Global Sickle Cell Disease Network
Small Study Group A Report from Benin Workshop
The Natural History of Sickle Cell Disease in Africa and Elsewhere; and Neonatal Screening Programs including Education, Counseling and Management.
Co-Chairs: Kwaku Ohene-Frempong (USA) and Cherif Rahimy (Benin)
While systematic screening of large populations has not been conducted in most African countries, fairly good data on prevalence of SCD exists in many countries.
Estimates of incidence: Incidence of SCD in sub-Saharan African countries ranges between 1-2%.
- Total population estimates for Africa : 855 million
- Average birth rate: 36.8/1000
- Total births: 29,632,980/year
- SCD incidence of 1%: 296,330 cases/year
- SCD incidence of 1.5%: 444,496 cases/year
- Newborn screening and screening of pregnant women have provided reliable prevalence data in several countries outside Africa. The reported prevalence rates have not changed much over the past few decades.
- New prevalence figures are probably not essential at this time, except perhaps to convince governments and other health policy makers about the need to develop programs and services
- Early diagnosis of SCD through the implementation of neonatal and early childhood screening programs
- Those not previously diagnosed should be identified in all clinical settings by incorporating Hb phenotype analysis as part of general health maintenance and clinical care.
Several categories of resources are needed:
- Human: Training of health workers through north-south and south-south partnerships.
- Technical (non-human): Commitment by countries to acquire equipment and supplies for laboratories, clinical care and data management.
- Education and Advocacy: Organize sustained educational programs for health workers as well as the general public, including incorporation of SCD education into school curricular.
- Political: Use of patient/parent support groups, advocacy organizations and national spokespersons to draw governments’ attention to SCD as a public health priority, as well as highlighting the needs of those affected.
Information for all developing countries was not available. Fairly reliable information is available for the following countries:
- National universal: Brazil (still in development)
- National, targeted: Cuba
- Pilot, universal and ongoing: Ghana
- Pilot, targeted and ongoing: Benin
- Pilot, universal, not ongoing: Burkina Faso, Burundi, Congo DRC, Guadeloupe, India, Jamaica, Rwanda, St. Lucia etc.
(Targeted means, not ALL newborns in the location are tested - only those determined by various parameters to be at risk for disease)
- Partnerships with countries with established newborn/neonatal screening programs for human and technical resource development.
- Partnership with international agencies to assist in the establishment of infrastructure for neonatal screening, follow-up and clinical management.
- Case management: Home visits by assigned public/community health nurses or other personnel for education, counseling and referral for clinical care following diagnosis.
- Primary care: Incorporation of clinical management of infants and young children with SCD into primary care programs (by training existing staff locally).
- Sickle Cell Centres: Establishment of specialized Sickle Cell Centres in regional and tertiary care institutions linked to a network of primary care centres (by training specialists locally or at other partnership sites abroad).
- Information and communication technologies (ICT): Establishment of networked database systems to link screening sites for data entry, management, monitoring and evaluation purposes, using existing systems where available.