The Measurement and Treatment of Respiratory Impairment in Patients with Amyotrophic Lateral Sclerosis

Johns Hopkins University

Goal:
Improve measurement of respiratory impairment and assess the impact of NIV

Aims:

• Determine the ability of upright and supine FVC to predict transdiaphragmatic pressure.
• Determine whether NIV cab improve lung compliance in patients with respiratory muscle weakness
• Determine whether NIV can alter the decline in pulmonary function in patients with mild respiratory muscle involvement.

Status:
Conclusion for Aim #1 is that FVC is an excellent and simple test of diaphragmatic weakness. Initial findings for Aim #2 suggests that NIPPV can improve lung compliance and correlates with diaphragmatic weakness. The study continues to address Aim #3.