Sickle cell disease (SCD) is a genetic blood disorder affecting red blood cells, with high morbidity and mortality rates. Sickle haemoglobin (HbS) is a structural variant of normal adult haemoglobin (HbA) (Chakravorty & Williams, 2015). SCD includes a series of pathological genotypes resulting from the inheritance of HbS. SCD affects 20–25 million people globally, and 50–80% of infants born with SCD in Africa die before the age of 5 years (Aygun & Odame, 2012). It is estimated that 240,000 children are born with SCD annually in sub-Saharan Africa (Makani et al., 2011) and with about 100,000 babies born with SCD living in Nigeria, according to the Centre for Disease Control (CDC).
In Lagos State University Teaching Hospital, Ikeja, a study conducted in the adult clinic for SCD shows age range of 14-42 years with mean age of 22±6 years as the mean age for the participant. (Dosunmu et al 2014).
The United Nations General Assembly has recognized SCD as a global public health concern due to the morbidity and mortality caused by the disease and the significant social and economic impact that results (United Nations General Assembly, 2009).
The incidence of the Sickle Cell Trait in Cameroon, the Democratic Republic of Congo, Gabon, Ghana, and Nigeria ranges from 20% to 30%, and in some parts of Uganda, the prevalence is 45% (Afolayan & Jolayemi, 2011; Agasa et al., 2010; Anie, Egunjobi, & Akinyanju, 2010; Serjeant & Ndugwa, 2004; World Health Organization, 2006). Chakravorty and Williams (2015) suggested that there are few places where the carrier rate for SCD is greater than 25% because of the disadvantages conferred by homozygosity.
The disease process in SCD causes complications in multiple body organs. Some of the most common morbidities associated with SCD include chronic pain and intermittent painful episodes, musculoskeletal problems, stroke, pulmonary hypertension, and septicemia. These complications often co-exist, affecting the quality of life for patients, and if untreated, they may lead to death.
Many children with SCD experience musculoskeletal complications due to avascular necrosis, osteomyelitis, and septic arthritis. Balogun et al. (2010) published findings in 2004 from a study of 318 SCD patients in Nigeria. Study participants ranged from 1 to 45 years of age, and 46% below age 10. These authors noted that children below age 10 years were more likely to have multiple musculoskeletal problems, and reported that among the children younger than 10 years in this study, 95% had septic arthritis, 63.3% had osteomyelitis, and 7.2% had avascular necrosis of the femoral head. Among children between 11 and 20 years of age, 46.4% had avascular necrosis of the femoral head, 30.6% had osteomyelitis, and 5% had septic arthritis.
Effective management of SCD revolves around genetic counselling, neonatal screening and early diagnosis; prophylaxis with immunizations; providing anti-malarial medications, antibiotics, and hydroxyurea; and prompt management of complications. Bone marrow transplantation in a selected segment of patients is the only proven cure for SCD to date (Walters et al., 2001), but this is an expensive treatment and in 2004 Sergeant and Ndugwa noted that this option is not feasible as a public health approach in low resource countries (Serjeant & Ndugwa, 2004).
In 2009, Abioye-Kuteyi et al., reported results of a survey of 320 government workers in Nigeria that though about 69% of intending couples had poor knowledge about SCD, 95% had positive attitudes towards premarital screening.
As noted by Serjeant in 2005 “We cannot yet cure sickle cell disease, but we have learnt that simple interventions significantly improve morbidity and mortality.
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