Appointments with the occupational therapist, physiotherapist, respiratory service, neurologist, GP and massage therapist take time, as do exercises and voice banking.
Time is spent sourcing and learning to use equipment to navigate deteriorating physical capabilities eg, walking sticks and dictus splints (lifts the toe to avoid tripping), eating utensils, wheelchairs, hoists, eye gaze equipment and seats and beds that help lift a person.
Some equipment is unfunded. MND provides a compelling argument for income protection insurance.
Motor neurone disease occurs in one in 300 to 400 people in a lifetime, 90 per cent with no family link.
However, relatively few are affected currently as MND can progress fast, with some dying three to 12 months after diagnosis.
Stephen Hawking’s 55 years with MND (ALS variant) is an aberration. Only 10 per cent live more than five years after symptom onset with ALS; few last a decade.
Other variants include progressive bulbar palsy, affecting muscles of speech and swallowing first, with shorter life expectancy than ALS; progressive muscular atrophy with longer life expectancy; and primary lateral sclerosis affecting only upper motor neurons, with good life expectancy.
Some have frontotemporal dementia, with cognitive changes.
I appear to be in the top 10 per cent for longevity, few have my capability 16 months from diagnosis and four years from symptom onset, but I am progressing.
There is no cure, but studies suggest riluzole provides a survival benefit of six months or more.1 Regular liver function tests are required.
Considerable research activity, including viral vectors for gene editing, provides hope for a cure or disease modifier.
New Zealand’s Centre for Brain Research is active in MND research, including holding an MND patient registry funded by Motor Neurone Disease New Zealand and brain tissue from MND sufferers. MND NZ funded the first patient treatment trial recently.