What is ALS and What is the Philosophy of Care?

Abstract

have no financial or nonfinancial relationships related to the content of this article. Amyotrophic lateral sclerosis (ALS) is a rapidly degenerative disease involving upper motor neuron (UMN) and lower motor neuron (LMN) impairments, for which there is currently no cure. It is necessary for speech-language pathologists to understand the underlying neurological pathophysiology and philosophy of care for individuals with ALS in order to facilitate effective assessment and intervention in this population. The authors of this article review the characteristics of ALS, as well as the general philosophy of care for individuals with ALS. The article covers the topics of assessment and intervention within the context of a multidisciplinary ALS clinic; surgical and pharmacological interventions; and monitoring disease progression, including tracking respiratory status. Periodic multidisciplinary assessment aids in appropriate medical and therapeutic interventions, symptom management, and quality of life. Periodic assessment of respiratory function is especially important to aid in timely delivery of appropriate interventions. Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease for which there is currently no cure. The cause of ALS is not known, and survival is, on average, approximately 3 years following symptom onset (Corcia & Gordon, 2012). The purpose of this article is to summarize the characteristics of ALS and related types of motor neuron disease (MND), as well as the current philosophy of care for individuals with ALS. Characteristics of ALS ALS is a rapidly degenerative disease involving motor neurons in the brain, brainstem, and spinal cord. It is commonly known as Lou Gehrig's disease in the United States, motor neuron disease (MND) in Great Britain, and Charot's disease in France. Typically, the first symptom is weakness; about two-thirds of patients first experience spinal muscle weakness in either the upper or lower extremities, while one-quarter of patients have bulbar weakness and initial dysarthria and dysphagia. Respiratory symptoms are rarely a first sign of disease (Gautier et al., 2010), yet breathing difficulties are a serious complication for almost all patients with ALS, regardless of whether primary weakness is spinal or bulbar.

The Safety and Efficacy of Expiratory Muscle Strength Training for Rehabilitation After Supracricoid Partial Laryngectomy: A Pilot Investigation

“…13,14,16,28 In contrast, values for PCF were below normal at baseline but closer to the normal range following intervention. 34 This finding was also consistent with patient reports of subjective improvement in the efficacy of their voluntary cough after treatment, although there was less improvement in symptoms of "chronic" or involuntary cough as demonstrated by a nonsignificant reduction in Cough Severity Index scores.…”

Respiratory Strength Training in Amyotrophic Lateral Sclerosis

“…9,10 Traditional ALS models of care have, therefore ,been primarily palliative in nature. 11 However, emerging evidence suggests that early mild-moderate intensity exercise programs may increase physiologic capacity and strength, prevent the development of disuse atrophy and deconditioning, mitigate pain and spasticity, and improve overall psychological well-being in individuals with ALS. 10,[12][13][14][15][16][17][18][19] Preliminary studies demonstrate that a moderate-intensity program targeting expiratory musculature (expiratory strength training) was safe and led to short-term improvements in airway clearance physiologic capacity (maximum expiratory pressure and peak expiratory cough flow) in mildly affected individuals with ALS.…”