What is ALS, a disease suffered by scientist Stephen Hawking?

Renowned British scientist, Stephen Hawking, died on Wednesday, March 14 2018. Stephen Hawking is the only person with ALS (Amyotrophic lateral sclerosis) who has survived to the age of 76. Yes, Stephen Hawking's ALS disease since he was 21 years is a disease with a life expectancy that is not too big. In fact, people diagnosed with ALS generally only have a life expectancy of about 3-5 years from the time the disease develops.

So what is ALS? Why is the life expectancy of people with this rare disease not big? Find out all about Stephen Hawking's ALS disease below.

What is ALS, Stephen Hawking's disease?

ALS disease is a disorder of the motor nerves or nerve cells in the brain and spine that regulate the movement of the striated muscles (muscles that are moved on their own accord). ALS stands for amyotrophic lateral sclerosis. This condition occurs when the nervous system in which certain cells (neurons) in the brain and bone marrow slowly die.

These cells send messages from the brain and bone marrow to the muscles. Mild muscle problems appear at first, but slowly the person will become paralyzed, as does Stephen Hawking. Some people have ALS for several years. Eventually the muscles will stop working. This disease is also called Lou Gehrig's disease, named after the famous baseball player from the United States died from this ALS disease.

There are two types of ALS:

1. Upper motor neurons: Nerve cells in the brain.
2. Lower motor neurons: Nerve cells in the spinal cord.

These motor neurons control all reflex or spontaneous movements in the muscles of your arms, legs and face. Motor neurons also tell your muscles to contract so you can walk, run, lift light objects around you, chew and swallow food, and even breathe.

ALS signs and symptoms

The onset of signs and symptoms of ALS is usually gradual, so the first time you feel symptoms, you may not be aware of the severity of the condition. ALS signs and symptoms are:

• The muscles in one of the arms or legs are weakened
• Speaking incoherently
• The weakened muscles slowly spread to the hands and feet and other parts of the body
• The back and neck muscles weaken, causing the head to hang limply
• Loss of muscle tissue (atrophy)
• Tongue twitching
• Paralyzed (unable to move, speak, eat and swallow, and breathe)

Stephen Hawking lived more than 50 years after being diagnosed with ALS. - Source: TIME Magazine

What causes ALS?

ALS is an event that is still being studied by experts. The cause is unknown and about 90 percent of cases occur sporadically. In about 10 percent of people, the disease is passed on to family members. Scientists also suspect imbalances in glutamate levels in the body and autoimmune diseases as the cause of ALS. Please note, ALS is a disease that is not contagious.

However, a person's risk of developing ALS such as Stephen Hawking will increase if a person:

• Have a family history of ALS
• 40-60 years old
• In the age group <65 years, men are more at risk of developing ALS than women
• Smoking or frequent exposure to secondhand smoke (secondhand smoke)
• Injury from impact

ALS is a condition that cannot be cured, but can be controlled

Yes, ALS is a condition that cannot be cured completely. The treatment provided by the doctor is only intended to control the symptoms and support the patient for as long as possible. One drug is riluzole, which may prolong life and in some people may slow the progression of ALS, but its effect is limited.

Other medications may help control seizure symptoms, difficulty swallowing, cramps, constipation, pain, and depression. A stomach tube may be used for feeding if the patient is choking. Nutritionists play a role in helping prevent weight loss. Education and counseling are also important to help calm the psychological condition of a person who has ALS.

Physical, occupational, and speech therapy can help patients stay strong and independent. Assistive devices such as braces, metal leg wraps, wheelchairs and breathing machines are also required during the treatment period. In the next stage, the main goal is to provide comfort to the condition of people who have ALS.

It should be noted, cases of ALS in Stephen Hawking who managed to survive more than 50 years from the first diagnosis are very rare. However, this does not mean that it is impossible. Always consult your doctor for the best treatment and make sure the people closest to you are always there for the patient, both mentally and physically.