OBJECTIVES: Sickle cell disease (SCD) is a commonly inherited haematological disorder associated with a huge economic burden to the healthcare systems. Approximately 300,000 infants are born with SCD annually. Routine and lifelong comprehensive care is needed for patients to treat serious complications. The data on economic burden associated with SCD are limited. This review aims to synthesize the economic burden of SCD worldwide.

METHODS: A targeted literature review was performed by conducting a comprehensive search in PubMed, Cochrane and Embase databases to locate articles written in English and published from January 2014 through June 2019. The target population included patients diagnosed with SCD. The outcomes studied included health care resource utilization (HCRU) - direct and indirect costs.

RESULTS: A total of 20 studies were reviewed to estimate the economic burden and HCRU associated with SCD. The total direct annual costs ranged from US$ 1 million to US$ 3 million in 2013 and the average annual indirect cost was found to be US$ 1,293 in 2015. The annual loss of transportation was estimated at US$ 2,372 in 2012. A massive disparity was observed among hospital admissions and emergency department (ED) visits which range from 183 to 1,101 and 24 to 1,990 visits respectively. The total length of stay associated with SCD was between 6 and 67 days. The total 94 inpatient and 1,060 outpatient visits were recorded, but a higher variation across the included studies.

CONCLUSIONS: The current literature review found wide discrepancies between HCRU data reported for SCD and illustrates its huge economic burden. This review highlights the need for cost effective treatments in the management of SCD.