David Niven lived with an extremely challenging disease

Motor Neurone Disease: Expert on early signs and symptoms

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Famed for Around The World In 80 Days (1956), Separate Tables (1958), The Pink Panther (1963), and Murder By Death (1976), the Oscar award-winning actor eventually couldn’t swallow, let alone act in films any more. In a letter penned to his dear friend, Anthony Quayle – a letter he had somebody else write – Niven shared how the condition had affected him. “I can’t write, swallow well, [or] walk properly,” the father-of-four revealed.

“But, apart from this, I’m fine,” he quipped. “Don’t, whatever you do, get motor neurone disease.”

Motor neurone disease

Considered a “rare” condition by the NHS, motor neurone disease “progressively damages parts of the nervous system”.

Otherwise referred to as “amyotrophic lateral sclerosis (ALS)”, the condition occurs when nerve cells in the brain and spine stop working properly.

“It’s not clear what causes motor neurones to stop working properly,” the NHS says.

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In the beginning, symptoms of motor neurone disease happen on one side of the body.

Common early symptoms might include:

  • A weakened grip, which can cause difficulty picking up or holding objects
  • Weakness at the shoulder that makes lifting the arm difficult
  • A “foot drop” caused by weak ankle muscles
  • Dragging of the leg
  • Slurred speech (dysarthria).

As the disease progresses, the other side of the body is affected and the symptoms become more severe.

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Eventually, a person who has motor neurone disease may be unable to move, communicate, swallow, or breathe easily.

Typically diagnosed in people over the age of 40, most people first develop symptoms in their 60s.

While there is still no cure for the condition, treatment is utilised so that the person affected has the best quality of life possible.

“For example, a breathing mask can greatly help the symptoms of breathing and weakness,” says the NHS.

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“And a feeding tube (called a gastrostomy) helps maintain nutrition and overall comfort.”

Motor neurone disease is a “severely life-shortening condition for most people”.

Niven, for example, died at the age of 73 at his home near Lake Geneva, Switzerland.

“Life expectancy for about half of those with the condition is three years from the start of symptoms,” the NHS adds.

Living with the disease has been described as “extremely challenging” and “terrifying”.

Most often, end-of-life care happens in the person’s own home, like it did with Niven.

“In most cases, a person with the condition will die in their sleep as the end stage of gradual weakness in their breathing muscles,” the NHS explains.

David Niven starred in A Matter Of Life And Death, which is showcasing on Saturday, December 10 at 2pm on BBC Two.

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