Disease Management
Î Specialized 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:
Î Supine forced vital capacity (FVC) and maximal inspiratory pressure
(MIP) may be considered useful in routine respiratory monitoring, in
addition to the erect FVC (C).
Î Nocturnal oximetry may be considered to detect hypoventilation
(regardless of the FVC) (C).
Î Sniff 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).
Î Noninvasive ventilation may be considered to enhance quality of life in
patients who have respiratory insufficiency (C).
Î Noninvasive ventilation may be considered at the earliest sign of
nocturnal hypoventilation or respiratory insufficiency in order to improve
compliance with noninvasive ventilation (C).
Î Tracheostomy invasive ventilation (TIV) may be considered to preserve
quality of life in patients who want long-term ventilatory support (C).
Î Mechanical insufflation/exsufflation (MIE) may be considered to clear
secretions in patients who have reduced peak cough flow, particularly
during an acute chest infection (C).
Î There are insufficient data to support or refute high-frequency chest wall
oscillation (HFCWO) for clearing airway secretions (U).
Late-stage ALS (U)
Î There 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 Warning Side Effects
Riluzole
Rilutek
50 mg q12h Liver injury (rare),
neutropenia (rare)
Fatigue, nausea
