‘It felt impossible to believe the fun-loving sister of just a year ago now faced an unutterably bleak future’

THE date 15 September 2022 will stay with me for the rest of my life for its sheer hopelessness. Sitting by the phone waiting for Joanna to call to give me the results of the second series of nerve tests was excruciating. The utter desolation of her diagnosis left me winded, doubled-over with an almost physical pain of what the future now held for her – and her family and friends.

My beautiful, generous and kind-hearted sister started the conversation with ‘I’m so sorry. It’s MND’. Typically – before any thought about the impact this hideous diagnosis would have on her – she was thinking about those closest to her.

I’ve never been one for consulting Dr Google about medical matters but MND was my one exception. A quick scroll left me terrified as the stark realisation of what must be one of the cruellest diseases imaginable became clear.

Parts of your body simply stop working. In most cases only the brain remains unaffected, which means even when you can no longer move, or speak, or eat, you’re still fully aware of the awfulness that is MND.

And then the question none of us really wanted to ask because it can differ so widely from person to person. How long does Joanna have? Three to five years from when the symptoms first started, we’re told, although it might be longer.

I’m quickly learning there are very few ‘definites’ in the progression of MND. We are clinging on to the hope that she can retain her independence and quality of life for as much of that time as possible.

It felt impossible to believe the happy, kind, vibrant and fun-loving sister of just a year ago now faced an unutterably bleak future. And it seemed like a cruel twist of fate that Joanna had been diagnosed with a life-limiting disease at exactly the same age our mum was when she was told she had terminal pancreatic cancer.

The effect on our family has been all-consuming and our lives have changed beyond all recognition. I moved back to Guernsey with my husband in October to support Joanna and our family through whatever is to come. What became clear very early on is, however bleak the prognosis, there is help out there – both physical and emotional – for people like Joanna with MND and other neurological diseases.

That said, there is no cure – at least not yet. There are very few drugs. One called Riluzole offers the possibility of a 10% reduction in symptoms – but it doesn’t help everyone and the ‘benefits’ may not be worth the potential side-effects. We still have no power over this disease or definitive idea about how long we’ll have Joanna in our lives.

For me, the complete lack of control over what’s happening made me determined to do something to help. So while I can’t cure MND, I can use my journalistic skills of old to talk about the incredible team of people in the island making the lives of those diagnosed with MND more bearable, to raise awareness of the work that’s being done to make MND a disease of the past and – perhaps most importantly – to tell some of the stories of those currently living with MND in Guernsey, starting with my sister, Joanna.

JOANNA’S STORY

YOU never hear anyone with a bad word to say about Joanna. Her love of life (and dogs), her diligence and professionalism at work, her amazing baking skills and her incredible kindness are just a few of the many qualities that set her apart.

She first noticed something wasn’t right in September 2020 when she began to feel a disconnect between her brain and her right leg, making her feel like she was going to fall over. With no improvement – and after a bad ankle sprain, the long process began to get a diagnosis. Two years on from the first symptoms, she was told she had MND.

‘In one way it was a relief to finally get a diagnosis,’ she said. ‘I felt upset knowing how this would impact on other people and it takes a while to process. I’ve gone through every emotion, from anger to denial to despair since then. I’ve always been one to cope in the moment and then, when the dust has settled, fallen apart. It’s the first thing you think about when you wake up and the last thing you think about when you go to bed. I always pooh-poohed mental health until this happened. I get good days and bad days and if I have a really bad day it will linger with me for about a week. At times I feel as though I’m becoming a bit more withdrawn from people.’

The progression and initial symptoms of MND differ widely from person to person, which may explain why it’s so difficult to diagnose. Joanna was told she had a slowly progressing, lower limb MND but a definite prognosis of how long she may have to live was impossible. The average is one to three years from diagnosis.

‘The not knowing is the really tough bit. It all goes back to being concerned about other people. If you’ve got a diagnosis of a terminal illness and there’s no hope, then why make it drawn out? And when people say you’re “lucky” because it’s the slow progressing one – it doesn’t help. I’m 18 months down the line – I can’t work, I’m exhausted all the time, I’ve no interest in things, I’ve got no motivation, I can barely walk, I fall over. I take my little dog Oscar out on my mobility scooter every day and maybe try to do one other activity for an hour and that’s a lot better quality of life than a lot of people have got. But from being an energetic, hard-working person, it’s very difficult to accept and I just think it would be easier all round if they said it’s progressing quickly and you’ve got a year – for both me and for my friends and family.

‘I’m very lucky to have a lot of good friends. I can remember Mum saying, if you can count your good friends on the fingers of one hand, then you’re doing very well in life. So I’m doing very well in life! But I also feel guilt for putting people through this, even though I know I’m not responsible. You try to make them feel better but ultimately you’re the cause of their pain.’

Re-homing Oscar has brought a new lease of life to Joanna, who lost her two dogs back in 2022. She never intended to get another dog but there followed a moment of serendipity…

‘I started reducing my hours at work and then in September 2023, my husband said he thought it was time to give up altogether. My amazing doctor (Dr Jocelyn Aitken from Cobo Health Centre) agreed and when she asked what would make me happy, I said “a little old dog that would just snuggle with me”. The next day a friend rang and said 10-year-old terrier Oscar needed rehoming and did I know anyone who might be able to help? So I stopped working in the October and collected him two days later. He’s my constant little companion and it was just meant to be.’

Determined to make the most of the time ahead, Joanna and husband Richard have been taking some of the holidays they’d planned to do when they retired. With a career spanning nearly 40 years in finance, Joanna has made sure the figures all add up to a secure future for Richard.

‘If he can have that money to make his life easier and to be able to do the lovely stuff that we could have done together – do it for us – for me – that’s what I want,’ she said. ‘It feels like the right thing to do for me and him.’

As for what keeps her awake at night, Joanna’s biggest fear is watching her body give up working while her brain stays pin sharp.

‘Death doesn’t worry me. What worries me is not being able to communicate or do anything for myself but still having an active mind,’ she said. ‘I’m not religious – when I’m gone, I’m gone. I think a lot of people would say I’m “lucky” to be slow progressing. But today is going to be better than tomorrow and tomorrow is going to be better than the day after. It’s a downhill slope and you just think, why prolong it?’

MND patients in Guernsey – and people suffering from other neurological conditions like Parkinson’s and MS – have a whole raft of support available to help them with everyday life, and planning for whatever time they have left. From an MND nurse to an occupational therapist, a neuro- physiotherapist to a speech and language coordinator, there are no shortage of medical professionals on hand to help. That’s in addition to the local GPs and UK-based neurological experts who provide the initial diagnosis and routine check-ups every six months or so. Joanna has been overwhelmed by both them and Guernsey Motor Neurone – the local group which coordinates equipment, wheelchair provision and voice banking – a system to make sure sufferers can continue speaking with their own voice for as long as possible. They also support anyone wanting to raise money for MND.

For now it’s about coping with the extreme tiredness and coming to terms with the progression of the disease. Joanna and Richard celebrated their 21st wedding anniversary last September and decided to renew their vows to each other as a way of bringing their family and friends from different parts of their lives together.

Existing friendships – like those from her university days in Liverpool – remain a constant source of joy for Joanna who is adamant she wants to be remembered how she currently is – good fun, positive, happy, sociable and kind.

‘It’s funny but when I read the comments in the card my work colleagues gave me, they are just so lovely. Normally when someone leaves you put “sorry, take care, bye!” But there are some really lovely comments and it makes you realise how people see you and how much they care. I’m not a bad person. I’ve always known I was quite a tough old cookie but I’m more fragile than I thought. And I’m very loved.’

• For more information about MND and where to go to for help, visit www.guernseymotorneurone.co.uk. You can also contact Susie at susiehunt2024@gmail.com

MND – THE FACTS

• MND is short for motor neurone disease and affects nerves known as the motor neurones which are found in the brain and spinal cord and help tell your muscles what to do.

• It affects up to 5,000 adults in the UK at any one time and there’s a one in 300 risk of getting it across a lifetime.

• MND is also known as ALS, which is used as an umbrella term for MND in some countries.

• It affects adults of any age but is more likely to affect the over-50s.

• MND symptoms will vary from person to person and progress at varying speeds which makes the course of the disease difficult to predict.

• The disease is life-shortening and there is no cure but there are ways to manage symptoms to help achieve the best possible quality of life.