A disease that knows no boundaries
Motor neurone disease robs sufferers of so much, but community and family support can make a world of difference, writes Toni Jordan.
In 1995, Phil Brady, a member of CPA Australia, was a busy Sydney accountant, living in the suburbs and working in the city. He enjoyed his job, was happily married and a proud father to four children. For a while, he'd noticed a difficulty handling chopsticks nothing serious, just a strange awkwardness he'd never had before. Then his right hand began cramping up.
'I didn't take any notice at first,' Brady says, but eventually visited his GP.
Diagnosis took a long time the symptoms seemed so minor at the beginning but after six months Brady was sent for tests with a neurologist. The results weren't good.
Brady became one of the 120,000 people around the world who are diagnosed with motor neurone disease every year. It can be a slow death.
Motor neurone disease, or MND, is an umbrella-name for a range of diseases that affect the motor neurones. In the US, it's often called Lou Gehrig's disease, after a famous baseball player who had the condition. A common sub-type of the condition is amyotrophic lateral sclerosis (ALS). A well-known sufferer is the British physicist Professor Stephen Hawking.
Motor neurones are the nerve cells that control the movement of muscles. MND sufferers can't control the muscles of their arms or legs, or those that control breathing, swallowing or speaking.
'It's a disease of progressive loss that affects people in their prime, 40 to 50 years old, often with teenage children,' says Carol Birks, national executive director of MND Australia.
As the muscles progressively fail, sufferers need help with formerly simple tasks, like feeding, moving, communicating and dressing.
'You lose your job, you lose your social connections,' Birks says. 'It's a spiralling series of losses. They're robbed of their future.'
In Australia, 1400 people have MND, but a glance at the internet site of the International Alliance of ALS / MND Associations shows the global spread of the illness. There are ALS / MND associations around the world providing information and support, and promoting the best possible care for people living with ALS / MND in their community.
This is a disease that knows no geographical boundaries and can initially present in many different ways.
'Sometimes it starts with a hoarse voice or difficulty swallowing,' says Birks, or, as in Brady's case, it might begin with weakness, twitching or cramping in the hands or feet. In the majority of cases, intellectual capacity and memory are not affected, sufferers are very aware of the failings of their muscles. And, although survival time varies between sufferers and some cases progress more slowly than others, there are no remissions.
Support for further research in MND is vital, because the cause is 'only known in a very small percentage of people, around 2 per cent, with a specific gene,' says Birks.
'Environmental and genetic risk factors are being investigated, but as yet there's nothing definitive.'
Vital work into possible cures is also under way, with researchers investigating the way nerve cells are controlled, the potential for stem cell therapy, and the way ageing motor neurones repair.
'There is one current treatment that slows down the progression of MND, so the earlier the treatment begins, the better,' says Birks, emphasising the importance of an early diagnosis.
Everyday support for sufferers is also crucial. To move around the house, Brady uses a walking frame. When outside he uses an electric scooter or a wheelchair. He is increasingly fatigued, finds it difficult to concentrate and, despite the support of family and friends, finds it frustrating to ask people to do things for him. He is embarrassed speaking on the phone to people he doesn't know, worried that he won't be understood or will be thought drunk. Managing sufficient food intake is another challenge: he is never hungry and has no appetite. Even with medication, he finds eating a chore, and relies on advice from his dietician and food supplements to maintain his body weight.
All this brings a large responsibility to the family carer, usually a spouse, child or other family member. Brady's wife Trish writes his correspondence, helps him dress (bending to pull up socks or manipulating fingers to do up buttons is near impossible), makes his phone calls and cooks and cuts up his food. MND Australia reports that carers and families also live with the impact of MND, and that the emotional needs of carers should be considered. Another factor is the loss of income: not only are sufferers unable to work, but caring for someone with MND is frequently a full-time job. This can have financial implications on an entire extended family.
MND progresses at different rates, and Brady considers himself 'lucky' that his disease has progressed slowly. Still, he hasn't taken MND lying down. Already well past the average survival time after diagnosis of two or three years, he's still enjoying life and planning for the future.
'I get a lot of support from my neurologist, dietician and the local disability support network,' says Brady, who is often touched by the public at large. 'People are most willing to lend a hand.'
If you are keen to help, or have some ideas about raising awareness of MND and support for it, CPA Australia president Alex Malley would like to hear from you. Post your comments on his blog or send him an email.
Toni Jordan is a Melbourne-based writer.
Further information
Reference: March 2008, volume 78:02, p. 75 76
