Amyotrophic Lateral Sclerosis (ALS) Series-Disabled Legend Professor Stephen Hawking

Prof. Stephen Hawking was born on 8 January 1942 (300 years after the death of Galileo) in Oxford, England. Prof. Stephen Hawking parents’ house was in north London, but during the second world war Oxford was considered a safer place to have babies. When he was 8, his family moved to St Albans, a town about 20 miles north of London.

At the age of 11 Prof. Stephen Hawking went to St Albans School, and then on to University College, Oxford, his father’s old college. Prof. Stephen Hawking wanted to do Mathematics, although his father would have preferred medicine. Mathematics was not available at University College, so he did Physics instead. After 3 years and not very much work he was awarded a first class honours degree in Natural Science.

Stephen then went on to Cambridge to do research in Cosmology, there being no-one working in that area in Oxford at the time. Prof. Stephen Hawking’s supervisor was Denis Sciama, although he had hoped to get Fred Hoyle who was working in Cambridge. After gaining his Ph.D. he became first a Research Fellow, and later on a Professorial Fellow at Gonville and Caius College.

After leaving the Institute of Astronomy in 1973 Prof. Stephen Hawking came to the Department of Applied Mathematics and Theoretical Physics, and since 1979 has held the post of Lucasian Professor of Mathematics. The chair was founded in 1663 with money left in the will of the Reverend Henry Lucas, who had been the Member of Parliament for the University. It was first held by Isaac Barrow, and then in 1669 by Isaac Newton.

Prof. Stephen Hawking has worked on the basic laws which govern the universe. With Roger Penrose he showed that Einstein’s General Theory of Relativity implied space and time would have a beginning in the Big Bang and an end in black holes. These results indicated it was necessary to unify General Relativity with Quantum Theory, the other great Scientific development of the first half of the 20th Century. One consequence of such a unification that he discovered was that black holes should not be completely black, but should emit radiation and eventually evaporate and disappear. Another conjecture is that the universe has no edge or boundary in imaginary time. This would imply that the way the universe began was completely determined by the laws of science.

Prof. Stephen Hawking’s many publications include: The Large Scale Structure of Spacetime with G F R Ellis, General Relativity: An Einstein Centenary Survey, with W Israel, and 300 Years of Gravity, with W Israel. Prof. Stephen Hawking has 3 popular books published; his best seller A Brief History of Time, Black Holes and Baby Universes and Other Essays and most recently in 2001, The Universe in a Nutshell.

There are .pdf and .ps versions of his full publication list. Prof. Stephen Hawking has 12 honorary degrees, was awarded the CBE in 1982, and was made a Companion of Honour in 1989. Prof. Stephen Hawking is the recipient of many awards, medals and prizes and is a Fellow of The Royal Society and a Member of the US National Academy of Sciences.

Prof. Stephen Hawking continues to combine family life (he has three children and one grandchild), and his research into theoretical physics together with an extensive programme of travel and public lectures. Prof. Stephen Hawking suffers from ALS.

What is ALS?

ALS stands for Amyotrophic Lateral Sclerosis, sometimes called Lou Gehrig’s disease. It is a rapidly progressive and fatal neuromuscular disease that is characterized by degeneration of a select group of nerve cells and pathways (motor neurons) in the brain and spinal cord. This loss of motor neurons leads to progressive paralysis of the voluntary muscles. The heart is not a voluntary muscle, and therefore, remains unaffected by the disease. However, since breathing is controlled voluntarily by the chest muscles, death usually occurs when the chest muscles are no longer able to help the lungs achieve adequate oxygenation. Generally, there is little impairment of the brain or the senses.

“Amyotrophic” means:A = absence ofmyo = muscletrophic = nourishmentlateral = side (of spine)sclerosis = hardening or scarring ALS is not contagious, but it is fatal.For the most part, the battle is short, with 80% losing their lives within three to five years of diagnosis. While between 10% and 20% live ten years or more after diagnosis, others live only a few months. While the cause is unknown, research is being conducted in areas relating to genetic predispositions, viral or infectious agents, environmental toxins and immunological changes. For some people, the muscles for speaking, swallowing or breathing are the first to be affected. This is known as bulbar ALS.

The term “bulbar” refers to the motor neurons located in the brain stem, that control the muscles used for chewing, swallowing, and speaking. ALS symptoms, and the order in which they occur, vary from one person to another. In 85% of cases, ALS effects the lower portion of the spinal cord first. This is known as limb onset ALS. In these cases, muscle weakness, cramps and weakened reflexes affects the muscles in the arms and legs as the first signs of ALS. The rate of muscle loss can vary significantly from person to person with some patients having long periods with very slow degeneration. Signs and Symptoms Upper Motor Neuron Degeneration muscle stiffness or rigidity emotional lability (decreased ability to control emotions) excessive fatigue dysphagia (difficulty swallowing) dyspnea (shortness of breath) dysarthria (a speech disorder caused by impairment of the muscles used for speaking) incresed or ‘b risk’ reflexesgait spasticiy Lower Motor Neuron Degeneration muscle weakness and atrophy involuntary contraction of muscle fibres muscle cramps weakened reflexes flaccidity (decreased muscle tone)difficulty swallowing disordered articulation shortness of breath at rest.

Is ALS a Rare Disease?

ALS is not considered a rare disease. Approximately 2,500-3,000 Canadians currently live with ALS. 2 or 3 Canadians lose their battle to this devastating disease every day. In Ontario, roughly 1,000 people have ALS at any one time. “ALS is clearly the most common cause of neurological death on an annual basis,” Dr. Michael Strong, clinician at the University Health Sciences Centre and research scientist at the Robarts Research Institute, London, Ontario.

What Causes ALS?

We don’t really know what causes ALS, but we do know that it can strike any adult at any time. While the usual age at onset is between 45 and 65, people as young as 17 have been diagnosed in the past. Between 5 and 10% of ALS cases are found in the same families, meaning that they are “familial”, and are definitely linked genetically. But for the most part, diagnosis is sporadic and we don’t know how it is caused.

What are the early symptoms?

ALS usually becomes apparent either in the legs, the arms, the throat or the upper chest area. Some people begin to trip and fall, some may notice muscle loss in their hands and arms and some find it hard to swallow and slur their speech. ALS is difficult to diagnose. There is no specific test available that will either rule out or confirm the presence of ALS. Diagnosis is usually made through a ‘diagnosis of exclusions’. Neurologists conduct a number of tests, thereby ruling out other disorders that may cause similar symptoms, such as strokes or multiple sclerosis and if nothing else is positive and yet the symptoms continue to worsen, ALS is often the reason.

What are the effects of ALS?

Because ALS frequently takes its toll before being positively diagnosed, many patients are debilitated before learning they have ALS. The disease usually does not affect the senses – taste, touch, sight, smell, and hearing – or the mind. ALS has a devastating effect on patients and their families. As they cope with the prospect of advancing disability and eventually death, it consumes their financial and emotional reserves. It is a costly disease in its later stages, demanding both extensive nursing care and expensive equipment.

What can be done about ALS?

There is no known cure at this time and very little in the way of treatment that will have an effect on the disease itself.

Is there hope for people with ALS?

Research is looking to find not only the cause of the disease so that a cure can be developed but also other medications or treatments that can help until a cure is found. With improved knowledge about ALS, healthcare providers and families can help people living with ALS live life more fully. The services offered by the ALS Society of Ontario help improve the quality of life for those who live with ALS and their families.

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