Sickle cell disease is one of the most common public health problems in the world today. It affects 25 percent of the world’s population, more than 1.6 billion people (Benoist et al. 2008). The Ministry of Health for the last two decades has focused on the prevention and treatment of diseases like malaria, HIV/AIDS and the provision of child immunization against measles and polio. Sickle cell disease in Uganda is not among the health conditions which have been prioritized in terms of resource allocation and it has remained a major silent killer.
Sickle cell is an inherited condition which changes the normal shape of the red blood cells. The red colour of blood is made up of a pigment called haemoglobin which carries oxygen with protein to the body tissues for normal function and removes carbon dioxide. The modified haemoglobin causes round blood cells to become stiff, sticky and sickle shaped. The deformed cells can block blood flow, causing severe pain, organ damage and stroke.
How many people suffer from Sickle Cell Disease?
In Uganda, the disease has been a major public health problem for many years, according to data from the 2001 Uganda Demographic and Health Survey (UDHS) 71% of children under five and 37% of women of reproductive age (WRA) were anemic. Five years later, these rates increased to 73% and 42%, respectively (Uganda Bureau of Statistics and ICF International, Inc. 2007). In 2015 the Uganda Sickle Cell surveillance study (US3) was conducted and it indicated that the country still has a big number of sickle cell patients.
The study revealed that districts from the northern region had the highest prevalence of sickle cell trait amounting to 18.6%. The East-Central region was second at 16.7%, followed by Mid-Eastern Uganda with 16.5% and South-Western region had the lowest at 4.1%. During the study it was discovered that out of 23, 000, children who were tested from different regions, 2,800 tested positive for sickle cell gene which amounts to 12% of the tested sample. This implies that one in every ten babies born in Uganda carry the gene.
In infants signs of sickle cells start to emerge as early as six months, the common signs include swelling body parts especially the hands and feet during the first year of life. Patients with sickle cells have low blood levels or anemia, yellow eyes especially when dehydrated and experience episodes of pain when sickle cell block blood flow and decrease oxygen delivery. In adults, many have ulcers and bone infections and in men they can experience uncontrolled painful erection.
Though the number of children born with sickle cell gene in the country is high, only 7,000 patients are registered with the Sickle Cell Clinic at Mulago National Referral Hospital. On average the clinic receives 200 patients over five days according to the information from the Sickle Cell Association of Uganda. Mulago Hospital has the only sickle cell clinic and it is a day care centre operating from Monday to Friday.
Malaria and Sickle Cell Disease
Malaria is widely considered to be one of the major causes of illness and death in patients living with sickle cell disease in Uganda. This has created a need to scale up malaria control, prevention and treatment interventions. Results from the Uganda Sickle Cell surveillance study revealed that the Northern region which had the highest prevalence of sickle cell also had the highest prevalence of malaria at 63% and the south western region which had the lowest prevalence of sickle cells also had the lowest prevalence of malaria at 13%. This implies that interventions to prevent and treat malaria can reduce and control the increasing number of children born with sickle cell genre and also improve the lives of sickle cell patients. The government needs to revise and improve strategies to fight malaria especially in the Northern and East-Central regions where prevalence of malaria is still high.
Tackling Sickle Cell Disease
Awareness about sickle cell disease is still low especially in rural areas where most of the patients live, there is need for government and non-government organizations to come up with countywide programs to create awareness on how sickle cells can be prevented, encourage couples to go for genetic testing, highlight the importance of newborn screening and available treatment options in case the baby is tested positive. This will reduce the number of children born with sickle cells and also increase the number of patients seeking for treatment.
Interventions that can help to reduce and control sickle cells are genetic counseling and new born screening services for early detection, prevention and provision of vaccines, clinical care for children and pregnant women and also capacity building of human resource to improve quality of life of sickle cell patients. These interventions should be made available in lower health centres which most Ugandans have access to. Health centres should be equipped with enough pharmaceuticals and trained human resource to respond to the needs of sickle cell patients. For patients with sickle cell diseases to live a better life, interventions that improve survival, prevent complications, treat acute events, and reduce end organ damage must be made available at all times especially in lower health centres.
Prevention and control strategies including encouraging Ugandans to sleep in insecticide-treated mosquito bed nets should be scaled up since it has been established that there is a direct relationship between sickle cells and malaria. Also supplementation of women and children with iron and vitamin A, deworming, food fortification particularly vitamin A-fortified oil Comprehensive care programs for sickle cell patients especially infants should be introduced at lower health centres to prevent complications which negatively affect their survival and quality of life.
Finally, strong political commitment and continued investment in strategies to reduce the disease are needed to help patient/parent support groups and advocacy organizations scale up countrywide interventions and draw government’s attention to sickle cell disease as a public health priority. There is greater need for proactive measures and collaboration on the part of the government and its partners to effectively implement population screening and counseling programs as a prevention strategy is much more economical than spending on managing the burden of sickle cell disease in children.
By Ssekajoolo Mathew,
The writer is a social worker and advocate for health equity and social justice.