Effect of HbF Level among Different Severity of Sickle Cell Disease

Background: Fetal hemoglobin (HbF) can inhibit the deoxygenation induced polymerization of sickle hemoglobin (HbS) that drives the Pathophysiology of sickle cell disease. The aim of this study was to determine fetal Hb level in Sudanese sickle cell disease patients as well as to find out the effect of fetal hemoglobin level on different severity groups.

Materials and Methods: This was descriptive cross sectional study included 100 Patients with sickle cell disease diagnosed by Positive sickling test and Hemoglobin electrophoresis. The Patients were attended Sudan sickle cell anemia center (SSCAC), Elobied-Sudan during September 2015 – July 2016. Clinical history was obtained to perform the severity of the disease according to Hedo et al. scoring. Fetal hemoglobin was estimated by Betke's method. Data were analyzed using SPSS software computer program version 21.

Results: The mean of HbF level among the studied population was 7.6%. The descriptive analysis showed that, the mean level of HbF in 38 (38%) patients with mild disease was 7.7%, while in 54 (54%) patients with moderate disease the mean level of HbF was 7.6% and the last 8(8%) patients with severe disease showed HbF level 7%. There was no statistical significant differences observed when HbF level was less than 10% (P value = 0.146), while the statistical significant differences was observed among patients with HbF level more than10% (P value = 0.03).

Conclusion: The study concluded that Hb F level has no effect in severity of the disease among studied sickle cell patients, unless HbF level more than 10%.