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Stephen Hawking's medical condition | Stephen Hawking's medical condition |
(20 minutes later) | |
Professor Stephen Hawking suffers from amyotrophic lateral sclerosis (ALS), the most common form of motor neurone disease. | Professor Stephen Hawking suffers from amyotrophic lateral sclerosis (ALS), the most common form of motor neurone disease. |
What causes it? | What causes it? |
ALS, which accounts for more than 90% of all cases of motor neurone disease, causes nerve cells in the brain and spinal cord called motor neurones to degenerate and eventually die. | ALS, which accounts for more than 90% of all cases of motor neurone disease, causes nerve cells in the brain and spinal cord called motor neurones to degenerate and eventually die. |
These cells play an essential role in passing messages to the muscles. | These cells play an essential role in passing messages to the muscles. |
Without them the brain cannot control movement properly, and the muscles cease to work well. | Without them the brain cannot control movement properly, and the muscles cease to work well. |
Around one in 10 cases of ALS are thought to be due to genetics, but the trigger for the other 90% of cases remains a mystery. | Around one in 10 cases of ALS are thought to be due to genetics, but the trigger for the other 90% of cases remains a mystery. |
It is estimated that 3,500 people in the UK have ALS. | It is estimated that 3,500 people in the UK have ALS. |
What are the symptoms? | What are the symptoms? |
As more and more motor neurones are lost, the muscles - particularly in the limbs - begin to waste. | As more and more motor neurones are lost, the muscles - particularly in the limbs - begin to waste. |
Early symptoms include tripping up when walking, or dropping things. | Early symptoms include tripping up when walking, or dropping things. |
Twitching and "cramping" of the muscles is also common, especially in the hands and feet. | Twitching and "cramping" of the muscles is also common, especially in the hands and feet. |
In the more advanced stages, people often have difficulty speaking, swallowing or breathing and experience paralysis. | In the more advanced stages, people often have difficulty speaking, swallowing or breathing and experience paralysis. |
Death is usually caused by a failure of the respiratory muscles. | Death is usually caused by a failure of the respiratory muscles. |
What is the prognosis? | What is the prognosis? |
Poor. The average life expectancy for somebody with ALS is just two to five years from the time symptoms first appear. | Poor. The average life expectancy for somebody with ALS is just two to five years from the time symptoms first appear. |
Half of patients die within 14 months of their diagnosis. | Half of patients die within 14 months of their diagnosis. |
Mel Barry, of the Motor Neurone Disease Association, said: "ALS is quite a complicated and mysterious disease, and often by the time a diagnosis is made people have had symptoms for up to a year." | Mel Barry, of the Motor Neurone Disease Association, said: "ALS is quite a complicated and mysterious disease, and often by the time a diagnosis is made people have had symptoms for up to a year." |
However, not all people with ALS have the same symptoms, and the rate of progression of the disease can vary greatly | However, not all people with ALS have the same symptoms, and the rate of progression of the disease can vary greatly |
Professor Hawking first developed the disease when he was just 21 years old, and has lived with it for more than 40 years. | Professor Hawking first developed the disease when he was just 21 years old, and has lived with it for more than 40 years. |
The fact that he has lived for long with the condition has been described as remarkable. | The fact that he has lived for long with the condition has been described as remarkable. |
It is estimated that only about 5% of people with ALS survive for more than 10 years. | It is estimated that only about 5% of people with ALS survive for more than 10 years. |
Ms Barry said: "The fact that Professor Hawking has survived for so long is very, very unusual." | Ms Barry said: "The fact that Professor Hawking has survived for so long is very, very unusual." |
Is there any treatment? | Is there any treatment? |
Only one drug - Rilutek - is licensed as a treatment for ALS. | Only one drug - Rilutek - is licensed as a treatment for ALS. |
The drug works by blocking release of a key chemical called glutamate by the central nervous system, but its effect is limited, extending survival by three to six months. | |
Trials are currently taking place using another drug, lithium, which has produced promising results in mice, but work is still at an early stage. | Trials are currently taking place using another drug, lithium, which has produced promising results in mice, but work is still at an early stage. |
Most other treatment is simply palliative, attempting to minimise the effect of the disease. | Most other treatment is simply palliative, attempting to minimise the effect of the disease. |
This can include ventilation systems to help with breathing, feeding tubes if swallowing is a problem and muscle relaxants for muscle cramping. | This can include ventilation systems to help with breathing, feeding tubes if swallowing is a problem and muscle relaxants for muscle cramping. |
How was Professor Hawking diagnosed? | How was Professor Hawking diagnosed? |
On his website, Professor Hawking says that he tries to live as normal a life as possible, and not to think about his condition. | On his website, Professor Hawking says that he tries to live as normal a life as possible, and not to think about his condition. |
He said his diagnosis came as a great shock. | He said his diagnosis came as a great shock. |
He had been physically uncoordinated as a child, but had taken up rowing when he went to Oxford. | He had been physically uncoordinated as a child, but had taken up rowing when he went to Oxford. |
"In my third year at Oxford, however, I noticed that I seemed to be getting more clumsy, and I fell over once or twice for no apparent reason. | "In my third year at Oxford, however, I noticed that I seemed to be getting more clumsy, and I fell over once or twice for no apparent reason. |
"But it was not until I was at Cambridge, in the following year, that my father noticed and took me to the family doctor. | "But it was not until I was at Cambridge, in the following year, that my father noticed and took me to the family doctor. |
"He referred me to a specialist and shortly after my 21st birthday I went into hospital for tests." | "He referred me to a specialist and shortly after my 21st birthday I went into hospital for tests." |
What course has his condition taken? | What course has his condition taken? |
Professor Hawking was able to feed himself and get in and out of bed until 1974. | Professor Hawking was able to feed himself and get in and out of bed until 1974. |
Until that point he and his wife were able to manage without outside help, but then had to rely on live-in help from one of his research students. | Until that point he and his wife were able to manage without outside help, but then had to rely on live-in help from one of his research students. |
In 1980, he changed to a system of community and private nurses, who came in for an hour or two in the morning and evening. | In 1980, he changed to a system of community and private nurses, who came in for an hour or two in the morning and evening. |
This lasted until he caught pneumonia in 1985, and had to have a tracheotomy operation. | This lasted until he caught pneumonia in 1985, and had to have a tracheotomy operation. |
After this, he needed 24-hour nursing care. | After this, he needed 24-hour nursing care. |
Before the operation, his speech had been getting more slurred, so that only a few people who knew him well could understand him. | Before the operation, his speech had been getting more slurred, so that only a few people who knew him well could understand him. |
However, he could could communicate. He wrote scientific papers by dictating to a secretary, and gave seminars through an interpreter. | However, he could could communicate. He wrote scientific papers by dictating to a secretary, and gave seminars through an interpreter. |
The tracheotomy operation removed his ability to speak altogether, and he had to rely on a small portable computer and a speech synthesizer fitted to his wheel chair. | The tracheotomy operation removed his ability to speak altogether, and he had to rely on a small portable computer and a speech synthesizer fitted to his wheel chair. |