Stephen Hawking - Longest Survivor of Amyotrophic Lateral Sclerosis (ALS) in History

Stephen Hawking, who recently died at the age of 76 had Amyotrophic Lateral Sclerosis (ALS), which confined him to a wheel-chair. However, the neuromuscular disease could never defeat his spirit nor blunt his razor-sharp brain. When Hawking was diagnosed with ALS, he was told that he would not live more than two years. But with sheer determination and powerful positive thought, he proved his doctors wrong by defeating the disease.

He lived a life of dignity and extraordinary achievement for 53 more years.

Hawking spent 30 years as a Mathematics Professor at the University of Cambridge and was the Director of research at the school's Center for Theoretical Cosmology.

Amyotrophic Lateral Sclerosis (ALS), a neuromuscular disease is a rapidly progressive and fatal disease. ALS causes degeneration of voluntary muscles and nerves of the body. Voluntary muscles help in chewing, walking, and talking. Often, ALS begins with muscle twitching and weakness in a limb or slurred speech

ALS is called Lou Gehrig's disease, named after the famous American baseballer who died of ALS in 1941 at age 37. ALS is also referred to as Maladie de Charcot, and Motor Neurone Disease (MND).

Currently, there is no cure available for ALS, and there is no effective treatment to halt or reverse, the progression of the disease.

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Symptoms of Amyotrophic Lateral Sclerosis

In three-fourths of those with ALS, either the limb or hand may be affected first and later spreads to other parts of the body. Patients are unaware of the symptoms, as the onset of the disease is subtle.

Early signs and symptoms include:

• Difficulty in walking
• Tripping and falling
• Weakness in leg, feet or ankles
• Weakness or clumsiness in hands
• Slurred speech
• Muscle atrophy

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Causes and Risk Factors of Amyotrophic Lateral Sclerosis

Scientists are still researching the exact cause of ALS, although there are a plethora of factors, such as gene mutation, chemical imbalance, disorganized immune response, and protein mishandling.

Around 5 to 10 percent of all ALS cases are inherited, while the rest have no known cause. The risk factors for ALS include the following:

• Age - People who are between 40 and 60 years are at a higher risk of ALS
• Sex- Men are more affected than women
• Surgeries related to spinal cord could also damage nerve fibers and can cause ALS
• Exposure to neurotoxins, heavy metals or viruses could also be the trigger factors
• Individuals in the military have a 60 percent increased chance of developing the disease

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Diagnosis of Amyotrophic Lateral Sclerosis

Diagnosing ALS is difficult, as the symptoms resemble other neurological conditions. Diagnostic tests need to be conducted to rule out other nervous disorders that resemble ALS. Some of the tests include:

• Electromyography (EMG)
• Nerve conduction velocity (NCV)
• Magnetic resonance imaging (MRI)
• Spinal tap or lumbar puncture
• Muscle biopsy
• Blood and urine tests

Treatment and Management of Amyotrophic Lateral Sclerosis

So far, a specific cure is not available for ALS. Hence, the treatment is focused mainly on slowing down the progression of the disease and making it manageable. 

• The Food and Drug Administration (FDA) approved the very first drug riluzole (Rilutek) to minimize motor neuron damage.
• Physical therapy encourages the patient to do simple, low-impact exercises like walking, swimming, or stationary bicycling.
• Speech therapy is recommended for patients as communication becomes increasingly difficult with disease progression.
• Caregivers of ALS patients need to prepare small nutritious meals that can be at various intervals to the patient throughout the day.
• Suction devices help remove excess fluid or saliva from the patient to prevent choking.
• Nocturnal ventilatory support will be used to inflate the lungs and help the patient to breathe while sleeping.

Medical science is relentlessly working hard in finding ways to diagnose and cure the disease, which might take years, even decades. Meanwhile, with Hawking as a role model, let us train our brains in the right direction to help others in the society.

Reference:

1. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet - (https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/A myotrophic-Lateral-Sclerosis-ALS-Fact-Sheet)
2. ALS: Amyotrophic Lateral Sclerosis - (https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms)
3. About ALS - (http://www.alsa.org/about-als/)
4. Lou Gehrig's Disease? (ALS) - (https://kidshealth.org/en/kids/als.html)

Source-Medindia