New ALS Guidelines Outline Best Practices to Prolong Length and Quality of Life

Susan Jeffrey

October 14, 2009

October 14, 2009 — Two new American Academy of Neurology practice parameters review the optimal management of patients with amyotrophic lateral sclerosis (ALS) and make evidence-based recommendations to maximize the length and quality of life for these patients.

Although the disease is still rapidly progressive and fatal, the documents aim to help optimize care. One document looks at drug, nutritional, and respiratory therapies, and the other reviews multidisciplinary care, symptom management, and cognitive and behavioral impairment in these patients.

Data taken from the ALS CARE database, which tracks outcomes for these patients, have shown that underuse of many therapies has persisted since the original American Academy of Neurology practice parameter on this topic was published in 1999, the authors, led by Robert G. Miller, MD, from California Pacific Medical Center in San Francisco, write, "though there have been gains."

Still, "the persistent underutilization of therapies that improve survival and quality of life poses a challenge for ALS clinicians to continue to raise the standard of care for patients with ALS," they conclude.

The practice parameters are published in the October 13 issue of Neurology.

Drug, Nutritional, and Respiratory Therapies

For the first parameter on drug, nutritional, and respiratory therapies, the authors reviewed 8 class 1 studies, 5 class 2 studies, and 43 class 3 studies in ALS conducted between 1998 and 2007. In general, the authors conclude that, "important treatments are available for ALS that are underutilized. Noninvasive ventilation..., percutaneous endoscopic gastrostomy (PEG), and riluzole are particularly important and have the best evidence."

Among the main recommendations in this document are:

  • Riluzole should be offered to slow disease progression in these patients (level A); there are inadequate data to support use of lithium carbonate, and more study on this agent is needed, the authors note.

  • PEG should be considered to stabilize weight and to prolong survival in patients with ALS (level B). There are insufficient data to support or refute specific timing of PEG insertion, the authors note, or to support or refute that PEG can improve quality of life in these patients (level U).

  • Noninvasive ventilation should be considered to treat respiratory insufficiency and to lengthen survival (level B) and may be considered to slow the decline of forced vital capacity (level C) and improve quality of life (level C). Early initiation of noninvasive ventilation at the earliest sign of nocturnal hypoventilation or respiratory insufficiency may increase compliance, the authors add (level C). Tracheostomy invasive ventilation may be considered to preserve quality of life in ALS patients who want long-term ventilatory support (level C).

  • Insufflation/exsufflation may be considered to help clear secretions (level C), and mechanical insufflation/exsufflation devices may be considered for this purpose in patients who have reduced peak cough flow, particularly during a chest infection (level C); however, the authors conclude there are insufficient data to draw conclusions about high-frequency chest wall oscillation to clear airway secretions (level U).

  • The authors counsel against use of creatine in doses of 5 to 10 g daily, "because it is not effective in slowing disease progression (Level A)," they write. "High-dose vitamin E should not be considered as treatment for ALS (Level B), while the equivocal evidence regarding low-dose vitamin E permits no recommendation (Level U)."

More studies are needed to examine the best tests of respiratory function in ALS, as well as the optimal time for starting PEG, the effect of PEG on quality of life and survival, and the effects of vitamins and supplements on ALS, the authors add. They also suggest, among other future research directions, that more work is needed to establish whether use of lithium carbonate slows disease progression or prolongs survival in ALS and whether more and larger trials of novel antioxidants should be considered.

Multidisciplinary Care, Symptom Management, and Cognitive Behavioral Therapy

In the second document, the same group of authors looks at other aspects of care for ALS patients including how to break the news of the diagnosis, the role of multidisciplinary clinics, symptom management, cognitive and behavioral management, and communication and palliative care for these patients.

For this analysis, the authors found 2 class 1 studies, 8 class 2 studies, and 30 class 3 studies, but note that many important areas have not been investigated. "More high-quality, controlled studies of symptomatic therapies and palliative care are needed to guide management and assess outcomes in patients with ALS," they conclude.

Among their main recommendations on these topics are:

  • Multidisciplinary clinic referral should be considered for managing patients with ALS to optimize healthcare delivery and prolong survival (level B) and may be considered to enhance quality of life, the authors conclude (level C).

  • For the treatment of refractory sialorrhea, or drooling, botulinum toxin B should be considered (level B), and low-dose radiation therapy to the salivary glands may be considered (level C).

  • A new treatment for pseudobulbar affect or emotional lability, a combination of dextromethorphan and quinidine (Avanir Pharmaceuticals), is now under review by the US Food and Drug Administration, and the authors suggest that this treatment should be considered in the event it is approved and if its adverse effects are acceptable (level B).

  • For patients who develop fatigue while taking riluzole, withholding the drug may be considered once the balance of risk between fatigue and the modest survival benefit of the drug have been discussed with the patient (level C).

  • A significant proportion of patients with ALS demonstrate cognitive impairment, the authors write, and in some cases, they meet criteria for dementia. In light of this, the authors recommend that screening for cognitive and behavioral impairment should be considered (level B). Screening tests of executive function may be considered to detect cognitive impairment in patients with ALS before confirmation with formal neuropsychological evaluation (level C).

  • Neither behavioral impairment nor the natural history of cognitive or behavioral impairments has been adequately studied, the authors add. There are insufficient data to support or refute the effect of these impairments on the management of ALS or to support or refute treatment of cognitive or behavioral impairment in the setting of ALS (level U).

  • Other management strategies, including those for cramps, spasticity, depression, anxiety, and insomnia in the setting of ALS, all lack sufficient evidence to support their use (level U).

More research is also needed into ways to optimize communication for ALS patients, specific treatments for pain and dyspnea in late-stage ALS, or the use of hospice, spiritual care, or advance directives and withdrawal of ventilation in ALS.

Although some progress has been made in evaluating therapies leading to more confident recommendations about multidisciplinary clinics and treatment for pseudobulbar affect and sialorrhea, future research is "still greatly needed" in a number of different areas, they conclude; areas such as different strategies for diagnostic disclosure, clinical trials of treatments for cramps, constipation, depression, anxiety, fatigue and therapeutic exercise, more investigation of the natural history and possible treatments for behavioral and cognitive problems, and finally, design controlled trials of terminal symptom management, advance directives, hospice, and spiritual care.

Dr. Miller has disclosed that he serves on the editorial board of the ALS Journal; received a speaker honorarium from the American Association of Neuromuscular & Electrodiagnostic Medicine; served as a consultant to Celgene, Knopp Neurosciences Inc, Teva Pharmaceutical Industries Ltd, Taiji Biomedical Inc, Sanofi-Aventis, Novartis, and Neuraltus; and receives research support from the National Institutes of Health and the Muscular Dystrophy Association. Disclosures for coauthors appear in the document.

Neurology. 2009;73:1218–1226, 1227–1233. Abstract Abstract

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