New Guidelines for Treating Patients With Amyotrophic Lateral Sclerosis
New Guidelines for Treating Patients With Amyotrophic Lateral Sclerosis
Can you discuss the current guidelines for the treatment of patients with amyotrophic lateral sclerosis?
The American Academy of Neurology (AAN) has published a 2-part Practice Parameter update about the care of the patient with amyotrophic lateral sclerosis (ALS). The Practice Parameter update was developed by a team of ALS experts, convened by the AAN, led by Robert G. Miller, MD, of the Department of Neurology, California Pacific Medical Center, San Francisco, California.
The chief message of this practice parameter is that while there is no cure for ALS, much can be done to ease the burden of the disease on patients and their caregivers and, to a certain degree, to extend their survival.
In 1999, the AAN published an evidence-based practice parameter for managing patients with ALS, but new studies in the past 10 years made an update appropriate. To update the 1999 practice parameter, the authors systematically reviewed and analyzed studies from 1998 to 2007 and classified them using the AAN's evidence-based methodology.
The authors identified 8 Class 1 studies, 5 Class 2 studies, and 43 Class 3 studies addressing the questions of slowing disease progression, nutrition, and respiratory management for patients with ALS, and 2 Class 1 studies, 8 Class 2 studies, and 30 Class 3 studies addressing the questions of breaking the news, multidisciplinary clinics, symptom management, cognitive and behavioral impairment, communication, and palliative care for patients with ALS. Under this classification system, Class 1 and Class 2 studies are the most reliable: they constitute only 20%-25% of the articles informing the conclusions. The authors state therefore that more studies are needed to examine the best tests of respiratory function in ALS, as well as the optimal time for starting percutaneous endoscopic gastrostomy (PEG), the impact of PEG on quality of life and survival, and the effect of vitamins and supplements on ALS, as well as of symptomatic therapies and palliative care.
The chief findings of the practice parameter are that for extending life or slowing disease progression. The best evidence is for use of noninvasive ventilation (NIV), PEG, and riluzole. The authors comment that these treatments are often underutilized and recommend that they should be offered to patients, in the case of riluzole, or considered by the physician, in the case of NIV and PEG.
The median extension of life by riluzole in placebo-controlled studies was 2-3 months. The median extension of life by NIV or PEG may be approximately 6 months, provided the treatments are applied early and adhered to. The efficacy of NIV is supported by a randomized controlled trial.
The authors also recommend that multidisciplinary clinic referral should be considered for treating patients with ALS to optimize healthcare delivery and prolong survival, and may be considered to enhance quality of life. They recommend that botulinum toxin B should be considered for the treatment of refractory sialorrhea and that low-dose radiation therapy to the salivary glands may be considered.
Medication treatments for sialorrhea are typically tried first. Patients who gain insufficient relief with medications are considered "refractory." There is a manufacturer's warning about use of botulinum toxin in patients with ALS, due to concern of spread of the toxin to ventilatory muscles, leading to exacerbation of ventilatory failure. In my opinion, this may tilt the balance in terms of safety toward salivary gland irradiation, except in patients with exceptional ventilatory reserve.
Finally, the authors of the practice parameter recommend that dextromethorphan and quinidine should be considered for pseudobulbar affect if approved by the US Food and Drug Administration and that for patients in whom fatigue develops while taking riluzole, withholding the drug may be considered.
The care of most patients with ALS will exceed the resources available to any single practitioner. For that reason, referral to a multidisciplinary clinic is very effective in helping patients manage the course of their disease. Its impact on prolonging survival depends in part on the patients' acceptance of treatments available to them. This, in turn, hinges on the value they place on extending survival in the later stages of their disease. Having practiced in different places, I am aware that this aspect of patient care may vary from place to place.
The updated Practice Parameter, taken together with the original parameter, places excellent current treatment information at the finger tips of those caring for patients with ALS. Clinician Summaries and Patient Summaries are available on the AAN Website.
Question
Can you discuss the current guidelines for the treatment of patients with amyotrophic lateral sclerosis?
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Response from Carmel Armon, MD, MSc, MHS Professor of Neurology, Tufts University School of Medicine, Boston, Massachusetts; Chief, Division of Neurology, Baystate Medical Center, Springfield, Massachusetts |
The American Academy of Neurology (AAN) has published a 2-part Practice Parameter update about the care of the patient with amyotrophic lateral sclerosis (ALS). The Practice Parameter update was developed by a team of ALS experts, convened by the AAN, led by Robert G. Miller, MD, of the Department of Neurology, California Pacific Medical Center, San Francisco, California.
The chief message of this practice parameter is that while there is no cure for ALS, much can be done to ease the burden of the disease on patients and their caregivers and, to a certain degree, to extend their survival.
In 1999, the AAN published an evidence-based practice parameter for managing patients with ALS, but new studies in the past 10 years made an update appropriate. To update the 1999 practice parameter, the authors systematically reviewed and analyzed studies from 1998 to 2007 and classified them using the AAN's evidence-based methodology.
The authors identified 8 Class 1 studies, 5 Class 2 studies, and 43 Class 3 studies addressing the questions of slowing disease progression, nutrition, and respiratory management for patients with ALS, and 2 Class 1 studies, 8 Class 2 studies, and 30 Class 3 studies addressing the questions of breaking the news, multidisciplinary clinics, symptom management, cognitive and behavioral impairment, communication, and palliative care for patients with ALS. Under this classification system, Class 1 and Class 2 studies are the most reliable: they constitute only 20%-25% of the articles informing the conclusions. The authors state therefore that more studies are needed to examine the best tests of respiratory function in ALS, as well as the optimal time for starting percutaneous endoscopic gastrostomy (PEG), the impact of PEG on quality of life and survival, and the effect of vitamins and supplements on ALS, as well as of symptomatic therapies and palliative care.
The chief findings of the practice parameter are that for extending life or slowing disease progression. The best evidence is for use of noninvasive ventilation (NIV), PEG, and riluzole. The authors comment that these treatments are often underutilized and recommend that they should be offered to patients, in the case of riluzole, or considered by the physician, in the case of NIV and PEG.
The median extension of life by riluzole in placebo-controlled studies was 2-3 months. The median extension of life by NIV or PEG may be approximately 6 months, provided the treatments are applied early and adhered to. The efficacy of NIV is supported by a randomized controlled trial.
The authors also recommend that multidisciplinary clinic referral should be considered for treating patients with ALS to optimize healthcare delivery and prolong survival, and may be considered to enhance quality of life. They recommend that botulinum toxin B should be considered for the treatment of refractory sialorrhea and that low-dose radiation therapy to the salivary glands may be considered.
Medication treatments for sialorrhea are typically tried first. Patients who gain insufficient relief with medications are considered "refractory." There is a manufacturer's warning about use of botulinum toxin in patients with ALS, due to concern of spread of the toxin to ventilatory muscles, leading to exacerbation of ventilatory failure. In my opinion, this may tilt the balance in terms of safety toward salivary gland irradiation, except in patients with exceptional ventilatory reserve.
Finally, the authors of the practice parameter recommend that dextromethorphan and quinidine should be considered for pseudobulbar affect if approved by the US Food and Drug Administration and that for patients in whom fatigue develops while taking riluzole, withholding the drug may be considered.
The care of most patients with ALS will exceed the resources available to any single practitioner. For that reason, referral to a multidisciplinary clinic is very effective in helping patients manage the course of their disease. Its impact on prolonging survival depends in part on the patients' acceptance of treatments available to them. This, in turn, hinges on the value they place on extending survival in the later stages of their disease. Having practiced in different places, I am aware that this aspect of patient care may vary from place to place.
The updated Practice Parameter, taken together with the original parameter, places excellent current treatment information at the finger tips of those caring for patients with ALS. Clinician Summaries and Patient Summaries are available on the AAN Website.
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