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!

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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!

A sign?

Sam's war wounds after blood test

Yesterday, I took Samuel into hospital for a blood test which was to be followed by a clinic appointment with Sam’s pediatrician, Dr H. Samuel had to fast for the blood test as it was in relation to starting the ketogenic diet, so we turned off his feeding pump at midnight instead of letting it continue until 8am.

As there was time in between appointments I thought I’d take his pump bag & feed with us so I could give him a quick feed before seeing Dr H. But no, it didn’t work out as planned. I think they were either running late or forgot about us, but we got called in late for the blood test, which took a while to do as his veins aren’t very cooperative, oh yes and they had to pause while he had a seizure.

Anyway, we finally got into see Dr H. I normally arrive to appointments early and am always calm and quite relaxed. But on this occasion I was late and very flustered. And then it hit me. Sam had essentially been fasting for 13 hours and the poor boy was really unsettled and very twitchy (I should add that although I might refer to Sam having ‘twitches’ sometimes, it is in fact all seizure activity).

You may  not think it is surprising that he was unsettled as the poor boy was probably hungry. But you see, the ketogenic diet is a high fact diet, used to mimic starvation. I understand from Dr H that back in good old days, that was how doctors treated epileptic patients, by essentially starving them. And Samuel, well there was no visible change to him whatsoever. In fact, it appeared that we were having quite a bad day. I was thinking about it but chose not to  say anything out loud about it to Dr H. But I didn’t need to. While looking at Samuel as we were chatting, Dr H said what I really didn’t want him to say. That he was concerned that this was a sign that the ketogenic diet might not work for Samuel.

Right from the start when we started talking ketogenic, although extremely keen to start it (anything is worth a try), I have continued to remind myself that it certainly isn’t a cure and not guaranteed to work on everyone. I didn’t really need to remind myself what a tricky customer our boy is. In fact I’ve almost told myself it wont work, just so I’m prepared because I’ve seen the ‘conventional’ epilepsy drugs that have been tried on Samuel not even touch the sides,  as the doctors have put it, his epilepsy ‘is off the scale’.  And I guess because I’m use to preparing for the worst.

Samuel is on three strong medicines, which he has twice a day (this doesn’t include his bedtime medicine to help him sleep or his emergency epileptic medicine). If this diet works, we could take him off one or more of these medicines. If it works it could mean he has far less seizures. Less seizures mean less distress for him, less pain and discomfort. It would give his brain a rest. May give his brain a chance to develop – could he even reach a milestone? Oh my goodness, if it works, I can’t begin to tell you what that could mean for Sam and us. But I can’t let myself get too excited. It may make no difference at all.

But I must remain hopeful. I mean Samuel has already surprised the doctors and proved them wrong. He’s still here isn’t he? He’s still fighting. And I can’t help but think of one day last November (it was a Thursday) when he had bad diarrhoea and on one day when he must have been pretty empty because of his upset stomach, he was so still it was like he was a different child. He was wide awake, but there was no twitching and ‘abnormal movements’.  Auntie C visited that day and couldn’t believe that it was the same boy. So I think we can still have a little hope.

Time will tell. We are hoping to start the diet in April and it becomes apparant if it’s working within the first three months.

I just pray that it does make a difference to Samuel. Even the smallest difference could change everything. So, dear reader, I ask one small thing of you. Say a little prayer for Samuel. Pray that somehow this diet will work for our boy. Because we really need it to. Samuel needs it to.

More information about ketogenic diet:  A useful website to visit to find out more information about the ketogenic diet is matthewsfriends.org and their document about the different types of ketogenic diet is particularly useful.