Today, June 19th marks the World Sickle Cell Day, a day dedicated to raising awareness about sickle cell disease (SCD), a genetic blood disorder that affects millions of people around the world. The name sickle cell comes from the fact that the red blood cells of individuals with SCD become "sickle" shaped or crescent-shaped rather than the normal round shape.
Sickle cell disease is a significant health concern in Nigeria, as it has one of the highest burdens of the disease in the world. The incidence of SCD in Nigeria is very high the country has the highest number of new-borns with sickle cell disease in the world, accounting for about 150,000 births per year (World Health Organization, 2021). The disease cut across ethnic groups, affecting Yorubas, Igbos, and Hausas. It is estimated that about 25% to 30% of Nigerians carry the sickle cell trait, which increases the risk of having children with sickle cell disease when both parents are carriers. Sickle cell disease affects the quality of life of individuals and is associated with a reduced lifespan.
The high incidence of SCD in Nigeria is attributed to a combination of factors, including cultural practices, lack of awareness, limited access to healthcare services, and insufficient genetic counselling. It is important that the public are educated about this disease that will result in the reduction of the suffering and impact of sickle cell disease. Early diagnosis and comprehensive care are crucial to improving the quality of life for individuals with SCD in Nigeria.
People living with sickle cell disease go through a lot of misery especially when the abnormal sickle cell causes blockage of the blood flow, leading to severe pain, damage to organ such as the lungs, kidneys, liver, and spleen. This can lead to serious complications such as infections, stroke, pulmonary hypertension, and kidney failure. It results in multiple visits to hospitals when patients have the sickle cell crisis and the excruciating pain can last for hours or days. Individuals living with sickle cell may have characteristics facial features and when jaundiced, have yellow discolouration of their eyes due to the breakdown of red blood cells, and when anaemic, are pale. Children with SCD may exhibit delayed growth and development, which can affect facial proportions relative to their age, prominent cheekbones, and sunken appearance of the cheeks.
The diagnosis of SCD is confirmed through laboratory tests that detect the presence of abnormal haemoglobin, specifically haemoglobin S (HbS). Sickle cell disease patients have both SS genes (homozygous) and sickle cell carriers have a single S (heterozygous). It is important that patients are under the care of a doctor from childhood and continue receiving care as they grow into adulthood. Historically, treatment options for SCD have been limited. However, advancements in medical research have led to the development of new therapies, including gene therapy and targeted drugs such as hydroxyurea, offering hope for better management and potential cures. The cost of treatment makes it not easily affordable by patients in Nigeria and the multiple hospitalisations cause a lot of financial hardships. We should take steps through awareness and educational activities to eliminate the stigma associated with sickle cell disease.
Patients now live well into adulthood and old age, in good health, with proper healthcare.
The theme for 2023 World Sickle Cell Day is Empowering Change and Building Hope. One hopes that that the Nigerian government will invest in resources for screening, diagnosis, treatment and psychological support for patients and research into the disease. Patients with sickle cell deserve an improved quality of life.