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a long time and he is on quite a high dose (14mls twice a day) and I think Clobazam and his other drug Vigabatrin have superceded it.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!

Love rules

In our home epilepsy and love rule. Both are so very strong and both feel impossible sometimes to control. Love gives us the strength to fight epilepsy and gives us hope. Without love epilepsy would surely beat us. Epilepsy and love rule this house. But love will always be stronger, no matter what.

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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.

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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!

What colour is your grass?

Is your grass greener than mine? Probably.

I was catching up on blog posts and came across the latest post by Single Dad/Disabled Daughter. The post was called ‘I like my grass brown, thank you very much..’ and he talks about comparing disabilities and looking at how he’d rather his children had less painful, less limiting conditions than they do. He has two children Pearlsky and David, I think both are in their teens and both have extremely life limiting (as in they cannot do anything for themselves) conditions with a bit of epilepsy thrown in for good measure. I may have just done his children a total disservice by not explaining their conditions properly and in detail, so if you want to find out more than have a look at his blog.

I like my grass brown, thank you very much

I seem to have launched a discussion a few posts ago about comparing disabilities when I said “Autistic? Screw you. I’d kill for a kid that was autistic.” Let me take that a bit further …

I would rather be deaf than blind. I don’t think I will ever have a choice, and the exercise is academic, but the fact is, I think one is less onerous than the other. I would rather be a paraplegic than have uncontrolled epilepsy. Hence, I can “rank” disabilities. Would I rather have a child that is autistic or has an in-born error of metabolism of a certain amino acid? I can voice an opinion.

It does get much harder when there are multiple disabilities. Would I rather be deaf with epilepsy or blind and have a g-tube? Now the discussion gets a bit bizarre and I need not go further, except to say that when talking about multiple disabilities, the combinations are infinite and the comparisons are impossible.

I can also say that I would take any disability that is not genetically inheritable for my child than any one that is. Sophie, of A Moon, Worn As If It Had Been a Shell, can walk and has some forms of communication, but has many break-through seizures (and is severely disabled). Pearlsky has no communication, cannot walk, but does not really have break-through seizures much anymore. Would I trade? Again, a moot question and one not easy to answer. BUT, Sophie has two seemingly wonderful brothers, “normal” brothers. Pearlsky, alas has no “normal” sibling. That I would trade. Of that, I am jealous. I chose to marry into a gene pool that unfortunately did not match mine (actually, matched it too well). We all make mistakes … but I would greatly have preferred an non-genetic based disability. David, my son, would have preferred that as well, I am sure.

As parents of “this” population, I agree, we can’t really compare. And yes, we must support each other.

Another reason we cannot compare has a lot has to do with our own level on the universal shit-o-meter. Let’s say that due to my upbringing, beliefs, education, spirituality, experiences, etc. I can handle up to an 8 on the universal shit-o-meter. At that point I collapse into a quivering heap on the floor and need a Pearlsky-tini to continue (three parts VOX vodka, one part Valium (brand, not generic), chilled). You, on the other hand, may reach quivering-heap-on-the-floor status at a 6 on the universal shit-o-meter. Does not make you less of a person? No, just different. So, your 6 is actually equal in pain to you as my 8 is to me. Your life seems easy to me, I wonder why you can’t deal and may envy your situation where you think my life is beyond impossible, but the truth is we both feel an equivalent level of pain, we have the same level of angst.

The point? You should not feel bad that you have it easier and can’t deal, and I should not think you a wimp for not being able to deal with something I would find easy. Life is not comparable like that. Severe disabilities are not comparable. Shit is shit, though.

Yet, I can still legitimately be envious of your problems.

“You and your friends get together. Everyone puts their problems on the table. When you look at them all, you’ll take back your own.” ~ my maternal grandmother, Pearl, and boy do I miss her.

The grass may be greener, but I guess I like mine brown.

Reading through his blog post I totally understood what he was saying. It might not be the right thing to say, but I completely understood. I’m in contact with a lot fantastic parents of children with a diverse range of special needs and disabilities. I read and hear about their problems and difficulties and while I feel sorry for what the child has to go through and for the parent who has to care for them and worry about them, I do harbour this secret feeling that I would give everything I own, every limb of mine for Samuel to have their condition.

Samuel also has a genetic condition, actually to be pedantic, I too have a genetic condition, but Mother Nature decided that I should escape symptoms, but pass them on to my children.

I don’t want those parents to feel guilty that their children in a bizarre way may be ‘better off’ than Samuel. They’ve got a better deal than we have. And I hope that the parents will understand that in no way is my intention to undermine or belittle their child’s condition and the problems that go with it.

But I can’t help feeling the way I do. Samuel doesn’t and will probably never smile, laugh, give eye contact, sit up, walk, doesn’t acknowledge us, cannot eat orally, doesn’t communicate, has daily breakthrough seizures…I’m sure if we were able to do deals, I’d easily find someone who would have a much better condition to have, but would anyone want to trade for what Samuel has? I doubt it.  But this is the hand we’ve been dealt and yes, we may have been dealt a much harder hand than you. But as Disabled Dad says, shit is still shit, whichever way you look at it.

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

Hate

Hate is a strong word. I think we should only use the word with caution. Really think about what we are saying before we say it. But what I’m about to say I mean with every fibre of my being. I hate ARX and I hate epilepsy.

I hate watching what the seizures do to my beloved, precious boy every single day. The way his body stiffens and twists, how he cries sometimes before, during and after. How his lips briefly go blue. How it can take him such a long time to unravel and to come out of it. The fact that sometimes we have to medicate some of his seizures and in effect sedate him. I hate that we have to put all these medicines into his little body every day.

I hate the fact that I feel relieved and lucky that he breathes independently and is fortunate not to have oxygen or a tracheotomy.

I hate watching him snooze peacefully, then be ripped out of his lovely sleep by a seizure.

I hate the damage the seizures must do to the good parts of his brain. I hate the fact that he has seizures that we cannot see. Seizures going on his brain that don’t show physically.

I hate that his epilepsy has been described by his doctor as ‘off the scale’ and the moment a doctor or nurse unfamiliar with him see Samuel move or twitch, they want to medicate him.

I hate the fact that these seizures don’t give his brain a chance to develop and allow him to develop and grow intellectually.

But despite all this, everyday I feel so full of love for my boy, my husband and the little bubble we seem to have created in which we feel safe and normal. I love looking at my beautiful boy and stroking his hands, squidging his cheeks and just generally admiring his gorgeousness. I love Samuel so very much and no damn epilepsy will ever dampen that.

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.

Getting it right

Samuel and I spent the day today with our friends at Julia’s House. I’ve come home feeling knackered, which is probably because I did a lot of nattering (I am a chatterbox to be honest) and having to think even more about Samuel’s epilepsy and trying to explain to the carers and nurses about how we medicate Sam’s seizures.

I’ve talked before in my post Early days about the difficulties in explaining just when is the appropriate time to medicate Samuel with Midazolam (his emergency medicine) and when you just give him a good cuddle and ride it out. It isn’t a perfect science and I’m sure I get it wrong sometimes but it can be so difficult, especially as he really does live up to being a tricky customer!

Samuel started off today really snoozy but then as the day went on he became more unsettled. I was hoping that it might be his teeth giving him agro so I gave him some paracetamol as his cheeks were really pink but that didn’t make much of a difference. He progressed to an afternoon of on and off clustering. If I hadn’t have been there he would have had Midazolam, we all knew that.

But the problem is the grey area within his protocol.  There are some seizures you count, you look at your watch and mentally log, others you just give him a good old cuddle. But should we be ignoring those seizures? Are we pushing the boundaries too much? I think everyone understands that by nature Samuel is a twitchy boy and those twitches (which are in actual fact seizure activity) are inevitable due to the set-up of his brain, but it’s the other things he does, the seizures. How can I expect people to ignore some but count others? We just don’t want him medicated every day which realistically could happen. Samuel has seizures EVERY day.

I think the nurses, well one in particular, could see the difficulty I have and advised me to talk to Sam’s doctors about it to clarify his protocol. The ridiculous thing is I do agree with the written protocol, in fact I co-wrote it with the doctor over the phone BUT it’s about how it works in practice. She said that she felt that it is such a huge responsibility on my shoulders to make sure that they get it right when I leave him in their care.

You see I love Julia’s House, I really do. The whole place, the people, I just love it there. I cannot explain how amazing the nurses and carers are, how kind, lovely and understanding they are. They make me feel normal. Nothing I tell them phases them. I doubt there is much I can tell them that would surprise them or they hadn’t heard before. They must be the best childminders in the world!!! And I can’t begin to tell you just how lovely they are with Samuel. Right from day one, the way they talk to him, stroke him, cuddle him. As his mother who loves him with every grain of my being, watching these people hold him so gently, look after him with so much care. It just makes my heart swell.

If it wasn’t for this situation with his epilepsy and emergency medication I would have left him there on his own after the first day. I trust them, I really do.

We did end up giving him Midazolam in the end. He did need it to break the cycle he’d got himself into. But although it’s always disappointing when he has to have it, I really did feel we achieved something today. They saw what he does. They witnessed what his epilepsy can be like and they saw how much I want him to be awake and alert as much as possible. They saw the difficult situation I’m in, the hard decisions I often have to make about medicating him and I really felt that they were there behind me, supporting me.

Why is no-one cuddling me?

The nurse was keen to make me see that it isn’t a test of me. It’s not about right or wrong or whether we are looking after Samuel correctly. It’s about us all being in the same team and understanding how we can care for Samuel together.

We will get it sorted. We must. I’ve already put forward some dates to have community sits which is where carers come over to the house to look after Samuel. I will be home, just for the time being, but do have every confidence in them, Samuel and me, that we will get there.

There is a sign up in Julia’s House that says ‘Julia’s House is a happy place’ but it is more than that. Julia’s House is a happy, loving, caring, supportive, cuddly, fun, positive, sunny, friendly place. The sun ALWAYS shines at Julia’s House.

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.

Take the rough with the smooth

Well readers, we seem to be having quite a good period at the moment. Very good in fact.

Please don’t get me wrong, Samuel is still having seizures every day. But at the moment he doesn’t seem to be having as many in a day as previously and instead of being quite low, they are quite short and sharp.

We are often asked how many seizures he has in a day and as we don’t keep a log it is an impossible question to answer exactly. But our staple answer is between five and 15. It just depends on what kind of day we are having and often no two days are the same. For no reason, he could have two dramatically different days. It is just the way things are.

When things are going well we average about two weeks in between giving him Buccal Midazolam. Midazolam is his emergency medicine. The dose we give him at the moment is 0.5ml and as it is a buccal medicine you give it orally (the only thing he has orally). You put the syringe into the mouth push it against the inside of the cheek and then squirt. His system absorbs it very quickly, although it can take between five and 10 minutes for him to unravel from the intensity of the seizure and go to sleep.

Samuel’s current emergency plan is that we give him one dose of Midazolam if he’s been having a seizure for over 10 minutes or he has had a cluster (of say about four or five short ones) in half an hour. We can give him two doses in 24 hours but if he needs more than two doses within 24 hours or when he wakes up from the second dose and we are worried we have to take him into hospital. So far we’ve not needed to call an ambulance and only taken him in twice. We have open access at our hospital which means we don’t have to go via A&E, and we can go straight to the children’s assessment ward.

I should mention at this point that an important note on Sam’s emergency plan is cuddles. Yes, it’s true. When he’s having a seizure, giving him a cuddle not only provides him with comfort, we find it can sometimes help to take the edge off the seizure (what the NICU doctors would call containment) and it can also help you feel the seizure. You can feel its intensity and when it starts to slow down.

We often find that Samuel’s worst time is during the evening. Between 7pm and 9pm is what we call the ‘danger zone’ as this is when the level of Sam’s drugs in him has dropped and we are waiting for the evening dose to top him up. He is often extremely unsettled and more likely to have a seizure. BUT over the last few days (possibly the week) he’s said ‘up yours’ to the danger zone and slept through it!

He has been far more settled and we’ve had a lot more settled awake periods which is lovely. Although it is so nice to see him like this, we still have to take one day at a time because although he’s been so good like this recently, it doesn’t mean it will continue. So we enjoy every minute that he’s calm, settled and relaxed and make sure that we remember it as often you can forget what it is like when you hit a really rough patch.

Epilepsy, plus ARX seem to make up their own rules and just when you think ‘yeah, everything is going really, really well’ it pokes you in the eye!

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.

Seizure Vs Fit

I just wanted to make a comment about the word ‘seizure’.

Sam’s pediatrician and neurologist say ‘seizure’.

When my husband and I talk about what Samuel has been up to we mostly use the word ‘fit’. When we discuss Sam’s condition with friends and family we use the word ‘fit’. And most of the nurses and doctors during our recent hospital stay say ‘fit’. This seems to be the word that people find easier to digest and understand and actually my mum said she finds it easier to think of her precious Grandson as having a fit rather than a seizure. Daft I know.

However, speaking to an adult who has epilepsy they said they hated the word ‘fit’ and much preferred it if people said ‘seizure’. And I guess, because of that I feel inclined when talking about it on twitter and on here to say seizure.

I’m not sure really what is right and what is wrong, but to us both words mean exactly the same it is just the habit we’ve got into.

Edit: Just popped back to add that when Sam was in NICU they would refer to him having a seizure as an ‘episode’. We’d come into the unit in the morning to be greeted by a nurse saying, ‘Hi Mum and Dad. Unfortunately Samuel had an ‘episode’ this morning’.

The first post

I am mum to the most special and beautiful boy in the world, Samuel Luke, who was born on 10 Nov 2010. He is the most beautiful baby I’ve seen in my whole life. He has the most amazing blue eyes and the most riduclously long eyelashes any woman would be proud of. My son has an extremely rare genetic disorder called ARX which causes severe epilepsy. Sam spent the first two months of his life in Neonatal Intensive Care, cared for by the amazing doctors and nurses who we owe so much to. He continues to have seizures every day but they are much better than what they were. He is still very much a baby baby, but boy, he does love his cuddles. That’s when you know he is happy when he is having a good long cuddle.Because of his condition, we know that we may not have him for anywhere near as long as we should, that is why we celebrate every day with him. Every day is a beautiful miracle.But if you held him, he may not look at you, but you would feel how much he likes to be cuddled and you would come away realising how much you enjoy cuddles too and perhaps even how much you needed that warmth at that moment.He is our boy Sam. xx