AMYOTROPHIC LATERAL SCLEROSIS

Submitted By: Demdam, Akea Camille Teodoro, Michelle Torralba, Mary Ann Nocon, Maria Giezel Quibod, Cristina

 Amyotrophic lateral sclerosis (ALS)

Also referred to as motor neurone disease in some Commonwealth of Nations countries and as Lou Gehrig's disease in the United States is a debilitating disease with varied etiology characterized by rapidly progressive weakness, muscle atrophy and fasciculations, muscle spasticity, difficulty speaking (dysarthria), difficulty swallowing (dysphagia), and difficulty breathing (dyspnea). ALS is the most common of the five motor neuron diseases.

Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons) and from them to particular muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and twitch (fasciculations) . Eventually, the ability of the brain to start and control voluntary movement is lost.

 Signs and Symptoms:

Muscle weakness Muscle Atrophy Muscle Fasciculation (twitching) Cramping of muscles Slurred speech Dropped Foot Difficulty in moving Difficulty in swallowing Difficulty in speaking or forming words Spasticity Exaggerated reflexes

Risk Factors:
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Hereditary - Up to 10 percent of the people who have ALS inherited it from their parents. If you have this type of ALS, your children have a 50-50 chance of developing the disease. Age - ALS most commonly occurs in people between the ages of 40 and 60. Sex - Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70. Smoking - Smoking cigarettes appears to increase a person's risk of ALS to almost twice the risk of nonsmokers. The more years spent smoking, the greater the risk. On the other hand, quitting smoking can eventually lower this increased risk to that of a nonsmoker. Lead Exposure - Some evidence suggests that exposure to lead in the workplace may be associated with the development of ALS. Military Service - Recent studies indicate that people who have served in the military are at higher risk of ALS. Exactly what about military service may trigger the development of ALS is uncertain, but it may include exposure to certain metals or chemicals, traumatic injuries, viral infections and intense exertion.

 Possible Complications:
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Breathing in food or fluid (aspiration) Loss of ability to care for self Lung Failure Pneumonia Pressure sores Weight loss Dementia

Pathophysiology:

Mutations in the gene that produces the SOD1 enzyme were associated with some cases of familial ALS. This enzyme is a powerful antioxidant that protects the body from damage caused by free radicals. Free radicals are highly reactive molecules produced by cells during normal metabolism. If not neutralized, free radicals can accumulate and cause random damage to the DNA and proteins within cells. Although it is not yet clear how the SOD1 gene mutation leads to motor neuron degeneration, researchers have theorized that an accumulation of free radicals may result from the faulty functioning of this gene. In support of this, animal studies have shown that motor neuron degeneration and deficits in motor function accompany the presence of the SOD1 mutation. Studies also have focused on the role of glutamate in motor neuron degeneration. Glutamate is one of the chemical messengers or neurotransmitters in the brain. Scientists have found that, compared to healthy people, ALS patients have higher levels of glutamate in the serum and spinal fluid. Laboratory studies have demonstrated that neurons begin to die off when they are exposed over long periods to excessive amounts of glutamate. Now, scientists are trying to understand what mechanisms lead to a buildup of unneeded glutamate in the spinal fluid and how this imbalance could contribute to the development of ALS.

 Treatment:

Because there's no reversing the course of amyotrophic lateral sclerosis, treatments mainly involve efforts to slow the progression of symptoms and make you more comfortable and independent. The drug riluzole (Rilutek) is the first and only medication approved by the Food and Drug Administration for slowing ALS. The drug appears to slow the disease's progression in some people, perhaps by reducing levels of glutamate a chemical messenger in the brain that's often present in higher levels in people with ALS.

Treatments to control symptoms are also helpful: Baclofen or diazepam may be used to control spasticity that interferes with daily activities. Trihexyphenidyl or amitriptyline may be prescribed for people with problems swallowing their own saliva. Physical therapy, rehabilitation, use of braces or a wheelchair, or other orthopedic measures may be needed to maximize muscle function and general health. Choking is common. Patients may decide to have a tube placed into their stomach for feeding. This is called a gastrostomy. A nutritionist is very important. Patients with ALS tend to lose weight. The illness itself increases the need for food and calories. At the same time, problems with swallowing make it hard to eat enough. Breathing devices include machines that are used only at night, and constant mechanical ventilation. Patients should discuss their wishes regarding artificial ventilation with their families and doctors.

Nursing Interventions for Amyotrophic Sclerosis:

1. Provide emotional and psychological support to the patient and his family. 2. Implement a rehabilitation program to help the patient maintain his independence as long as possible. 3. Have the patient perform active exercises and range-of-motion exerciseson un affected muscles to help strengthen them. 4. Depending on the patients muscular capacity, assist with bathing, personal hygiene, and transfers from wheelchair to bed. 5. Help establish a regular bowel and bladder elimination routine. 6. Provide good skin care when the patients mobility decreases. 7. Reposition the patient often. Keep his skin clean and dry, and use pressure reducing devices such as an alternating air mattress. 8. Help the patient obtain equipment, such as a walker or wheelchair, when necessary. 9. If the patient cant talk, provide an alternate means of communication such as message boards, or computers. 10.Administer ordered medications as necessary to relieve patients symptoms. 11.Have the patient who has breathing difficulty perform deep breathinganc coughing exercises. 12.If the patient has trouble swallowing, give him soft, semisolid foods and position him upright during meals. 13.Inspect the patients skin regularly for evidence of breakdown.