Central Hypoventilation: A Case Study of Issues Associated with Travel Medicine and Respiratory Infection

Hon, Kam Lun; Leung, Alexander K. C.; Li, Albert M. C.; Ng, Daniel K. K.

In the summer of 2013, the pediatric intensive care unit (PICU) of a hospital in Hong Kong received an 8-year-old boy with central hypoventilation with respiratory infection and decompensation en route to Hong Kong on a make-a-wish campaign. He was diagnosed with central hypoventilation (medullary atrophy) or Ondine's curse and had been well managed in Toronto, Canada. He was ambulatory, only needed home ventilatory support at night via tracheostomy, and inhaled salbutamol puffs on a prn basis, and he was on PEG (percutaneous endoscopic gastrostomy) feeding with puree food. Advanced fitness for air-travel arrangement was well negotiated with the respective commercial airline. However, he developed symptoms of respiratory infections with intermittent fever (up to 39 ∘ C), cough, and sputum for 2 days prior to departure. The child was seen at the emergency department of a children's hospital in Toronto and was treated with an oral course of cefuroxime. During the long-haul flight, symptoms of respiratory infections persisted and desaturations (86%) developed. The patient had his own oxygen monitoring and air compressor on board which needed to be increased to 1 L/min. On arrival in Hong Kong, he was taken to the emergency department. His vital signs were as follows: tympanic temperature 38.6 ∘ C, heart rate 157/min, and SpO 2 98% on own ventilator with flow 1 L/min. The home ventilator's electric plug was in Canadian style and did not fit the Hong Kong standard socket. Chest radiograph revealed mild right sided haziness. He was admitted to PICU for management. He weighed 23.2 kg and his vital signs were as follows: temperature 36.5 ∘ C, heart rate 121/min, respiratory rate 23/min, BP 97/57 mmHg, and SpO 2 97% in room air on arrival at PICU. The child received physiotherapy and the tracheostomy was temporarily connected to the ICU ventilator on SIMV mode with pressure control (PC) and pressure support (PS). Settings were FiO 2 0.25, inspiratory time (Ti) 0.9 seconds, intermittent mandatory ventilation (IMV) rate 20/min, positive end expiratory pressure (PEEP) 5 cm H 2 O, pressure control 15 cm H 2 O above PEEP, and PS 13 cm H 2 O above PEEP. There was no further desaturation, and the settings were gradually reduced to IMV 10/min and FiO 2 of 0.21. The child gave a history of drug allergy to Ativan (lorazepam) and gluten sensitivity. Sedation was not needed. He received a course of intravenous amoxicillin/clavulanate (30 mg/kg/dose, 8 hourly). The patient remained playful, talkative, and not in distress. Laboratory data were normal complete blood count with white blood cell count of 16.1 × 10 9 /L, neutrophil differential of 77%, and elevated C-reactive protein of 44.1 (normal < 9.9 mg/L). There was no bacterial or respiratory viral isolation in the tracheal aspirate. Blood culture was negative. The patient was discharged from the PICU 2 days later and went on with his make-a-wish journey to Disneyland in Hong Kong prior to returning home.