title: Auto-adjusting positive airway pressure in children with sickle cell anemia: results of a phase I randomized controlled trial.
abstract: Low nocturnal oxygen saturation (SpO(2)) is implicated in complications of Sickle Cell Anemia (SCA). Twenty-four children with SCA were randomized to receive overnight auto-adjusting continuous positive airway pressure (auto-CPAP) with supplemental oxygen, if required, to maintain SpO(2) >or=94% or as controls. We assessed adherence, safety, sleep parameters, cognition and pain. Twelve participants randomized to auto-CPAP (3 with oxygen) showed improvement in Apnea/Hypopnea Index (p<0.001), average desaturation events >3%/hour (p=0.02), mean nocturnal SpO(2) (p=0.02) and cognition. Primary efficacy endpoint (Processing Speed Index) showed no group differences (p=0.67), but a second measure of processing speed and attention (Cancellation) improved in those receiving treatment (p=0.01). No bone marrow suppression, rebound pain or serious adverse event resulting from auto-CPAP use was observed. Six weeks of auto-CPAP therapy is feasible and safe in children with SCA, significantly improving sleep-related breathing disorders and at least one aspect of cognition.
authors: Neurosciences Unit, UCL Institute of Child Health, The Wolfson Centre, London, UK.
authors: Melanie J
authors: MJ
authors: Marshall
authors: Romola S
authors: RS
authors: Bucks
authors: Alexandra M
authors: AM
authors: Hogan
authors: Ian R
authors: IR
authors: Hambleton
authors: Susan E
authors: SE
authors: Height
authors: Moira C
authors: MC
authors: Dick
authors: Fenella J
authors: FJ
authors: Kirkham
authors: David C
authors: DC
authors: Rees
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000503:physiopathology* / Q000628:therapy*; D002648:Child; D045422:Continuous Positive Airway Pressure / Q000379:methods*; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D009483:Neuropsychological Tests; D010100:Oxygen / Q000378:metabolism; D010349:Patient Compliance; D017286:Polysomnography / Q000379:methods; D011788:Quality of Life; D012890:Sleep; D016896:Treatment Outcome
chemical_list: D010100:Oxygen