The Cochrane Library and sickle cell disease: an overview of reviews

Abstract

AbstractBackgroundSickle cell disease is a genetically inherited red blood cell disorder which features pain as a predominant manifestation.ObjectivesTo review the evidence within Cochrane systematic reviews on the management of pain associated with vaso‐occlusive crises and priapism secondary to sickle cell disease in paediatric populations.MethodsThe Cochrane Library of Systematic Reviews was searched for all reviews that addressed interventions to relieve pain associated with sickle cell disease. Reviews that primarily addressed the reduction of sickle cell crises were excluded. Data were extracted from included reviews.ResultsThree reviews were included. One review examined pharmacological interventions to relieve pain associated with vaso‐occlusive pain crises. One trial in children reported a significant reduction in VAS scores associated with ketorolac administration versus pethidine, but another trial performed in adults showed no significant reduction in VAS scores when ketorolac was compared to placebo. Two trials found that parenterally delivered corticosteroids reduced the length of analgesic therapy compared to placebo, and another trial found that there was no difference in the pain scores of children when morphine was delivered either parenterally or orally. Two trials reported on long‐term psychological interventions to manage pain. One found that coping skills training in children resulted in a reduction in the tendency to report pain, while the other trial found that cognitive behavioural therapy in adolescents and adults resulted in a decrease in the emotional component of pain but not the sensory component. One review addressed the treatment of stuttering priapism in boys and men using stilboestrol, but included an insufficient number of patients to draw a conclusion.Authors' ConclusionsPharmacological interventions to treat vaso‐occlusive pain crises show mixed results in the use of ketorolac and no difference between parenteral versus oral administration of morphine. Psychological interventions for vaso‐occlusive crises demonstrated benefit in improving some components of pain. Evidence regarding treatment of priapism was weak. Further studies should employ multi‐centre trials and begin to address generalisability concerns by more focus in Africa, where the majority of patients with sickle cell disease reside. Copyright © 2008 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd. The Cochrane Collaboration