Managing respiratory problems in Prader–Willi syndrome

Children with Prader Willi syndrome (PWS) are at risk of developing both central and obstructive sleep apnoea in the context of dysfunctional respiratory control, a small upper airway and obesity. Growth hormone (GH) promotes body composition, psychosocial development and quality of life. Despite evidence of its stimulatory effect on the ventilatory drive, GH has been associated with unexpected nocturnal deaths in high risk patient groups such as the obese and those with pre existing respiratory problems. Using a case study, the present article explores issues around respiratory investigations, the safe use of GH and non invasive ventilation in children with PWS.