David Yates: A disease that takes everything from you

My very honest, kind, yet frank neurologist - a colleague and friend of 49 years - was quite correct when he told us that "MND is the worst way to die", after he had confirmed my self-diagnosis.

It has certainly been a challenge to our character and endurance, but it has still not been without it's lighter moments. It is important for one to endure the challenges - by maintaining a positive attitude, plus sensible physical and mental activity - in the hope that a treatment breakthrough might happen "on our shift".

Stephen Hawking, the brilliant British applied mathematician and theoretical physicist has apparently said there was not a lot of media coverage about motor neurone disease when compared with, say multiple sclerosis, yet the numbers affected are about the same. Hawking has fought with a type of the disease for years.

Many MND sufferers - surely the best advocates for the disease - lose the ability to speak, early in the process. As a result, I am motivated to use my middle finger to type this article, forged from personal experience to redress, in a small way, this gap in advocacy. This is a matter-of-fact presentation, giving insight into the life of a family touched by MND.

MND is one of the more common neurodegenerative diseases of adult onset, involving voluntary muscle groups. Sensation - touch, smell and taste - and cognition are not affected (apart from 10 per cent who develop a form of dementia).

The incidence is one or two per 100,000. About 10 per cent of cases are familial (transmitted between family members). Glutamate is the main facilitator of normal nerve transmission.

The excitatory signal ends with removal of glutamate, via transporter proteins. Glutamate in excess can kill certain cells involved in nerve transmission.

Italian researchers have recently discovered a flaw in the enzyme responsible for cleaning protein debris - including glutamate - that builds up around the motor neurone. Future treatment will involve boosting production of the cleaning enzyme.

I withdrew from medical practice on health grounds in 2000, having endured nine years of chronic pain syndrome, the legacy of being knocked off my bike by a car. Significant improvement in pain levels in March 2004 was replaced by the onset of symptoms, later identified as the commencement of MND.

I consider this to be more than coincidental. Brooking et al, have discovered elevated cerebrospinal fluid levels of glutamate in patients with CPS, in common with some MND patients.

The disease involves voluntary muscle, by causing progressive removal of myelin from the upper motor and in some cases, lower motor neurone sheath - myelin sheath - supplying the affected muscles.

Denervated muscle is a feature and - at least in the early stages - looks like a butterfly fluttering it's wings under the skin. It eventually gives way to twitching of digits and even long muscle tracts, with progression of the disease.

Voluntary muscle function inexorably slows to a glacial pace, before eventually stopping.

By contrast most if not all other diseases spare all or part of other bodily functions, such as speech, singing, eating, mobility and dexterity. It is difficult for the nervous system to accommodate, when the disease process is progressing.

Activities increasingly become single-minded uni-tasks. Balance problems are caused by an ongoing break in the feedback loop between peripheral muscle and the central nervous systems.

The addition of bulbar palsy transforms one into a combination of mewling babyhood and accelerated ageing. (Motor neurones gradually decline in number with advancing age.) Bulbar palsy is very real, stripping one of any semblance of dignity, through loss of control of emotions.

There can be a happy response, then tears, or unhappy response, then laughter, or a mixture of both in response to something that might be said by someone in person, or on TV, or other media, or even if one thinks of something that evokes memories. So, if I am seen crying in response to a word, sound or thought, I could be happy or sad, or both. Sympathy only aggravates the situation!

Music and singing evoke powerful emotions and memories, such that bulbar palsy invariably takes over. Patients with MND regress from normal food, through mechanically blended food, to nutrition by drip.

Eating and swallowing food is very antisocial, because they have to adopt the "navel gazing" position, while avoiding vocalising - all in order to prevent choking. I have been very fortunate that my wife, and later our daughter, both wonderful, adventurous cooks, have made my meals more appetising for me, in my time of need.

I now tend to feast on the memories of decades of eating wonderful cooking (including having to be the willing taster for trial runs of dinner party courses). One learns to cope with watching others eat delicious meals, but occasionally I can yearn for something simple like porridge, brown sugar, grated apples and walnuts, or homemade marmalade, toast and tea.

Eventually even swallowing mucus becomes a big problem because of the diminishing ability to cough. It often sits over the vocal cords.

Motor neurone disease also denies one the opportunity to talk with family and friends, to enable closure of one's life with them. Spare a thought for the "well" partner, who may have no family members on hand, or be part of a small family.

He or she has to do their usual chores about the house and property, plus those previously done by the unwell partner, as well as being the 24/7 caregiver. I salute them as the heroines and heroes of MND, with the researchers and helping agencies.

* David Yates is a retired family doctor and has advanced motor neurone disease. The first symptoms appeared in March 2004.

Motor Neurone Awareness week runs from June 21 to 27.

How you can help: Donations can be made to the MND Association, PO Box 24036, Royal Oak, Auckland 1345, through any branch of Westpac or via the MND Association of New Zealand website - http://www.mnda.org.nz/index.html.