SickleCell Disease (SCD) is a group of haemoglobin disorder resulting from the
inheritance of the sickle β-globin gene. The homozygous state (Hb SS or sickle
cell anaemia) is the most common form of the disease, however the interaction
of the abnormal haemoglobin S (HbS) with thalassemia and other haemoglobin
variants also result in sickling.
SCDis a major public health problem and the most common genetic disease disorder
that affect mankind. The disease has a global distribution, but certain regions
of the world have high prevalence for the sickle cell gene including Equatorial
Africa, Sicily in Southern Italy, Middle East and Central India.
In
Nigeria, the magnitude of the problem is much more pronounced given that 25% of
the population carry the sickle cell gene. The World Health Organization (WHO)
estimates the prevalence of SCA to be 20 per 1000 live birth annually which
translate to about 150,000 children born annually with SCA in Nigeria, thus
making Nigeria the country with the highest disease burden globally.
The
common and most distressing clinical manifestation of SCA is the episodic and
unpredictable sickle cell vaso-occlusive crisis (VOC). The crisis occurs
unpredictably over a life-time and account for most common cause of emergency
room visits and hospitalization. The most common acute VOC is the Bone Pain
Crisis (BPC). BPC is defined as painful episodes involving one or more sites of
bones in the absence of preceding trauma or obvious infection. The most common
sites are the long bones of the limbs, lumbar spine, and thoracic cage.
Malaria bacterial and
viral infection, are the most common precipitating factors for VOC and leading
cause of mortality in SCD. SCA patients are prone to infection because of a
defect in the alternative pathway of complement activation and opsonisation reduced
ability of neutrophils to kill pathogens and functional
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