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What Is ALS: Everything You Need to Know About Lou Gehrig's Disease

Updated on March 26, 2018
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Srikanth strongly believes that prevention is better than cure. He is of the opinion that awareness is a key to prevent diseases.

What Is ALS?

What is ALS? This question is at the top of the minds of many people worldwide. Amyotrophic lateral sclerosis is the full form of ALS. It is a progressive neurodegenerative medical condition that affects nerve cells in the brain and the spinal cord. It is also known as Lou Gehrig’s disease. Some people also call it Lou Gehrig disease.

Physicist Stephen Hawking Suffered From ALS

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I try to lead as normal a life as possible, and not think about my condition, or regret the things it prevents me from doing, which are not that many.... I have been lucky that my condition has progressed more slowly than is often the case. But it shows that one need not lose hope.

— Stephen Hawking, theoretical physicist, cosmologist and author

ALS Symptoms

The onset of this medical condition is very subtle. It is so subtle that usually its symptoms are overlooked. Early symptoms of Lou Gehrig’s disease may include tight muscles, difficulty in swallowing, cramps, fasciculations, stiff muscles, slurred speech, muscle weakness affecting an arm or a leg, nasal speech or difficulty in chewing. Usually, death occurs three to five years after symptoms begin.

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What Are the Causes of ALS?

Respiratory failure is the most common cause of death in people affected by ALS. Various genetic mutations can cause inherited ALS. Chemical imbalance is another possible reason. People affected by this medical condition generally have higher than normal levels of glutamate. Excessive levels of glutamate may not be good for nerve cells.

Disorganized Immune Response May Cause ALS

Disorganized immune response may also cause ALS. Sometimes a person’s immune system attacks his/her body’s own normal cells. This may kill nerve cells. Protein mishandling is another possible cause of ALS. Mishandled proteins within the nerve cells may result in a gradual accumulation of abnormal forms of these proteins in the cells. This will kill the nerve cells.

Rogue Genes May Cause ALS

A research study suggests that gene mutations may cause up to 17% of cases ALS in patients with no family history of the disease. Results of the study were published online in the Neurology, a journal.

Our results highlight that genetic factors play a significant role in the disease, which is important in an era of genetic-focused treatments.

— Dr Summer Gibson, University of Utah School of Medicine

Complete Neuron Cell

Image in public domain
Image in public domain | Source

ALS Risk Factors

Many research studies are investigating possible risk factors that may be associated with ALS. Heredity is one established risk factor of this medical condition. Five to ten percent of people with ALS inherited it (familial ALS). Their children have a 50-50 chance of developing the disease.

ALS is more common among people aged between 40 and 60. Before the age of 65, slightly more men than women develop this medical condition. However, the sex difference disappears after the age 70 years.

In some patients, this disease is triggered by some environmental factors. Cigarette smoking may enhance a person’s risk of ALS to almost twice that of a non smoker. Quitting smoking is known to lower the risk.

Lead exposure may be associated with the development of this medical condition. According to some studies, people who have served in the armed forces are at higher risk of ALS. This may be due to exposure to certain metals, intense exertion, viral infections and traumatic injuries.

Some research studies found many genetic variations that were common in patients with familial ALS and patients with non inherited ALS. These genetic variations may enhance susceptibility to this medical condition.

Research studies conducted at the Harvard T.H. Chan School of Public Health in Boston indicate that truck drivers and others who are exposed to diesel fumes while on the job may face a greater chance of developing ALS.

Smoking Doubles ALS Risk

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ALS Complications

ALS is a progressive medical condition. As the disease progresses the patient experiences complications. These include breathing problems, speech issues, dementia and eating problems.

Lou Gerhig’s disease paralyses muscles related to breathing. It impacts muscles that control swallowing negatively. People with ALS are known to develop malnutrition and dehydration.

People with ALS usually develop speech problems. It usually starts as mild slurring of words. However, severity of this problem increases as the disease progresses. It becomes difficult to understand their speech.

ALS Diagnosis

ALS diagnosis involves administration of EMG (electromyogram), to detect damage to the nerves. Neurologists usually perform additional tests to rule out multiple sclerosis, muscular dystrophy, spinal cord tumors and other medical conditions.

Three Different Electromyograms

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ALS Treatments

ALS cures have been eluding experts in the field of medicine. As on today, no treatment halts the progression of this medical condition. ALS treatment strategy involves patient education, adaptive or supportive treatment and mechanism specific treatment.

Some medicines and devices help control the symptoms of the condition and make living with the disease easier. FDA approved the use of riluzole (Rilutek) to treat ALS patients in 1995. This drug reduces damage to motor neurons by reducing the release of glutamate.

Today many doctors worldwide are using this medicine to treat ALS disease. However, this drug is known to have side effects related to the liver. It is advisable to have a doctor monitor your condition regularly while using this medicine.

Physical therapy is also used to treat Lou Gerigs disease. It is known to improve circulation and to help prolong muscle use during the early stages of this medical condition.

Some doctors prescribe Baclofen, which provides relief from stiffness in the throat and the limbs. Branched chain amino acids (BCAAs) are nutritional supplements that slow down weight loss and muscle decline.

Some doctors use Phenytoin to ease cramps. Excess saliva production (an ALS symptom) can be controlled by tricyclic antidepressants. Antidepressants also help in dealing with depression, which usually accompanies medical conditions like ALS.

One experimental therapy involves synthetic forms of an insulin-like nerve growth factor known as cell-derived neurotrophic factor. Some experts believe that this may protect motor neurons and stimulate the regeneration of damaged cells. However, this experiment is highly controversial.

Doctors use some devices to assist the patient in breathing. These are worn at night and are similar to devices used by people affected by sleep apnea. For instance, the patient may be given non invasive positive pressure ventilation to help with breathing at night. A feeding tube may be recommended to reduce the risk of getting food, liquids or saliva into the lungs.

At the later stages of this medical condition, doctors may recommend tracheostomy to enable full-time use of a respirator that inflates and deflates the lungs. Tracheostomy is a surgically created hole at the front of the neck leading to the trachea.

Tracheotomy

ALS Awareness

Efforts are on to spread awareness about this medical condition. Ice Bucket Challenge or ALS Ice Bucket Challenge is one such initiative. It is an activity involving pouring a bucket of ice water on someone’s head to spread awareness about ALS and to encourage contributions to fund research activities related to this medical condition.

Nominated participants are filmed having a bucket of ice water poured on their heads and then nominating others to participate. The nominated person has one day to comply or forfeit by way of a donation.

The Ice Bucket Challenge has been a success as it has been able to generate substantial amount of money for organizations like ALS Association, Project ALS, ALS Foundation Netherlands and ALS Therapy Development Institute. In fact the movement has raised more than $5.5 million for the ALS Association between July 29 2014 and August 15 2014, compared to $32,000 in the same period in 2013.

The rich and the famous people of this world, like Bill Gates, Justin Timberlake, Satya Nadella, Ethel Kennedy, Martha Stewart and Mark Zuckerberg have taken up the Ice Bucket Challenge.

ALS research efforts are on all over the world. We will hope that an effective ALS cure is found soon. Today there are many ALS support groups. There are many helping hands for ALS victims. If you or any one close to you is affected by A. L. S., it is definitely advisable to seek help.

Summary

  • ALS is the short form of Amyotrophic lateral sclerosis.
  • ALS is also known as Lou Gehrig’s disease.
  • ALS is a progressive medical condition.
  • Cigarette smoking enhances the risk of ALS.
  • There is no cure for ALS.
  • Ice Bucket Challenge is an initiative to spread awareness about ALS.

© 2014 Srikanth R

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