had Epidermolysis Bullosa (EB), a very rare genetic condition in which the skin and internal body linings blister at the slightest knock or rub, causing painful, open wounds. It is often said that people with EB have skin as delicate as a butterfly’s. Both boys also had a tracheotomy to help them breath.

When Samuel started at Julia’s House, he was often on session with Harry and then joined by Harry’s little brother Cody, before Harry left the pre-school sessions to start school.

Both the boys were very bright – it made me smile watching Harry show the carers how to use his iPad and both boys would always join in with all the songs – they knew all the actions. They were wonderful and brave little boys. Despite everything they had to go through each day they were always so happy and brought so much joy to so many people.

Losing these two amazing boys has broken so many hearts…sleep tight Harry and Cody. x

Despite starting off quite well, yesterday turned into a horrendous day. We’d had a good appointment at Orthotics (believe it or not they were impressed with Samuel’s new Superman shoes and think they will be perfect for him), then we popped into NICU and had a nice catch-up with the nurses and one of my favourite NICU doctors.

But then it all went wrong.

In the afternoon we had an epilepsy clinic appointment with Samuel’s doctor, Dr H. The appointment didn’t started off brilliantly as we had to give Samuel a dose of Midazolam in Dr H’s office. It had been eight days since Sam last had it which given our recent bad patch isn’t actually too bad.

We talked about Samuel’s medicines and our plan for them moving forward (I’ll tell you about this another time).

He then began saying that because we go the extra mile with our care of Samuel and react quickly, we’ve given him longer quality time. Which of course is nice to hear. But without saying the exact words, he said that Samuel is living on borrowed time. He’s outstripped his life expectancy. Which of course we are very aware of.

This led him to say that he wants us to think about an advanced care plan for Samuel which basically means what to do if we are faced with the possibility of Samuel going into intensive care.

Dr H said he doesn’t think we should put Samuel through intensive care as it could be gruelling for him & even if he recovers it won’t change his underlying condition. There is ‘no light at the end of the tunnel’ (he compared it to a scenario where a child has meningitis and then makes a good recovery). This came as quite a blow to me. I’m very fond of Dr H and always feel he knows and says the right thing about Samuel’s treatment and care. So I felt so disappointment that he felt that we shouldn’t throw everything at Samuel.

He wanted to discuss it while things were okish (which turned out to be quite ironic thing to say given the evening we then had), rather than when things could be quite fraught if things get crappy. He explained the different scenarios of intensive care and how gruelling it could be for Samuel. He said that he didn’t think he should go through that given that at best he would come home the same, his underlying condition would never improve. But in the cold light of day, when things are ok, in my mind I could overlook Sam having a line put in or being ‘worked on’ to be brought back. If that meant he would be able to come home and be just as he is now….but then if we do find ourselves in that scenario and I see it before my eyes and what it all really means then I may feel different. We may feel very different.

I just never want Samuel to be in pain or his seizures worse. But how on earth could they be any worse?

So we went home feeling quite low. And just to add to it all, Samuel had a really bad evening and overnight, so it was all a bit rubbish really.

But he seems to be sleeping (well snoring) it off this morning, so who knows with Samuel, today could be a very different day.

I don’t really want to dwell on yesterday too much. I don’t want to waste good Samuel time by worrying about what decisions we will need to make. I feel strongly that we will know what to do when decisions need to be made. I think our gut will tell us.

Oh and before you say that we are so brave and so on, we aren’t. The idea of making those decisions and maybe one day we wont bring Samuel home, well it broke my heart. The idea of not having my baby Bear to cuddle anymore is just unimaginable. I’m not daft, I know he’s living on borrowed time, but to have things spelled out to you, well it is never easy.

We are just like you, we are normal people, normal parents. But we have to deal with the very hard side of what life throws up. We just want to do everything we can for Samuel and everything right. We will never give up, we will just keep swimming.

Do you have an Instagram account? Enjoy baking? Fancy winning some great prizes (including a trophy) and help a good cause?

Then enter Sam’s Instagram Bake-Off 2013. (Added bonus is you get to eat all the cake afterwards!)

It is really easy to enter:

1. Make a donation (if you live outside the UK please keep in mind the increased postage costs for sending out prizes when making your donation) to Samuel’s Fund by sending a Paypal payment to welovechip@yahoo.co.uk (in the message box please include your full name & Instagram Bake-Off)
2. Decide what you want to bake, whether it be cupcakes or a cake. Give your creation a name, then email the recipe (& its name) to samuelsfund@yahoo.co.uk The only rule is that you must include the word SAM somewhere on the cake. Perhaps by using icing, chocolate chips…however you like!
   
3. Then bake the cake and from Friday 1 March to Saturday 2 March, post photos of your creation on Instagram using hashtag #samsbakeoff

The winner will be announced on Sunday 3 March.

Your cakes will be judged on creativity, individuality and how darn tasty they look.

Prizes will be awarded for 1st and 2nd place.

All the recipes and photos will be compiled into Samuel’s Fund Bake-Off recipe book which will be sold online and at fundraising events.

All proceeds will go to Samuel’s Fund, to help buy Samuel equipment and toys to make his world more fun and comfortable.

Don’t worry if you don’t currently use Instagram as you can easily download the app. You can also follow us on Instagram by searching @samuelsfund or by clicking below:

If you have any queries, please do get in touch.

All money raised will go towards toys and equipment to make Samuel’s world more fun and comfortable.

Good luck.

Team Sam. x

PS If you are in Dorset then you also have the chance to take part in Sam’s Dorset Bake-Off!

Winners prizes

To help fundraise to buy Samuel some new equipment, I’ve asked people to donate a children’s story that we can compile into a little book and sell online and at fundraising events. Our friend Katie Kitchen used Samuel as her inspiration to write this poem for our little book and I thought it was so wonderful, I just had to share:

Super Sam

Sam is a superhero,

A caped crusader in his own right.

His powers mean that he captures your heart,

And forever holds it tight.

In fact from the minute you see him,

Before you even know his name,

Something inside you already knows,

Your life will never be the same.

Is it his lashes thick and long?

Or his gorgeous big blue eyes?

Is it his amazing snuggly cuddles,

Or his super squishy thighs?

Is it the way his story has helped,

Join friends from around the globe?

How does he weave his magic spell?

I  don’t think we will ever know.

But quietly he enchants us,

You cannot help but love this little man,

And once a  part  of  your heart belongs to him…

You’re  always a member of ‘Team Sam’.

by Katie Kitchen.

I’ve been away for a bit, sorry. Not away away, just away from blogging. Everything has been ok, ish, but as Samuel’s seizures have been all over the place which has now led to an increase in both his Clobazam and Vigabatrin doses, plus starting to plan fundraising activities while also trying to reclaim my craft mojo, didn’t feel like there was much room left in my brain for the blog!

We’ve been getting to know some of our Julia’s House friends better. This is little Eiden. Eiden is 2 years old and has complex problems including cleft palate, scoliosis, hypermobile joints, developmental delay and gastroeosophagal reflux. But more importantly he is extremely cute, cuddly and very bendy!

Samuel recently hit a milestone. He had his first salon haircut (and only his second haircut ever). He wasn’t particularly bothered by the experience as he snoozed all the way through!

Heavy snow meant that Samuel’s Dad’s school had to close which gave Samuel a bonus day of Daddy cuddles!

We’ve started to plan fundraising activities this year for Samuel. We hope to raise money to be able to buy Samuel some special toys and equipment to make his world more fun and comfortable.

This is a P Pod chair and one of the first things we want to get with the money we raise

Coming up, we are hoping to be approved for funding to get Samuel a special standing frame. I’m preparing to go into battle with the wheelchair unit to get Samuel a better buggy. We will also be trialing a sleep system next as Samuel’s position overnight is quite poor and I’m going to be trained this week on how to use suction for us to use at home, just for when Samuel has a heavy phlegmy cough and cold.

Oh and by the way, ‘The Bear’? That would be Samuel. He’s our real-life bear.