Disease Management
ÎSpecialized multidisciplinary clinic referral should be considered to optimize health care delivery and prolong survival (B) and may be considered to enhance quality of life (C).
Respiratory Management Assessment:
ÎSupine forced vital capacity (FVC) and maximal inspiratory pressure (MIP) may be considered useful in routine respiratory monitoring, in addition to the erect FVC (C).
ÎNocturnal oximetry may be considered to detect hypoventilation (regardless of the FVC) (C).
ÎSniff nasal pressure (SNP) may be considered to detect hypercapnia and nocturnal hypoxemia (C).
Treatment:
ÎNoninvasive ventilation should be considered to treat respiratory insufficiency, both to lengthen survival and to slow the rate of FVC decline (B).
ÎNoninvasive ventilation may be considered to enhance quality of life in patients who have respiratory insufficiency (C).
ÎNoninvasive ventilation may be considered at the earliest sign of nocturnal hypoventilation or respiratory insufficiency in order to improve compliance with noninvasive ventilation (C).
ÎTracheostomy invasive ventilation (TIV) may be considered to preserve quality of life in patients who want long-term ventilatory support (C).
ÎMechanical insufflation/exsufflation (MIE) may be considered to clear secretions in patients who have reduced peak cough flow, particularly during an acute chest infection (C).
ÎThere are insufficient data to support or refute high-frequency chest wall oscillation (HFCWO) for clearing airway secretions (U).
Late-stage ALS (U)
ÎThere are insufficient data to support or refute > Specific treatments for pain and dyspnea > Hospice, spiritual care, or advance directives > Specific strategies for withdrawal of ventilation
Table 1. FDA-Approved Drugs for ALS Drug
Dose
Riluzole Rilutek
50 mg q12h Warning
Liver injury (rare), neutropenia (rare)
Side Effects Fatigue, nausea
