Monday, 18 August 2014

#IceBucketChallenge - suddenly everyone's heard of ALS

Have you heard of the Ice Bucket Challenge? It's the latest social media craze. Everyone who's someone (or wants to be) is doing it - and not just in the States. If you can view it, the BBC has picked up on it...

Most notably to date I think, even Bill Gates took it - using his own contraption! Oprah did it, Mark Zuckerberg did it, President Obama has been challenged as well, but has yet to respond.

Why are people doing this? It's a fundraiser for ALS. And why does it matter? Because, according to the New York times, this harmless bit of fun has been powerful...
As of Sunday, the [ALS] association said it had received $13.3 million in donations since July 29, compared with $1.7 million during the same period last year.
So, why am I writing about this? I'm impressed, no awed, at the positive power of the Internet to bring to the attention of millions a disease that few people have, and therefore hardly any research is being done into. But it's not an academic interest for me... it's personal. My mother died from ALS. And that's what I want to write about - because people's stories need telling, and my mother's illness and death is not something I tend to revisit much in my mind; but this challenge has brought memories back, which I want to honour and share. Share, because I want you to know what ALS is, its true and awful face. May you never be acquainted with it personally.

So little research is being done that I don't know if I will develop ALS like my mother did. There's a 10% chance, but no test. No known cause. No cure. No relief.

Perhaps the research funded by this lighthearted challenge will change that? I hope and pray.

The rest of this post will detail my personal experience, but for the facts about what ALS is, here's an article about it from The Independent. This is not a lighthearted post, I'll warn you now, but please read it anyway.

To a diagnosis

My mum, doing what she loved the most.
My mother was 52 and a passionate accordion teacher when she noticed the fingers on her left hand were slowing down. Thinking this might be age related - don't we all slow down?, she did eventually consult a doctor when she could no longer raise her left arm higher than shoulder level.

A pinched nerve was diagnosed.

Not long after that, her legs weakened. Walking became difficult. Her solution: take the bike instead! More doctor's visits, more diagnoses. Pinched nerves in the spine? A muscle wasting disease? A virus?

It wasn't until she could barely walk that ALS was finally diagnosed. It was a death sentence, and everyone knew it - everyone, that is, except for my sister and me, because against all predictions, my mother chose to believe she would be well again. She would fight this. From that hospital bed after she was diagnosed, she went straight back to work and refused to give up her position.

Her bosses, her friends, the doctors - she stood up to them all. She would not give up her cherished teaching job. They might have to get a temporary stand-in while she was on illness sabbatical, but advertise for a permanent position? No, she would not give up, give in.

Life with ALS

Wheelchair bound - winter '95/'96
What's difficult to get across is the relentless, unstoppable advance of this disease and how, piece by piece, body part by body part, it takes away a person's life. ALS affects only muscles we choose to move (skeletal muscles) by disabling the nerves that command these muscles to move. Then, because they don't get used, these muscles atrophy and waste away - like a leg in a cast. The smooth muscles, which control things like digestion, aren't affected. The brain isn't affected. In essence, ALS traps a person in an ever less responsive prison of their body. Their body won't die for a long time - internal organs, heart, brain, all continue unaffected. But how my mother kept her soul alive, I'll never know... she hung on to her beloved job as hope, but it probably helped that she had never seen anyone go through it.

To cut to the chase, I'll answer your question now - so how does someone die from ALS then? They suffocate. Breathing is a voluntary function - you can hold your breath, speed it up, etc. - therefore, those are affected muscles. Eventually, those muscles will cease working too. And the brain is present and conscious of it all throughout.

But before death, there is life with ALS. A slow, agonising descent with no ascent possible. For my mom, this meant going into a wheelchair for a few months; when she could no longer hold her body upright, she became bedridden. I cannot fathom what it must feel like to depend on your 14/15 year-old child to feed, wash, toilet (bedpan) your body; to turn and reposition your body when it becomes to painful to stay in the same position at night - because the nerves that die are those that control the muscles, not those that control sensation; you feel everything. To slowly lose the power of speech (tongue - voluntary muscle!) and be understood only by those who are closest to you. In the end, I was her only interpreter until she lost the ability to speak completely.

How long between diagnosis and death? For my mother, just under a year.

Fighting to the end

My mum fought, and fought hard. I never believed she might die. She always talked about being back to teaching after the summer, just needing a few months' physiotherapy once she had beaten the disease.

With my sister - this is how I remember her.
Always in her 40's. Full of life.
She was only sick for a year.
One day in February 1996, she choked on some soup a friend was feeding her. We called an ambulance, which took her to hospital. It was evening, and we had to leave her there overnight.

In the morning, she was distraught. The nurses had not understood her attempts at speech and had no time to be patient. When she asked to be repositioned, they told her to stop fussing or they'd give her morphine (her big fear - being drugged 'out of it'). She'd had no water, because drinking was difficult for her and it would take time to help her drink.

I was 15, this was my mother, and I was outraged. I decided to stay in hospital with her overnight the next night, hospital policies notwithstanding: they would not remove me. And they allowed it. I cared for her as I had done at home, and that night was as normal as normal had become: a few repositionings, some water to drink. Friends and family visited the next day and I slept through much of it - hearing only later that she had stopped breathing a few times and was being revived again and again by those around her, by lifting her up to a sitting position, slapping her back, shouting. On that day, her last day, she finally made her will. A lawyer came to her hospital bedside and asked her yes/no questions, which she answered by looking either to the left or to the right. It became clear even to her that she wasn't going to be able to breathe for much longer, and she asked for an iron lung (an external breathing apparatus). It would keep her 'alive' indefinitely... but to what end? She wasn't given it.

In the evening, her accordion students came to visit. She said her goodbyes. At about 9pm, everyone had left and I was alone in the room - family were around, but all had gone off somewhere: for a snack, a cigarette, a break. I was by her bedside, cheering and encouraging her every breath. But then she stopped.

I tried what the others had done throughout the day, sitting her up, shouting, slapping her back... but she had gone. I could feel her body turning cold under my hands. So this is what death is like - it has a distinctive facial expression, which I had not seen on her before, and a spreading cold. Or perhaps, the dissipation of warmth describes it better.

ALS has taken my mother. These memories are not happy memories. This disease is as close to a living hell as I can imagine because, despite the absence of physical pain, the mental anguish of being clear and conscious throughout it all must be soul crushing. At least pain gives a focus, a distraction - ALS gives no such luxuries. It is merciless.

It must be eradicated. 

That's why we (well, my Mr.) did the Ice Bucket Challenge - and we have put our own money to the cause as well. Please join us and donate!

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Thanks so much for sharing!