Management of children with sickle cell disease: a comprehensive review of the literature

Abstract

Objective: Sickle cell disease (SCD) affects 70 000 to 100 000 people in the United States, and 2000 infants are born with the disease each year. The purpose of this study was to review the quality of the literature for preventive interventions and treatment of complications for children with SCD to facilitate the use of evidence-based medicine in clinical practice and identify areas in need of additional research.

Methods: We searched the Ovid Medline database and the Cochrane Library for articles published between January 1995 and April 2010 for English-language abstracts on 28 topics thought to be important for the care of children with SCD. We also added pertinent references cited by studies identified in our search. Each abstract was reviewed independently by 2 authors. Data from articles retrieved for full review were abstracted by using a common form.

Results: There were 3188 abstracts screened, and 321 articles underwent full review. Twenty-six articles (<1% of abstracts initially screened), which consisted of 25 randomized controlled trials and 1 meta-analysis, were rated as having level I evidence. Eighteen of the 28 topics selected for this review did not have level I evidence studies published. The management and prevention of pain episodes accounted for more than one-third of the level I studies.

Conclusions: Although significant strides have been made in the care of children with SCD in the past 2 decades, more research needs to be performed, especially for acute events associated with SCD, to ensure that the health and well-being of children with SCD continues to improve.