Remote respiratory resistance exercise training improves respiratory function in individuals with VCP multisystem...

Area of Science:

• Neurology
• Pulmonology
• Genetics

Background:

• Valosin-containing protein (VCP) disease is a genetic disorder causing multisystem proteinopathy.
• Respiratory muscle weakness is a common and life-threatening complication of VCP disease.
• Effective interventions for respiratory dysfunction in VCP disease are limited.

Purpose of the Study:

• To evaluate the feasibility, safety, and efficacy of a remotely administered inspiratory muscle training program in individuals with VCP disease.
• To assess the impact of inspiratory muscle training on respiratory muscle strength and functional outcomes.

Main Methods:

• A 40-week remote study involving nine adults with VCP mutation-positive familial myopathy.
• An 8-week baseline period followed by 32 weeks of inspiratory muscle training.
• Primary outcome: maximum inspiratory pressure (MIP); Secondary outcomes: spirometry, grip strength, functional rating scales (IBMFRS, ALSFRS), and mobility tests (TUG, 6MWT).

Main Results:

• Significant weekly increase in maximum inspiratory pressure (MIP) by 0.392 cm H2O (p=0.023) during the training phase.
• Expected significant decreases in grip strength and ALSFRS scores, reflecting disease progression.
• The training program was feasible, safe, and well-tolerated by participants.

Conclusions:

• Remotely administered inspiratory muscle training can significantly improve inspiratory muscle strength in individuals with VCP disease.
• This intervention offers a promising, accessible approach to managing respiratory complications in VCP disease.
• Further research is warranted to explore long-term effects and broader clinical benefits.