Paraldehyde – the fragrant rescue medicine

Samuel’s rescue medicine has been Midazolam for a very long time. I think most (if not all) of the epilepsy world know about Midazolam. Samuel has had it since the beginning where he was given it several times a day – until his seizure treatment plan was tightened and he wasn’t given it for every single seizure. It’s always worked well. It does the job. If he exceeds his protocol (I will post that on here sometime in case anyone is curious) he gets a sqiurt of the prefilled syringe of Midazolam into his cheek. It generally works. But as Samuel has got older it’s had a more noticeable effect on his breathing and that was why we now have oxygen cylinders at home (and an oxygen protocol).

The other issue with having Midazolam in our armoury is it’s relationship with Clobazam. Now we love Clobazam. It’s been one of Samuel’s epileptic medicines for a long time and it is very effective. He has it twice a day and it is one of his most crucial medicines. But Midaz and Clobazam are almost like sister medicines. Give them too close together and you are at risk of over sedating him (which could effect his breathing), or as they work with the same receptors of the brain, Midazolam can almost dilute Clobazam making it less effective.

Prior to Samuel being admitted to hospital before the summer, he had earned himself Midazolam three days in a row. Not ideal. Think it was a contributing factor to why he got so poorly and had to be admitted.

So this is where Paraldehyde comes in. It was my suggestion to Samuel’s doctor about the possibility of introducing Paraldehyde as either an alternative to or replacement of Midazolam.  Dr H thought it was a good idea (I occasionally do have them) and we agreed that we would trial it to see if we found it effective and it did the job adequately. Well it did, and it is now Samuel’s first rescue medicine (although we will still continue to have a good stock of Midazolam in the cupboard as back up).

Now you might be thinking, ‘why didn’t you have Paraldehyde before given that Midaz is obviously quite a dirty drug?’. Unfortunately you don’t give Paraldeyhyde in quite the same way as Midazolam. It’s given rectally, plus the medicine smells. The moment you open the bottle the room is filled with, let’s just say, a unique smell. Once the bottle has been opened you have to act quickly and fill the syringe (which has a tube attached) and ‘insert’ immediately. You need to be quick as the medicine will block the syringe and wont be useable. On the wards they use to give the medicine in glass syringes but I guess budget cuts and health and safety stopped that and now you’ve just got to use it fast.

It’s not the perfect drug (are any of them?), but it’s the lesser of two evils. Unfortunately given the terrible summer Samuel had I’ve gotten very experienced (and quick) at giving it, so don’t think much of it. The only thing that bugs me about it is the way it makes him smell of it for the next few days. It’s like it fills his pores and makes his breath smell. But it doesn’t stop me going in for a smooch. NOTHING would stop me giving my boy a big fat kiss!

Uncle Midaz

Midazolam has been in our lives since the early days. It has become a very important part of our armoury in the battle against Samuel’s seizures. It is his emergency drug.

The protocol of when to give Midazolam to Samuel is if he’s had a seizure lasting more than 10 minutes or if he’s had a cluster of four or five short ones in half an hour. It is only given for full on focal seizures and not his general twitching.

In the very early days, Samuel had Midazolam a lot. On average he’d have about five doses A DAY! There wasn’t really a clear protocol at that time so he was given it almost as soon as he’d seizure. Problem was, Samuel would seizure a lot and medical staff are trained to zap seizure activity immediately! But the neurologist stepped in and changed the rules. She made us and his hospital team wait. It was hard. I’d be there holding my boy who’d been having a seizure for 8 long minutes and I wasn’t giving him anything. But it worked. It showed us all that more often that not, Samuel will self resolve after about 10 minutes and then be fine.

When the going is good and things are quite balanced, Samuel on average has Midazolam about once every two weeks. Although when the Ketogenic Diet was at it’s best he went without it for about 60 days!

Midazolam is the only thing Samuel has orally. It is squirted into his cheek and absorbed that way. After Samuel has been given it, he can take a bit of time to wind down. Sometimes he will even have another seizure after he’s had it. But then he eventually calms down and relaxes and usually goes off to sleep for about three hours.

Giving Midazolam is at last much easier after the introduction of pre-filled syringes. Before, Midazolam came in a little brown bottle and you would have to push the syringe through the stopper to draw up the Midazolam. This wasn’t always as simple as it sounds. There was always a big label over the bottle making it awkward to see what you were doing, if you’d already used the bottle a couple of times and the liquid was getting low, you’d have to angle the bottle a certain way to ensure you got enough in your syringe. Then you have the problem of air bubbles. When drawing up a medicine, you have to get rid of air bubbles as where air bubbles are, medicine isn’t. Don’t forget this is an emergency medicine. You are trying to do all this as quickly as possible too!

So at last we have pre-filled syringes. All you do is remove the seal of the tube, take out the syringe, break off the tab and it’s all ready to give.

We have a bit of a love & hate relationship with Midazolam. We love the effect it has. How it works. How it stops what is happening. But we hate it too. We hate having to give it. We hate what it symbolises…that Samuel’s had a bad patch.

But the relief you feel when you’ve been watching Samuel seizure for over 10 minutes and then suddenly he stops, relaxes and yawns and goes off to sleep. All is calm. Samuel is safe and content once more. That is why Uncle Midaz is a very important part of Team Sam.

Fats and Fits

TUESDAY

So, day two of our hospital stay. Not quite sure what to say really. We’ve started Samuel on a medicine called Pancrex to help him absorb fats better. He started on 1.55ml yesterday but we’ve cranked up the dose today. So far we’ve not seen a difference. Dietician is still not happy with Samuel’s nappies and quite frankly, as the one changing them, neither am I!

Pancrex is an odd gloopy medicine that I make from a powder and water. It is probably the ugliest medicine I have seen with a very creepy smell.

Seizure wise? They are getting worse. He had Midazolam yesterday afternoon but woke up after a three hour snooze unsettled, which carried on through the evening. At 10pm he had 6ml of Chloral Hydrate and thank goodness for that as he finally relaxed and went off to sleep.

He woke then at 3.30am and was twitchy on and off from then. Today has so far been very strange, his sleeping pattern is very different and he is very twitchy.

We are all hoping that this is to do with his weight loss. He’s not absorbing his fats, so losing weight which is reducing his ketone levels (0.5 today) which could be why his seizures are rapidly increasing. Hopefully if this new medicine works & he puts on weight then we will see an improvement in him. Well that’s what we are hoping and praying for.

I want to end this post all chirpy and upbeat but struggling with that to be honest. It’s obvious that we will be here for most of the week, but at least the nurses, doctors and our poor overworked dietician are all fantastic and all trying their damndest to help Samuel.

WEDNESDAY

After ketones of 0.5 and 0.4 yesterday, it was quite a nice way to start the day with reading of 0.6. Still ridiculously low but at least it’s an increase, if only a slight one.

The dietician had asked me to save her Samuel’s morning dirty nappies (brave woman) and I was pleased to see they looked better. Less, well, fatty. A poo sample is also being sent off to the lab.

Our plan now is to stay in another 48 hours, increase the enzyme medicine some more and keep monitoring his nappies and ketones. It will take a bit of time for us to see a weight gain though.

In the meantime we just have to brace ourselves and use the medicines we have in our armoury to keep the boy comfortable.

So far today has started off much better than yesterday, so I’m feeling cautiously optimistic. We’ve just got to wait and see.

Oh and you may be interested to know that this situation of a child on the ketogenic diet having to use these enzymes is rather unusual. In fact they don’t know of many any in the country it would seem. Why am I not surprised?!

UPDATE: This evening it was discovered (despite numerous different people listening to his chest since he was admitted) that Samuel has a chest infection on his right side. Chest infections are one of the things I fear for Samuel so did panic me initially but he’s been started on a course of antibiotics. Infections can reduce ketones. Is this why his have been so low? Will my son ever stop throwing us curve balls?

THURSDAY

8.30am: Despite having Midazolam at about 4pm yesterday, he ended up having another dose around 9pm. He then settled and had a good night. So far this morning he has been very relaxed and it’s nice to see him so settled even before his morning medicines.

10.30am: It has been suggested that slowing down Samuel’s feed may help his fat absorption. I was asked whether it would work at home having Samuel on a 20 hour continuos pump feed. My response was a big NO, it wouldn’t work. I think the plan may be then to have a very long feed over night and a couple of slightly slower feeds during the day. This should fit in ok hopefully with our daily routine. Well I very much hope so.

9pm: Today has been a much better day. Samuel was very snoozy this morning and although twitchy and did have a number of seizures, he didn’t have any Midazolam and has gone off to sleep on just his usual drugs.

We can also report from Camp Sam that his ketones were surprisingly high this evening, 1.9. Not going to get too excited yet as who knows what is in store for us tomorrow.

FRIDAY

8am: Samuel had a good night overnight, he slept through like a good boy. But, he was put on a saturation monitor overnight and it was showing that his SATs were low so he needed oxygen a lot of the night. I think this will probably mean we will be in hospital for at least another night. Because of the chest infection.

Back to the original reason we came into hospital, we’ve got a new feeding plan which I’m trying to get my head around. It’s confused me as we are reintroducing night feeds and the day feeds are so long (2 hours) I’m not sure what that will be like at home to manage. But if it helps his gut, then who am I to complain!

His ketones were 0.9 this morning so at least they are moving in the right direction.

It’s all a waiting game really.

MONDAY (17 September)

Chest infection: Samuel’s sat levels overnight are causing a headache. They keep sitting at 86 which nurses aren’t happy with and have to put him on oxygen. They want his sats to be in the 90s, preferably above 95. It could be because it’s just Samuel, the medicines he’s on at bedtime, or it’s still a bit of a hangover from his chest infection.

Diet/fats: This weekend Samuel ketones were 0.8 and 0.6 but this morning they were 1.0 which was very pleasing to see. We also weighed him and his weight has gone back up to what it was when he was admitted (it dipped during the week which was thought to be because the antibiotics were causing loose nappies).

The dietician has been to see us and wants to move Samuel into a tailor made version of the ketogenic diet which should be better for his gut. She has also suggested we increase the speed of his feeds as long feeds may affect ketone levels. She will come back later with a new recipient and feed plan.

The only complication is his saturation levels and whether he needs oxygen. That is what is going to keep us in hospital if the doctors are concerned. The gastro doctor is away today but Sam’s paediatrician is due to visit later so hopefully I will be able to report back later with some good (fingers crossed) news.

TUESDAY

I have some good news for you. We had a pow wow late yesterday with Dr H, Samuel’s paediatrician. We agreed last night to halve Chloral and get nurse just to do spot checks of his sats (instead of leaving him hooked up to the monitor all night) and if Samuel behaves himself then we can go home Tuesday/Today.

Well he did behave and wasn’t put on oxygen at all. Our nurse was fab too (but they’ve all been wonderful I must say).

We now just need the dietician to be on the ball today. We are going to move to the new feed recipe of some ketocal & a new concoction. The dietician said she should have everything ready to start at lunchtime. So if we are freed today, it probably won’t be until late afternoon. Or possibly early evening as we will need to get some bits from pharmacy.

So there is light at the end of the tunnel. But I’m not getting too excited yet. Who knows what the day holds. I’ll save my excitement for when we are actually home and my boy is back where he belongs.

3pm: Well, we are being freed!! The dieticians want to have a play with the new concoction we’ll be giving Samuel. So we’ve agreed that we’ll go home this afternoon BUT will come in as a day patient for a few hours tomorrow to start the new recipe and to weigh the boy. We may also have to come in again on Friday but we are going to see what happens. It’s a pain to have to come back in but knowing that we’ll be able to go home afterwards is fine. And if it helps my little boy then…..

A Samuel update

(Warning: This post contains poo references)

It’s been a while since I updated you on how Samuel is doing on the Ketogenic diet, so I thought I’d bring you up to speed.

Right, where to start. Things have been going quite well. Samuel had been having only a few seizures a day, mostly (if not all) in the evening and, I can’t believe I’m saying this, but we’ve had a few (just a few but even one is amazing) seizure-free days. As I write this, Samuel has so far gone 59 days since his last dose of his emergency medicine Midazolam. Pre-Ketogenic diet he averaged 14 days between doses, so we think this is pretty damn good.

Over the last few weeks we had noticed that his ketones had stuck on around 0.8. This is much lower than we’d like but the diet was still having a positive effect so we weren’t worried. The thing to make note of is what Samuel is actually doing rather than what the numbers say.

But, over the last week we’ve noticed more seizures have crept in during the day and although they may be short, they are very sharp and seem to unsettle him quite a bit. We’ve also noticed that he might have lost some weight. Samuel has always been like me, long and lean, but lately we’ve felt that he seemed a bit less podgy in certain areas.

Anyway, we got him weighed and sure enough he’s dropped some weight, which was particularly frustrating as we’ve been trying to fatten him up.

But don’t worry reader, we think we are on to it.

Today we handed in two poo samples to the hospital. One is to check whether Samuel has an infection which could affect weight gain and possibly ketone levels. The second sample is to check for fat globules in his poo – it could be that Samuel is effectively pooing out the fats so therefore he isn’t benefiting from them (this is what the dietician thinks the problem is, especially after seeing the consistency of his poo). If this proves to be the case, they can give Samuel a supplement which will him help him properly absorb the fats.

While we wait for the results, we are changing Samuel’s feed from powder to liquid. It is essentially the same feed, but there is thought that sometimes children who use a powder or liquid feed, tolerate one better than the other, so we are giving it a try. The liquid form of the feeds contains more fibre, so that might add a bit of extra excitement to nappy time!

Samuel’s new liquid feed

Drug wean 1 update – We are in the processing of weaning Samuel off one of his drugs, Topiramate. The wean is going well and sometime mid-September, Samuel should be off the medicine completely. He will still be on two anti-seizure medicines but the fantastic thing is that for the first time we wont be replacing this drug with another seizure medicine.

UPDATE: Since posting this, Samuel’s seizures have got worse and we had to give him Midazolam on Friday afternoon, ending his fantastic 60 day run. His poo nappies have got worse too, so we are hoping that once the lab results come back we can then start him on the supplement to help him absorb his fats better.

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!

Our nest

I love our home. We’ve lived here for five years now and after a dodgy couple of years of living next door to the neighbours from hell (which saw us having to appear in Magistrate Court to act as witness for their landlord who was desperately trying to evict them), we’ve really fallen in love with our little nest.

We also live two doors down from our Guardian Angel Auntie C.

Our house is a small three-bedroom end of terrace. We have a sweet little garden that we are desperately trying to make into something that we can really enjoy and take Samuel out into for picnics, swishing about in the paddling pool, cuddles and sensory play. I also need to figure out how we can squeeze in one of those fabulous basket nest swings.

Although it has been a lovely home I’ve started to think about the future. For a long time I’ve avoided thinking of the future – there was a time that we didn’t think we’d get Sam home from NICU. What about his first birthday? Second Christmas? But he made it. So I’m starting to allow myself to think that maybe we will be blessed for quite a while, despite his prognosis.

Samuel is getting a big boy now. A big and long boy. I can still carry him up and down the stairs but I must admit it’s a relief when I put him down – I’m not the strongest chick on the block and it is quite awkward as he cannot support his head or neck and when you carry him, he doesn’t cooperate or hold on. Our staircase is quite narrow and it’s tricky when he has a seizure while we are going up or down the stairs.

He is only 19 months (yes world, my boy has reached 19 months!) but I’ve started to think about whether we need to make some changes to the house to make everything easier and safer for us all, but especially Samuel. Being an end of terrace, I’ve been wondering about whether we can extend out the side. My dream would be to have a double-decker extension but have a feeling that may be hugely out of our budget! But I would love to make his room into an amazing sensory room.

I want to make our house beautiful. I want it to feel like a perfect family home for all of us. We have started to talk more about extending our family and are seriously looking at the possibility at our options for having more. So it would be amazing for us to make our home into something amazing for other children who join our family.

We’ll get there. Somehow. But for all the space or beautiful design this house may lack, it’s full to the brim of love.

(If there are any genies reading this and in the mood to grant some wishes, we could really do with a new kitchen too! x)

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.

Zzzz

One question we are asked a lot is about Samuel’s bedtime. ‘Does Samuel sleep well at bedtime?’ This is often asked by other parents, especially when they see Samuel in a very long, deep sleep and I think they worry that he wont sleep for us at bedtime.

But he sleeps very well at bedtime and that is because we cheat. At 10pm each evening Samuel has a medicine called Chloral Hydrate which we call his sleepy drug. It is also thought that Chloral Hydrate has some anticonvulsant properties which of course is an extra bonus.

He’s been on this medicine since he was around two months old as it was felt that he didn’t have a proper sense of night and day. He is only on a small dose of the medicine (about half of what he could have for his weight) but it is very effective. He still does wake in the night if his seizures are very bad, he has a wet or dirty nappy that has disturbed him or he is unwell, but generally our nights are very good.

With the Ketogenic Diet being effective now, I feel a bit more confident about looking at weaning Samuel (and us!) off Chloral Hydrate as he may be better now at night and perhaps he doesn’t actually need it anymore. But I will still keep it in our armoury, just in case!

If you don’t understand about Samuel’s condition this may surprise you but he stays up with us until we go to bed. He is not really aware of the world around him and isn’t disturbed by noise or light so he snoozes with us in the lounge until we decide to go to bed. That way his Dad (who misses him dreadfully when he is at work) gets extra cuddle time in the evening and I suppose is a habit we’ve got into.

Samuel moved in to his own bedroom on his first birthday. Before then he slept in his cot next to our bed on my side of the room and I’d often shuffle down the bed so I could sleep while holding his hand or just lay there staring at him. We both found it extremely difficult to come to the decision to move him out into his own room, but my father bought us a video monitor and that made things much easier (I do have the volume up very high and still lay in bed staring at him during the night!). We’ve so far (will just quickly touch everything in sight that may be wooden) not had any problems with Samuel’s breathing, so feel that he is safe in his own room.

Samuel can snooze anywhere!

Another little achievement we made is taking Samuel off his saturation monitor. Saturation monitors are used to monitor heart rate and oxygen in the blood. This has always been a frustrating piece of equipment as they aren’t designed for children who twitch and jerk a lot and it would often have a poor trace and give an inaccurate reading. There were times that I would notice that it didn’t seem to react when Sam had a seizure but would alarm when he cried afterwards! I told Samuel’s pediatrician that I was going to stop using the monitor and he said that he was very happy for us to do so as we have so far not had any problems with his saturation levels or his breathing. We are keeping the monitor though as we may wish to use it when he is poorly.

I wonder if you are reading this thinking that this all sounds so very alien to you. Quite bizarre in fact. But to us it is normal and works very well. Samuel is like a very little baby, so isn’t distracted by things that other 18 month old children would be. But anyway, what matters is it works and that is all that matters.

This post was inspired by Little Mamma Said and her post Bedtime. Please do pop over and have a read.

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.

Celebrate: The diet dream has come true

I’ve been debating all morning about whether to publish this post. I’m not sure why. I mean do I really think I’ll be tempting fate or am I worried I’m just wrong? But I really don’t think I am.

I think I need to finally say this out loud because everyone around me is saying it and perhaps I just need to get a grip and say it myself.

So here goes,

the ketogenic diet is working.

There I said it. It shouldn’t be working quite as effectively as it is. His ketones are very low and not in the therapeutic range where you could say he is in ketosis. But the diet is working. It seems that Samuel is as usual making up his own rules once again.

It’s not just me that has noticed a difference in Samuel. He spent a whole day last week at Julia’s House and they were all in agreement that he was far more settled and barely displayed any of his normal twitchy behaviour. His Dad, who only seems him during evenings and weekends because of work has barely seen him seizure at all over the last couple of weeks. I’m keeping a note of all his seizure activity and it tells me that he is only having about three seizures a day and they are all quite short ranging from about 30 seconds to just over two minutes. For Samuel that is incredible.

The diet is working. It’s only a month in and I am saying out loud. The diet is working.

To spend the day with Samuel and not have to sit and watch him regularly seizure, not have to see his little body regularly twist and stiffen as he cries, not have to keep an eye on the clock in case I have to start thinking of getting out his emergency medicine – it’s a luxury, a dream, a wonderful feeling that I can’t explain.

We still have a couple of months to go on the trial before it is agreed that Samuel stays on the diet long-term. But a month in, things are looking good, very good.  My dear reader I will say it one more time. The ketogenic diet is bloody working. But if it can have this effect when his ketones are low, what can we expect when they are high? That thought just makes my heart flutter.

The Ketogenic diet is a high fat and protein but low carbohydrate diet that is used to treat difficult to control 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 moved 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.

More information: If you want to find out more, 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 (and explain things much better than I do!).

This post is part of the Celebrate Blogging Challenge. This world of special needs and disabilities has many rocky roads, we will find ourselves on many rollercoasters along the way. But, there are a lot of positives. Through our adventures so far, we have met a lot of amazing, supportive people. It has made us stronger. We have learned to look at the world through new, wider eyes and find ourselves celebrating what to others may seem such a small achievement but to us, it’s the world. It’s a tough world, but there is so much to celebrate. Our children. So please join me each week and post about what there is to celebrate in your world during the past week. I’m looking forward to reading your posts and celebrating with you.

Share your celebration post below and then grab the Celebrate Blogging Challenge badge for your blog! Don’t forget to tweet about it too using #celebratebloghop

Click here to enter your link and view this Linky Tools list…

My previous ‘Celebrate’ blog hop posts:

• Celebrate: A day to remember
• Celebrate: 10 things
• Celebrate: Ketogenic Diet begins
• Celebrate: The ultimate milestone

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.

Celebrate: A day to remember

What happened (or not happened) yesterday may be just a one-off, never to be repeated again. It may have just been a miraculous day, coincidence, a fluke, just a bloody good day.

Samuel had NO seizures yesterday. None. Samuel can have something like anywhere between 5 and twenty seizures a day. Yesterday, Samuel had none. He has had seizures every single day of his life. Until yesterday.

Is it the ketogenic diet? Did the seizure monster just decide to have the day off yesterday?

Yes, I must admit he was a bit grumpy for a lot of the day, which I’m assuming is teething. Again. But no seizures?

What happened yesterday may never be repeated. Today, tomorrow we may have a terrible day with seizures in full force. But even if that is the case, I will always appreciate and celebrate our day yesterday.

The Ketogenic diet is a high fat and protein but low carbohydrate diet that is used to treat difficult to control 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 moved 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.

More information: If you want to find out more, 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 (and explain things much better than I do!).

This post is part of the Celebrate Blogging Challenge. This world of special needs and disabilities has many rocky roads, we will find ourselves on many rollercoasters along the way. But, there are a lot of positives. Through our adventures so far, we have met a lot of amazing, supportive people. It has made us stronger. We have learned to look at the world through new, wider eyes and find ourselves celebrating what to others may seem such a small achievement but to us, it’s the world. It’s a tough world, but there is so much to celebrate. Our children. So please join me each week and post about what there is to celebrate in your world during the past week. I’m looking forward to reading your posts and celebrating with you.

Share your celebration post below and then grab the Celebrate Blogging Challenge badge for your blog! Don’t forget to tweet about it too using #celebratebloghop

Click here to enter your link and view this Linky Tools list…

My previous ‘Celebrate’ blog hop posts:

• Celebrate: 10 things
• Celebrate: Ketogenic Diet begins
• Celebrate: The ultimate milestone

Keep calm and ask Mum

As you know we are in hospital for Samuel to start the Ketogenic Diet. I wrote in a previous blog post, Tricky Customer, that I was mainly nervous about the doctors and nurses and their approach to Samuel’s epilepsy.

I’m all too aware that Samuel has unusual epilepsy and that you don’t medicate all his seizure activity (because that would mean you’d be sedating him every single day when he does usually come out of the seizure by himself). But nurses and doctors are trained to resolve seizure activity as soon as possible.

To add to it all, Samuel didn’t have a great start to the week. We came into hospital on Monday and that evening we gave him Midazolam. But we, as in his Dad and I, decided to give it to him. He also had another dose on Wednesday which was given under my instruction.

And that’s been the theme for this week. Everyone has been told to follow our lead and it is up to us to decide when to give the emergency medication. In fact we’ve been pretty much left to it. I’m doing everything for Samuel as I normally do, doing all his medicines and feed plus managing his seizures. They’ve not even taken his medicines from us, they are here in the room with us for me to use when we need to.

The nurses are lovely as usual and very interested in Samuel and I’ve told them all about him. They notice his twitches and jerks and I explain that they are normal for Samuel and they tell him that he’s a brave boy and stroke his hair.

Being honest I think some of the nurses are relieved they don’t have to make any judgement calls and it can all be left up to Mum. I don’t mind. I don’t mind at all. It’s my job after all and although I might not be trained or be able to do lots of complicated nursing and medical stuff, I’m the best nurse Samuel will ever have.

Tricky customer

Samuel is going into hospital on Monday as an inpatient to start the ketogenic diet. I like our hospital, well as much as anyone likes a hospital. I’m use to it. We got there a lot for appointments or to pick up something – like tubes or new feed. To me it’s just a building where they treat poorly people. The doctors and nurses I’ve met there are really nice. The nurses always seem to fall in love with Samuel quite quickly and are really kind and gentle with him. Also, we are very lucky that Samuel’s pediatrician is wonderful (I’m a bit of a fan of his).

But I must admit I am nervous about going in next week. Samuel isn’t having any invasive procedures, he’s not going under the knife or being given general anesthetic. But I still am nervous.

I’m nervous because of his seizures. As the pediatrician put it, Samuel has off the scale epilepsy. Doctors and nurses are trained, are programmed, to treat seizure activity when it occurs. But you don’t do that with Samuel. He has seizures every day, so you would be sedating him every day. As I’ve said before, some seizures you just let happen, you cuddle Samuel and hold on for the bumpy ride until he’s settled again. Sometimes he twitches a lot. That is seizure activity. It unnerves doctors.

Last year we had to take Samuel into hospital because of his seizures (Better late than never). The doctor was a really nice guy. But I almost had to wrestle the emergency seizure medicine off him. We were having a rest in the parents room when the nurse called us to say that they might have to medicate Sam. As we approached the cot the doctor was prepping Samuel’s canula to give him some intravenous medicine. Samuel was twitching. Just twitching. The doctor said that he had seen what Samuel had done the night before (the previous evening was a very rough ride for us all) and said he anticipated that Samuel was about to do the same. I said no. That’s not how we treat his epilepsy. We have to wait and let it become something before we medicate, we don’t medicate just in case (that would mean he’d be medicated every day). I asked the doctor for five minutes more, just give him five minutes. And he did. I got Sam out of his cot and we had a cuddle. The twitches, the ‘seizure activity’ stopped. He settled. Oh yeah, he then went off to sleep.

When we see new doctors, doctors that haven’t met Samuel before, there are always the same questions. Now I know doctors have to ask these questions, it would probably be wrong if they didn’t. But I just find it difficult when the doctors first meet Samuel and are still looking at him through ‘textbook epilepsy’ eyes (I do of course appreciate that there isn’t textbook epilepsy):

• How many seizures does he have in a day?
• You realise that this is seizure activity? (Pointing at the twitchy, jerky movements that I haven’t appeared to acknowledge. But I always know every movement my boy does, I just don’t make a song and dance about it every time)
• What do his other seizures look like?
• How long do they normally go on for?
• Is this normal for Samuel? And this is the question I do like because I respond by very enthusiastically saying ‘YES! This is very normal for Samuel.’

And that’s it, what Samuel does is normal for Samuel. It isn’t normal outside of our bubble, but in his bubble it is what he does. He has an abnormal brain, that is why every EEG he has had has always come back the same. As we always say it ‘abnormal, but normal for Samuel’.

The other thing that although doesn’t annoy me, I do find strange is the enthusiastic wafting around of oxygen. Samuel doesn’t have oxygen at home. We have never had oxygen at home. When we came home from NICU we came home with a barrel full of medicines but no oxygen. It was always felt by the doctors in NICU that oxygen didn’t really help. He’d either come out of the seizure by himself or once we’ve given him emergency medicine. This really surprises people but his body seems to cope. with one type of his seizures, when he first starts, his lips very briefly go blue, but then they return to normal. I do thank our lucky stars every day (yes readers, we are extremely lucky) that Samuel doesn’t need help breathing and know that in the future it might all change. But for now it’s a nice feeling I can tell you, knowing your child can breathe independently and not require help.

So when the nurses say ‘shall we give him some oxygen?’ I always respond with ‘well you are very welcome to but we don’t have it at home’ And they do give him a big old waft of oxygen.

Samuel’s pediatrician, have I mentioned I’m a fan? will be around in the background and he is very in tune with our boy, so I do keep reminding myself of that which makes me feel more relaxed. I know he will do everything he can to ensure that the doctors on shift understand as much as anyone can about the quirks of Samuel and what to do and when. And hopefully the doctors and nurses will quickly learn the mantra of: ‘KEEP CALM AND ASK MUM’

I’m not sure the point of this post. But I think it just confirms that for nurses and doctors, Samuel is most definitely a tricky customer. I probably should get him a t-shirt made with that on!

All we’ve ever known

I’ve been reading more of the #definenormal blog posts which are part of the Define Normal blog hop challenge at Just Bring The Chocolate. I can relate to all of them in some way and to me, they sound all very, well, normal.

And that’s the thing. I’m so used to our little world, our little bubble, that sometimes I struggle to step back and realise what isn’t perhaps the mainstream normal.

It might be because Samuel is our only child. This is our only experience of being parents & having children. This is all we know.

Samuel has always had seizures. Medicines have always been a crucial part of Samuel’s care.

Samuel has never given us eye contact. He has never smiled. Never laughed. The only time we see him smile is when he is having a seizure and boy he would have one fantastic cheeky smile. But we’ve never had that so it doesn’t feel strange. But it does make me feel sad.

We get asked if Samuel likes this or likes that. We don’t know. How do you tell if a six week baby likes something? Sam might be 17 months old but is only really like a little baby.

But that is how he has always been. This is our normal.

Believer in hope

We finally have a date for Samuel to go into hospital to start the ketogenic diet. Assuming that he doesn’t suddenly come down with a bug and that there is a bed available, we are booked to go in on 23 April.

If you have not heard of the ketogenic diet, well it is a high-fat, adequate-protein, low-carbohydrate diet that in medicine is used primarily to treat difficult-to-control 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.

I have butterflies in my stomach. I so want this diet to work. To have a positive effect on Samuel. Even at the very least if it means that he just comes off one medicine, that would be fantastic. But I know for some children it has done so much more and made a huge difference to them. I know of a little girl that belongs to Samuel’s hospice and by a year after she started the diet she had come off all her medicines. The diet has made a huge difference to her in other ways too.

The idea of it having such an impact on Samuel, well I can’t put into words what that would mean to all of us. Could it mean that we come of one/some/all of his medicines? Would it allow him to develop – could he finally reach a milestone? Will he be almost/totally seizure free?

Samuel’s doctor has told us that they give the diet three months to see whether it is working. Some children show an improvement very quickly, while some show a slower response. If after three months sufficient ketones are being maintained but there have been no beneficial changes then the diet is stopped. For Samuel it would mean that the doctors return to the drug book and review the cocktail he is on and possibly take him off one and add a new medicine to our drug box.

I hope with every part of me, I pray, oh goodness do I pray and have everything crossed that this does work for our boy. If this works reader, oh my god, if this works it could change everything.

Please hope and pray with us. Team Sam..x

For more information about the ketogenic diet visit www.matthewsfriends.org