The Ice bucket challenge or ALS Ice Bucket Challenge, which went viral on social media with millions of videos was started to collect donation and spread awareness among the people about ALS disease which is an acute and incurable disease. The ALS Ice Bucket Challenge is taken by millions of people including famous Entertainers, Entrepreneurs, Sports persons, Media persons etc. The ALS Ice Bucket Challenge has become instrumental in receiving the large amount of donations for ALS Association which is fighting against the ALS and generating awareness among the masses.
- ALS is responsible for death of 2/100000 population annually and approximately 5,600 cases in the U.S. are diagnosed with ALS each year. It is estimated that as many as 30,000 Americans may have the disease at any given time.
- According to the ALS CARE Database, 60% of the people with ALS in the Database are men and 93% of patients in the Database are Caucasian. ALS is 20% more common in male than female.
- About 20% of people with ALS live 5 or more years, 10% live 10 or more years, 10% survive for 20 years, in some people ALS progression stops after a certain period and there are only small number of people in whom is ALS reversed.
What is ALS?
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenrative disease which is characterised by muscle stiffness, muscle weakness, twitching and cramps in arms and legs muscles, difficult in speaking, swallowing and in some cases shortening of breath. ALS is also known as Motor Neuron Disease (MND).
ALS becomes a cause célèbre in the United States when baseball legend Lou Gehrig’s life ended by the disease. That is why the ALS disease is famously known as Lou Gehrig’s disease in USA.
Amyotrophic is a Greek word which is made by combining A, Myo and Trophic, where “A means no/negative”, “Myo means muscles” and “Trophic means nourishment”. In Amyotrophic Lateral Sclerosis, lateral refers to the portion of Spinal chord from where the nerve cells transfer the signals and controls the muscles. Sclerosis means abnormal hardening of body tissues.
So overall ALS means a disease caused by genetic mutation in which there is a progressive loss of structure or function of neurons and/or the death of neurons. This results into no or negative nourishment of the muscle and thus muscle becomes weak and stiff. The nerves which are affected by ALS are motor neurons (that originates in the motor region of the cerebral cortex, whose cell body is located in the spinal chord) which provide the voluntary movement to the muscles. The main muscles under the voluntary control are the muscles of legs and arms. That’s why these muscles are the primary target of the ALS. However in ALS the muscles of heart and digestive system are remain unaffected because they functions automatically and does not come under the voluntary control of motor neurons.
There are mainly three types of ALS diseases:
- Spordic ALS (SALS): It is most common ALS disease in USA and around 90% to 95% patient belongs to this category. The SALS is non hereditary which means it can occur to any person.
- Familial ALS (FALS): This type of ALS are usually found in people who receives it from their ancestors which means it is hereditary and occurs more than once in family lineage. 5% to 10% cases of the ALS belongs to this category. The main cause of FALS is the mutation of SOD1(superoxide dismutase 1) gene which is located on Chromosome 21. The SOD1 binds Copper and zinc and act as an antioxidant which provides protection against superoxides (free radicals) which can damage DNA and Protein in cell.
- Guamanian ALS (GALS) : an extremely high incidence of ALS was observed in 1950 in Guam, a territory of US in west Pacific Ocean.
Symptoms:
During the onset or initial stage of ALS, the symptoms are very slight and not easy to observe but with the progression of the ALS the symptoms becomes more visible and can be observe easily which include:
- Muscle weakness especially of legs, arms, hands, mouth
- impairment of the use of arms and legs
- twitching and cramping of the muscles
- difficulty in speaking and thick voice and
- difficulty in breathing and shortening of breath.
Out of these symptoms, muscle weakness and paralysing are universal whereas other symptoms varies from patient to patient during its initial stage.
The hands and feet may be affected first, causing difficulty in lifting, walking or using the hands for the daily regular activities. With further weakening and paralysing the ALS effects speech, swallowing, chewing and breathing. When the breathing muscles become affected, ultimately, the patient will need permanent ventilatory support in order to survive. However the sense of sight, taste, hearing, touch and smell of patient remains unaffected (because they are not controlled by motor neurons).
Diagnosis and Treatment:
The ALS is difficult to diagnose and there is no proper test or procedure is available to diagnose it. The only method to diagnose the ALS is doing the series of tests and eliminating the mimic ALS. A comprehensive diagnostic examination includes the following tests:
- electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV)
- blood and urine studies including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24-hour urine collection for heavy metals
- spinal tap
- x-rays, including magnetic resonance imaging (MRI)
- myelogram of cervical spine
- muscle and/or nerve biopsy
- thorough neurological examination
There is currently no treatment available for ALS but the Food and Drug Agency of US has approved a drug called Riluzole (Rilutek) in 1996 this drug is marketed by Sanofi Aventis, a multinational pharmaceeutical comapany . The Riluzole slow downs the progression of ALS and increases the survival by approximately two or three months.
Donate for ALS Assoctaion: http://www.alsa.org
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