Understanding motor neurone disease: Motor Neurone Disease Awareness Day--June 21--aims to make the public and health professionals aware of the urgent practical and emotional needs of those with the disease and their families.

MOTOR NEURONE Disease (MND) is a rapidly progressive and disabling disease affecting the motor neurones and resulting in weakness of the voluntary muscles. Onset is insidious and there is no cure at the present time. First symptoms may include stumbling, foot drop, weakened grip, hoarse voice, slurred speech, cramp or muscle wasting and/or tiredness.

Eighty New Zealanders each year will die from this condition, many in mid life. They will lose their ability to perform even the simplest of daily tasks. As a result, they and their family will experience considerable emotional, psychological and spiritual distress throughout the course of the disease.

My interest in MND was heightened two years ago after I attended a palliative care conference in Tasmania. Keynote speaker was medical director and palliative care consultant at Wisdom Hospice, Kent, the United Kingdom, David Oliver, an international authority on this subject. Oliver inspired me with the depth of his knowledge and his compassion for the people who suffer this most debilitating disease. Oliver and New Zealand palliative care physician Anne MacLennan also toured New Zealand last year, sharing their knowledge with a wide range of health professionals. Videos of their presentations are available to hire or buy from the Motor Neurone Association.

As a palliative care nurse specialist, I have had the opportunity to share parts of patients' journeys with MND. I am also aware many health professionals are often not able to attain a sufficiently high level of knowledge of MND to provide the quality of care required by someone with the disease. At the same time, improved ways of managing the disease are constantly emerging. The rapid progression of the disease, combined with the complexity of needs associated with it, present a particular challenge to people with the disease and health and social services.

The disease takes three main forms. Amyotrophic Lateral Sclerosis (ALS) is the most common form and involves both the upper and lower motor neurones. It causes muscle weakness, spasticity, hyperactive reflexes, emotional lability, fasciculation and weight loss. People will survive from two to five years, from the onset of the disease.

Progressive Bulbar Palsy (PBP) affects about 25 percent of sufferers and also involves both the upper and lower motor neurones but is characterised by dysarthria and dysphagia, and predominantly affects older women. The average survival is six months to three years.

Progressive Muscular Atrophy (PMA) is the least common form, affecting about 10 percent of sufferers and predominantly affects the lower motor neurones, causing muscle weakness and wasting, weight loss and fasciculation. It affects men most commonly and the average survival is five years.

As there is no cure for MND/ALS, treatment should be palliative care from the time of diagnosis. The patient may experience many symptoms and the interdisciplinary team must carefully assess them. The approach at all times must be an holistic one, with the patient and their significant others being the centre of all discussions and decisions. It is important to address the significant issues while the patient is still able to speak and can make informed choices and advance directives for the future.

The aim of palliative care for MND/ALS is to reduce the effects of the disease, maintain the remaining power and enable the patient and family to live as full a life as possible.

Common symptoms

The most common symptoms for the person with MND/ALS cover physical, emotional, spiritual and social aspects. Physical symptoms include pain due to stiffness and immobility; dyspnoea due to diaphragmatic and intercostal weakness (this is often very frightening); dysphagia due to weakness of the face, mouth and oropharynx; drooling due to an inability to swallow saliva; emotional lability due to frontal lobe changes; cough due to reduced swallowing and increased secretions; insomnia due to fear, pain and insecurity; and constipation due to poor diet, reduced fibre intake and immobility.

Emotional symptoms centre around fears about the diagnosis, disability, sexuality, becoming dependent and fear of how they will die. Social problems include concerns about the family, change of role, communication difficulties, isolation and financial problems.

An interdisciplinary approach

Managing symptoms in MND demands strict attention to detail and requires an interdisciplinary approach. This is vital to maximise the quality of life for the person and to prevent difficult symptom issues in the terminal stages of the illness. A variety of health professionals such as physiotherapists, occupational therapists, speech-language therapists and district nurses can help manage and alleviate symptoms. Management must be discussed with the patient and family well before symptoms become a problem.

Pain: Non-steroidal, anti-inflammatory medications, muscle relaxants (baclofen) and opioids (morphine), which requires titration, are used, before long-acting opioids are commenced. Physiotherapy is useful in decreasing joint stiffness and muscle spasm

Dyspnoea: This is a very distressing symptom and the only measurable parameter that predicts survival. A calm approach is important and medications such as morphine can be helpful. Clonazepam drops are most useful in decreasing anxiety.

Non-invasive ventilation (BiPAP) can improve the person's quality of life but embarking on invasive ventilation requires very careful discussion. It is crucial advance directives are obtained and ventilation discussed early in the disease process, so appropriate decisions will be made in a crisis. A common misconception is that the MND patient will choke to death. The term "choking" is emotionally charged and, typically, death follows a rapid and sudden deterioration due to respiratory failure.

Dysphagia: This is very distressing and common symptom and the insertion of a percutaneous endoscopic gastrostomy (PEG) feeding tube may be helpful in improving quality and length of life. It needs careful assessment and early insertion if the patient should choose this option.

Terminal care: As the person's condition deteriorates, further assessments of their needs and adaptations to care will need to be made. Crises must be anticipated and medications must be available to attend to these crises, ie morphine for pain and distress, midazolam for sedation, and hyoscine for secretions and respiratory distress. Recent studies have shown that if good palliative care is provided for people with MND, most will die peacefully in their own home.

The care of the person with MND and their family presents a huge challenge to all involved. Care must be well coordinated, with strict attention to detail in assessing symptoms and planning care. The person with MND and their family must at all times be fully informed and supported to enable them to make wise choices. As health professionals, we must be creative about how we maximise each individual's potential throughout this progressive physical deterioration.

Useful resources for health professionals include:

(1) Oliver, D. et al (2000) Palliative Care in Amyotrophic Lateral Sclerosis. Oxford, New York: Oxford University Press.

(2) Leigh, N., Williams, V. and Abrahams, S. (2001) Motor Neuron Disease. European Journal of Palliative Care; 8: l, 10-12.

(3) MacLennan, A. (ed). A Problem Solving Approach for Health Professionals (flipchart) Motor Neurone Disease Association of New Zealand. This and other information is available on the MND Association website (www.mndanz.org-nz).

RELATED ARTICLE: coping with the disease's progression.

ELIZABETH Street of Wellington experienced her first symptoms of motor neurone disease in November 1999. However, she thought the difficulties she was experiencing turning her car keys and front door keys were symptoms of occupational overuse syndrome, not of MND.

A few months earlier, Street had resigned from her senior library position in order to work on short-term contracts and be at home for her teenage son. The two of them had recently moved to an apartment, following the break-up of her marriage. As luck would have it, the apartment had a rift and was in easy reach of the city and its network of social services.

Around six months later, Street began experiencing the same symptoms in her left hand as in her right. She had also been experiencing a lot of nerve twitching. Following an appointment with her GP, she was referred to a neurologist who ordered an electromyogram, the main--and very painful--means of diagnosing MND. "Most people spend a lot of time trying to have their condition correctly diagnosed," said Street. "I was lucky. Most GPs would barely see one MND case in their entire career and most don't like it because there's no treatment or cure. I'm lucky to have a neurologist who does take a professional interest in the disease and in me, although I didn't begin seeing him until a year after my diagnosis. Now he sees me every six months."

Street finds the progressive nature of the disease very frustrating. On the other hand, she and her family have had time to adjust to this reality, as opposed to someone having a stroke and therefore no warning their lives are about to change. "Going from being an independent, employed and useful member of the community to someone who is increasingly dependent on others and on an invalids benefit has been very difficult However, I am lucky to have a very supportive family and friends. You last longer if you have these networks."

Street relies on help from her sister and 16-year-old son for many daily tasks like showering and dressing. She was assessed two years ago for help with housework and is about to be reassessed for home help and personal care. She believes she has deteriorated more in the last six months than in the previous two years and expects she may be wheelchair-bound within two years. Fortunately, she still has some strength and grip in her hands and may soon use a walking stick to help her balance. The hospital occupational therapy team will also visit her this year to assess whether changes need to be made to her bathroom, to enable her to use less energy.

Street has a multi-disciplinary team on her case--a physiotherapist, neurologist, GP, palliative care co-ordinator, a respiratory physician, gastroenterologist, occupational therapist, speech therapist, the local MND Association field officer (a nurse currently researching the quality of care MND patients receive) and psychologist. Her sessions with the psychologist have given her techniques for keeping the cycle of anxiety and depression to a minimum. She has also found it helpful to discuss the issue of suicide openly and now takes a minimal dose of prozac to manage depression. "I now see this disease as part of life. Fortunately I am not in chronic pain and I can manage many of my symptoms. Having the opportunity to educate myself and my family about death is one of the good things about having a progressive disease. We prepare ourselves for giving birth but most of us are not given the opportunity to prepare for death. Most of my family and friends have now accepted that I will just slow down and stop one day. It won't be a sudden death."

At the same time, Street spends a lot of time keeping up to date with the latest research into MND. She is currently on a six- month trial of the antibiotic minocycline as well as a trial for creatine, a muscle-building protein. Although she admits there are "a lot of snake oil treatments out there", she is also aware of the positive research being done to manufacture a drug that will prolong the life of those with MND and delay the disease's progression. "Who knows when there'll be a research breakthrough? I accept, however, that I am unlikely to benefit."

Some months ago, Street made an appointment with a gastroenterologist to discuss the insertion of a PEG feeding tube. Deciding when to begin using the device was quite subjective. "I guess that will be when I am too tired to swallow or am taking too long to eat. Taking in calories in this way makes sense, as it then gives you time and energy for things other than eating. Some people have their PEG plugged in over night while they are asleep, then drink and eat as they wish during the day. I often eat standing at the kitchen bench, as this gives me somewhere to rest my arms."

Street is presently trialling a voice amplifier, provided by Enable New Zealand. As her disease progresses, she is finding it more tiring to speak and articulate her words clearly, a condition known as dysarthria. She has also looked at portable Light Writers and desk-top software like Hands Off. A speech therapist suggests she speaks more slowly, thus conserving energy.

"Talking and eating, particularly together, have always been my favourite pastimes. My social life was based around having people for dinner and going out for dinner myself. Now I prefer to eat first, then go out and socialise. As the disease progresses, the image I have of myself as a social being changes. You just have to think of other ways of enjoying social contact."

Mobility issues dominate Street's life. Now she has to choose carefully who she visits, depending on access, or she has to go with someone who will help her. "I encourage people to visit me at home now and for around an hour. Fortunately, I still get a lot of pleasure from reading and doing crosswords, but walking is getting harder."

On diagnosis, Street was told her prospects were two to five years. Her ambition was to see her then 13-year son through secondary school. "My timing is on track at the moment."

--Profile by Anne Manchester.

--Anne Morgan, RGON, DipN (London), PGCADN (palliative care), is a palliative care nurse specialist with the Canterbury District Health Board.