Amyotrophic Lateral Sclerosis or Lou Gehrig’s Disease
Frederick Aladad

Abstract
Amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease is a neurodegenerative disease that portrays progressive muscle paralysis, and eventually ends with death. As many as twenty thousand to thirty thousand people in the United States have amyotrophic lateral sclerosis, and an estimated five thousand people in the United States are diagnosed with the disease each year (Shiel, 2012). The etiology of the disease is unknown, but it plays a genetic factor. Less than ten percent has been shown to be inherited in families. ALS begins with the client with muscle weakness, stiffness, eventually to paralysis, then death caused by respiratory failure. Dysphagia occurs at onset in about one third of case, although generally it occurs in later stage of the disease (Noh, 2010). The nursing diagnosis is risk for aspiration related to impaired swallowing. Intervention for the diagnosis includes positioning the client in a 90 degree angle while in bed, a wheel chair or a chair. There is no known cure for the disease, but symptoms can be treated. Riluzole is administered to slow the progression of the disease.

Pathophysiology
Amyotrophic lateral sclerosis is a neurodegenerative disease that begins with rapid, progressive muscle weakness. It attacks the neurons that are responsible for moving voluntary muscles. Once the person is diagnosed with the disease, the person will inevitably face death during the later stages of the disease. Respiratory failure limits survival to 2-5 years after disease onset (Noh, 2010). The etiology of ALS is unknown, but research shows that a small percentage is inherited in families. Only about 5 to 10 percent of all ALS cases are inherited in the family's genes (Shiel, 2012). It is common in the geriatric population, but younger people can get the disease. ALS causes a wide range of disabilities. Early signs include clumsiness, tripping, dropping objects, fatigue of the extremities. The person complains of spasticity, and muscle weakness to the extremities. Some people will first experience weakness in the hand or arm, and experience difficulty simple tasks such as writing, turning a lock on a door to buttoning a shirt. Then, the person will exhibit slurred and nasal speech, or dysphagia. Paralysis occurs later in the disease process. The person may die from respiratory failure or aspiration pneumonia. Although there is no cure for ALS, it can be treated by relieving the symptoms. Riluzole is given to slow the progression of the disease. Other treatments include physical therapy to improve circulation and improving quality of life.

Case Study A 54 year old man was admitted to the hospital complaining of dysphagia and hoarseness for three months. A year before admission, the man developed weakness on both arms, having difficulty lifting objects about head level. The man experienced shortness of breath while going up stairs or hills, and had trouble sleeping. Dysphagia wasn’t present at the time. Dysphagia and hoarseness evaluation was performed, as well as medical history. The man’s nutritional status was poor due to difficulty swallowing. Vital signs were stable, but the man appeared chronically ill. The chemistry lab was performed, which came out normal. CT scan and abdominal scan had no significant findings. Neurologic examination revealed jaw jerk signs and positive Hoffman’s sign. The deep tendon reflexes of both the upper and lower extremities were positive for upper motor signs with atrophy of the tongue. In some cases, for example, if a physician suspects that the patient may have a myopathy rather than ALS, a muscle biopsy may be performed (Shiel, 2012). Brain MRI was recommended, but the man refused. Electromyography had to be performed to see the electrical activity performed by the skeletal muscles. The electromyogram revealed degeneration of the skeletal muscles. An endoscopy was performed, but with difficulty due to client having hypoxemia during the procedure. The case was clinically diagnosed as amyotrophic lateral sclerosis. At the present time, supportive care to relieve symptoms and improve quality of life, with bi-level positive airway pressure to assist in weakened breathing during sleep were applied to the patient and percutaneous endoscopic gastrostomy was performed to avoid airway aspiration due to swallowing difficulty (Noh, 2010).

Nursing Care Plan Nursing diagnosis for the patient is risk for aspiration related to impaired swallowing. Both acute and chronic conditions can place patients at risk for aspiration (Galanes, 2012). According to Shiel, difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking (2012). Nutritional status of the patient is poor due to complaints of difficulty swallowing. The patient couldn’t swallow porridge or water, and had to spit it out. The short term goal is the patient’s risk for aspiration decreasing as a result of ongoing assessment and early intervention (Galanes, 2012). Nursing intervention begins with assessing pulmonary status for signs of aspiration. Auscultate the lungs for crackles and/or rhonchi. Aspiration of small amounts can happen without sudden onset of respiratory distress, specifically in patients with decreased levels of consciousness. Then, position the client in a 90 degree angle while in a bed, wheel chair or chair. Providing cushions will maintain position. Proper positioning of patients with swallowing difficulties is of primary importance during and after feeding (Galanes, 2012). The patient wasn’t able to swallow saliva, so a percutaneous endoscopic gastronomy was performed to insert a tube for feeding and to prevent aspiration from occurring.

Conclusion The nursing intervention partially worked to decrease the client’s risk for aspiration. Since the client wasn’t able to swallow anything, including saliva, a PEG tube had to be inserted. It did relieve patient’s shortness of breath. ALS can’t be prevented. The disease strikes all ethnic and race groups. ALS most commonly strikes people between 40 and 60 years of age, but younger and older people also can develop the disease (Shiel, 2012). Since amyotrophic lateral sclerosis cannot be cured, it can be maintained by treating the symptoms. Riluzole is the first FDA approved drug choice that can treat ALS. Physical therapy is recommended to improve circulation in the muscles and cardiovascular strength. It can also enhance patient’s independence and safety throughout the disease process. Gentle activities such as swimming and walking can help strengthen the unaffected muscles. Speech therapy is also recommended if the client is exhibiting slurred speech. Positional changes during meal, dietary modifications and augmented feeding technique were provided to treat dysphagia of the patient (Noh, 2010). The disease cannot be prevented, but treating the symptoms and maintaining quality of life can prolong life.