A patient who was diagnosed 1 year ago with amyotrophic lateral sclerosis is being seen in his primary care physician's office. The patient is complaining of fatigue and inability to concentrate at work
The patient's FVC is 45% of predicted, the PaCO2 is 47 mm Hg, and the MIP is 54 cm H2O. Which of the following should be considered for this patient?
a. Continuation of current therapy
b. Supplemental home oxygen
c. Nocturnal CPAP
d. Nocturnal NIV
ANS: D
Amyotrophic lateral sclerosis is a progressive neurodegenerative disease that eventually leads to total paralysis. This patient has degenerated to the point where he meets the physiological criteria for the use of NIV, as evidenced by the FVC below 50% of predicted, the MIP below 60 cm H2O, and the PaCO2 above 45 mm Hg. This patient needs to be monitored closely for loss of oropharyngeal muscle strength and ability to generate an effective cough.
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