‘Normal’ for a day

Last weekend we went to our first Julia’s House family BBQ. We weren’t sure what to expect but we knew it would be a good experience for us.

Being honest, truly honest, at first it was a bit daunting. We had never seen so many wheelchairs or special buggies in one place at a time. There was a fantastic turn-out and the place was full of children with all manner of additional needs/medical problems/disabilities etc.

But that feeling literally only lasted a few minutes and as we looked around, we saw what seemed like every other family with a feed pump or some kind of equipment attached to their wheelchair. We smiled to each other and my husband said: ‘at last we are normal!’.

No one batted an eyelid at us. No one stared. No one wondered. No one stood looking awkwardly at Samuel wondering if perhaps something wasn’t quite right. No one patronised us, felt sorry for us, told us how brave we were or asked awkward questions. Everyone just got on enjoying themselves (and trying to keep track of the more mobile children!).

I’m starting to get to know more Mum’s from Julia’s House so of course it was wonderful to see them and have a good old natter about all the things that are part of our everyday life – gastrostomy buttons, therapists, seizures, ketogenic diet and so on.

It was also lovely to see the carers and nurses there too, who all took the time to come around and chat with the families and children. Quite often I’d look up to see one of Sam’s carers or nurses holding his hand and chatting to him.

There were fun activities for all the children and their siblings – bouncy castle, craft tent, music, a pet corner (I believe a snake was available for ‘cuddles’), play sessions with the Play Maker, face painting, BBQ, ice cream and to end the day there was an amazing (and brave) fire display.

This wasn’t a fundraising event for Julia’s House. They organise this every year for families to get together and just enjoy themselves and enjoy being part of a very special community. I think Samuel, his Dad and I fell in love with Julia’s House all over again that day.

James is the cousin of Sam’s Dad and is climbing Kilimanjaro to raise money for Julia’s House to help them continue all the wonderful things they do. If you’d like to support Julia’s House and what they do for children like Samuel and families like ours. You can sponsor James at: justgiving.com/James-Day1

To find out more about the wonderful work Julia’s House does watch this video.

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Meeting with Dr H

This coming week we have an appointment with Samuel’s pediatrician and we have a lot to discuss regarding the Ketogenic Diet.

We are still feeling that it is working and certainly making a difference to Samuel’s epilepsy but we have noticed a slight increase in seizure activity in the evening (but still amazing compared to before) and he is far more sleepy during the day. Also his ketones are still really low. Really low. We seem to be stuck between 1.4 and 1.6 and despite changing the recipe (Samuel is fed a special formula through his gastrostomy, so I’m referring to changing the concentration of the feed) we seem to be stuck.

When I last spoke to Samuel’s doctor and mentioned about the excessive sleepiness (with the diet working, it is now like he is a bit over medicated), he suggested that when we come into clinic for our appointment we discuss reducing Clobazam. Now we love Clobazam. It bloody works. It is a very strong drug (it is sister to Midazolam, Sam’s emergency drug) and can cause extra drowsiness. We are nervous about reducing this medicine but do see that it could really help us have more awake time with Samuel.

But the drug I do want to discuss with Dr H is Topiramate. Now we do have a fondness for Topiramate as it basically got Samuel home from NICU. But he has been on it a long time and he is on quite a high dose (14mls twice a day) and I think Clobazam and his other drug Vigabatrin have superceded it. Last weekend, while frustrated with Samuel’s ketones and no one around at the hospital to speak to, we phoned Matthew’s Friends. We were lucky enough to speak to Emma, Matthew’s Mum who advised that Topiramate can cause acidosis in the stomach which can reduce ketone levels. Well isn’t that something? So that will be high on my list to discuss with Dr H.

Although his brain and body does seem to be responding to the diet, I’m not sure what it is doing with his ketones but I really want to see them increase and increase soon as it’s the one nagging thing about the diet. We need Samuel’s ketones to remain at a stable level between 2 and 5. Even when they have been high (they have been above 2 a few times), it hasn’t been consistent and they’ve come back down again.

Do I dare wonder what his epilepsy would be like when his ketones are at a stable high level? That is just too exciting to think about. But in the meantime, maybe this is just Samuel changing the rules again. I just wish he’d let us in on the new rules!

Meal times

A friend of Samuel’s, Little H (his mum blogs at Little Mamma Said), will be having his gastrostomy operation tomorrow and it got me thinking about when Samuel had his operation and was finally able to get rid of the NG tube.

Samuel has never taken food orally. On his first day of life we were in postnatal with the scarily enthusiastic midwives thrusting him on my breast every five minutes. Samuel wasn’t having any of it. Literally. He would either get agitated and upset or go to sleep. Then on his second day of life we found ourselves in NICU. The (male) Consultants (who were lovely I must add) would say, ‘lets try him on the breast today’. We’d try but have the usual result. Sleep or agitated and crying. But then as days passed and they realised that his seizures were more serious than they’d thought, they started to sedate him more and I was just left to express milk.

As time went on Samuel continued to be sedated so breast-feeding, any kind of feeding other than NG tube feeding, was a no no. He was finally brought out of sedation and I started to ask about whether we could try oral feeding again. Problem was, by this time they needed the Speech and Language Therapist to come over and assess him before they gave him anything orally. But she was the only person in that role and she worked part-time. So we had to wait several days for her to come over and when she did, more often than not, Sam was sleeping, having a seizure or just not performing with his dummy.

The ridiculous thing is on his notes it says something like that he has a poor swallow. But that is simply not the case. Samuel has never once needed suction because of a poor swallow and excess saliva. He has always swallowed his saliva. He only dribbles very occasionally when he is teething or had a big seizure.

But we carried on feeding him via his NG tube and was delighted when in June last year (2011) he had an operation to have a gastrostomy button put in. It was the first operation he had ever had and he was absolutely miserable when he came around from the anesthetic. He was very sore and uncomfortable and he was having increased seizure activity, so our old friend Midazolam did make an appearance. I was also nervous about picking up him to cuddle him as I was so afraid of knocking his new button. But within a few days I was much more confident and before long he was back to having lots of tummy time.

Having the gastrostomy and getting rid of Samuel’s NG tube had so many benefits. I no longer had to pass a tube up his nose and down his throat which wasn’t pleasant for either Sam or I. The tube would be pulled out accidentally a number of times. The tape we had to use to stick the tube to the side of his face, would really aggravate his skin and he would always have a big sore red patch on his face when I’d removed the tape. We also of course got looks and questions too when out in public (by strangers). We were often asked how premature he was (he wasn’t, he was born full-term).

All medicines are given via syringe through Sam’s gastrostomy button on his tummy

Having the gastrostomy button now meant we could feed Samuel by a pump which was fantastic. I hated bolus feeding. With bolus feeding, you fill a syringe with feed or water, connect it to the NG tube and gravity takes the liquid down through the tube. However, hold the syringe too high and the fluid goes down too fas. If he gets agitated, cries, has a seizure, the fluid comes all the way back up the tube and syringe. I wasn’t very good at it!

We noticed quite early on that it was much easier to discreetly feed him when out in public (The pump is really simple to use and we have a specially designed bag that we can put the pump and feed in to take out and about and we use it everywhere!). We’d just hook him up to the pump and it takes care of itself. We only really get attention when the pump beeps or when we are attaching or removing the tubes.

I’m not sure whether we will ever get Samuel eating orally. I really wish that in the early days of NICU I’d insisted that we tried him with a bottle instead of trying to breast feed him. He may have had a better chance. Although in the past we have tried him with tasters, it has only been very tiny amounts of food and has been more for sensory than anything else. I’m hoping that once things are more settled with his new diet we can start the sensory tasters again. I’d love him to have that experience in his mouth. When we have to give him Calpol I always give him the syringe afterwards to suck and he does seem to like it. Perhaps because of his global development delay, he may never get the coordination right or not instinctively know what to do, but I’d like us to give it a good try.

This post has been included in the the #definenormal blogging challenge. Pop over to Just Bring The Chocolate to find out more and read other #definenormal posts.

Seizure monster has gone missing (and left his lazy assistant in charge!)

I have absolutely no idea where I should start with what I want to say, what I want to tell you. In my previous blog post A day to remember, I told you that for one day last Friday, Samuel had no seizures. None. Zilch.

I couldn’t understand it because his ketones were so low (to anyone new reading this, Samuel is on the Ketogenic Diet – pop over to my ketogenic blog posts page for a quick round-up of what’s it all about), so it couldn’t be the new diet.

But it wasn’t all plain sailing. He was miserable. Grumpy like I’ve never seen him grumpy before. But by Friday evening we realised why. The area around his gastrostomy site was very red, angry and sore looking. Plus the tip of one of his little fingers was very red too. We took him into hospital Saturday morning (and spent 5.5 hours there waiting to be seen and then waiting for antibiotics) and were told that he had a skin infection.

That would explain why he was so very grumpy. And we discovered that infections can reduce ketone levels which is why his ketones were so very low. (For those who still haven’t got their heads around ketones or haven’t been over to my Ketogenic pages to find out more – basically we need Samuel’s ketones to be high and at a consistent stable level for him to be in ketosis and therefore for the diet to work).

But dear reader, and perhaps this is the point of the post, so well done for sticking with me, you will be rewarded, Samuel didn’t have a seizure on Saturday either. And this time his Dad was there, so I have a witness. He didn’t have one seizure on Saturday. And Sunday? Sunday he did have a one minute seizure but that was all. That was all. Samuel has severe ‘off the scale epilepsy’ (to quote his padetrician) and has seizures every single day. Well he did.

And today? I’m not going to jinx it, but so far he’s had two 30 second seizures and that is all.

Original image ‘borrowed’ from http://www.littlemammasaid.blogspot.co.uk

So is it the new diet? Is it actually working? I still can’t answer that. And it’s not me being coy or over-cautious, I just really don’t know. His ketones are low, very low, so the diet shouldn’t really be working. But it makes me wonder if Samuel is again making up his own rules, even for this new diet.

I might blog again and tell you that we’ve had a shocking evening tonight, had to give him his emergency medicine (Midazolam) and the rest of the week is dire. It is normal for us to not have two days the same. What is normal is to have two dramatically different days. So I’m confused. Feeling rather shell-shocked actually.

But to sit here with my precious boy and not have to watch his little body twist and stiffen, his arms and legs jerking manically, him screaming and crying, is an overwhelming, wonderful feeling. I wish I could bottle it.#

I’m not sure if the Seizure Monster has just gone away on a short holiday and left his lazy assistant in his place. The Seizure Monster may be missing. But we aren’t missing him.

UPDATE (15/05/12) – Samuel didn’t have any more seizures after I posted yesterday, so that was just two small ones he had yesterday. Today? Well, it’s 8pm and Samuel has only had three very brief seizures today. You may be thinking, ‘well three seizures, that doesn’t sound good’. But three very short seizures in one day, for Samuel, is brilliant. Whatever happens tonight or tomorrow, I am such a very proud mum.

More information: If you want to find out more about the Ketogenic diet, please have a look at Samuel’s Ketogenic journey and you can also read a round-up of all my blog posts about Samuel’s Ketogenic adventures. Matthewsfriends.org is a fantastic resource full of really useful information and case studies about the Ketogenic Diet.

Medicine cabinet

You may not be surprised to know that we have a rather bulging medicine cabinet (well actually it’s a red box rather than a cabinet) with all the medicines needed to manage Samuel’s epilepsy, reflux and general bits and bobs:

FAQs

I was reading Little Mamma said‘s blog post about being asked questions about her son’s condition – well actually in this case she was asked outright ‘what’s wrong with him?’

I’ve been asked that about Sam. It was worse when he had his NG tube in because that was stuck on his face and impossible not to be seen. Now it is really when we are out and pump feeding him or when people (who we may know a little, or don’t know at all) stop and chat to us and notice something might be a bit different with Samuel.

I  know people don’t mean any harm or offense. We, as humans, are just terribly flawed when it comes to putting our foot in it. We say things without thinking. People essentially do mean well. If you are reading this thinking that you might have asked a daft question, please please don’t worry about it. But I do wonder though whether I should have a list ready of frequently asked questions to give to people before they come out with their questions.

An example could be, in no particular order:

Question/Comment: Gosh, isn’t he a big boy? My cousin has just had a big baby too. (This is said a lot, but it is because I have to hold Samuel as if he was a little baby because he is unable to support his own head and neck)  Answer: No, not really, he’s just right length and weight actually. He was only 6lb 4oz when he was born.

Question/Comment: Was he a premature baby? How early was he? Answer: He was full term, well one week short of being bang on full term.

Question/Comment: What’s that? (Said after spotting him being gastrostomy fed) Answer: He is having a feed via his gastrostomy button, it goes straight into his tummy.

Question/Comment: Oh, how bizarre (looking very confused), can’t he eat normally then? Answer: He hasn’t got a reliable swallow so all his feed and medicines go through his gastrostomy button.

Question/Comment: Was very sorry to hear about your baby. We’ve been thinking about you a lot but didn’t want to get in touch as didn’t want to bother you. Answer: No need to feel sorry for us, we have our child that we’ve always wanted and who we love very much. He has brought so much joy into our lives.

Question/Comment: What’s wrong with him?  Answer: He has a rare condition that affects the brain, causes severe epilepsy and global development delay. (It’s a funny question because with strangers or people I don’t know very well, how much detail do I go into? So I just mention the epilepsy and special needs in the hope that is enough to satisfy interest but not boring or scaring them with more info than perhaps they needed!)

Question/Comment: Will he grow out of it? Answer: No, unfortunately not.

Question/Comment: Would an operation help? Can they cure his condition. Answer: No unfortunately not. Samuel is missing a part of his brain & has lissencephaly which refers to the smoothness of his brain. This cannot be repaired or cured.

Question/Comment: I don’t think he likes me/is interested in what’s going on/he looks bored. (This is normally said when someone tries to interact with Sam, but Sam just ignores them)  Answer: (I’m never quite sure what to say here) He isn’t ignoring you really, he has special needs so doesn’t realise that you are chatting to him.

Question/Comment: I heard you were visiting the hospice. I didn’t realise things were that bad. Answer: There are a lot of unknowns with Sam’s condition, particularly as it is very rare. But we do know that he has a life limiting condition and that not only means that he is extremely limited in what he will be able to do, it does also mean that how long we have to enjoy him is very limited too. The hospice offers us great emotional and practical support and respite. They also have fantastic facilities and are great at looking after children like Samuel.

Question/Comment: So will you be having more children? Or when do you think you’ll start trying again for another one? Answer: Err, it’s not quite that straightforward and Sam is only 15 months so we will take our time.
Question/Comment: Does he sleep ok at night? Do you have to stay up with him all  night? Do you get to sleep? Answer: We are lucky in that Sam has a drug called Chloral Hydrate that helps him sleep at night. Doesn’t guarantee he will sleep straight through but helps him settle and gives him more of a night and day. He’s on a feeding pump for 10 hours overnight so is bound to do a massive wee nappy which can disturb him so often get up to do one nappy at night. Much like a lot of ‘normal’ parents I imagine.

Question/Comment: You are very brave. I don’t think my husband and I could cope with a disabled/special needs child.  Answer: Yes you could, you are a parent, you love your child and would do anything for them. You would move heaven and earth to help them. We are just like any other good parents who love their child.

Question/Comment: Is it ok to give him a cuddle? Do I need to hold him a special way? I wont hurt him will I? Answer: Cuddling Sam is just like cuddling a big baby. Just hold him close and if he has a seizure then just hold him tight.

Question/Comment: Oh is he having a little laugh? Answer: No, unfortunately he’s just about to have a seizure.

Maybe I should just give them a link to my blog post about how damn perfect my boy is.

Other random comments we get:

  • Oh my goodness aren’t his eyelashes amazing, why do boys always get stunning eyelashes?!
  • You would never tell anything was wrong with him when he is calm. He just looks so perfect.
  • My friends step-son has Downs Syndrome.
  • Could I have a cuddle with Sam please as I’ve had a stressful day and need a Samuel cuddle to relax me.
  • (Stranger looking at him snoozing or calm in his buggy, normally said when we are in a lift) Ah, sometimes you just want to keep them little like that. Enjoy the peace while you can as they grow up quick and will be running around causing havoc in no time. (My dream would be for Sam to be running around causing havoc!)
But one of my favourite things said to me recently by a stranger has to be, ‘I have to tell you that I think your baby is one of the most beautiful little creatures I’ve ever seen’.
If this post sounds all too familier to you, Kate from The life and Times of Team Kitchen blog wrote a great poem called Staring. Pop over and have a nosey.

Meet the gang

Samuel likes to hang out with some special friends at home.

Edgar and Max hang out with Sam in his nest downstairs. Edgar was given to him by his Auntie T and Max was given to Sam by Auntie C when he was in hospital having his gastrostomy to keep him company.

Grannie Bear was knitted for Sam by, well yes you’ve guessed it, his Grannie and she looked after him when he was in his cot in NICU. Grannie Bear stays up in his cot (also known as The Cottage) and looks after it for when he comes up at bedtime.

Sam and The Gang.The coolest gang around.

Sam with Edgar and Max

Sam with his boys: Edgar (the elephant) and Max (the dog)

Sam with Grannie Bear (in NICU)

On days like these…

Today has been a bit of a frustrating day. It started with me complaining to the manager of our doctors surgery for their continued incompetence with dealing with the boy’s prescriptions. If it wasn’t for the adjacent chemist who have simply been amazing we’d have found ourselves in trouble a number of times.

Because of the strict use-by dates on his medicines we can’t order them too early or we will end up wasting a lot of the medicine to then go on the new bottle. But I always give us and the surgery plenty of time to process the request. But more often than not they make a mistake. They either lose the prescription request (“sorry it’s been left in the drawer” or “sorry it had fallen through the tray and we hadn’t noticed” or just “we don’t know what’s happened to it”), or they change what I’ve requested and put through a request for a medicine that Sam no longer has – thankfully the chemist queried it as he hadn’t had the medicine in quite a while. A number of times we’ve been told that the form was in the pile about to be taken over to the chemist but then I speak to them next day and it hasn’t even been signed off by the doctor! Aaaarrghh!

Anyway, I called the manager, found myself angry one minute then quite emotional, particularly as I said that these weren’t just vitamins, these medicines stop him having a seizure that could kill him.

Then I had a really annoying appointment at the hospital. It wasn’t to do with Sam’s head or seizures in fact it was to do with the other end. When he had his gastrostomy operation they dropped one of his testes but decided not to do the other. They are now talking about doing the other one. I’m not keen. He wees just fine and I really aren’t keen on him having an operation at the moment (especially as we’ve just got over a series of bugs) and will it really make a difference?

The surgeon we met today hadn’t met us before. Had probably only seen Sam’s notes briefly before our appointment. He didn’t understand Sam’s condition. I had to explain. I had to explain Sam’s life expectancy and whether he is expected to reach his teenage years. Bearing in mind 50% of children with Sam’s condition don’t reach one year old, the answer is unlikely. I had to go over this stuff with him more than once. I asked him to go back and discuss this with Samuel’s Neurologist and then come back to me as they are the medical experts. This chaps colleague (who did the previous surgery on Samuel but had been called away today) actually shares an office with Samuel’s Neurologist so the phrase ‘get a room and discuss’ seems a bit daft. Anyway, I was pretty hacked off.

(By the way the surgeon told me right at the end of the appointment that there is a slight risk that if the other testical isn’t dropped into place then it could become cancerous. I’ll just add that to the list of stuff to worry about then.)

But then afterwards I had a coffee with my friend T or Auntie T as I should call her as she is Sam’s other Godmother. On days like these you need a friend like her. I’ve been banging on recently about needing people in ‘our world’ to talk to but I already have a great friend in T. Yes her son J is fine, developing so quickly he’s going to be running about before long and I may be the only person she knows with a disabled/special needs child, but you know, I think she gets it. When we had Sam’s diagnosis and prognosis I rang her to tell her. We both cried on the phone. She demanded that she had to come over and see us and have a cuddle with Sam. She’s always been there and I don’t think I’ve ever really appreciated that. Although she has her little man now and he is quite a handful, it is so obvious how much she loves Sam and relishes being his Godmother.

So although I’ve felt really quite pissed off today I have learned something. Sometimes what you are looking for can be just under your nose. Auntie C and Auntie T, friends for life. x

Day in the life

Well there is no such thing really as a typical day for us. Sam makes up his own rules as he goes along – no two days are ever the same. But we do have to have a rough routine for his medicines so I guess that does keep us organised. This is how we roll:

  • 8am – Turn off the pump feed & flush through with sterilised water.
  • 9am – Give Samuel his medicines with flushes of water in between – Topiramate, Vigabatrin and Clobazam.
  • 11.15am – Give Samuel his reflux medicine (this is a new thing and I’ll talk about this more another time).
  • Noon – Sam starts his pump feed of good old Pepti Junior. After the feed we flush him through with water.
  • 3pm – Sam starts his pump feed of good old Pepti Junior with a sachet of baby Gaviscon mixed in. After the feed we flush him through with water.
  • 6pm – Sam starts his pump feed of good old Pepti Junior with a sachet of baby Gaviscon mixed in. After the feed we flush him through with water.
  • 9pm – Give Samuel his medicines with flushes of water in between – Topiramate, Vigabatrin and Clobazam.
  • 10pm – Give Samuel his bedtime medicine Chloral Hydrate which helps him sleep and flush with water.
  • 10.10pm – Start his overnight pump feed (500ml to run over 10 hours).

This is of course a bog standard day for us. This doesn’t include if Sam has had a bad seizure

Infinity Pump bag

and we’ve had to give him some Midazolam. We medicate if his seizure has been going on for 10 minutes or if he’s clustered and had four/five in 30 minutes. Midazolam is squirted into the cheek and then you rub the cheek to make sure it is absorbed.

Also this doesn’t include if we’ve given him Calpol etc. It still feels strange when we give him a ‘normal’ medicine as we are so used to it being prescription only and doses tightly controlled.

In addition to this I have to do regular care of his gastrostomy button. Turn it every day and give the skin area around the button a good wipe. I also have to replace the water in the button once a week.

Sam’s pump is very portable, we have a nifty rucksack to take it in so he can have a feed anywhere – the boy has eaten in restaurants, cafes, shopping centres, parks and the car.

So there you go. That’s how we roll in our world. Updated to be included as part of the BlogHop #definenormal. Pop over to Just Bring the Chocolate to find out more.

Ketogenic diet?

Just a note to say that Sam’s doctors are looking into the possibility of putting him on a special diet which has been proven to help epilepsy.

I’ve nicked this from Wikipedia:

The ketogenic diet is a high-fat, adequate-protein, low-carbohydrate diet that in medicine is used primarily to treat difficult-to-control (refractory) epilepsy in children. The diet mimics aspects of starvation by forcing the body to burn fats rather than carbohydrates. Normally, the carbohydrates contained in food are converted into glucose, which is then transported around the body and is particularly important in fuelling brain function. However, if there is very little carbohydrate in the diet, the liver converts fat into fatty acids and ketone bodies. The ketone bodies pass into the brain and replace glucose as an energy source. An elevated level of ketone bodies in the blood, a state known as ketosis, leads to a reduction in the frequency of epileptic seizures.

As Sam has a gastrostomy and is tube fed it is much more straight fed then if he ate normally as there is a special made up Ketogenic formula feed available (sounds bit of a nightmare if you eat properly).

The one downside is that it will mean Samuel has to have a short stay in hospital – should only be a few days – just while he starts the new diet. I’m a bit nervous about that as the last time he was in hospital the three of us caught a bad tummy bug, but if there is a chance that it could help him then we aren’t going to hold back.

So watch this space.

UPDATE: We may be starting the diet during the first week of April (2012). Fingers croseed.

New set of wheels

We picked up Sam’s new buggy on Friday, a new ‘special’ buggy. The Occupational Therapist wasn’t happy with how unsuported he was in his old one – we use to have to put in rolled up towels to try and improve his positioning – and he was growing out of it fast.

I think husband and I both felt a bit hesistant at first about it, as yes it does look a bit ‘special needs’ but that lasted about 5 minutes once we saw him in it and how well supported he is.

We took the new buggy out at the weekend but did make the mistake of picking a day when the German market was in town and it was absolutely heaving with people. I did feel extra protective of him as he seems much more exposed in his new buggy and people seemed to be walking really close to us. But we then went for a picnic in the park and set up Sam’s gastrostomy pump and we all ate al fresco. People walked past us and I did notice them looking at Samuel, but they didn’t stare in that awful way, but instead titled their head to one side and smiled. That’s Samuel’s magic, he can touch anyone’s heart.