Uncle Midaz

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

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

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

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

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

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

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

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

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

God save our NHS

I talk a lot about Team Sam. Team Sam is headed up by, well Samuel of course, and consists of not only his Dad and I, but all the people who love and care for him – that includes Julia’s House who look after him and his therapists and doctors who oversee his care.

But I must say one massive component in Team Sam, I mean without it we wouldn’t be able to do what we do so easily, is our old faithful NHS.

Hopefully during our lives most of us will only have to rely on the NHS a few times and for only minor things. We, however, are so reliant on the NHS for everything. As a family our biggest financial cost of having Samuel was me having to give up work. When I was pregnant our intention was for me to go back part-time, whether that was for three or four days (we were still negotiating that right up until his arrival). But we soon realised that there was no option and I was to  give up work and be his carer.

But wow, Samuel must cost the NHS a pretty penny. Off the top of my head they pay for:

• All his medicines (and don’t forget he has quite a few and I have been reliably informed by my local chemist that they aren’t cheap)
• Ketogenic formula
• Pump equipment and bits and bobs
• Gastrostomy care and related bits and bobs
• Syringes
• Various therapies including hydrotherapy and physiotherapy
• Special buggy
• Special tumble form seat
• Special high chair
• EEGs (recording of electrical activity in the brain)
• Stuff to test Samuel’s blood ketones and glucose – monitor, lancers and the test strips (one box of 10 ketone strips costs £30 & we use two a day)
• Paedetrician, Neurologist, Genetics, Child Development, Community Nurse, Speech and Language Therapist, Dietician
• Treatment and care when admitted as an inpatient (although Samuel has only been admitted once as an emergency patient since coming out of NICU)

I know I’ve forgotten a number of things but I think you get the idea. Anyway, the fantastic NHS pays for all of that. Its all free. We don’t have to have special medical or health insurance. We just get it all because we live in England. I love the NHS. The NHS has helped our little lad defy the odds.

One of Samuel’s best friends Liam lives in America. They don’t have the NHS like us and his Mum and Dad have to worry about medical and health insurance. I think they may not even be covered for Liam starting the Ketogenic Diet which we can’t even imagine happening here. I must admit that I do feel guilty about how lucky we are and it makes me feel all the more grateful.

So to you NHS here is a massive thank you from Samuel, our family and also all the families in this country who benefit like we do.

And so a message to Mr Cameron. The NHS is vital for children like Samuel and without our free medical care, I can’t even imagine what we’d do and the effect it would have on Samuel. Would it mean that we’d have to make certain choices as to which therapies, or worse, which medicines he could have because we, or our insurance company, would be paying and couldn’t afford (or wouldn’t pay out) for certain things? Would we have to look at cheaper alternatives for some of Samuel’s care and treatment? Hands off our NHS Mr Cameron, I’m warning you.

Rollercoaster Ride

Samuel spent eight weeks in NICU. Eight long weeks. I can’t begin to tell you of the different emotions we felt during that time. Excitement, joy and love and pride at being parents to this beautiful little boy. Fear, pure and utter fear that we may never take him home. We may lose him. Despair. But also we found huge comfort in NICU and the support we received.

It is so difficult to put into words how wonderful the doctors and nurses were. We learned so much from the nurses, we even learned about survival. But all those nurses and doctors were just spectacular. The nurses would regularly pick us up off the floor, dust us down and gave us so much support and comfort we could carry on to see another day.

Our wonderful relationship with Auntie C blossomed while we were there which is something we will always treasure.

We also formed some great friendships with other parents and one particular family has a very special place in our hearts. Leela and Anthony are parents to twins Willow and Stanley who were born 14 weeks early. I’m proud to tell you that Leela and Stanley are raising money for NICU along with the family of Oliver who is also a graduate of NICU. Willow and Stanley and their friend, Oliver, spent a combined 385 days fighting for their little lives in NICU at Poole Hospital. Their journeys were full of ups and downs and quite a rollercoaster ride. For more of an insight into what these babies went through, check out The Early Adventures of Willow and Stanley.

In the summer of 2012, a group of close family and friends will be cycling one mile for each day these three babies endured in hospital. They will start at Windermere in the Lake District on 1 August, finishing (hopefully) 4 days and 385 miles later at Poole Hospital. If you were able to give just a few pounds, your money would go towards buying much-needed equipment to help babies like Samuel, Oliver, Willow and Stanley. You can find out more about their cycle ride and sponsoring them at www.justgiving.com/RollercoasterRide

Even if you don’t want to sponsor the team at the moment, please please watch this beautiful video about Samuel’s very special friends Willow and Stanley. Thank you. x

Our nest

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

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

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

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

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

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

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

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

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

Meal times

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

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

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

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

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

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

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

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

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

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

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

Quality not quantity

I’ve blogged before about friends (Days like these) and what they mean to me, in particular my online friends (I get by a little bit with help of my (cyber) friends).

When a friend is in trouble, don’t annoy him by asking if there is anything you can do. Think up something appropriate and do it.
– Edgar Watson Howe

I lost friends when I had Samuel. Some people backed off completely as soon as they heard there were problems. They would probably say they didn’t want to intrude. They wanted to give us space. I’m the age where a lot of my friends are having children. I think some of them backed off (or just disappeared) because they didn’t really want to know about what could go wrong, what could happen. Perhaps they felt guilty that their child was fine and healthy. Maybe they thought we’d be miserable and rain on their ‘new baby’ parade.

I backed off too. Having Samuel and finding ourselves in our situation, made me look at people differently. Casual acquaintances or people I just gossiped with, I didn’t need them or have time for them anymore. It made me refocus on the people who mattered. In the early days I found many relationships, friendships difficult, hard work. Having to explain everything to them so they understand about Samuel’s condition and abilities (or lack of), seeing their apologetic, sympathetic looks. Being rubbed on the shoulder and being told rather patrionisingly, with a head tilted to one side, that we were doing really well and were both very brave, by people who would then go back to their neat little worlds. I know they meant well, but it did my head in.

I felt at first that having Samuel had shrunk my world. My world had got dramatically smaller. How wrong I was.

But having Samuel opened my eyes and my heart to those I needed in my life. Those people who are pure gems that you just need to have around.

Friends like Sam’s Auntie T, JJ, Gem and Kate were there before and they are here still with bells on. When Sam was in NICU they no doubt had absolutely no idea what to do or say, but they put themselves forward. They’d email and text and offer to get us shopping, to come and visit in NICU, to just be there for us. I know damn well that I could send out an SOS to each of them and they’d get here as fast as they can. I’ve even got a former work friend of mine who is much younger than me and a bit of a party animal, but she still finds the time to regularly check in with me to see I’m ok. Likewise with an old school friend who has had her own heartache to deal with, but she’s always there you know, there in the background.

And of course there is Auntie C. She is our guardian angel. We knew her before we had Samuel of course (to those who don’t know, she is our neighbour and was one of Sam’s NICU nurses) but not the way we know her now. She is a crucial person in our lives and means means the world to us.

And apart from our bond with Auntie C, our time in NICU brought us together with three sets of families who are so very dear to me. Each of their little chimps had various problems. They are all miracles in their own way. And their mum’s adore Samuel. They hold him so close and tightly like he was one of theirs. They keep up-to-date with how he is doing and treat him like he is a real little VIP.

And then my mum-friends I’ve met through Julia’s House. I’ve made friends with three fabulous, intelligent and strong women who really do ‘get it’. Nothing I can say will particularly shock them and they certainly wont sit there patting me on the shoulder, telling me I’m doing really well and so brave. They treat me like a normal parent with just extra challenges like them. Their children are much older than Samuel and I’ve been able to learn so much from them already. It’s not quite like the girls from Sex and The City when we get together. We talk about wheelchairs and medicines, but we do have a great laugh together too.

When you die, if you’ve got five real friends, then you’ve had a great life.

It is because of Samuel I have these amazing people in my life. Even my friendships that I had before I believe have been strengthened because of him. Samuel has opened the door to a whole new world for us and do you know what? It’s big, warm and full of people with golden hearts (and a great sense of humour). Thank you Samuel. x

Celebrate: The ultimate milestone

Under ‘normal’ circumstances (whatever that may be), in my 17 months career so far as a Mum, I’d have noted and celebrated Samuel’s milestones. When he said his first word and what it was, the first time he laughed, when he started to roll over, crawl, walk, when we started getting him onto solids and so on.

But these are milestones that we have never reached and may never do. But I think Samuel has reached milestones that beat all these.

Milestone 1 – Coming home from NICU. We were warned that it might never happen. We might never be able to take him home because we’d either lose him to his seizures or he’d be so heavily sedated, he’d have to stay in hospital indefinitely.

Milestone 2 – First birthday. Something we felt we could only dream of reaching. And we did and had a fantastic family birthday party.

Milestone 3 – Christmas. Samuel spent his first Christmas in hospital, but he spent his second at home with us and it was absolutely wonderful.

Milestone 4 – Our one year anniversary of being home from NICU.

Milestone 5 – Beating the genetics prognosis. Last April (2011), we were told that it was very likely that we would only have Samuel for months. His condition would deteriorate and finally beat him. Beat us. That was a year ago and look at him now. His neurologist has said herself how impressed she is with how well he is doing. Since we’ve been home from NICU we have only had one emergency admission. I think this is the ultimate milestone and Samuel has reached it with bells on.

My brave little solder. Still here, still fighting, still gorgeous.

Team Sam. x

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

Share your celebration post below and once you are done grab the Celebrate Blogging Challenge badge for your blog!

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A brief escape

A few weeks before we came home from NICU, the nurses let us take Samuel out in the NICU pram for a walk. We didn’t go very far as we were quite nervous but we felt very proud pushing our beautiful baby around the neighbourhood. It was the first time we’d been properly alone with Samuel and it was wonderful.

The NICU 'chav pram' (apologies to any Burberry fans). His little friend is Grannie Bear (named after Grannie who knitted her).

 

Samuel's cardigan and bobble hat were knitted by Grannie.

Diagnosis – what’s in a name?

Samuel got his official diagnosis last April (2011). We were told he had ARX. But we’d already suspected that would be the case because his neurologist (who I continue to be in awe of) suspected that this is what he had and was keen to have him tested for it, so it came as no big surprise.

So we have a label, a name. I’ve been thinking lately if having that diagnosis, you  know, being able to read about ARX, has actually made much of a difference to us and I’m not sure it has. From MRI scans we already knew what his brain looked like. We already knew his seizures were severe and hard to control. We already anticipated that he would have some kind of development delay. I suppose for some people and their parents it’s like putting the bits of the jigsaw puzzle together to be able to understand what they are dealing with.

At Samuel’s diagnosis meeting last Spring we discussed prognosis (ie life expectancy). The genetics doctor told us that it was very difficult to be precise of course and it would take a brave and bold doctor to start talking actual numbers with parents. But then my husband asked if it was months. Did we have only months with Samuel? And her answer? She said yes. That was almost a year ago. The neurologist, who was unfortunately not at that meeting, said later that with Samuel’s condition being so rare, it is extremely difficult to be so definite. There are only a small number of children known to have had ARX in this country, so they are basing their information on such a small number of case studies (plus Samuel has a rare mutation of the (rare) condition). I gather the stats are that 1 in 400,000 babies are born with ARX. So that didn’t help us at all.

It makes form filling a bit easier. We actually have something to fill in the box when asked about the condition. But no one has heard of ARX, so we have to explain in some detail what it actually means.

I know for some parents and I’m sure the individuals themselves, having a diagnosis will at least be that missing part of the jigsaw. Instead of having to just explain the child’s symptoms they can actually give a name. But for us, we tell people that Samuel has ARX but then have to give the symptoms as no one has heard of it! I always wonder if life would be that little simpler if Samuel had an ‘off the shelf’ condition. Something that people have heard of, seen on the TV and have a vague understanding of.

Despite our experiences, I do believe that a diagnosis can be such a positive thing. Last week I was encouraging a close relative to refer her child to be assessed as suspicions are that the child maybe on the autistic spectrum and if that is confirmed at least their parents and school will be better informed about how they can help them.

But for our Samuel, well he is still here, battling away each day. He’s surprised all the doctors with just how well he is doing. Since we came home from NICU last January (2011) we have only had one emergency admission and Samuel seems to otherwise be quite a healthy boy.

The important message to all parents seeking the golden chalice of diagnosis is when you get it, don’t let it define the child. Because Samuel may have ARX but that boy is making up his own rules.

I would be really interested to hear your views and experiences of the difference of having, or not having a diagnosis and what it has meant for your family.

UPDATED: Since publishing this post, we’ve had an appointment with the genetics consultant to talk about our options for future pregnancies. I talked about the appointment in Is the impossible, possible? and talked about the options we’ve been given from taking on a dangerous gamble and conceive naturally, have IVF PGD or be on the receiving end of egg donation. The genetic doctors are able to tell me the likelihood of having more children with ARX what our risks are because we have a diagnosis. We have something that the guys in the lab can work with. Since Samuel’s diagnosis they were able to find out if I was a carrier which means we can make decisions about future pregnancies. We know how to not pass this on again. That’s what a diagnosis has done for us. It has stopped us passing on this dreadful condition. It ends here with Sam and I.

Hayley from the blog SwanFreddie has also written a post about diagnosis and why it is important to her family, so please do pop over and have a read. Eric from Pressure Support has also done his usual thing and written a fantastic post about his son Liam’s diagnosis. Definitely worth a read.

• Find out more about Samuel’s condition ARX

Admin duties

Since we came home from NICU last January (2011), I have had to become quite a pro at form filling, admin & managing the diary. I’ve never worked as a Personal Assistant before, but certainly think along with my mummy and caring duties I’m proving to be one fine PA to the boy.

One of the forms people will always tell you is a demon to fill out is the paperwork for Disability Living Allowance. It’s not actually that bad and the amazing Cerebra have produced an absolutely fantastic guide to help. The guide takes you through each stage of applying for DLA, starting from whether you should apply at all, through the claim pack box-by-box, with lots of useful hints and tips on preparing for a medical visit, obtaining supporting evidence, keeping a DLA diary and much more.

You can request a printed copy of the DLA guide by calling 0800 328 1159 or emailing info@cerebra.org.uk or you can download and print it yourself.

Personally I would recommend that when you complete the DLA form for the first time you make a photocopy. I know doing so will be very time-consuming but when you come to renew, it will make your life much easier!

Do have a look at Cerebra’s website for other useful financial information.

Anyway, it’s renewal time for us so..

It's DLA time again

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.

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!

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?

 

Meet the gang

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

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

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

Sam and The Gang.The coolest gang around.

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

Sam with Grannie Bear (in NICU)

If I was to write a book…

If I was to write a book about Samuel’s story, then probably the first line would be: “Do small penises run in your family?” Now you can’t just read that and leave, you must read on….

As we knew there may be issues with Samuel, as soon as he was born one of the NICU baby doctors came down and examined him. I was left lying there all a mess, obviously a little sore and my husband was outside the room holding my baby. I’d not even held him, barely touched him at that point.

The midwife, Tessa I think her name was, popped out the room for a few minutes then came back and said that everything appeared fine at the moment with the baby, but the baby doctor wasn’t happy to confirm the baby’s sex. I looked at her blankly, but he’s a boy, he’s got a penis, he’s a boy. She smiled awkwardly at me and said that yes, she thinks he’s a boy, after all he did wee on her when he was born, but his genitales are small and she can’t feel any testes.

Samuel’s dad came back in the room with him and I got my first cuddle. We tried some breastfeeding (which wasn’t successful) then I had to waddle to the showers to get myself sorted. When I came back I was really dizzy and was put in a wheelchair to go up to postnatal. Tessa was examining Sam’s bits again and said that she could definitely feel a tube in his penis, “look mum have a feel, there is a tube isn’t there?” Then she looked up at my dear husband and said the words, “Do small penises run in your family do you know?” It would have been funny if it wasn’t so bizarre.

Sam’s dad didn’t know what to say, so I said that he was fine, a very normal size but how would he know about his brother and dad – hardly something they would talk about!

So we were shuffled off to postnatal very confused. Then the head of NICU arrived, Dr K, (we’d met him in the last week of the pregnancy) and examined Sam and studied his bits and had a feel about. He decided to send Sam for an abdomen scan to see about his testes and if there was anything there that shouldn’t be. Then Dr K said that perhaps when we tell people that the baby was born we could not mention the gender, just to be on the safe side. I looked at him blankly. I looked at Sam, who to me did actually look like a boy, just with slightly smaller bits.

So he had the scan. His dad went with him and saw the scan for himself. After seeing the scan he was very confident that yes, Sam was a boy. There were no signs of anything else (ie girls internal bits) and there was a blur where the testes were – they just hadn’t dropped. So when they came back to postnatal we made the phone calls telling our parents of Sam’s arrival. The Grandparents couldn’t understand why we had taken so long to call them and we didn’t really want to go into it. Because it wasn’t over.

Dr K said that they still wouldn’t confirm the sex until they had results of a chromosome test. Fantastic.

About 10 days later we got the result back. Sam was a boy.

It turns out that the slightly smaller genitales is part of his condition, ARX, but it doesn’t affect him really and surgery has brought down his testes. Having been peed on a number of times, we can say that everything works fine!