Symptom Management of the Patient With Amyotrophic Lateral Sclerosis: A Guide for Hospice Nurses

Gail Houseman, APRN

Disclosures

Journal of Hospice and Palliative Nursing. 2008;10(4):214-215. 

In This Article

Abstract and Introduction

The hospice patient with amyotrophic lateral sclerosis may present with a variety of problematic symptoms. The hospice nurse needs to be knowledgeable of these potential symptoms, along with treatment options, to provide the best care possible to a person with amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive, neuromuscular disorder that involves the degeneration of both the upper and lower motor neurons, leading to eventual wasting and atrophy of all voluntary muscles, including muscles that are used for respiration. The usual cause of death in ALS is related to respiratory failure. ALS occurs in roughly one to two per 100,000 people worldwide.[1] The average life expectancy after diagnosis is 2 to 5 years; however, the progression of the disease is unpredictable, and approximately 10% of all people with ALS live more than 10 years.[2] ALS is slightly more common in men, until the age of 65 years. Onset of ALS symptoms can occur in people aged 20 through 90 years, although the incidence peaks between the ages of 40 and 70 years. Research published within the last few years indicates that more than 50% of people with ALS have some cognitive impairment, specifically problems with executive functioning or judgment.[3,4]

The onset of symptoms of ALS varies with each individual, although unexplained weakness in a limb is the most common site. Onset of ALS symptoms can also occur in the swallowing and/speaking muscles, which is referred to as bulbar ALS. Some people with ALS develop initial symptoms in their respiratory muscles; a person with ALS who reports onset of symptoms in the respiratory muscles tends to have the poorest prognosis with regard to life expectancy. Nearly all people with ALS develop symptoms of respiratory insufficiency. Ideally, people with ALS and their loved ones will be carefully educated about the disease and asked to make a decision regarding choosing (or not choosing) invasive mechanical ventilation. Most (90%-95%) people with ALS choose not to use invasive mechanical ventilation.[5,6]

People with ALS on hospice care may have a variety of onset and symptom presentations. The site of onset, along with the speed of progression, varies from person to person. One person with ALS on hospice could be quadriplegic but have little or no trouble speaking, chewing, and swallowing. Another person with ALS might have significant problems speaking, chewing, and swallowing but still be able to ambulate safely. Most people with ALS on hospice care have problems with respiratory insufficiency. Many people with ALS have significant weight loss, either because of muscle loss or difficulty maintaining adequate nutritional intake because of dysphagia.

In 1999, the American Academy of Neurology (AAN) published its practice parameter guidelines for care of a person with ALS.[7] Since this time, the guidelines have been re-evaluated and are scheduled to be published sometime next year.[8] The AAN 2007 practice parameters for care of a person with ALS indicate that there is a paucity of controlled studies specific to ALS and symptom management. There seems to be a lack of consensus with regard to symptom management among neurologists who treat people with ALS. Forshew and Bromberg[9] queried principal neurologists from 61 ALS centers specifically about symptom management in a person with ALS. Of the 64% who replied to the questionnaire, the authors concluded that consensus for treatment of symptoms was rare and that efficacy of all treatments for symptom management was judged to be moderate at best.[9] Given the lack of research-based recommendations for the treatment of symptoms in the person with ALS, the following suggestions for care of the person with ALS on hospice is based primarily on the author's participation in a multidisciplinary team at a National ALS Association-certified ALS center in Philadelphia, PA.

The hospice nurse who cares for the person with ALS needs to be aware of the symptoms associated with this illness. Most people with ALS experience several, if not many, of the following symptoms: respiratory insufficiency, fatigue, problems with mobility, pain, dysarthria, dysphagia, problems with secretions, involuntary emotion expression disorder (IEED), depression, insomnia, and constipation. These symptoms and less common symptoms are addressed along with possible interventions.

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