Key Points
ÎAmyotrophic lateral sclerosis (ALS) is a relentlessly progressive, paralyzing, neurodegenerative disease. It is characterized by loss of motor neurons in the spinal cord, brainstem, and motor cortex.
ÎMost patients with ALS die within 2 to 5 years of onset. ÎThe mainstay of ALS management is symptomatic treatment and palliative care. ÎRiluzole has FDA approval for slowing disease progress in ALS. ÎMultidisciplinary clinics are the optimal means to coordinate ALS treatment. Figure 1. Algorithm for Respiratory Management
Text in bold = evidence-based Text in italics = consensus-based
Diagnosis: ALS
Initiate noninvasive ventilation orientation Pneumovax and flu vaccine
Symptom evaluationa and PFTs
nocturnal oximetry or FVC < 50% Consider noninvasive ventilation
Orthopnea or SNP < 40 cm or MIP < -60 cm or abnormal
Noninvasive ventilation tolerated?
NO
Further education regarding documented benefits. Evaluate reasons for noncompliance
Reintroduce noninvasive ventilation
Not Successfulb
Hospice referral for palliative care
YES
Ongoing evaluations and adjustment of pressures
Successful
Discuss invasive ventilation
a Symptoms suggestive of nocturnal hypoventilation: frequent arousals, morning headaches, excessive daytime sleepiness, vivid dreams
b If noninvasive ventilation is not tolerated or accepted in the setting of advancing respiratory compromise, consider invasive ventilation or referral to hospice.
pO2 or unable to manage secretions
Unable to maintain > 90%, pCO2
< 50 mmHg PCEF < 270 L/min
Mechanical inexsufflator Treat sialorrhea/phlegm
Manual assisted cough Suction machine
FVC, forced vital capacity; SNP, sniff nasal pressure; MIP, maximal inspiratory pressure; PCCEF, peak cough expiratory flow
