• Degeneration

THE recent death of Stephen Hawking from amyotrophic lateral sclerosis (ALS) had great resonance in China.

The British physicist was an icon in the country, not just because of his intellect and the visits he made, but also because he was an inspirational figure for Chinese people with ALS, a fatal illness.

Despite being given just a couple of years to live after being diagnosed at age 21, Hawking lived for another 55 years — becoming the world’s longest-surviving ALS patient — married twice and had three children. While his body was in a wheelchair, his mind travelled the universe.

During a visit to China in 2006, Hawking made a point of speaking publicly on June 21 — on which Global ALS Day is marked every year — telling Chinese patients, “Physical disability should not stop you from moving forward, just as I do.”

Cui Liying, director of the department of neurology at Peking Union Medical College Hospital, said an estimated 100,000 people in China have ALS, which is known in the West as Lou Gehrig’s disease after a well-known baseball player who had the illness.

Although the average life expectancy of Chinese patients is three and a half years, about half of them die within 30 months, while 20 per cent live about five years, and only 10pc survive for more than 10 years, Cui said.

She added that global research into the illness has progressed slowly, and researchers have not yet been able to determine the cause or identify a cure.

“Although ALS is not yet curable, that doesn’t mean it cannot be treated. Treatment refers to not just extending people’s life spans by the use of drugs, but also careful nursing, which is important to reduce pain and maintain a good quality of life,” she said. “We have found that the average life span of patients in China is rising as a result of better medical treatment and nutrition, but we haven’t done a thorough survey to prove it.”

Degeneration

“In China, ALS is known as jiandong, or ‘gradual freezing’, which is an apt description of how the disease develops,” Cui said. “The patient’s muscles gradually become weak, limiting their activity as though they are freezing.”

Medically, it is the progressive degeneration of the motor neurons that eventually leads to death, she said.

The technical name provides a precise explanation of the illness. In “amyotrophic”, the “a” refers to “no”, “myo” means muscle, and “trophic” is “nourishment”. When muscles lack nourishment, they atrophy or waste away.

“Lateral” identifies the part of the spinal cord where the nerve cells that send signals to the muscles are located. As this area degenerates, it leads to “sclerosis”, or hardening.

Clinically, ALS usually starts with muscle atrophy in the hands that gradually develops into limb weakness, and eventually patients lose the ability to speak, eat, move or breathe, Cui said.

In theory, patients with advanced ALS can survive by constant use of a respirator, but that can result in complications, including lung infections and blockage of the airways by sputum or even saliva.

“The cruellest part is that the patient’s feelings, emotions, and cognitive functions are all normal, and they have to watch themselves dying step by step. They can’t scratch a place that itches or cry out when they are in pain,” Cui said. “In China, the average onset age is about 52, so the majority of patients are middle-aged; the family breadwinner. They endure great pain inside.”

The World Health Organisation lists ALS among the top five fatal illnesses, along with cancer and AIDS.

However, the illness is also designated a rare disease as a result of its low incidence, with between four to eight patients per every 100,000 people.

Cui said few patients receive a timely diagnosis because the early symptoms are not obvious and can easily be mistaken for other conditions.

“The clumsy fingers or weak limbs are ignored by many people, but the disease develops rapidly later and by then it’s too late. Early diagnosis is crucial to delaying the progress of the illness,” she said. China Daily

Published in Dawn, March 28th, 2018