Assessing Air Travel Safety in Neuromuscular Dystrophy: Standard Versus Prolonged High Altitude Simulation Tests

• Status: Completed
• Trial end date: 2018-04-01
• Target enrollment: 0
• Participant gender: All

Summary

To determine if a prolonged high altitude simulation test (HAST) lasting two hours, identifies more patients at risk of respiratory failure than the standard HAST lasting 20 minutes, in patients with neuromuscular disease (NMD) and severe pulmonary restriction. To evaluate the safety of supplemental oxygen administered in those with a positive HAST in the NMD population.

• Phase: N/A
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: The Hospital for Sick Children

Criteria

Inclusion Criteria:

• individuals with a confirmed diagnosis of NMD

• age ≥ 5 years of age

• absence of hypoxia at rest, defined as oxygen saturation ≥ 92% for two consecutive minutes while at rest and breathing room air

• absence of daytime hypercapnea at rest, defined by partial pressure of carbon dioxide (pCO2) less than 45 mmHg on capillary or arterial blood gas at rest

• patient or authorized substitute decision maker provides consent to study participation.

Exclusion Criteria:

• current respiratory tract infection

• inability to comply with the study procedures

• severe heart failure as defined by uncontrolled symptoms of heart failure, or left ventricular ejection fraction < 30%

• presence of a tracheostomy

• use of domiciliary bilevel positive airway pressure (BPAP) for more than 12 hours daily

• recent hospital admission within the past 4 weeks.