Back in 2014, our Facebook timelines were filled with people drenching one another with buckets of ice water. It was light-hearted fun, and entertaining to watch and participate in, but there was a serious message behind it all. That message was all too familiar to Amy Senior; the previous year, her dad Ian had passed away after suffering from Motor Neurone Disease (MND): the same illness for which the so-called Ice Bucket Challenge was raising money and awareness. Three years on from the Challenge, MND remains incurable, but there have been significant research developments – and York’s own Department of Health Sciences is helping to make a difference.
“If you asked anyone who knew him, they would tell you that my Dad was the life and soul of any party,” Amy tells me. But after his MND diagnosis, Ian “lost his speech almost immediately” and, over the following four years, his ability to eat, drink and move were all taken from him by the disease. He was fed through a PEG tube, and “by the end, he was only able to move his thumb very slightly which helped us to understand if he needed anything.”
The Motor Neurone Disease Association (MNDA) received the majority of UK Ice Bucket Challenge donations. According to their website, MND affects the nerves, called motor neurones, in the brain and spine which ‘tell your muscles what to do.’ The disease causes the messages sent from these nerves to ‘gradually stop reaching the muscles, leading them to weaken, stiffen and waste.’ As a result, movement, speech, eating and breathing all become affected. It is a significantly life shortening illness: Ian lived for four years post diagnosis, but Amy tells me that life expectancy is normally around two years. Treatment, therefore, is focused on managing symptoms to improve quality of life.
‘Motor Neurone Disease’ is actually an umbrella term for a number of illnesses which fall under this category. Adults can be diagnosed at any age, but people over 50 are more likely to be affected, and in the UK 5000 adults suffer at any one time from the disease. Amyotrophic Lateral Sclerosis (ALS) is the most common form, and is used as the umbrella term in the US rather than ‘MND’. The other types are Progressive Bulbar Palsy (PBP), which mainly affects the face, mouth and throat; Progressive Muscular Atrophy (PMA), which progresses slower than ALS; and Primary Lateral Sclerosis (PLS), the rarest and slowest form of MND, where symptoms tend to be focused on the lower limbs.
Amy remembers him “drinking a pint or two down his PEG tube” at family celebrations, despite this being strongly advised against
In all cases, however, MND is a debilitating and frightening disease, and like any serious illness it drastically affects friends, family and carers. When Ian was diagnosed, none of Amy’s family had ever heard of MND before, which made it “very scary.” Harder still was seeing her Dad be so altered by the disease. Amy is “one of four children that he spent every Saturday taking to football, dancing, or horse riding,” and when he was well, Ian “had military precision when organising fam- ily trips to London and Rome, loved cooking, had a brilliant sense of humour, and a not-so brilliant ability on the dancefloor (not that that stopped him from trying!).” But MND took all of that away from Ian and his family.
Little is known about the causes of MND. Although in some cases there is a family history of the illness, this number remains small; as a result, research is ongoing. The MNDA stresses that ‘MND affects everyone differently’: symptoms vary between patients, as does the progression of the illness. Very rarely, patients can develop a specific type of dementia as a result of the disease, but in most cases changes to thinking and behaviour are mild. Ian never lost his sense of humour: Amy remembers him “drinking a pint or two down his PEG tube” at family celebrations, despite this being strongly advised against – “he didn’t care!” He was “incredibly stubborn and hated what MND did to his body,” and this was just one way of fighting back while he could. Although some dismissed the Ice Bucket Challenge as ‘slacktivism’ and accused participants of only being involved for the likes and shares, the campaign actually went on to raise over $100m in just 30 days. These donations went on to fully fund numerous research projects into the causes and treatment of MND, and two years later, in 2016, came the news that one of these studies had identified a new gene associated with the disease.
It’s this kind of research that could have helped Ian and his family feel more supported. Amy tells me how in the week after his death the family was offered a device called Eyegaze which enables people to communi- cate using just their eyes. It would have been a “gamechanger” for the Senior family, had they been offered it in time. Instead, “it was a real kick in the teeth”. Their experience back in 2013 was that “nothing moved fast enough as healthcare professionals didn’t understand how serious MND was, and how quickly it progressed.”
But, thanks to the subsequent increase in awareness and research efforts, the situation could now be beginning to change for sufferers of MND and their carers. A flagship study funded by Marie Curie and the MNDA is being undertaken by Dr Kate Flemming of the University’s Health Sciences department. Dr Flemming’s research is part of a wider project of three studies at the Universities of York, Manchester and Sheffield, all focusing on different aspects of palliative care. Although the ultimate goal for researchers into MND is to find a cure, for today’s sufferers palliative or life-lengthening care is critical. The York study is being completed in conjunction with St Leonard’s Hospice, and focuses on developing a “more complete picture of the needs of patients and carers”.
These developments are important to Amy and her family, too. Since Ian’s diagnosis they have been committed to fundraising including multiple sponsored runs, completing the Three Peaks Challenge, “and an event to mark what would have been Dad’s 50th birthday, with music and all his friends and family there.” But last April they set themselves a bigger challenge: to raise an additional £5000 in the following year to commemorate the fifth anniversary of Ian’s passing. It’s been a busy few months: “So far we had a collection at my wedding in May, a tug-of-war event in July and a 10k race in September,” lists Amy. These efforts have brought them to a total of £3500, with over half a year still to go.
There is currently no cure, and only one treatment which may extend the life expectancy for some people.
That money will itself help to fund the same kind of research currently being undertaken by Dr Flemming. The family plan to split the total between the MNDA, which provide “practical and emotional support” and Marie Curie. “My mum was Dad’s full-time carer,” explains Amy, “and due to the nature of the illness it was really difficult for her to leave his side. Marie Curie nurses looked after my Dad during some nights, so my mum was able to get a good night’s sleep.” The charities offer these services alongside funding research projects, which are of equal importance to Amy, who stresses that “there is currently no cure, and only one treatment which may extend the life expectancy for some people. Nobody knows the cause of MND, and when a third of people of MND die within one year of diagnosis, that research is imperative.”
You can donate to the Senior family’s fundraising efforts by clicking here.