Amyotrophic Lateral Sclerosis - Research Paper Example

?Amyotrophic lateral sclerosis (ALS) “Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's Disease, is a progressive brain disease thatattacks the nerve cells (neurons) that control voluntary muscles” (ASHA, 2011). Amyotrophic lateral sclerosis (ALS) is a brain disease that is fatal in nature. It is also known as Lou Gehrig’s disease. ALS progresses rapidly and attacks the motor neurons that fundamentally control the function of voluntary muscles in the body. ALS is one of the several motor neuron diseases in which, the motor neurons degenerate and expire. Located in the brain and the spinal cord, motor neurons are the links of vital communication between the voluntary muscles and nervous system. Upper motor neurons which are located in the brain send messages to the lower motor neurons that are located in the spinal cord. The receiving motor neurons in turn, generate instructions for the voluntary muscles in the body. ALS occurs with the simultaneous death and degeneration of the upper and lower motor neurons, which essentially distorts the passage of messages to the voluntary muscles. Lack of function first causes the muscles to weaken and eventually, the muscles waste away. The brain ultimately loses its capability to control the voluntary movement of muscles in the body. The weakness caused by ALS is accompanied by a whole range of disabilities. Loss of strength of the chest and diaphragm muscles reduces the ventilatory support and thus, saps the patient’s ability to breathe. In a vast majority of cases, it only takes the patients between 3 and 5 years to die because of respiratory failure after the symptoms have started to show up, while only 10 per cent of the patients of ALS live for 10 or more years after the onset of symptoms. Although it may be possible to prolong the survival of patient using tracheostomy intermittent positive-pressure (TPPV) for about 5 years on average, “many clinicians have ethical reservations about recommending it for ALS patients and, at least in some states, < 10% of ALS patients are offered or consent to tracheotomy” (Bach, 2002). In the past, it was believed that ALS did not have any effect upon an individual’s intelligence, though as more and more research is being conducted, it is being increasingly believed that ALS causes changes in cognitive functions including memory loss and depression. Nevertheless, the patient’s ability to hear, see, taste, and smell are not altered by ALS. The patients are able to recognize it when they are touched though their own ability to move the hands may be lost. Patient’s bladder and bowel functions remain efficient but the patient does require help to and from the bathroom in the late stages of disease. Up to 5000 Americans are diagnosed with ALS on a yearly basis. ALS affects people of all ethnicities and races all over the world and is thus, a very common neuromuscular disease. People suffering from ALS are mostly aged from 40 to 60 years, though people younger and older than this are also susceptible to acquiring the disease (NIH, 2011). ALS is more common among men as compared to women. ALS occurs randomly in as many as 95 per cent of the cases and the risk factors in them are minimal. It is not an inherited disease. It is not necessary for the patients to have a family history for ALS. Accordingly, diagnosis of ALS in a parent is generally no reason for the child to have this disease as well. Cases in which ALS is inherited make no more than 5 to 10 per cent of the total. Only the familial type of ALS passes the disease through inheritance when one of the parents carries the gene with ALS. 20 per cent of all familial cases of ALS occur because of a particular defect in the genes which causes mutation of the superoxide dismutase 1 (SOD 1) enzyme (LEF, 2011). 80 per cent of the familial ALS cases occur because of unidentified genetic reasons. “Perhaps the most important disorder in the differential diagnosis is multifocal motor neuropathy, which is dominated by lower motor neuron signs and characterized by multiple motor-conduction blocks on electrical testing” (Rowland and Shneider, 2001). ALS’s symptoms are quite subtle and hence, most patients do not take them seriously. Some of the earliest symptoms include but are not limited to twitching, weakness of the muscles, stiffening of muscles, troubled swallowing and chewing, nasal speech and slurred speech. If adequate treatment is not taken soon, the general problems aggravate and form obvious weakness. Muscles of the body that are first damaged decide for the body parts that make be affected by the initial symptoms of the disease. Symptoms mostly start appearing in one of the legs. This causes the patient trouble when he/she wants to walk or run. Patients frequently experience tripping or stumbling. The symptoms may also begin in the arm and the patient may feel difficulty wearing or butting a shirt or typing on a keypad. Some patients experience problems with the speech. In the last stages of the disease, patients can not breathe because of the weakness induced in the respiratory system. Ultimately, patients can not breathe without ventilatory assistance. In the last stagesm the patient may also experience pneumonia. Irrespective of the point of initial appearance of the symptoms, weakness in the muscles protrudes into other body parts as the disease aggravates. This is when the patients start to experience problems in swallowing and moving. Dysphagia is often accompanied with dysarthria, and the patient can not form words. Doctors may not be able to diagnose ALS in a patient until both the lower and upper motor neurons are damaged and it is not possible to find other causes for the depicted weakness. When a patient is diagnosed with ALS, the health care professionals should explain the various options of treatment to the patient in order to enable him/her to make informed decision. To date, no cure has been identified for ALS. Doctors are yet in the exploring and learning phase of the cases and treatment of ALS as more research is being done on the diseases of brain. As a result, some medications have been determined for the treatment of ALS including Riluzole which is an antiglutamate agent that was the first drug approved by FDA for treating ALS in patients (LEF, 2011). Riluzole does not provide a patient with complete cure against ALS, yet it has been found to prolong life particularly of the bulbar patients. These days, doctors are trying to assess the synergistic effects of combination of drugs for treating ALS. Like all diseases that are rendered incurable, palliative care or symptom management is employed for the treatment of ALS in the state of the art practice. Such a care is essentially active care of the patient. Multidisciplinary clinical teams do a very good job in treating the patients of ALS particularly if the teams are specialized in neuromuscular disorders. Intervention manages emerging symptoms with the progress of disease. These teams work with patients and the caregivers to design a plan of physical and medical therapy for the individual patients. These teams also provide the patients with special equipment that enables them to move around comfortably. Medications may be prescribed for minimizing fatigue, controlling spasticity and easing cramps in the muscles. Depression, constipation and sleep disorders are also treated with drugs. Pharmacists suggest the patient ways to gain maximum benefit from the drugs and supervise the prescriptions in order to escape the risk of harmful interactions of drugs. References: American Speech-Language-Hearing Association. (2011). Amyotrophic Lateral Sclerosis (ALS). Retrieved from http://www.asha.org/public/speech/disorders/ALS.htm. Bach, J. R. (2002). Amyotrophic Lateral Sclerosis* Prolongation of Life by Noninvasive Respiratory Aids. CHEST. Retrieved from http://chestjournal.chestpubs.org/content/122/1/92.full. Life Extension Foundation. (2011). Amyotrophic Lateral Sclerosis (ALS). Retrieved from http://www.lef.org/protocols/neurological/als_01.htm. National Institutes of Health. (2011). Amyotrophic Lateral Sclerosis. Retrieved from http://www.nlm.nih.gov/medlineplus/amyotrophiclateralsclerosis.html. Rowland, L. P., and Shneider, N. A. (2001, May 31). Amyotrophic Lateral Sclerosis. The New England Journal of Medicine. 344: 1688-1700. Retrieved from http://www.nejm.org/doi/full/10.1056/NEJM200105313442207. Read More