‘Death sentence’ disease
Amanawatu¯ woman living with motor neurone disease remains hopeful of a miracle treatment to take back her life, after watching family members die young from the same condition.
Kylie Matena, 33, was diagnosed with the disease 10 years ago and has watched her family suffer at its pernicious hands.
Motor neurone disease kills nerve cells that control muscles enabling us to move, speak, swallow and breathe. With no nerves to activate them, these muscles gradually weaken and waste away.
Matena’s father died at 42, her grandfather was 39, her aunt 43, and her uncle was the first in the family with the disease to make it to his 50th birthday.
‘‘I try not to think about it. I just think I’mhanging on for some sort of treatment.’’
Matena, from Feilding, spoke to Stuff during Motor Neurone Disease Awareness Month, amonth to learn more about the disease and generate funding, research and conversation.
There is no cure for the disease, and it has an average life expectancy of between three and five years once symptoms appear and it’s diagnosed. ‘‘It’s not incurable, it’s underfunded.’’ Matena said the reality of the disease was that some people were diagnosed and then dead within six months.
She said she was fortunate that for her family, the disease progressed slowly. The death sentence had been extended to 10-15 years.
For many years Matena could hide her disability, walking small distances and not leaving the house.
Now, after 10 metres she’s exhausted and unable to lift her legs.
Her legs would ‘‘noodle’’ below her and she often collapsed to the ground until her strength returned.
‘‘I’monly just coming to terms with the physical deterioration myself.’’
She has to accept that she can’t do what she used to, and learn to ask for help.
Matena mostly suffers from lowerbody motor neurone symptoms and can’t stand nor walk for long periods without leaning on something or using her walking stick.
Fatigue is also a big problem and after a few hours out of the house she is exhausted.
She uses a ventilator at night to help her breathe and to allow her body to get restful sleep instead of fighting for the next breath.
The first thing she does when she wakes is take a cocktail of tablets to help with pain. Forty minutes later, they kick in and she is able to get up.
Mornings are the hardest. After rushing her children to school, showering, getting dressed and having breakfast, she is knackered.
Matena works from home running a business that supports people with disabilities, while completing her bachelor of science.
Her four children, aged 19, 17, 15 and 6, have a 50-50 chance of inheriting the disease. Matena is undergoing gene therapy to find the type of gene responsible so her children can be tested for the disease without the presence of symptoms.
Despite being heavily researched, it isn’t known what causesmotor neurone disease and public awareness about it is low.
‘‘The more attention this cruel disease gets, the closer we get to discovering a treatment or cure,’’ she said.
New Zealand has the highest known rate of the disease in the world, with an average of two people diagnosed every week.