ALS sufferer and Theoretical Physicist Stephen Hawking will appear as a guest on the Big Bang Theory tonight on E4 at 8pm.
[Stephen] Hawking has a motor neurone disease that is related to amyotrophic lateral sclerosis (ALS), a condition that has progressed over the years. He is now almost completely paralysed and communicates through a speech generating device. Hawking’s illness has progressed more slowly than typical cases of ALS: survival for more than 10 years after diagnosis is uncommon.
Symptoms of the disorder first appeared while he was enrolled at University of Cambridge; he lost his balance and fell down a flight of stairs, hitting his head. The diagnosis of motor neurone disease came when Hawking was 21, shortly before his first marriage, and doctors said he would not survive more than two or three years. By 1974, he was unable to feed himself or get out of bed. His speech became slurred so that he could be understood only by people who knew him well. During a visit to CERN in Geneva in 1985, Hawking contracted pneumonia, which in his condition was life-threatening as it further restricted his already limited respiratory capacity. He had an emergency tracheotomy, and as a result lost what remained of his ability to speak. A speech generating device was built in Cambridge, using software from an American company, that enabled Hawking to write onto a computer with small movements of his body, and then have a voice synthesiser speak what he typed.
The particular voice synthesiser hardware that he once used, which has an American English accent, is no longer being produced. Asked why he has still kept the same voice after so many years, Hawking stated that he has not heard a voice he likes better and that he identifies with it even though the synthesiser is both large and fragile by current standards. Although a mid-2009 corporate press release said that he had chosen NeoSpeech’s VoiceText speech synthesiser as his new voice, a 30 December 2011 interview with Hawking’s technician indicates that Hawking is still using an older synthesiser containing a card “which dates back to the 1980s” and that any upgrade would have to be the same voice, otherwise “it wouldn’t be Stephen’s voice any more”.
For lectures and media appearances, Hawking appears to speak fluently through his synthesiser; however when preparing answers his system produces words at a rate of about one per minute. Hawking’s setup uses a predictive text entry system, which requires only the first few characters in order to auto-complete the word, but as he is only able to use his cheek for data entry, constructing complete sentences takes time. During a TED Conference talk, it took him seven minutes to provide a brief answer to a question.
He describes himself as lucky, despite his disease. Its slow progression has allowed him time to make influential discoveries and has not hindered him from having, in his own words, “a very attractive family”. When his wife, Jane, was asked why she decided to marry a man with a three-year life expectancy, she responded, “Those were the days of atomic gloom and doom, so we all had a rather short life expectancy”.
Taken From [http://en.wikipedia.org/wiki/Stephen_Hawking]
Raising Awareness of Motor Neurone Disease 