Knowledge and Awareness of Sickle Cell Anemia: Cross Sectional Study among the General Population in Saudi Arabia

Amany  M. Khalifa; Mawaddah  F. Alhejaili; Amirah  H. Aledaili; Amani  M. Alharbi

doi:10.54393/pbmj.v5i3.328

Authors

Amany M. Khalifa Faculty of Medicine, University of Ha’il, Ha'il, Saudi Arabia
Mawaddah F. Alhejaili Faculty of Medicine, University of Ha’il, Ha'il, Saudi Arabia
Amirah H. Aledaili Faculty of Medicine, University of Ha’il, Ha'il, Saudi Arabia
Amani M. Alharbi Faculty of Medicine, University of Ha’il, Ha'il, Saudi Arabia

DOI:

https://doi.org/10.54393/pbmj.v5i3.328

Abstract

Sickle cell disease (SCD) is a hereditary blood disorder. The red blood cells are sickle in shape by polymerization of the Hemoglobin molecule caused by a β-globin gene defect that leads to Substitution of valine for glutamic acid in position 6 of the β-globin. It's an autosomal recessive inherited hematological disease. In Saudi Arabia, the prevalence of the sickle cell gene in the adult population is (4.2%) for sickle-cell trait and (0.26%) for SCD, with the highest prevalence was found in the eastern region. Consanguineous marriage gave a high incidence of the disease. The aim of the current study was to build interest to Knowledge assessment & awareness of Sickle Cell Anemia (SCA) in different regions in Saudi society and try to spread more information about it. Also, assessing the willingness of society to do premarital testing to prevent genetical diseases.Results: A total number of 400 participants filled in the present study questionnaire. The overall percentage of awareness was 60.16%

References

-Prithu sundd, Mark T. Gladwin, and Enrico M. Novelli.p pathophysiology of sickle cell disease. Annual review of pathology 2019,14: 263-292.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7053558/

- Mangla, Ankit, Moavia Ehsan, and Smita Maruvada. “Sickle cell anemia.” StatPearls [Internet], 2019

https://www.ncbi.nlm.nih.gov/books/NBK482164/

-Russell E Ware, M de Montalembert, L Tshilolo, MR Abboud. Sickle cell disease. The lancet 2017, 376(9): 848-855.

https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(17)30193-9.pdf

-Bender, Michael A., Hobbs W. “Sickle cell disease, gene reviews.” GeneReviews®[Internet] University of Washington, 2017:1993-2018.

https://www.ncbi.nlm.nih.gov/books/NBK1377/

- David C Rees. Thomas N Williams. Mark T Goadwin. Sickle-cell disease. PubMed. 2010,376(9757): 2018-31.

https://pubmed.ncbi.nlm.nih.gov/21131035/

- Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, Smith WR, Panepinto JA, Weatherall DJ, Costa FF, Vichinsky EP. Sickle cell disease. Nat Rev Dis Primers. 2018 Mar, 15(4): 18010.

https://doi.org/10.1172/JCI89741

- Ataga KI, Kutlar A, Kanter J, Liles D, Cancado R, Friedrisch J, Guthrie TH, Knight-Madden J, Alvarez OA, Gordeuk VR, Gualandro S, Colella MP, Smith WR, Rollins SA, Stocker JW, Rother RP. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017,376: 429–39. [PMC free article] [PubMed]

https://www.nejm.org/doi/10.1056/NEJMoa1611770?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200www.ncbi.nlm.nih.gov

- Nathalie Couque, Delphine Girard, Rolande Ducrocq, Priscilla Boizeau, Zinedine Haouari, Florence Missud, Laurent Holvoet, Ghislaine Ithier, Marie Belloy, Marie-Héléne Odièvre, Michel Benemou, Patricia Benhaim, Brigitte Retali, Philippe Bensaid, Brigitte Monier, Valentine Brousse, Roger Amira, Christine Orzechowski, Emmanuelle Lesprit, Laurent Mangyanda, Nathalie Garrec, Jacques Elion, Corinne Alberti, André Baruchel, Malika Benkerrou . Improvement of medical care in a cohort of newborns with sickle-cell disease in North Paris: impact of national guidelines. British Journal of Haematology 2016,173(6): 927-937.

https://onlinelibrary.wiley.com/doi/10.1111/bjh.14015

-Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med 2017,376: 1561-73.

https://www.nejm.org/doi/10.1056/NEJMra1510865

- Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014 Sep 10, 312(10): 1033-48.

https://jamanetwork.com/journals/jama/article-abstract/1902235

- Damilola Ashorobi1, Adam Ramsey, Siva Naga S. Yarrarapu, Ruchi Bhatt. Sickle Cell Trait. StatPearls. 2022

https://www.ncbi.nlm.nih.gov/books/NBK537130/

- Maha Mohammed Alotaibi. Sickle cell disease in Saudi Arabia: A challenge or not. J Epidemiol Glob Health 2017, 7(2): 99-101

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7320430/

-Wasil Jastaniah. Epidemiology of sickle cell disease in Saudi Arabia. Ann Saudi Med. 2011,31(3): 299-293.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3119971/

- Hassan M, Albagshi MH, Altaweel HA, Bushehab AA et al. “Sickle cell disease awareness among school children in Saudi Arabia.”International Journal of Medicine in Developing Countries 2019, 3 (1):001-04.

https://www.researchgate.net/publication/337533711_Sickle_cell_disease_awareness_among_school_children_in_Saudi_Arabia

- Shaikha A, Al Hajeri A. “Public awareness of sickle cell disease in Bahrain.” Annals of Saudi Medicine 2010, 30(4): 284-88.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2931779/

-Al-ghubishi S1,” Survey on Sickle Cell Disease (SCD) Awareness amongst High School Students in AlQunfudah” .Int J Med Res Health Sci 2021, 10(4): 9-18

https://www.ijmrhs.com/medical-research/survey-on-sickle-cell-disease-scd-awareness-amongst-high-school-students-in-alqunfudah-ksa.pdf

- Ibrahim NKR, Al-Bar H,, Al-Fakeeh A,, Al Ahmadi J,, Qadi M,, Al-Bar A,, Milaat W. “An educational program about premarital screening for unmarried female students in King Abdul-Aziz University, Jeddah.” Journal of Infection and Public Health, 2011, 4 (1): 30-40.

https://www.sciencedirect.com/science/article/pii/S1876034110000833

-, Maryam M, Shilbayeh S, Kabli A. “University students’ knowledge, attitudes, and practices towards the national premarital screening program of Saudi Arabia.” Journal of Egyptian Public Health Association 2017, 92(1): 36-43.

https://www.researchgate.net/publication/325125768_University_Students'_knowledge_attitudes_and_practices_towards_the_National_Premarital_Screening_Program_of_Saudi_Arabia

- Binshihon SM, Alsulami MO, Alogaibi WM, Saleh AHM, Mandourah HN, Albaity BS, Qari MH. “Knowledge and attitude toward hemoglobinopathies premarital screening program among unmarried population in western Saudi Arabia.” Saudi Medical Journal 2018; 39(12):1226- 31.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6344659/

- Alhowiti A, ,Shaqran T. “Premarital screening program knowledge and attitude among Saudi University students in Tabuk city 2019.” International Journal of Medical Research and Health Sciences 2019, 8(11): 75-84.

https://www.ijmrhs.com/medical-research/premarital-screening-program-knowledge-and-attitude-among-saudi-university-students-in-tabuk-city-2019.pdf