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.

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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.

Story of survival

When Samuel was in NICU we would regularly have a nurse called K. I really liked her, she had a great sense of humour (which really helped given the situation) and she was very kind and seemed to be very fond of Sam.

I think we’d been in NICU two or three weeks when the head consultant Dr MK asked us to have a chat with him. He told us that they were becoming increasingly concerned about Samuel’s condition and the fact they were finding his seizures so hard to control. At that point he was on a Midazolam infusion and totally out of it. Dr MK told us that we needed to prepare ourselves for the possibility that Samuel would not come home. Ever. We would either lose him to the seizures or that they would be so impossible to treat he would have to be permanently sedated and remain in hospital indefinitely. As you can imagine our already shaky world crumbled.

My husband got angry. Not at the doctor but at the unfairness of it all. At the women, pregnant women he saw outside the maternity unit in their dressing gowns smoking. We were both very emotional. We went back out to Samuel’s cot and K sat with us for quite a while before telling us her story.

She hesitated at first, I think she was unsure whether it was the right thing to do. But I’m so glad she did. She told us of her baby boy J, who had been born 24 years previously. He had a rare brain disorder, I can’t remember if she told me what it was or whether he had a diagnosis,  but she said it was bad. His brain was in a worse situation than Sam’s. He also had a severe cleft palate and in effect was missing his top lip. She was allowed to take him home, but everyone knew it was for ‘family time’ before he passed away. It was almost certain that her boy wouldn’t make it. If I remember correctly, he lived for around 8 months.

As she told me this story I kept staring at her thinking, but how are you here, walking, talking, how have you made it through? But the point is, she did. Her experience led her into nursing, she was a hairdresser when she had J, but after what she went through she wanted to help other babies, so she became a neonatal nurse.

K told us that things we said in the meeting with the doctor, the things that my husband had got angry about, she had said the same things herself all those years ago. She told me that I must not feel guilty, however hard it is, I must not feel guilty. She said that I must always remember that I brought Samuel into this world and whatever happens I will give him a life full of love.

Throughout our time in NICU when things got really bad and I had really dark thoughts I kept reminding myself that K’s been through this and she survived. She somehow made it through and she had worse odds than we did.

The fact that she wanted to help other poorly babies made me admire her even more. I don’t think I’ve got it in me to do that. Being a NICU nurse you see just how damn cruel nature can be. Our friend Auntie C, also a NICU nurse, has told me many stories of children she’s nursed and the different conditions. I don’t think I could deal with that cruelty every day.

When we finally got Samuel home, my husband and I made a deal. That we would truly treasure Samuel. We would enjoy every moment and celebrate the boy he is and try not to mourn the boy he will never be. We made that pact in memory of J.

Although he has surprised the doctors that he’s still here and seems to be doing ok, we know that we will outlive Samuel. He wont make old bones. But we’ve got this far and he is a fighter.

I know there will be parents reading this that have lost their babies. Samuel is 16 months and maybe their little ones didn’t make it that far. But I promise each and every one of you that we hold and squeeze him so tightly every day and know how lucky we are to still have him here with us.

Whatever happens in the future, somehow we will survive. We have to, as Samuel will always be here in our hearts.

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.

Early days

Well we’ve had four sessions now at Julia’s House and I do really feel that bringing them into our lives is certainly going to be really positive for us.

For three of those days Samuel slept for most of the day (which he is doing now as I write) so the carers didn’t really have a chance to play with him. One of the sessions he was much more awake, but very unsettled with some bigish seizures thrown in, so we didn’t think he was in the right mood for play time.

But I do think they are getting to know him. The only problem is (and I need to keep reminding myself that it is still early days) that to be able to leave him with them, they need to see and understand which of his seizures we medicate and which we just cuddle him through.

There is a protocol, which is a good protocol, but when it comes down to it, it isn’t helpful for a child like Sam who has a lot of seizures. So the protocol says to medicate when he’s been having a seizure for 10 minutes. The problem is, Samuel has a lot of seizures. When he’s twitching, that is actually a seizure. So there are some things he does that we don’t medicate, that we just have to overlook.

There are some seizures, even though he’d been doing it for several minutes, where I would just scoop him up onto the sofa with me and we’d cuddle for however long he needed. It wasn’t a seizure that particularly would worry me and in fact in our language at home we’d probably say that he was unsettled and having a bit of a fuss.

Apart from leaving him with Auntie C a few times for no longer than a couple of hours, I’ve never left Samuel since he came home from hospital. It has always been his dad or I with him (mainly me). I’ve never had to explain it before in quite such detail. This is different to when I’m discussing Sam’s condition and seizures to his doctors. This is so I can leave him confident that his new carers understand when to get the Midazolam out and when just to give him a good squeeze.

Because Julia’s House are booked up with training over the next couple of weeks, we’ve not got a session again until next month. But I’ve got a plan. With the use of our little video camera, I’m going to film Samuel to show what we do medicate and what we just cuddle through. I think this might help me show the nurses and carers because I have found it really quite difficult to explain and worry that I’ll just never be able to articulate it correctly. But I keep reminding myself, it’s still early days.

They are more than happy for me to stay with Samuel when he has his sessions and I can go off into another room and have a read or a coffee if I want some space. But I like being around the carers and seeing the other children play (I could write a whole post about how adorable the other children are) and watching people chat to Samuel and admire his gorgeousness.

They make me feel normal.

It is so nice to be around people who will chat and cuddle Samuel and aren’t put off by him not obviously reacting to them. They stroke his hand and chat to him about what the weather is like, what the other children are doing, they tell him that they like his shoes and his new jumper. They hold him tightly when he has a seizure and tell him that it will be alright, he will be ok. Very quickly they have become Samuel’s friends.

I know we’ll get there in time. After all, it’s still early days.

 

Related posts:

What is ARX?

Samuel’s condition is called ARX or Aristaless Related Homeobox. Although his neurologist suspected for quite some time that he had ARX, we got the confirmed diagnosis in April 2011. As with a lot of conditions there is a spectrum, from mild to most severe cases – Sam, unfortunately, is at the most severe end. This is based I think on the severity of his seizures and that they are so hard to treat.

Samuel’s particular form of the condition is called XLAG (an awful name, as is often the case for rare conditions) which stands for x-linked lissencephaly, ambiguous genitalia, absent corpus callosum.

The ARX gene is located on the X-chromosome which is where the name of the condition comes from. All of us have 22 pairs of standard chromosomes and then a final pair that are called our sex chromosomes. Girls have two X chromosomes and boys have one X chromosome with a smaller Y chromosome. The Y chromosome only really contains genes that turn that person into a male, but the X chromosome contains many different genes performing all sorts of functions around the body. Boys are more vulnerable than girls when it comes to changes on the X chromosome, as clearly girls have two copies of all the genes present, whereas boys only have one.

Mutations within the ARX gene in themselves are rare and the phenotype of X-linked lissencephaly with ambiguous genitalia (this is the medical phrase but in Sam’s case it means he has small genitalia and his testes had not descended at birth) is rarer still.

In some genetic documentation we have read, it says that 50% die with the first year. The problem is that the professionals are basing their information on such a small number of case studies and no condition, especially ARX, is particularly ‘textbook’. There are very few case reports in medical literature and although there is a very limited life expectancy in these groups of boys on the more severe end of the spectrum, Samuel’s neurologist has said that they cannot be definitive regarding a life expectancy.

How does it affect Sam? Well Samuel has global development delay. At over a year old he is still very much a little baby and has made no development progress. He cannot support his own neck or head. He has several seizures every day and the number of these can range, depending on whether we are having a good day, from five a day to 15.

He has poor swallow co-ordination (despite swallowing his own saliva, never having needed suction and not drooling) so takes no food orally and is instead fed through a gastrostomy button on his stomach.

Find out more about ARX

 

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If you meet Samuel

If you were to meet Samuel, I’d ask just a few things of you.

Whether he is sleeping, awake, or even on his food pump, come over to him and hold his hand. Samuel is good at holding hands. Stroke his hands and look at his beautiful little fingers.

Look into his big beautiful blue eyes and if he’s having a snooze, look at his beautiful long dark eyelashes.

Stroke his hair and feel how soft it is and see what a lovely blonde colour it is.

Talk to him. Tell him about yourself. He may not obviously react to you, but I’m sure he would know you were there. Chat to him.

If you can, give him a cuddle. Scoop him up and bring him into you. Listen to him purr as he leans into you. Feel the warmth of his cuddly little body.

If he has a seizure while he is in your arms, just pull him in closer to you. Let him feel safe and loved. The is the best of all his medicines.

If you get to have a Samuel cuddle, you’ll quickly understand how powerful his cuddles truly are and I promise you, he will touch your heart.

But please don’t look at him with sadness. He is so very loved and I believe in his own way he is a very content and happy little fellow.

 

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!

The guy in the sky

I don’t consider myself to be a particularly religious person. I did go to Catholic schools when I was younger, but my parents (especially my Dad) did leave it open to us to decide what we believed in.

I do believe in God. I can’t help it, it’s a feeling in my gut that I can’t control. I must believe in God because I get angry at Him (in my view God is more likely to be female being a creator and all that but for ease in this instance I will refer to him as male), I pray to Him – there have been times I’ve been desperate over Samuel’s condition where I have literally got down on my hands and knees and prayed. That must mean I believe.

You look at children like Samuel and those that go to Julia’s House and you wonder how nature, how God can be so cruel. How on earth can this be allowed to happen. But then you look at Samuel’s gorgeous eyes, the pleasure he gives us, the love we have for him, the goodness he brings out in people and wonder whether there is a reason for this. I am angry with God. But at the same time I’m so grateful to have Samuel and thankful that he’s my boy.

I don’t think it does any harm to believe in something like God. What if I’m wrong, what’s the worst that can happen? I mean if there isn’t anything then I’m not going to know that am I after I’ve popped my clogs?!

I don’t go to church regularly at all. I don’t feel connected to God in church. For me, my church is my home. My home is full of love, joy and laughter. People come over to see how we are and to see Samuel and for his cuddles. That’s my kind of church. Perhaps it is being a Catholic and the sedateness of Catholic Mass, sitting amongst strangers. When I do go I often look around and wonder whether the regular church goers, the ones that come to church religiously (excuse the pun) are actually good, gold hearted people. Just going to church every week doesn’t make you a good person. I’m probably surrounded by Christeningpeople who have their prejudices, who judge people who may live their lives differently.

When Sam came home from NICU we got him Christened. Not in case we lost him, but to give him all his chances. To make sure everyone was in his team. I’ve been a union member all my working life and like to cover all corners, just in case. We’d got to know the Chaplin in hospital, he’d pop over to NICU for a chat and to see Sam. He kindly agreed to Christen Samuel in our own home. So yes, I suppose my home really is like a church.

I think I need to believe in God because I need Heaven. My boy will have a much shorter life than he should. I need to know that the end, isn’t really the end. I like to think that when my time comes and I walk up to the pearly gates, there will be my boy waiting for me and I’ll be with him again.

My mum doesn’t go to church very regularly either, but she’s very spiritual. When we realised about Sam’s condition she looked up various Saints to pray to and keeps a photo of him by her bed with her rosary. My mum always says that because Samuel is so pure, so innocent he is like a Saint. She wrote to the nuns that taught her at her boarding school and they promised that they would pray for him. So my boy even has a group of nuns on his team!

When I pray to God for Samuel, I don’t ask him for a miracle, although I’m assuming He Sam and his mumknows that I wouldn’t mind if he did see it appropriate to perform one! To replace Sam’s missing Corpus Callosum. To stop the seizures, to let Sam start to reach development milestones, I would give anything and everything for a miracle like that. But all I ask is that Samuel is content and comfortable in his little world. We judge happiness on big smiles and laughter, but I think in his own little way, Samuel is a happy boy. And that is all we want.

It’s all just words

Just a quick note to answer a question someone sent me.

I’ve been asked why is my blog called Tricky Customer. Well, as the advert says, it does exactly what it says on the tin. When he was in NICU, Samuel was often reffered to as a tricky customer, because that was what he was. The doctors were quite literally scratching their heads trying to figure what to do and how to control his seizures while working through the drug book. He was anything but a straightforward patient and his seizures and condition was nothing that they had seen before.

Also while I’m here I thought I’d mention that I previously did a blog post called Seizure vs Fit where I explained how and when we use these two words. But when Sam was in NICU the nurses and doctors very rarely used these words and instead it was always ‘episode’. ‘Samuel had another episode this morning’. I found it quite strange but it seemed to be the language that was used. Interestingly I noticed that when a couple of the premature babies in neighbouring cots had breathing problems and needed urgent help, these instances would be refered to as ‘episodes’ too.

But in the end, they are all just words aren’t they?

 

New chapter

You know the advert where the family have booked a package, all-inclusive foreign holiday and throughout their journey right up to and including arriving at the hotel they are treated like VIPs?

Well I had that feeling today. That lovely fuzzy feeling where everyone is so very pleased you are there and they’ve been really looking forward to meet you.

But we weren’t on holiday, we were at Julia’s House. It was our very first session there this morning and it was wonderful. When we came in all the carers and nurses appeared and said hello and were keen to meet Samuel (and I). I kept hearing, ‘Oh is this the lovely Samuel, can I give him a cuddle?’. The whole morning session was spent with him being cuddled by different people who were all just so lovely.

The place does not at all feel like a hospice. It is very warm and homely and the bedrooms are really cute and cosy. We were given a quick tour and then I was made a lovely cup of tea and sat down with a lady called Pam who will be one of Sam’s carers and we just had a lovely chat about Samuel and our world. I think most if not all of what I said she had heard before which I found very comforting. We also met Ashley who will be another one of Sam’s carers, he was very sweet with him and didn’t seem to mind when Sam farted and burped on him which I thought was a good sign!

There were a couple of other children there (who were very sweet) whose parents weren’t there and I was interested to watch the nurses and carers play and interact with them. They were really kind and made a huge effort to make things fun and interesting.

Even very limited children like Samuel get involved with arts, crafts, baking (!), gardening and all sorts. I’ve been warned that my fridge will soon be covered in pictures made by Samuel. There is a fantastic play lady too who gets really involved and puts together an individual play plan for the child, I just hope that Sam doesn’t sleep through his play dates with her!

It was so nice to see them enjoy Samuel and take pleasure in cuddles with him (his cuddles are fantastic I must say). The lead nurse even said about how cuddles were part of his emergency care plan which she’s not come across before, but thought was fantastic.

I was only there for about three and a half hours but I could hear a little voice in my head say ‘Yes I trust these people, I really do.’

My only reason why I feel that it may take a few sessions before I’m happy to leave him there on his own is because I want to feel that they do completely understand his seizures (as much as anyone can understand his seizures!) and I’m confident that they know when to medicate and when just to cuddle him.

But I can’t explain the feeling I had in my chest as we left.

For quite a while I’ve very much wanted to be part of the Julia’s House community, to meet other parents like us, to be part of a world where we are normal. A world where there isn’t anything we say, or anything that Samuel does that is particularly surprising or unusual. Just normal. Our normal.

But above all, the most important, significant feeling I have is that they will really help us get the best out of Samuel and will definitely be a big part of our world.

This is the start of an exciting new chapter in the adventures of Samuel Luke.

On days like these…

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

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

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

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

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

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

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

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

Day in the life

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

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

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

Infinity Pump bag

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

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

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

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

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