A Pilot Study of Sickle Cell Anemia in the Belizean Population

Background: Sickle Cell Anemia (Sickle Cell Disease) is an inherited disease resulting from a genetic mutation in the hemoglobin protein found in red blood cells. Screening is a requirement in the United States and in U.S. territories, and is considered a component of routine prenatal care. In resource limited countries such as Belize, there are no screening programs in place for testing newborns for Sickle Cell Anemia, and the disease is largely undefined. Children often go undiagnosed until they exhibit clinical symptoms. Untreated Sickle Cell Anemia in children under the age of 5 can lead to poor clinical outcomes. Oftentimes, those suspected of having the disease obtain diagnostic testing from a foreign country, and the test frequently used is the Sickle Prep Test. While the Sickle Prep Test is able to identify the presence of hemoglobin mutations, it cannot differentiate between sickle cell disease and sickle cell trait, a distinction which has profound clinical implications. A new technology, the Sickle SCAN test, utilizes a chromatographic, immunologic approach to qualitatively identify the presence of select variants of the hemoglobin protein (HbS, HbA, HbC), thereby allowing clinicians to differentiate between sickle cell disease and sickle cell trait. This test is a powerful tool that it is cost effective and timely; it does not require complicated testing equipment, and can disclose one's hemoglobin genetic profile in under 6 minutes. Methods: 87 patients were enrolled and tested for sickle cell disease and sickle cell trait in the towns of Dangriga and Hopkins, Belize. Testing took place over the course of 4 days. A survey was administered to collect key demographic information and information regarding participant's knowledge about Sickle Cell Anemia, past diagnosis, and family history of disease. Results: 13% were found to have sickle cell disease (n=12), and 25% were found to have the trait (n=22). For those with the disease or trait, the majority were found to belong to the Garifuna ethnic group. 26% of the participants had no knowledge of Sickle Cell Anemia. It was also reported that 51% had a family member with the disease, and 23% were unsure whether a family member had the disease.

Conclusions: This pilot study demonstrates the feasibility ofusing the Sickle SCAN test to gather knowledge about the prevalence of Sickle Cell Anemia in a population where the disease remains largely undocumented.