Modified hypoxic challenge testing in children needing nocturnal ventilation: An observational study.

ABSTRACT

BACKGROUND: Guidelines for air passengers with respiratory disease focus on primary lung pathology. Little evidence exists to guide professionals advising children needing ventilatory support because of neuromuscular or central hypoventilation conditions; these children might risk hypoxia and hypercapnia if unable to mount an adequate hyperventilation response. OBJECTIVE: This study assessed the response to low ambient oxygen using a modified hypoxic challenge test. In addition to measuring pulse oximetry and response to supplementary oxygen, we also measured transcutaneous carbon dioxide and response to ventilatory support. METHODS: Twenty children on nocturnal ventilatory support aged 1.6-18 years were recruited in a pragmatic sample from outpatient clinics; 10 with neuromuscular weakness and 10 with central hypoventilation. Participants underwent a two-stage, modified hypoxic challenge test; a conventional stage, where oxygen alone was titrated according to SpO2, and a new stage, where participants used their routine ventilatory support with oxygen titrated if needed. Participants were interviewed to understand their experiences of testing and of air travel. RESULTS: Thirteen participants needed supplemental oxygen during the conventional stage, but only two did when using ventilatory support. Transcutaneous carbon dioxide remained within normal range for all participants, on or off ventilatory support. Whilst some participants found testing challenging, participants generally reported both testing and air travel to be valuable. CONCLUSIONS: Evaluating response to patients' usual ventilation through "fitness-to-fly" assessment aids decision making when considering whether children who receive nocturnal ventilation can travel by air, since for some using a ventilator reduces or avoids the need for supplemental oxygen.