On 19th June, Uganda joins the rest of the world to celebrate the World Sickle Cell disease. Sickle cell disease is a major public problem especially in Sub-Saharan Africa.
In Uganda 15,000 to 20,000 babies are born with sickle cell disease every year. Unfortunately, 80% of them die before their 5th birthday.
However, Uganda has made tremendous efforts towards the management and treatment of the disease as a means to rid the country of the burden.
Uganda has also been the first country in Africa to come out with national prevalence data, which has also been published in leading journal -The Lancet.
In 2014, the Ministry of Health carried out a survey to establish the prevalence of Sickle Cell trait and sickle cell disease across the country, which documented a high sickle cell burden, with a national trait average of 13.3%, and disease burden of 0.73%.
In some high burden districts, the prevalence of the trait is above 20% and that of the disease above 1.5%.
As a follow up, Uganda started targeted newborn screening in the high burden districts of Gulu, Lira, Kitgum, Dokolo, Oyam, Tororo, Jinja and Kampala, covering a total of 274 health facilities.
So far over 50,000 newborn babies have been tested. Health workers in these facilities have been trained in sickle cell management and sickle cell clinics have been opened up.
According to Prof. Mbonye, these clinics are stocked with prophylactic drugs like penicillin, anti-malarials, and folic acid which are key in managing the diseases.
‘ ‘This has been complemented by mass sensitization campaigns to create more awareness about the disease amongst communities. Mass screening campaigns and counseling have also been conducted in several districts. Pre-marital counseling and testing is being promoted in collaboration with religious leaders.’’
The Ministry of health also plans to scale up newborn screening services to more high burden districts and introduce hydroxyurea, which is a disease transforming drug. Some children are on it privately and are doing very well.
Prof. Mbonye says the Ministry also plans to implement a policy to administer pneumococcal vaccine to children with sickle cell disease above two years to protect them from recurrent infections.
These developments, coupled with Uganda’s efforts towards the building and strengthening of sickle cell research and clinical capacity, encouraged REDAC -a network dedicated to combating Sickle Cell disease across sub-Saharan Africa–to nominate Uganda as a host of the 6th International Symposium on Sickle Cell Disease.
The highly successful conference brought together distinguished scientists, clinicians, policy makers, researchers, advocacy groups, technology and pharmaceutical companies. The conference has raised the profile of sickle cell disease in Uganda.
In Uganda, the World Sickle Cell Day will be held in Bundibugyo. The district is one of the high burden districts with a prevalence of 21.7% of trait and 1.9% of disease. Surprisingly all districts around Bundibugyo apart from Ntoroko with a trait prevalence of 15%, the rest of the districts around it have a prevalence of trait of >10%.
Bundibugyo has always been known to have a high burden of sickle cell disease, since a study conducted in 1949 put the prevalence of trait among the Bamba in Bundibugyo at 45%!
The recent US3 study was quite representative of the country, but still put Bundibugyo among the high burden districts, quite different from the districts around it.
All other factors remaining constant of course, what promotes increased spread of sickle cell trait and disease amongst communities is increased intermarriages among carriers without knowledge, due to lack of premarital sickle cell testing and counseling.
Prof. Mbonye says if we are to reduce the incidence of sickle cell disease in Bundibugyo and any other part of the country, premarital sickle cell counseling and testing must be encouraged. For this to be done effectively, communities must be adequately sensitized, and several stakeholders involved especially religious, cultural and political leaders.
Patient management early testing and patient identification has been identified as one of the interventions for Sickle cell management. The Ministry of Health has started targeted new born screening in high burden districts, coupled with setting up of sickle cell clinics.
Though not curable, (except through very expensive and risky procedures like born marrow transplant), sickle cell disease is preventable through premarital testing and counseling and is also manageable through early patient identification and management.