By CAITLIN SCARR (2013)
Sally Strahan’s mother, Irene, died two days before Christmas. The year was 1993, and Irene had been battling motor neurone disease for 18 months.
Living interstate and pregnant with her second child, it was a traumatic experience for Sally, her mother, and their family.
“You’re totally helpless and that I think was the feeling of our family,” Sally said. “There was such a strain between us and yet we didn’t know how much to talk to each other, we didn’t know how the other person was feeling and how to take it.”
Twenty years have passed, and little has changed. Motor neurone disease is still relatively unknown, and remains untreatable and fatal.
Motor neurone disease, also known as amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, is a neurological disease in which the motor neurones die. Motor neurones are the nerve cells that control muscle movement, and when they die the muscles are unable to respond and begin to waste.
Motor neurone disease sufferers lose the ability to walk, write, speak, swallow, and eventually breathe. The muscles simply cannot respond. The majority of motor neurone disease sufferers are mentally alert and completely aware of what is happening to their bodies.
The cause of the disease is unknown, there is no treatment or cure, and the disease is always fatal. Experts are aware of a genetic component in about 10% of cases, but effective treatments are yet to be discovered.
Macquarie University have just unveiled their new motor neurone disease research laboratory. It is fitted with extensive equipment and a team of experts devoted to researching treatments for motor neurone disease.
Macquarie University dean of medicine Simon Foote said in an interview with The Australian that he hoped the research facility would lead to “some sort of major breakthrough within the next decade.”
Although this development in research is exciting, the reality of motor neurone disease is that most people currently living with the disease will die long before any successful treatment is developed.
The average life expectancy for motor neurone disease varies, but it is generally considered to be between two and three years. Half of sufferers will die in the first 14 months, the other half living for anywhere between two and 10 years, and occasionally even longer.
Retired neurologist and associate professor Dr Barrie Morley has noted that there is some correlation between the initial symptoms and life expectancy.
“People who present with the first signs in the feet,” Dr Morley said, “tend to have a somewhat longer prognosis than people who present with it up in the throat area.”
According to Dr Morley, most people first notice some wasting in the hand muscles, or “foot drop,” where the person is unable to properly lift their foot. The disease then progresses and affects nearly all the muscles in the body.
The only muscles not affected in motor neurone disease are in the eyes and eyelids. This can become the main source of communication as the disease progresses.
“If the patient has been rendered completely inarticulate,” Dr Morley said, “then by adopting a code of eye-blinking or moving of the eyes, it can be a means of communicating.”
The debilitating nature of the disease has stuck with Sally. She watched her mother lose the ability to write letters, to peel a potato, to brush away an insect, or even to chew her food. They had to blend Irene’s food into mush for her to eat it.
“She couldn’t swallow in the end,” said Sally. “She absolutely hated it. We also had to make sure that the food was room temperature before we gave it to her.”
Cherie Orchard is aware of the difficulties facing motor neurone disease patients and their carers. Cherie’s husband Sydney died of motor neurone disease in November 2012, three years after his diagnosis. Cherie was his primary carer.
“We felt so desperately unable to cope and desperately useless,” she said. “I think that’s how you actually feel, when you have someone with motor neurone disease. You just feel so unable to cope, make a difference to the patient, so you try to do it when it’s all over. It is a day-by-day thing, and then when it’s over, it’s dreadful, absolutely dreadful, because not only are you desperately sad, but you’re desperately tired.”
Cherie was exhausted caring for Sydney: “They can’t move, they can’t talk, it’s like they’re a baby. So you’re the whole time advocating for them, and getting angry on their behalf, and getting your liver in a twist, and you’ve got no time to be loving them. It’s either that or you’re wiping their bottoms or showering them or trying to dress them or trying to feed them.”
Not only is day-to-day care time-consuming, but organising visits to the doctor can be a difficult task. Emily Maher, Cherie and Sydney’s daughter, explained this process.
“A [general practitioner] (GP) is not trained in motor neurone disease,” Emily said. “So they try to pass you off to the neurologist, but he’s a specialist, so he doesn’t actually want to see you once a week. So [the neurologist] is palming you off to the GP.”
Sydney also received regular home visits from other health professionals, including occupational therapists, physiotherapists and speech pathologists.
“You’ve got people trooping back and forth through your house the whole time, but no one actually helps you,” Cherie said. “They’re never going to get better. It’s a terminal disease. They don’t need people prodding and poking and assessing them all the time. That’s just rubbish, and it just takes up my time and his. And they lose their dignity when they’re being prodded and poked.”
The energy and time it takes trying to organise care for motor neurone disease patients is detracting from the role loved ones would prefer to play.
“You’ve only got that little bit of time with your loved ones,” Cherie said, “and it’s just spent fighting with people. Well I did, anyway.”
When Scott Sullivan was diagnosed with motor neurone disease in October 2010, the first question he asked his neurologist was: “Isn’t that an old person’s disease?” Scott was in his late thirties.
That morning, he had dressed for work. He was executive manager at Suncorp, and was to go to his appointment before continuing on to work. His wife didn’t go with him. They thought he was just suffering from a pinched nerve.
“I didn’t end up going to work that day,” Scott said.
Scott believes that his life hasn’t changed too much yet. He has had adjustments made to his house, and has noticed a weakening in his body recently.
“I have two young kids,” Scott said. “I can’t play with them any more. I can’t pick up my son because I am afraid of dropping him… But all in all life is still really good… I have no right to be complaining at all.”
Ian Davis, 35, a doctor from Melbourne, diagnosed himself with motor neurone disease in September 2011. His quality of life hasn’t decreased too much.
“I’ve had to pull back a bit [at work],” Ian said. “I’m basically at a desk job now… stairs are getting harder.”
Weeks after his diagnosis, Ian saw Scott on television raising awareness for motor neurone disease, and immediately made contact. They quickly found a common interest in using their time to help others with the disease.
From May 3rd to 18th this year, the pair rode from Brisbane to Sydney to raise awareness and funds for motor neurone disease. The pair rode in tandem, on a custom-made bicycle that catered to their strengths: Ian’s arms and Scott’s legs.
“While we still can, we have to do something like this [ride],” Ian said. “It’s always been a big risk, us planning [the ride] three months ago because that’s the thing with this disease. You don’t know if it’s going to ramp up and go fast.”
The ride was a success, and all funds raised were donated to research for motor neurone disease. Donations are the main source of funding for research in Australia.
Janet Nash, the executive officer for research for Motor Neurone Disease Australia, is involved in distributing funding for motor neurone disease research.
“Last year we awarded $2 million in grants,” she said. “All of the money has come in donations, and it’s just been escalating so rapidly in the last few years.”
Despite the rise in donations, government funding for research is virtually non-existent.
“We don’t live long enough,” Ian explained. “There’s no incentive for drug companies or for the government to put enough money in [to research].”
Motor neurone disease patients have such a short life expectancy that their burden on the healthcare system is extremely light, in comparison with chronic diseases like cardiovascular disease or diabetes.
The state governments give some funding to their motor neurone disease organisations. Motor Neurone Disease Victoria receives approximately $2 million a year in government funding which helps with support and equipment.
Motor Neurone Disease Queensland receives annual government funding of about $40,000. That works out to about $133 per Queensland sufferer per year. A new electric wheelchair can cost more than $5000.
Scott established Queensland-based foundation MND and Me as a response to the disappointing support he encountered as a motor neurone disease sufferer.
“When I researched Motor Neurone Disease Queensland,” Scott said, “the services were really in a very sorry state, so there was an opportunity there to actually do something tangible and to improve the outcome for Queenslanders with motor neurone disease…I wasn’t going to sit around the house and twiddle my thumbs. There were jobs that needed to be done.”
For those unable to access sufficient care and support, there are some government care packages available. Eighteen months before Sydney Orchard died, he was approved for a high-care package to help with his day-to-day care.
According to Cherie, Sydney was entitled to 16 hours of care per week. They never received that amount of time.
They were told by various care providers that there wasn’t staff available for that level of care. Instead, they received three showers per week and a cleaner who would only clean the areas in their home where Sydney’s wheelchair had gone.
With such unreliable funding, sufferers and their families have taken it upon themselves to raise funds. Cherie and her family alone have raised about $50,000 through various fundraising events.
Ian and Scott’s bike ride has currently raised over $62,000. These combined are more than double what the government has contributed to Motor Neurone Disease Queensland.
Graham Opie, the chief executive officer of Motor Neurone Disease New South Wales, believes the national disability insurance scheme (NDIS), or DisabilityCare, is a step in the right direction.
“[Families] can determine what’s best for them,” Graham said. “The problem with the NDIS is if you’re diagnosed over the age of 65, you’re no longer eligible for it, which is a big setback. Nearly 60% of our members are diagnosed over the age of 65, so they fall into the aged care category, which can’t support people with motor neurone disease.”
He does believe that support is difficult to access due to lack of awareness of the disease. Cherie Orchard agrees.
“It’s very difficult trying to do something with motor neurone disease,” Cherie said. “Nobody seems to know what it is. Well I didn’t even know what it was until my husband got it. It’s terrible.”
The lack of awareness is a common theme among those with motor neurone disease experience. Ian says there is “definitely” a lack of awareness about the disease in the wider community.
“I think out of all of the diseases that there are,” Ian said, “this one has the greatest ability to scare people into doing something. I think [Scott and I are] prime examples. Neither one of us did anything to deserve this.”
Trying to get the wider community to engage with motor neurone disease is not easy.
“Dying people make other people nervous,” Ian said. “Nobody wants to be challenged by their own mortality.”
Mortality is inevitable in motor neurone disease sufferers. The Orchard family wanted to be prepared for Sydney’s death.
“No one will tell you how they die,” Emily said. They had been told that he would just stop breathing. “In that last week, Dad started coughing… You didn’t know it was a cough because he had no strength left in his lungs. So he was coughing, which was actually him choking and dying, and we didn’t know… It’s a shitty way to die, I can tell you that.”
Lyn, a Queensland resident, lost her husband to motor neurone disease about eight years ago. When he entered the hospital, dying, staff asked her what she would like them to do.
“I don’t know whether they were asking me to try and save him, or why. He hated his life. That’s something you don’t think of. You don’t think you’ve got to make a decision… I think it would have been stupid to tell someone to try and save his life so he could go on and be miserable for another six weeks.”
Sally’s mother Irene died due to complications from surgery. Doctors were trying to place a feeding PEG (a percutaneous gastrostomy) in her stomach.
“She couldn’t take the anaesthetic because of her chest,” Sally said. “The disease was all through her diaphragm and in her throat and in her mouth really badly by then. It was all down in her shoulders and her diaphragm and she had nothing there to breathe, to help her breathe. All her muscles had deteriorated.”
The doctors placed Irene on a ventilator, where she spent several hours before the family made the decision to turn the machine off. It still took hours for her to die, while her family sat with her and waited.
“There needed to be,” Sally said, “and I hope there is now, a lot more knowledge [of] what you are going to go through and the support to go through it.”
For those left behind, they only hope that awareness of the disease is growing. It is a difficult task, but Sally believes time has made a difference.
“What I’d been through and the experiences of my mum’s death was never discussed at all. Nobody knew what motor neurone disease was,” Sally said.
“[Now] when I say my mum died of motor neurone disease, more people know about it than they used to back then.”