Lifejackets on

“Always wear a lifejacket, it could save your life!”

Living right on the shoreline, we have learned to have a healthy respect for the water. For all its beauty, the sea is a powerful and dangerous thing. If I was the boating type, I would know that without a well fitting lifejacket, I would struggle to keep my head above water if I went overboard.

Given that I am absolutely terrified of ferries, yes ferries (thanks, TV news, for imprinting the Debrugge disaster into my brain at a young tender age), there won’t be much boating action happening anywhere round here anytime soon. But if I was to step onto a boat, I would certainly be doing so with my lifejacket fastened tight.

Sometimes I have likened our experiences of raising Orange to what I imagine it must be like to sail the high seas, if you are a sea-legged kind of person. Periods of calm, serenity, beauty, a feeling of deep connection with what life is all about (and, of course, rather a lot of fun), interspersed with raging storms that you have no choice but to keep your head and steer your ship through if you want to make it to the other side.

Life can be like that for any of us, I know, but in raising Orange we know that he will be dependent on us for life and we must equip ourselves for many a storm to come.

As Orange has got bigger, we have learned that we cannot do this on our own.

For the last few months, we have been testing waters of an entirely different kind. Orange has been going to stay with a respite carer for an overnight stay or two. He has just returned from his longest stay yet – two days and one night – that for the first time we were not too exhausted to enjoy.

For the last two days, while Orange has been waited upon hand and foot, utterly spoiled, and taken out to enjoy the winter sun at Mount Edgcumbe, Bea, Gavin and I have made the most of doing some of the most un-wheelchair-friendly activities we could find.

Seaton's Tower

We have climbed lighthouses. We have ridden bikes. We have walked the seafront. We have swum, splashed and goofed about in swimming pools. And we have eaten rather a lot of ribs. And ice cream.

Ice Cream

The time we have spent together has been golden and we have cherished it because we have been able to focus on Bea. And on ourselves. In a way that we never can when Orange is with us because his needs always have to come first. As they should, because he is the least able to help himself, but as much as I love us all being together and experiencing things as a family, I will be the first to admit that it is hard work.

Which wheelchair shall we take – the all-terrain or the one that actually supports him? Will the wheelchair get in the door? Will there be steps? Will it be warm enough for Orange? Will it be too noisy? Will there be any food we can feed him? Will there be anywhere to change him? What will we do if he doesn’t cope? Bags, medication, drinks, spare clothes, iPads, wheelchairs and an Ernie are minimum requirements for a family day out.

Sometimes it goes well, sometimes it doesn’t, but I don’t think I realised until we had a day out without him, how I am always on high alert and how this can impart an underlying aura of tension into any family activity, no matter how familiar or fun it might be.

Having a day just with Bea meant we could do things on a whim. It’s sunny, great! Quick trip to The Hoe.

Oh look, the lighthouse is open, shall we go up it, YES!

In our enthusiasm, neither Bea nor I remembered until we were halfway up and fully committed to the task, that we both harbour a fear of not only heights but tight spaces. Imagine our joy when we realised, half way up, that it was getting narrower and narrower, steeper and steeper… but because we could focus solely on each other, we worked our way through those fears together.

One step at a time, we climbed. One foot in front of the other, until we reached the top.

Lighthouse top

As a sibling-carer, Bea has learned too young that life can be frightening and unpredictable. That people get sick, and sometimes they die, and not always because they get old. I know she feels the weight of unpredictability in our lives and has taken on more responsibility that she should have for keeping us all afloat.

She has also made some big sacrifices in her life. Age seven, she knows how to give CPR and call an ambulance but she doesn’t know how to ride a bike. With respite care, we can ease some of that burden for her, and for ourselves too. As well as climbing a lighthouse, we were able to take Bea out to ride her bike today for the second time ever.

It’s taking a lot of adjustment to get used to sending our little boy away while we go off to have fun without him. I won’t pretend that I haven’t been eaten alive inside by guilt about that and I won’t pretend that I don’t feel sad when he is not with us.

I do.

And I wish he was right there with us, climbing lighthouses, riding bikes, and splashing and duck diving in the pool. But we have to be honest with ourselves, and fair to each other, in recognising that this isn’t the way our lives can be. They just can’t.

Without respite care, we would be overwhelmed and overwrought. Exhausted. And totally unable to steer our ship through the storm when it comes.

When I am feeling guilty about sauntering easily down the seafront, gazing out to sea and chatting to my little girl while my little boy is being cared for by another, I have to remember that actually it’s good for him too. If he is to have independence, relationships, freedom and confidence in his world he must learn that, with careful choices, he can have fun and be safe outside of his immediate nuclear family.

He has a right to adventure, too. I mean, just look what he gets up to when we’re not around.

Orange wig

“I knew, when I met you, an adventure was going to happen”

Winnie The Pooh

Lifejackets on, folks.

A Cornish Mum

Don’t lose your head

When our first babies reached a year old, my friends and I cheerily raised a drink or two to celebrate having kept alive our firstborns for an entire year. Giving birth to, feeding and growing a whole actual human seemed like such a momentous achievement and yet we were able to nonchalantly joke about keeping our offspring alive because we had never yet faced a situation where their lives were ever remotely at risk. We just had to, you know, feed them and love them and stuff.

Even with Orange, we’ve been extremely lucky that he was born healthy and in the first 18 months of his life, faced no immediately threatening health issues, despite his developmental complexities. Of course he threw us a major curveball in the autumn of 2012, when we were just days away from relocating to Cornwall, in that he developed a seizure disorder and gave us the fright of our lives by having six seizures in 24 hours that each caused him to stop breathing.

Our experiences of the NHS have shown us that there isn’t much that can fluster a High Dependency Unit paediatric nurse, but there were several moments during that hospital stay when we were not the only people in the room that had our hearts in our mouths wondering if our boy would ever take another breath.

But he did.

Mr K wrote a guest post for the blog at the time that said everything I was too numb to articulate.

It was the greatest fear I have ever felt and I never wanted to step inside that house or that hospital again. Of course we didn’t have to, for we soon left it all behind to start a new life that represented hope, serenity and a happier future. An escape.

Several days and one extremely nervous journey down the A303 later, we had left well behind us the memories of our boy, blue and lifeless on the kitchen table, the fluorescent hustle of paramedics, the sterility and freneticism of the resus unit, and the deep, deep fear of losing our boy to a type of epileptic seizure which not one specialist could accurately identify.

With a little trepidation, and a lot of hope, we settled into our new lives knowing that it was likely Orange would face these seizures again, but with every day, week and month that ticked by, we slipped further and further from a paralysing state of high alert to a more relaxed way of being.

We worried a little less about who we left Orange with. We allowed ourselves to be excited about him starting nursery. Even letting the telly babysit him while I went to the loo or made a cup of tea became a possibility again. We slept a little more, instead of lying with one ear attached to the baby monitor, tuned in to every rise and fall of his breathing.

With only a few absence seizures here and there, and one small seizure last summer with a slightly juddery but well oxygenated and still breathing boy, his epilepsy was well controlled. We felt confident we knew what we were doing, and I didn’t hold my breath every time Mr K travelled for work, wondering if now would be the time Orange would throw us a big one.

But last week, he did.

With Mr K in Dallas, I was on bedtime duty for the fourth night in a row, and in major need of a gin and tonic. Orange, home early from nursery with a fever, was in bed. Just Beep to contend with. At last, she was settled. And then a little voice piped up.

“Mummy, Orange’s breathing is giving me nightmares. He sounds like a monster.”

I listened at the door.

“He’s just snoring Beep, get back into bed and I’ll go and tuck him in.”

As I opened the door and unlatched his bed, the ‘monster breathing’ stopped. I raised the blanket to tuck him in. Pulling it over his little body I watched for the rise and fall of his chest before closing his bed and saying goodnight. Only that rise never came.

Lifting him from his bed, his stillness crushing me cold, I knew we were here now. This was it. It was a bad one. My head torn between fear for my boy’s life and sadness for my little girl that here she was again, watching her brother clinging on to life.

“He died, but he’s ok now…” Her three year old words echoed in my heart as I dialled 999 and held his airway open.

Desperately following the life support instructions of the emergency operator, I gave him rescue breaths, Beep watching his chest and his colour all the time.

“He’s breathing in mummy. He’s breathing in. But he’s grey mummy, he’s still grey. Orange, you’re going to be ok, the ambulance is coming. He’s breathing mummy, he’s breathing.”

Orange took tight, laboured breaths as the seizure constricted his chest. In, and out. In, but not out. A pause. In again, in again. And out. A pause too long. More rescue breaths.

As the ambulance arrived, my little six year old Beep took charge, running to open the door and directing the crew up to where we were. Bravely, she watched as Orange was intubated on the landing floor. Calmly, she cuddled a favourite toy and went without a tear or a fuss to sleep with our neighbours as I frantically grabbed medication, a hoodie, shorts and a phone charger and leapt into the ambulance with Orange, tubed and bagged and still in the grips of the longest seizure he had ever had.

His pyjamas cut, wires all over his body, oxygen mask over his little face, we raced through the dusky lanes to the whine of sirens and the flash of blue lights. Rescue medication given. But still no change in him. Holding on to the inside of the vehicle as we sped up and down through the windy Cornish countryside, I watched Orange, rigid and fighting for breath. And I lost hope. Gave in to fear.

Is this how it’s going to happen? With Mr K abroad and Beep with no family to comfort her? It’s been 30 minutes and he is still unconscious and struggling to breathe. We’re going to lose him, right here on the A38 in the back of an ambulance, aren’t we?

I wonder when to call Mr K. If we’re losing him now I don’t want to go through this alone. I text him.

In an ambulance. Orange not breathing.”

A second dose of diazepam. I watch the screen as his oxygen sats hover around 50.

A Texas number flashes up on my phone. But I don’t know what to say. Get home? But how? Your son might not make it? But maybe he will? And then I’ve created fear where none belonged.

But then the sats were creeping up. Up and up, 60, 65, 72, 88. The heavy silence in the ambulance was broken by the paramedics.

“He’s going to be alright, he’s doing alright now, he’s doing ok. He is, he really is. Go on, tell his dad he’s going to be ok!”

As we arrived at resus, a now distressed and angry Orange was wrapped in a blanket and carried gently into the hospital. Wired up to monitors, and soon falling into a post seizure unconsciousness, Orange let his exhausted little body rest and I completely fell apart in the arms of my mum who was waiting there for us, not knowing if he would arrive alive or dead.

We watched and waited for Orange to regain consciousness. We watched and waited for any sign of another seizure, expecting it to happen as it had in resus three years before. And again, and again on the ward.

But he surprised us. History did not repeat itself.

In fact, by midnight, riding up to the High Dependency ward he sat up on his trolley and had a good giggle about the fact he was naked in the hospital in the middle of the night.

We were looked after wonderfully on the ward. After a moment of black humour shared with friends about the prospect of a night upright in a hospital chair, a kind nurse brought me an incredible recliner and a pillow. Mum brought me an enormous bag of snacks and a duvet, and some of the essential items I’d forgone for shorts and a winter hoodie in my panic. And the next day, kind and wonderful friends brought in breakfast, coffee, books, toys and much needed hugs.

Our wonderful village and lovely friends, and mum, supported us and held us up when we needed it most.

I thank you all so much everyone who looked out for us, looked after Beep, visited, messaged and called Mr K to make sure he was ok too. I hope we can return these kindnesses in the future.

So now we are home, the adrenaline has subsided, and I am trying not to lose my head. We can’t run away from the fear this time. I can’t leave 250 miles behind me the landing where I kept my son alive while waiting for an ambulance. And I know it probably won’t be the last time I have to do so.

This time we have to face it.

Feel it.

Learn to live with it.

And not be paralysed with the fear of what may come. We must fill our heads and hearts with new dreams and new hopes instead.

 

About a Beep

‘And do you have any worries about your other child?’ A question I am asked a lot, by every professional we meet, in fact. A question that is always followed by a poignant pause, and a silence from me as I wonder what on earth to say, then mutter, ‘no, no, she’s fine’ and gaze at the floor, or twiddle with my earring, awkwardly, waiting for the next question. ‘Does she look like Orange?’ ‘Is she developmentally normal?’ (A phrase I have grown to dislike intensely, hence it is in italics as a way of demarking it physically from my own chosen words).

It is hard for me to sit down and write this, to dig around in the corners of my brain where uncertainty lies, since life has been so colourful and happy since we moved to our beautiful little coastal village, but in truth, yes, I do worry about Beep. I worry in all the ways a mother of any child does, of course, but there are some larger, looming spectres that trouble my mind now when I think about Beep, and her future.

You will have to forgive my pseudo attempt at explaining the science behind all this, but it is relevant, so stay with me… In simple terms, we don’t know the cause of Orange’s difficulties, but we have been told it is likely what they call a ‘single gene disorder’. In other words, one of Orange’s genes is somehow faulty. We have no idea which one, and trying to find out which one it is could take years and years of testing, with no guarantee of an answer at the end of it. For him, now, it doesn’t really matter. Whatever it is, we are told it is now in the realms of being so rare that even if we find out which gene it is, and we are given a diagnosis, or a name for his syndrome, there will be so few known cases worldwide that his prognosis will remain unknown. There are no comparables. He is just Orange.

It is possible that this troublesome little gene is a fluke of nature and that, in the moments when Orange was but a little speck of cells, something random occurred that left this one gene slightly out of shape. Or, it is also possible that this faulty gene is the result of something more calculated. Something more predictable, and measurable, in Mr Mavis and I, that means any and all of our offspring have a 50% chance of being affected, or perhaps carriers. And this is where Beep comes in. We have no idea if she is affected (seems not, but who knows what’s in her future given we have absolutely no idea what we’re dealing with), and equally we have no idea if she is a carrier. Her own childbearing right of passage, should she choose it, could turn out to be rather complex.

I also worry for her in school, growing up, being the sister of a child with disabilities, particularly learning disabilities. I have written about this before and I still don’t know quite how to help her, or protect her, from the hurt this might bring. For now, I am hanging on to the hope that this might be a little easier for Beep here in Cornwall than it would have been in Surrey, where we all too frequently found that disability is just not talked about in many circles, where a significant but often silent majority find it shocking, disgusting, funny, or at least something they don’t want to hear or see. Unkind, thoughtless comments, jeering, averted gazes, pretending that disability doesn’t exist. These are the things that cause hurt and harm. I have developed a thick enough skin to brush off a fair amount of that nonsense, or to challenge it when necessary, but a young girl shouldn’t have to do that.

There are some things more immediate, more pressing, that I so desperately want to fix for Beep. She coped so wonderfully, so bravely, when Orange had his first seizures, but has taken to playing a game, all by herself, that makes me want to scoop her up and erase from her sweet little mind that terrifying morning when Orange stopped breathing. ‘My baby’s got a blue face’, she says, carrying a doll under one arm. ‘He died, but he’s ok now’, she says, gently stroking her doll’s head and pretending to phone for an ambulance.

At times, her anxiety is palpable. Nothing but mummy cuddles will do. She is alternately quiet and manic, her young heart and mind working overtime to process what’s happening in her world. A whirlwind of hospitals, machines, worried faces, whispered words and exhausted parents and then without so much as five minutes to catch a breath, her home was packed up for the third time in a year, she waved goodbye to another nursery and was whisked away to start a new life, here in Cornwall.

I know there is no way of taking away the fear that she must have felt, the anxiety she will have absorbed, the sadness and confusion there has been at times, and the frustration and boredom she has endured while working around Orange’s appointment schedule. What we can offer her here though is something she won’t have felt for a long time, certainly not since Orange arrived, and that is security. A sense of place, a sense of self, a sense of belonging. Slowly, piece by piece, we are building a life here. And the healing, soothing power of being by the sea is even greater than I had hoped. Even though she is only three, I can see her responding to her new environment in a way that gives me such hope and joy. Just this weekend, I took this picture of Beep hitching a ride on daddy’s shoulders as we strolled down the beach enjoying the morning sunshine. Quietly contemplative, the sound of the waves in her ears and the salty air filling her lungs, she sang and chatted sweetly as we walked along. I just know this place is good for her.

 

How do you explain a Syndrome Without A Name to a three year old?

“Siblings have a lifelong need for information, they often experience social and emotional isolation, and have to cope with difficult situations…” 


A few days ago, something happened in our house that got me thinking about The Beep and her experience of having a sibling, her only sibling, with complex and undiagnosed disabilities. It was a typical sort of a weekend in the Mavis household – well, typical of our weekends of late, trying to fill the time while we are waiting to move house, everybody was mildly bored and irritable and it was raining outside, so we were cooped up, four humans, four cats, seeking out entertainment within the confines of someone else’s four walls (which, by the way, are desperately in need of redecorating. Can you tell I’m not a fan of renting?)

The Duplo was out. Snacks had been had. Some ridiculous Bollywood-esque electro-pop dad dancing had been done to The Bom Bom Song and Korea’s latest export, Gangnam Style, (not my choice, obviously). Bath time was approaching. Everybody was getting a little tired. Out of nowhere, The Beep flicked into dictator mode and all of a sudden if we failed to cooperate with her barking demands, we were branded poo-poo heads, silly pong pongs or boys with no dresses or earrings. Don’t you just love the way a three year old can hurl an insult? Having whipped her wrath around both parents and all four cats, she turned her attention to her brother.

“Baby Orange, you are stupid

And in a split second I was floored. Stuck fast between a searing hurt for my son who, let’s face it, is likely to have to brush off many such a slur during his lifetime, and a dawning realisation that the time is coming when we will have to start explaining to The Beep what’s different about Orange. She needs to be prepared for a future of dealing with other people saying just that about her brother. I desperately hope that they won’t and that he’ll be surrounded by love and affection everywhere he goes, but human beings just aren’t like that. He may or may not be conscious of the   cowardly vitriol aimed in his direction but I can be damned sure that his sister will be. I knew in that instant it was time to start arming her with the knowledge and ability to cope with other people’s discomfort about disability. Particularly learning disability, which Orange has in as yet unknown magnitude.

It is very hard to tell how much The Beep understands about her brother’s difficulties. Children are so perceptive, there is no doubt that she will have absorbed a great deal of our anxieties. From the age of two and a half, she accompanied me every week to private physiotherapy sessions for Orange, during which it became very obvious indeed that he was dealing with a great deal more than just the stiff neck we thought we were treating. She has come with her Dad to pick us up from hospital after Orange had surgery, and held his hand in A&E while his oxygen levels are checked. Just this very week, I have had to explain to her that yet another stranger was coming to the house to ‘help Orange learn to play/eat/sit/talk’ and that I needed her to be very good and quiet while they were here. She has sat in room after room while doctor after doctor, therapist after therapist, has asked me question after question about Orange’s development, disabilities, health and habits.

I am sure she must know, deep down, that most 18 month olds don’t need so much help to learn how to do basic things. And yet The Beep is so overwhelmingly positive about Orange. Yesterday, while eating a beef curry, he was doing a brilliant job of grabbing the spoon and shoving it into his little cake hole. We have been working on self-feeding for months. Whoops of joy, enthusiastic clapping and a cheerful ‘Well DONE Baby Orange, you’re doing SO WELL’ clamoured across the kitchen from where she stood, playing boats and pirates at the sink (read, flooding the kitchen…) The Beep has become a veritable Orange Cheer Leader.

But pretty soon, I am expecting her to ask me why her brother can’t do the things that her friend’s little brothers can do. It is crucial that we get the answers right. I don’t know yet what they are, and the fact that Orange remains undiagnosed, and could be a SWAN forever, makes that job just a little bit harder. How do you explain a Syndrome Without A Name to a three year old? Answers on a postcard please…

 

More about siblings

I came across this interview with Jo Whiley, whose sister has Cri du Chat, a genetic syndrome involving chromosome 5. She talks with honesty about her parents’ experience of caring for her sister, and what life was like growing up as the able-bodied, neuro-typical sibling of a girl with quite complex needs.

I commend the work that Jo is doing with Mencap. Her approach to changing attitudes toward disability is straightforward, honest and direct. She is out there in the mainstream talking about it. Exactly as it should be. So why this wonderful interview is hidden away in the ‘Talking about Disability’ section of the Guardian website I do not know.

C’mon Guardian, you know this deserves more profile:

Jo Whiley talkes about her life with her sister who has Cri du Chat