Extremely high birth prevalence of sickle cell disease in rural Tanzania

Pediatr Blood Cancer. 2020 Nov;67(11):e28620. doi: 10.1002/pbc.28620. Epub 2020 Aug 16.


Background/objectives: Sickle cell disease (SCD) is an important, hidden cause of childhood mortality worldwide. It is most prevalent in sub-Saharan Africa where national newborn screening programs remain unavailable and most children in rural areas are never diagnosed. We conducted a study at a rural district hospital in northern Tanzania to determine the birth prevalence and community awareness of SCD and to determine the feasibility of using point-of-care testing to enroll newborns in a new SCD clinic for ongoing treatment.

Design/methods: We screened infants at Shirati KMT hospital for SCD using HemoTypeSC, an inexpensive point-of-care test. Infants who screened positive were enrolled in the SCD clinic and instructed to return at 6-12 weeks for confirmatory testing, counseling, and preventive care.

Results: A total of 999 newborns were screened from February to September 2019. Among these, 31.6% (315/999) had sickle cell trait and 3.9% (39/999) had SCD. No hemoglobin C was detected. Very few parents knew their own sickle cell status (0.3%). At 5 months after completion, 12 infants from the screening study and 30 additional children had been seen at the SCD clinic for ongoing counseling and care.

Conclusions: Birth prevalence of SCD in rural Tanzania is extremely high and community awareness is low. Newborn point-of-care testing enhances case finding and enables early enrollment in preventive care for SCD, even in rural sub-Saharan Africa with minimal laboratory capacity. SCD-specific clinical services implemented at the district hospital level could expand access to many children and significantly reduce early SCD morbidity and mortality.

Keywords: linkage-to-care; newborn screening; prevalence; sickle cell disease; sub-Saharan Africa.

MeSH terms

  • Anemia, Sickle Cell / diagnosis
  • Anemia, Sickle Cell / epidemiology*
  • Female
  • Follow-Up Studies
  • Humans
  • Infant, Newborn
  • Male
  • Neonatal Screening / methods*
  • Prevalence
  • Prognosis
  • Prospective Studies
  • Rural Population / statistics & numerical data*
  • Sickle Cell Trait / diagnosis
  • Sickle Cell Trait / epidemiology*
  • Tanzania / epidemiology