Nigeria has the largest population of individuals with the condition globally. Reports indicate that about 25% of Nigerians carry the gene that causes it and 2% to 3% of the population suffer from sickle cell disease.
The mortality rate for children ranges from 50% to 80%. Clinical presentations vary among sickle cell patients. Some include leg ulcers, fatigue, dizziness and end organ damage.
Some centred on hydroxyurea in the management of the condition. This drug treats anaemia by helping to prevent the formation of sickle-shaped red blood cells. It has contributed towards reducing the burden of the disease globally. Furthermore, it was observed that the drug has robust efficacy and safety in the treatment of children with sickle cell anaemia.
Niprisan has also proven to be effective in the management of sickle cell disease. This intervention is classified as a phytomedicine, which refers to medicines derived from plants in their original state. Developed by Nigeria’s National Institute for Pharmaceutical Research and Development, it contributed towards reducing the number of hospital admissions among patients.
A lack of adequate knowledge about sickle cell disease in countries like Nigeria, DR Congo, Cameroon and Nigeria contributed to the high prevalence of the condition. A more informed populace will reduce the disease burden. Similarly, poor prioritisation of premarital genetic counselling and screening severely limited its utility in the prevention of the condition. In countries like Cyprus, Greece and Italy, premarital genetic testing is standard practice. However, in most of the reviewed countries, most couples were not aware of genetic counselling. Others did not know the location of facilities which offered the service.
Sickle cell related interventions are expensive. It exposes families to financial hardships as most have to seek loans to pay hospital bills and other healthcare costs. In Nigeria for example, the findings of a study showed that few participants (7.2%) were enrolled in the National Health Insurance Scheme.
Health insurance can be an invaluable tool that can help ease the financial burden associated with the management of sickle cell disease.
There is a need to increase public awareness regarding sickle cell disease. This will correct some of the negative attitudes and wrong practices such as disregard for sickle cell screening prior to marriages, as well as a willingness to marry amongst individuals with genotype incompatibility. Campaigns should be targeted at correcting misconceptions that the disease is a death warrant and contagious.
People should know it is possible to live a long life with proper healthcare after diagnosis.
A Guest Editorial