Exercise for Better ALS Management?

Abstract

You have accessThe ASHA LeaderFeature1 Sep 2012Exercise for Better ALS Management? Rachel Harkawik andCScD, CCC-SLP James L. CoylePhD, CCC-SLP BRS-S Rachel Harkawik Google Scholar More articles by this author , CScD, CCC-SLP and James L. Coyle Google Scholar More articles by this author , PhD, CCC-SLP BRS-S https://doi.org/10.1044/leader.FTR5.17112012.np SectionsAbout ToolsAdd to favorites ShareFacebookTwitterLinked In In a major departure from previous thinking, new research suggests moderate exercise could potentially benefit patients with amyotrophic lateral sclerosis (ALS). In fact, some moderate intensity resistance, endurance, and cardiovascular exercise may preserve function for a longer period of time. Speech-language pathologists—like other rehabilitation professionals—typically focus on improving independence for patients who have experienced a decline in their communication and swallowing functions. But in our growing role as medical SLPs, we often seek to lower the risk of adverse outcomes by using preventive measures (ASHA, 2004). In the case of ALS, these new findings could bolster our role in providing secondary prevention services (see box for definitions) and help us educate other professionals on the scope of SLPs’ preventive care. Clinical Markers of Decline Patients with ALS often undergo repeated routine testing to assess their current level of functioning, predict the rate of disease progression, and ensure timely management of symptoms signaling clinical risk. Although testing reveals declining functional capacity as ALS progresses, it might also provide intervention targets previously avoided for fear of accelerating patient deterioration. Pulmonary function testing is one test battery that assesses ventilation—the ability to move air into and out of the respiratory system—and is critical to the patient’s physiologic need to exchange respiratory gases between the blood and the atmosphere. Forced vital capacity (FVC) is produced by abdominal musculature responsible for forced exhalation and coughing, both of which are necessary to clear contaminants from the respiratory system and to produce speech. Decreased FVC diminishes speech intelligibility by lowering expiratory airflow and pressure necessary for sufficiently loud speech and length of utterance. According to the American Academy of Neurology’s Practice Parameter, decreased FVC was the single most relevant prognostic factor for mortality in patients with ALS (Chio` et al., 2009). An FVC below 50% of normal ( based on a person’s height, age, and gender) indicates impending respiratory failure, and should prompt consideration of mechanical ventilation (Benditt, 2002). Often, concurrent consideration of a feeding tube to mitigate the risk of large volume aspiration and to maintain hydration and nutrition, is recommended (Benditt, 2002). However, interventions should be determined on a patient-by-patient basis. Progressive deterioration of muscle function in the structures responsible for articulation, phonation, and regulation of resonance also can affect speech. But emerging evidence suggests that in aging people and those with stroke, lingual muscle exercise can produce increased lingual strength and pressure generation while swallowing. Likewise, studies of expiratory muscle strengthening have demonstrated improvements in maximum expiratory pressure. Lingual and expiratory muscle exercise may retard the progression of age-related loss of ventilatory and speech muscle function. Exercise in ALS: Good or Bad? Clinicians have used epidemiological studies and anecdotes to justify exercise avoidance in ALS. For example, Bello-Hass and colleagues (2007) reported that people who performed intense physical activity at work or leisure prior to the onset of disease were at higher risk of developing ALS. Other studies have reported a higher incidence of ALS in veterans and professional athletes whose professions involved regular intensive exercise (Chio` et al. 2005; Haley, 2003). The nickname for the condition—Lou Gehrig’s disease, after the professional athlete who had the disease and raised its profile—has amplified this notion. Given reports linking exercise and onset of ALS, it is understandable why avoiding exercise was thought to be beneficial. The medical management methods of ALS patients may play a role in why exercise is thought to be detrimental. Fatigue is a common symptom in ALS. In response to this symptom, clinicians encourage conservation to preserve available energy for important activities of daily living. It seems to make “common sense” that for patients with excessive fatigue the unnecessary use of declining muscles would be counterproductive, “wasting” an already-diminishing resource. In fact, most patients with ALS are encouraged to minimize overworking declining muscles (Drory, et Al., 2001). But now researchers are taking a closer look at the effects of exercise on patients with ALS, finding reasons that may justify some forms of exercise with this population. Exercise has been shown to increase mitochondrial content, enhance blood flow to muscles, and increase muscle strength and endurance in other neurological conditions such as post-polio syndrome. These adaptations may help to increase the efficiency of the neuromuscular system and reduce fatigue (Drory et al., 2001). Other research suggests that general stretching and light exercise may reduce other complications of neuromuscular disease, such as ALS-related musculoskeletal pain and contractures (Krivickas, 2003). Animal models have shown that exercise may have neuroprotective properties, such as enhancing new neuronal formations (Kirkinezos, 2003). Also, oxidative stress has been hypothesized to lead to cell death in ALS. Since endurance exercise has been found to reduce oxidative stress in muscles, researchers are beginning to question avoidance of exercise in ALS, and more research is being conducted in this area (Mahoney et Al,. 2004). As for the question of whether exercise hurts patients with ALS, research indicates the contrary-at least when it’s moderate. Early experiments were conducted with mice that were bred to have ALS. A study by Kirkinezos and colleagues (2003) found that moderate—but not strenuous or minimal—exercise (walking) significantly increased the animals’ life span by 8% for males and 3% for females, while not increasing the progression rate of the disease. Another study found that male mice receiving intensive exercise exhibited more rapidly declining performance and died more quickly than females over time (Mahoney et al., 2004). Researchers also examined the effects of exercise in humans with ALS. In 2001, Drory and team divided 25 patients with ALS into a moderate regular exercise group (n=14) and a control group (n= 11) performing regular daily activity. Members of the exercise group did not have a faster rate of decline in muscle strength, nor did they experience greater fatigue or increased musculoskeletal pain than the control subjects. The exercise group benefitted from significantly decreased spasticity at three months and was clinically less disabled than control subjects at three and six months. A study looking at a resistance exercise program found similar benefits. Participants had significantly greater combined upper and lower extremity scores than controls on the ALS functional rating scale, and better overall scores at six months. Neither leg strength nor FVC declined, and participants in the resistance exercise group experienced a higher quality of life at six months after exercise began (Bello-Haas et al., 2007). It is clear that the endpoint of ALS is death, and all of the studies cited above underscore that benefits of any ALS intervention will always be finite. But as a preventive modality, exercise may reduce the rate and intensity of future related conditions. Gosselink and colleagues (2000) found expiratory muscle weakness to be directly related to a decreased FVC-which is related to overall survival. No studies could be found to date on inspiratory (diaphragm) or expiratory (abdominal) exercise in patients with ALS, although there are promising findings from research on patients with multiple sclerosis, who are similar to patients with ALS in that both have respiratory weakness due to upper motor neuron damage. Expiratory muscle training was used with 12 patients with multiple sclerosis. Participants breathed against resistance, using a positive expiratory pressure threshold training device. Results from training indicated that patients with moderate disability improved their maximum expiratory pressure more than those with a mild disability. Gains were maintained above baseline following training. Participants with multiple sclerosis increased their words per minute and reported a subjective benefit for their dysarthria (Chiara, Martin, & Sapienza, 2007). Taking into account all the identified research—extremity or expiratory/respiratory—it appears that exercise may be beneficial earlier in the disease’s progression rather than later. An Evidence-Based Care Model These studies show gains in extremity functioning. Could exercise have the same potential effects on other skeletal muscles affected by ALS, such as those related to respiration, communication, and swallowing (Eberstein & Googgold, 1968; Llado et al., 2006)? Given the benefits of exercise seen in some patients with ALS and multiple sclerosis, it is possible that exercise will find its way into the management plans of SLPs working with ALS patients in an effort to slow deterioration. If respiratory exercise can increase maximal expiratory pressure, it could help slow the decline of FVC which, in turn, may delay or mitigate the need for mechanical ventilation and some of the negative secondary effects of dysphagia. During speech, the increase in words per minute could possibly delay the need for AAC or allow more time for additional training before AAC is implemented fully. Slowing the progression of respiratory failure offers the opportunity to provide patients more time to receive counseling about their medical options and end-of-life care. SLPs planning to recommend exercise should secure clearance from the patient’s physician to prevent inadvertent cardiovascular complications when performing swallowing maneuvers (Chaudhuri et al., 2002). For further guidance, we propose a theoretical model of secondary prevention of communication and swallowing deterioration for SLPs working with patients with ALS, (see box, page XX). While this model has not been explored yet in scientific studies, we present it to stimulate future research on the subject and provide SLPs with evidence to help them make objective decisions about the potential use of exercise. Professionals should be considerate of the disease progression when exercise is recommended, and recognize when patients can no longer benefit from exercise due to advanced disease. As SLPs, we can take a more active role in providing preventive services for patients with ALS. We now have evidence to support that fact that exercise may not be determetal as it was once was thought. However, we need more information to draw decisive conclusions on the benefits of aerodigestive tract exercise therapy in patients with ALS. We encourage researchers and clincians to explore factors that may enhance the benefit of exercise, including identification of optimal candidates, intensity and duration of exercise, specific training routines, and candidates for whom expiratory exercise could be harmful. It will be important to emphasize early intervention and preemptive measures alongside traditional symptomatic treatment. Acknowledgment: The authors thank Paula Leslie, PhD, CCC-SLP, for editorial assistance with this article. The Relentless Progression of ALS Each year, 5,000 people are diagnosed with ALS in the United States. In 2011, as many as 20,000-30,000 Americans were living with ALS (NIH, 2011). This disease systematically destroys the upper and lower motor neurons responsible for controlling voluntary movement, causing progressive loss of muscle function and resulting in progressive muscle degeneration and atrophy. During this process, the patient experiences advancing weakness and eventual paralysis, causing deterioration of aerodigestive tract functioning responsible for swallowing, breathing, and communication. Its natural clinical endpoint is paralysis of the respiratory musculature (Mayo Clinic, 2011). Although the cause of ALS is not yet known, the disease causes diminishing muscle fiber population, which probably explains why patients commonly report symptoms of fatigue as ALS progresses (Mahoney et al., 2004). As more muscle fibers become denervated, physical endurance and available energy diminish. Subsequent symptoms includeprogressive loss of speech intelligibility (dysarthria), ventilatory competence that may result in ventilator dependence, and oropharyngeal swallowing disorders that endanger airway protection, hydration, and nutrition. Dysarthria directly diminishes communication abilities, safety, and independence; dysphagia is associated with secondary adverse outcomes such as malnutrition, airway obstruction, and an increased risk for lower respiratory tract infection (pneumonia) that increases steeply as pulmonary function declines. Levels of Prevention SLPs provide three levels of prevention, according to ASHA (1988): Primary prevention is the elimination or inhibition of the onset and development of a communication or swallowing disorder. An example of primary prevention is intervention with dysphagic patients to lower their risk of pneumonia. Secondary prevention is the early detection and treatment of communication disorders that may lead to elimination or retardation of the disorder’s progress. Interventions may preserve communication and swallowing function during a disease’s progression. Tertiary prevention is the reduction of a disability by attempting to restore effective functioning, largely through the provision of active rehabilitative and compensatory services (ASHA, 1988). Given the nature of ALS, primary prevention of communication and swallowing disorders is not possible, and SLPs typically provide tertiary prevention via compensatory interventions. Sources American Speech-Language-Hearing Association [ASHA]. (1988). Prevention of communication disorders [Position Statement]. Available from www.asha.org/policy. Google Scholar American Speech-Language-Hearing Association [ASHA]. (2004). Preferred practice patterns for the profession of speech-language pathology [Preferred Practice Patterns]. Available from www.asha.org/policy. Google Scholar Bello-Haas V., Florence J., Scheirbecker J., Lopate G., Hayes S., Pioro E., & Mitsumoto H. (2007). A randomized controlled trial of resistance exercise in individuals with ALS.Neurology, 68, 2003–2007. 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She is an affiliate of Special Interest Group 12, Augmentative and Alternative Communication. Contact her at[email protected]. James L. Coyle, PhD, CCC-SLP BRS-S, is an assistant professor of communication sciences and disorders at the University of Pittsburgh. His research interests include dysphagia outcomes measurement and attribution of health risks associated with dysphagia. He is a Board Recognized Specialist in Swallowing and Swallowing Disorders and member of the Board, an affiliate of Special Interest Group 13, Swallowing and Swallowing Disorders (Dysphagia). Contact him at[email protected]. Advertising Disclaimer | Advertise With Us Advertising Disclaimer | Advertise With Us Additional Resources FiguresSourcesRelatedDetailsCited ByPerspectives of the ASHA Special Interest Groups5:6 (1603-1615)17 Dec 2020The Safety, Tolerability, and Impact of Respiratory–Swallow Coordination Training and Expiratory Muscle Strength Training on Pulmonary, Cough, and Swallow Function Surrogates in Amyotrophic Lateral SclerosisCara Donohue and James L. Coyle Volume 17Issue 11September 2012 Get Permissions Add to your Mendeley library History Published in print: Sep 1, 2012 Metrics Downloaded 2,990 times Topicsasha-topicsleader_do_tagasha-article-typesCopyright & Permissions© 2012 American Speech-Language-Hearing AssociationLoading ...