Haematologica, Vol 94, Issue 7, 1006-1010 doi:10.3324/haematol.2008.005215
Copyright © 2009 by Ferrata Storti Foundation
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Sickle Cell Disease

Auto-adjusting positive airway pressure in children with sickle cell anemia: results of a phase I randomized controlled trial

Melanie J. Marshall1, Romola S. Bucks2, Alexandra M. Hogan3, Ian R. Hambleton4, Susan E. Height5, Moira C. Dick, Fenella J. Kirkham1, David C. Rees5

1 Neurosciences Unit &
3 Developmental Cognitive Neuroscience Unit, UCL Institute of Child Health, London;
2 School of Psychology, University of Western Australia;
4 Chronic Disease Research Centre, The University of the West Indies, Barbados and
5 Department of Paediatric Haematology, King's College Hospital, London, UK

Correspondence: Fenella J Kirkham, Neurosciences Unit, UCL Institute of Child Health, The Wolfson Centre, Mecklenburgh Square, London WC1N 2AP, UK. E-mail:f.kirkham{at}ich.ucl.ac.uk The online version of this article contains a supplementary appendix

Low nocturnal oxygen saturation (SpO2) 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 SpO2 ≥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 SpO2 (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.

Key words: red cells, hemoglobinopathies, quality of life, continuous positive airway pressure, oxygen saturation.