ALS Ice Bucket Challenge: What is ALS?

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Unless you have been living on the moon for the past few months, you have heard about or seen the ALS Ice Bucket Challenge. The first question that came to my mind when my facebook wall was flooded with people upturning ice-filled buckets over their heads, was- Why are they doing this and more importantly, if this is just an Internet trend then why are people like Sir Ian McKellen taking part? Well, it’s for a noble cause. Just to clarify things, the actual act of dumping of ice water on yourself has no connections with the disease ALS. So what is ALS?

Amyotrophic lateral sclerosis or ALS is also known as motor neurone disorder (MND), Charcot disorder, and, in the United States, Lou Gehrig’s disorder. It has not one, but many causes which we will discuss in some detail. ALS is normally a genetic disorder. The condition runs in certain families. It is caused by a defect on Chromosome 21. This accounts for 20% of the familiar ALS cases and 2% of the overall ALS cases. The mutation is inherited in an autosomal dominant manner. These mutations can be of a hundred different kinds. The most common ALS causing mutation is a mutant SOD1 gene. The most common mutation found in Scandinavian countries is D90A-SOD1. While the former is rapidly progressing, the latter is slower and patients may survive upto 11 years.

But in around 90% of the reported cases, no genetic cause is present. It cannot be said for certain but other factors have been blamed for this disease like head trauma, military service, participation in contact sports, intake of food contaminated by blue-green algae, diet enriched with branched-chain amino acids, chemical exposure, electromagnetic field exposure, occupation, physical trauma, and electric shock. Lesions to the motor system in the frontotemporal lobes are also major causes of ALS.

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Sadly there is no definite test for the diagnosis of ALS. Doctors can only suspect it from the presence of upper and lower motor neuron signs in a single limb. Then the symptoms like muscle weakness, atrophy of muscles, hyperreflexia, and spasticity are judged. But ALS has these primary symptoms common with some other disorders with are much more treatable. So doctors have to conduct neurological examinations at regular intervals and conduct many more tests to rule out all other diseases. HIV, Human T-cell leukaemia virus (HTLV), Lyme disease, syphilis and tick-borne encephalitis all show ALS-like symptoms and these must not be confused. In fact the number of “ALS mimic syndromes” are so high that a specialist neurological opinion is necessary in the initial stages of the disease itself.

The initial symptoms are weakness are muscular atrophy, accompanied by trouble swallowing, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. In most of the cases, the symptoms start in the limbs though bulbar onset or respiratory onset are also possible. In case of a limb onset- awkwardness while running, walking, tripping, the dragging of one foot (dropped foot), the loss of dexterity in winding a clock, buttoning one’s clothes or writing- is common. In case of a bulbar onset- difficulty is speaking, swallowing, a nasal tone is speaking, slurring and tongue immobility is common. In case of a respiratory onset, the person faces a difficulty in breathing. ALS can also result in frontotemporal dementia but since the last two symptoms are common for a lot of other disorders, it becomes difficult for a common diagnostician to point at ALS in the first go. As the disease progresses, the patient will have a difficulty in forming words and even control voluntary functions of the body like the bowel moment or eye ball movement.

Riluzole is the only treatment for ALS but it has only worked upto a certain extent. In most cases, the cure is impossible but measures have been taken to improve the living conditions of patients. Medications only serve to relieve the patient of the discomfort caused by the symptoms. Breathing support and therapy are necessary in the later stages. Changing the food habits does not cure ALS but since it is difficult for the patients to swallow, their meals must be regulated and carefully supervised. In the later stages, a feeding pipe is the only option.

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Many NGOs have stepped up to provide any help required by the patients and the family members. The ALS Ice Bucket Challenge has been a brilliant initiative. Though some people have claimed that it decreases the seriousness of the disease, the fund raising programme has been very successful. The original Ice Bucket Challenge was this- as soon as you were nominated, you had to douse yourself in ice water and nominate 3 other people within 24 hours or forfeit and donate $100 to the research programme for ALS. But thankfully, almost everyone who has taken the Ice Bucket challenge has also donated to the fund. The best part of this challenge is that it is fun and it has gone viral. Many celebrities have not only taken the challenge but given their own creative twist to this. Charlie Sheen and Sir Patrick Stewart have straightforward paid the donation instead of taking the challenge. My personal favorite ice bucket challenge is that of the Foo Fighters. They actually imitated the famous bucket of blood scene from Stephen King’s Carrie.

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It is one of the best things that has gone viral till date. So I nominate everyone reading this article for the ALS Ice Bucket Challenge. Go pour freezing water all over yourself. Or don’t, if you don’t want to. But do donate now that you know what it’s for.