Hydroxyurea for children with sickle cell disease in sub‐Saharan Africa: A summary of the evidence, opportunities, and challenges

Abstract

Sickle cell disease (SCD) is a common and life‐threatening inherited blood disorder that affects more than 300,000 newborns per year. Because of the origins of the sickle gene mutation as a protective mechanism against malaria for those with sickle cell trait, more than 90% of annual SCD births are in sub‐Saharan Africa (sSA). Over the past several decades, there have been many important advances in the care of individuals with SCD, including early diagnosis through newborn screening programs (NBS), prophylactic penicillin, the development of vaccines to prevent invasive bacterial infections, and the emergence of hydroxyurea as the primary disease‐modifying pharmacologic therapy. These relatively simple and inexpensive interventions have significantly reduced the morbidity and mortality of sickle cell anemia (SCA) such that individuals with SCD can live longer and more complete lives. Unfortunately, although these interventions are relatively inexpensive and evidence‐based, they are only readily available for affected individuals living in high‐income settings, representing <5% of the global SCD population. In sSA, where >90% of the global SCD burden exists, SCD still results in early mortality with 50–90% of infants likely dying before reaching 5 years of age. Recently, there are an increasing number of efforts in many African countries to prioritize SCA with pilot NBS programs, improved diagnostics, and expanded SCD education for health care professionals and the general public. It is essential to include access to hydroxyurea as a core component of any SCD care program, but there are still many barriers that prevent the widespread global use of hydroxyurea. Here, we summarize what we know about SCD and hydroxyurea use in Africa and discuss a strategy to respond to what we consider to be a public health imperative to maximize access to and appropriate use of hydroxyurea for all individuals with SCD using innovative dosing and monitoring strategies.

Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

“…However, underdeveloped countries, particularly in sub-Saharan Africa, experience a high mortality rate of 50% -80% within the first five years of life. Recognizing the severity of this issue, the WHO declared Sickle cell disease a world health problem in 2006, emphasizing the urgent need for accessible and effective treatment options worldwide [1] [2] [3].…”

Sickle Cell Disease Stories on YouTube: A Comparison of Nigeria, Ghana, Uganda, and Kenya

“…It tries to combat misinformation about the disease and recommends screening for the trait before marriage. Newborn screening raises questions about access and expense, and comprehensive care relies on access to medications, such as hydroxyurea and penicillin, which many families in the region cannot afford (Dexter & McGann, 2023). Thus, increasing awareness about sickle cell disease is one of the most viable interventions, and research examining message content, form, and platform is urgently needed.…”