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Essay: Amyotrophic Lateral Sclerosis

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  • Subject area(s): Health essays
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  • Published: 15 October 2019*
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  • Words: 1,748 (approx)
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The disease Amyotrophic Lateral Sclerosis, also known as ALS, has always interested me. When I was in sixth grade I saw a man in a wheel chair who could not speak for himself. Wires were connected to his head and a computer spoke for him. This man was my best friend’s father and he was diagnosed with ALS when he was in his forties. Two years after that day my friend’s father passed away, he was in his fifties. He was in the hospital for three days, then his lungs stopped working and gave out. Living with this disease for about ten years was a miracle but ever since then I always found this disease interesting because the cause is unknown and it is completely fatal.

Amyotrophic has a prefix, a root word, and a suffix, like most medical words. The word amyotrophic comes from the Greek language (ALS Association). The prefix “a” means no, the root word “myo” means muscle, and the suffix “trophic” means nourishment (ALS Association). So, amyotrophic means “no muscle nourishment” (ALS Association). Lateral pinpoints the places on the spinal cord where percentages of nerve cells are that signal and control a person’s muscles (ALS Association). Sclerosis is the hardening and/or scarring of body tissue where the muscles deteriorate (ALS Association). When there is no muscle nourishment, like when a person has ALS, the muscles cannot do their job and enable a person to walk, talk, or even breathe.

Amyotrophic Lateral Sclerosis is a progressive neurological disease that destroys nerve cells and causes disability (Mayo Clinic). It affects neurons in the brain that send messages to the spinal cord then to the muscles, in both the superior and the inferior parts of the body (ALS Association). These nerves lose their ability to activate certain muscles which causes them to become feeble and can often result in paralysis and eventually death.

According to the Center for Disease Control and Prevention, the CDC, reports suggest that 20,000 to 30,000 people have ALS in the United States and every year about 5,000 plus are told they have ALS. This disease is more common in men than women but studies have shown that this difference has been growing smaller (CDC). ALS is age related, most people diagnosed with ALS are in their fifties to seventies (CDC). This disease is extremely fatal and most people with ALS live for about two to five years after their symptoms develop and they are diagnosed by a physician, but people diagnosed earlier tend to live longer (CDC). The average survival rate is about three years but about twenty percent live for five years after diagnosis, about ten percent will survive ten years, and five percent can live twenty years or more with this disease (ALS Association).

There is no clear answer as to what causes ALS but there are different types varying the signs and symptoms. Sporadic ALS comes randomly with no clear risk factor or cause (McIntosh, 2017). This type of ALS accounts for ninety to ninety-five percent of cases (McIntosh, 2017). The second type of ALS is familial ALS and is inherited, a child of a person with ALS has a fifty percent chance of developing the disease (McIntosh, 2017). This type accounts for five to ten percent of cases (McIntosh, 2017). Researchers are looking into what genes and gene mutations may cause ALS along with other possible factors. Some possible causes are a disorganized immune response where the immune system may attack their own body and kill nerve cells, a chemical imbalance of high levels of glutamate which it known to be toxic, or a mishandling of proteins where abnormal proteins can build up and cause nerve cells to die (McIntosh, 2017). Researchers are still trying to discover if there is a biological factor or a specific gene that causes this disease or if there are environmental factors that help it develop, or if it is a combination of the two, the answers are still unknown.

A person who has ALS will often see symptoms in the hands, feet, or limbs first with the symptoms progressively spreading to other parts of the body (Mayo Clinic). Some early signs and symptoms include: having a hard time walking or doing average daily activities, tripping or falling, weakness in the legs, feet, or ankles, weakness in the hands, slurred speech, trouble swallowing, muscle cramps, twitching in the arms, shoulders, and tongue, difficulty holding the head up or keeping good posture (Mayo Clinic). Eventually, ALS will affect mastication, swallowing, conversation, and ultimately breathing. Several people with ALS can experience a brief lapse of control over emotional expressions or they may experience some behavioral changes (McIntosh, 2017). Some symptoms may not be noticeable or even thought to be ALS, but, with time, the weakness becomes more evident and a person will experience much pain and/or fatigue (McIntosh, 2017).

Diagnosing ALS early can be very difficult because some of the symptoms mimic other neurological diseases. There is no one test to diagnose ALS, rather it is a variety of tests, exams, and scans to rule out other possible diseases and see if ALS can be a possibility. A series of nerve and muscle tests can be done.  An electromyogram can be completed, where a doctor inserts a needle electrode into the skin and into various muscles (Mayo Clinic). This test assesses how quick the muscles conduct electricity and whether they are damaged or not (Mayo Clinic). This test allows a doctor to see irregular patterns of muscle movement. A nerve conduction study can also be done, which measures the ability of nerves to send signals to muscles in different parts of the body (Mayo Clinic). A muscle biopsy can also be done where a doctor will remove a sample of muscle tissue and send it to a lab where they will examine it for any muscle diseases that can be causing the symptoms a person is experiencing (Mayo Clinic). Scans can also be administered, like magnetic resonance imaging, that produces detailed images of the brain and spinal cord. Although it cannot directly diagnose ALS, doctors use this scan to rule out any other possible diseases that the experienced symptoms can relate to (Mayo Clinic). The magnetic resonance imaging can also show if the person experiencing these symptoms has a spinal cord tumor or a herniated disk in the neck that can be a cause (McIntosh, 2017). Blood and urine tests or a spinal tap can also be used to rule out other reasons for the said symptoms (Mayo Clinic).

There is no cure for amyotrophic lateral sclerosis. There is no medication to take to get rid of it nor is there a surgery to reverse it. The only kinds of treatment there is is medications and therapies to manage and slow the development of the symptoms. There are two medications currently that the U.S. Food and Drug Administration have approved called riluzole and edaravone (NINDH). Rilozule is thought to lessen the number of damaged motor neurons by lessening the levels of glutamate in the body (NINDH). This drug can give a person with ALS a few extra months to live but it does not reverse the damage already done to the other motor neurons (NINDH). Edaravone is believed to slow the deterioration in clinical assessment of day by day functioning in people with ALS (NINDH). These two drugs are directly effective with the cause of ALS but a doctor can also prescribe medications that suppress the symptoms being experienced. Some medications can help with muscle cramps and spasms, depression, fatigue, pain, or even the lapse of control over emotions. Physical therapy can help a person with ALS. Low impact aerobic exercise like walking, swimming, or bicycling can help strengthen muscles not affected by the disease and can help prevent fatigue and depression (NINDH). Some occupational therapists may suggest braces, walkers, or wheelchairs that can help a person with ALS preserve energy while still remaining mobile (NINDH). If a person with ALS loses the ability to speak or speak clearly a speech therapist can help them adapt strategies to be more clear and remain able to communicate verbally (NINDH). As ALS progresses some may recommend using a computer-based speech synthesizer to communicate which uses eye-tracking technology (NINDH). As ALS progresses a person can lose nutrition and strength so a nutritionalist may teach the person or caregiver how to prepare certain meals that provide enough nutrients and steer them away from foods that can be hard to swallow (NINDH). Along with losing strength, as ALS progresses a persons muscles that help them breathe weaken and eventually stop working. As it gets worse a doctor may suggest noninvasive ventilation, or NIV for short, which helps a person breathe through a mask connected to a ventilator (NINDH). The NIV can increase the quality of life for a person with ALS as well as lengthen their endurance. When breathing gets too hard to do through the mask a doctor will then suggest a respirator that inflates and deflates the lungs for a person or they can perform a tracheostomy, where a doctor surgically creates a hole in the neck where a tube is inserted and connects to the windpipe and helps a person breathe (NINDH). Although there is no cure scientist are looking into what can either cause ALS or treat is. They are looking into cellular defects, stem cell lines, biomarkers, and new treatment options involving the SOD1 gene/mutation (NINDH).

Recently, in 2012, a man by the name of Ted Harada lived with ALS and was able to complete a two-and-a-half-mile walk to try and beat the disease (Thiessen, 2017). In 2010, Ted was diagnosed with ALS and, after time, simple tasks became too hard for him to do (Thiessen, 2017). He was then saved by an experimental ALS treatment where doctors opened his spinal cord and injected neural stem cells into the damaged areas in hopes that these new cells would either fix or replace the damaged one (Thiessen, 2017). They believed that this treatment would slow or stop the deterioration of motor neurons. This trial not only slowed his progression of ALS but completely reversed it (Thiessen, 2017). This man who was basically sentenced to death got another chance for life after this experimental treatment. In 2016 Ted died of brain cancer and no one knows whether his ALS would have remained reversed or if the symptoms would have returned (Thiessen, 2017). It is trials and experiments like these that can help find a cure for this horrific disease.

 

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