Abstract:
Background: Foetal haemoglobin has been implicated
in the modulation of sickle cell crisis. Its level is generally
inversely proportional to the severity of sickle cell
disease (SCD) for a given sickle cell phenotypes. The
main aim of therapy for vaso-occlusive crisis (VOC),
which is the hallmark of SCD, is to reduce the chances
of sickling through the prevention of polymerization of
HbS. One way of preventing this polymerization is by
increasing foetal haemoglobin levels.
Objectives: To determine the relationship between
HbF levels and the frequency of crisis in SCD patients
in Ghana.
Method: A longitudinal retrospective survey covering
a period of 30 months was carried out on adult SCD
patients at the Sickle Cell Clinic of the Korle-Bu
Teaching Hospital.
Results: Eighty-three adults aged 15 to 65 years made
up of 40 males and 43 femalea were studied. Analysis
of variance (ANOVA) gave significant results in Hb
and HbF levels. Higher HbF levels were positively
related to less frequent crisis and were significantly
high in SCD patients than in controls. HbF effects on
the clinical manifestations on SCD were variable.
Conclusion: Threshold values of HbF play a role in
reducing the frequency of vaso-occlusive crisis in SCD
patients and this finding contributes to the body of
available literature on SCD severity. However our
work does not give the apparent threshold level of
helpful HBF Level in SCD.