Vol 75, No 1 (2024)
Original article
Published online: 2024-03-29

open access

Page views 87
Article views/downloads 51
Get Citation

Connect on Social Media

Connect on Social Media

Sickle cell disease in the Zanzibar Archipelago, the Republic of Tanzania

Wanesa Richert1, Mohammad Othman Atumani2, Daria Kołodziej1, Krzysztof Korzeniewski1
DOI: 10.5603/imh.99311
Pubmed: 38647058
IMH 2024;75(1):43-48.

Abstract

Background: Sickle cell disease (SCD) is one of the most severe haemoglobinopathies, a group of blood
disorders, typically inherited. The condition affects over 7.7 million people globally and results in more
than 370,000 deaths per year. The highest morbidity and mortality rates are seen in Africa and most children
with SCD are born in Tanzania. The available literature on SCD morbidity in Tanzania focus primarily
on the residents of the mainland, while there is little data available on SCD morbidity among residents
of the Tanzanian islands in the Indian Ocean. The aim of the present study was to confirm the presence
of sickle cell disease among residents of the Zanzibar Archipelago.

Material and methods: The study group consisted of 27 people, residents of Pemba Island in the Zanzibar
Archipelago, aged between 2 months and 26 years old, whose at least one parent has been diagnosed with
sickle cell anaemia. Blood samples collected from the study participants were tested using HemoTypeSCTM,
a rapid, point-of-care diagnostic test. The tests were performed at the Amal Hospital (Chake Chake town,
Pemba Island) in June 2023.

Results: Sickle cell disease was diagnosed in 11 study subjects (40.7%); their haemoglobin concentration
ranged between 6.6 and 8.5 g/dL. The presence of the sickle cell trait (HbAS phenotype) was confirmed
in 14 patients (51.9%). Only two of the tested patients had normal haemoglobin phenotype.

Conclusions: The results of the present study support the necessity to introduce large-scale population-
-based screening for SCD in the Zanzibar Archipelago, especially in infants whose family members have
sickle cell anaemia. The introduction of such a programme will help monitor the number of new SCD cases
in the region and may potentially reduce infant mortality due to SCD as well as minimize complications
from SCD in older children through the adoption of effective disease prevention measures.

Article available in PDF format

View PDF Download PDF file

References

  1. GBD 2021 Sickle Cell Disease Collaborators. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of Disease Study 2021. Lancet Haematol. 2023; 10(8): e585–e599.
  2. Piccin A, Murphy C, Eakins E, et al. Insight into the complex pathophysiology of sickle cell anaemia and possible treatment. Eur J Haematol. 2019; 102(4): 319–330.
  3. Tisdale JF, Thein SL, Eaton WA. Treating sickle cell anemia. Science. 2020; 367(6483): 1198–1199.
  4. Onimoe G, Rotz S. Sickle cell disease: A primary care update. Cleve Clin J Med. 2020; 87(1): 19–27.
  5. Hebbel RP, Hedlund BoE. Sickle hemoglobin oxygen affinity-shifting strategies have unequal cerebrovascular risks. Am J Hematol. 2018; 93(3): 321–325.
  6. Payne AB, Mehal JM, Chapman C, et al. Trends in sickle cell disease-related mortality in the united states, 1979 to 2017. Ann Emerg Med. 2020; 76(3S): S28–S36.
  7. Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004; 364(9442): 1343–1360.
  8. Szczeklik A, Gajewski P. Interna Szczeklika 2017. Kraków: Wydawnictwo Medycy-na Praktyczna, 2017, s. 1728–1729. ISBN 978-83-7430-517-4 [in Polish] .
  9. Piel FB, Patil AP, Howes RE, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet. 2013; 381(9861): 142–151.
  10. Detemmerman L, Olivier S, Bours V, et al. Innovative PCR without DNA extraction for African sickle cell disease diagnosis. Hematology. 2018; 23(3): 181–186.
  11. Christopher H, Josephat E, Kaywanga F, et al. Potential of point of care tests for newborn screening for sickle cell disease: Evaluation of HemotypeSC™ and sickle SCAN® in Tanzania. Int J Lab Hematol. 2022; 44(5): 959–965.
  12. Makani J. Sickle Cell Programme, Muhimbili University of Health and Allied Sciences. Curative options for sickle cell disease in africa: Approach in Tanzania. Hematol Oncol Stem Cell Ther. 2020; 13(2): 66–70.
  13. Ambrose EE, Makani J, Chami N, et al. High birth prevalence of sickle cell disease in Northwestern Tanzania. Pediatr Blood Cancer. 2018; 65(1).
  14. Musyoka AM, Zebedayo K, Mmbaga BT. Prevalence of Sickle Cell Disease Among Anaemic Children Attending Mbeya Referral Hospital in Southern Tanzania. East Afr Health Res J. 2018; 2(2): 142–146.
  15. Makani J, Cox SE, Soka D, et al. Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania. PLoS One. 2011; 6(2): e14699.
  16. Redhead D, Maliti E, Andrews JB, et al. The interdependence of relational and material wealth inequality in Pemba, Zanzibar. Philos Trans R Soc Lond B Biol Sci. 2023; 378(1883): 20220288.
  17. Ambrose EE, Smart LR, Charles M, et al. Surveillance for sickle cell disease, United Republic of Tanzania. Bull World Health Organ. 2020; 98(12): 859–868.
  18. Tluway F, Makani J. Sickle cell disease in Africa: an overview of the integrated approach to health, research, education and advocacy in Tanzania, 2004-2016. Br J Haematol. 2017; 177(6): 919–929.
  19. Grosse SD, Odame I, Atrash HK, et al. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med. 2011; 41(6 Suppl 4): S398–S405.
  20. Arishi WA, Alhadrami HA, Zourob M. Techniques for the detection of sickle cell disease: a review. Micromachines (Basel). 2021; 12(5).
  21. Mukherjee MB, Colah RB, Mehta PR, et al. Multicenter evaluation of hemotypeSC as a point-of-care sickle cell disease rapid diagnostic test for newborns and adults across India. Am J Clin Pathol. 2020; 153(1): 82–87.
  22. Yanni E, Grosse SD, Yang Q, et al. Trends in pediatric sickle cell disease-related mortality in the United States, 1983-2002. J Pediatr. 2009; 154(4): 541–545.
  23. El-Hazmi MAF, Al-Hazmi AM, Warsy AS. Sickle cell disease in Middle East Arab countries. Indian J Med Res. 2011; 134(5): 597–610.