P138: WHITE PAPER: ASSESSMENT OF INTERNATIONAL SICKLE CELL DISEASE GUIDELINES IN HIGH-, MIDDLE-, AND LOW-INCOME COUNTRIES

Sickle cell disease (SCD) is a common genetic condition defined by abnormalities in hemoglobin, an oxygen carrying protein in red blood cells. About 5% of the world's population carries genes for hemoglobin disorders, with the highest prevalence in countries in Subsaharan Africa (SSA). SCD causes significant morbidity, mortality, and economic burden in the countries and populations affected. Various countries have established national guidelines for SCD management, which serve to standardize care and improve patient outcomes. Ten such guidelines were identified for compilation and analysis in this paper as of summer 2021. Qualitative analysis of the guidelines has identified the priorities of each government and medical institutions’ stance on best practices across high-, middle-, and low-income countries. The analysis identified that care for acute complications of SCD is more frequently endorsed in the guidelines of low-and-middle income countries (LMIC), which also have higher disease burden, over high-income countries (HIC). Chronic preventative care for SCD disease, which is shown to be an evidence-based effective approach to improve patient outcomes, is more frequently recommended in guidelines of HIC over LMIC. Furthermore, national newborn screening guidelines are scarce in the countries with high prevalence rates and adolescent mortality. While most countries recommend screening programs, LMIC have limited recommendations for newborn preventative measures. Incorporation and prioritization of preventive care guidelines in LMIC may serve as a path to inform sustainable nationwide interventions to improve patient outcomes for SCD. Implementation of these guidelines would contribute to preventing acute complications and improving morbidity and mortality for individuals with SCD. In addition, prioritization and inclusion of preventive and chronic care measures in healthcare services may mitigate the economic burden of SCD in LMIC.

References

1. Yawn et al. JAMA 2014. 312;10.

2. Piel et al, PLOS Medicine 2013; 10:7

3. Kato et al, Nat Rev Dis Primers 2018; 15:4.

4. Ryan et al. Br J Haematol 2010; 149;1.

5. Alli et al. S Afr Med J 2014; 104;11.