Sickle cell disease and the adherence to guidelines for the use of blood transfusions in Duhok, Kurdistan, Iraq

Authors

  • Fahad Abdulwahab Jameel Department of Hematology, Azadi Teaching Hospital, Duhok, Iraq.
  • Adnan Anwer Sadeeq Department of Pathology, College of Medicine, University of Duhok, Duhok, Iraq.

DOI:

https://doi.org/10.15218/zjms.2021.011

Keywords:

Transfusion guidelines for sickle cell disease (BCSH 2017), Sickle cell disease, Iraq

Abstract

Background and objective: One of the important health problems in Duhok city, Iraqi Kurdistan Region, is sickle cell disease. Blood transfusion remains a significant therapeutic intervention in patients with sickle cell disease that reduces complications related to vaso-occlusions. This study aimed to assess compliance to guidelines for the use of blood transfusionsin Duhok, Kurdistan, Iraq.

Methods: This is a cross-sectional study that included 135 patients with sickle cell disease registered at Jeen center of pediatric hematological diseases in Duhok, Kurdistan, Iraq. Between April 1st and July 31st, 2019, 205 medical visits of sickle cell disease were registered. Every patient was evaluated to record the clinical setting and explanations behind visiting and indications for transfusion.

Results: Of 135 patients,65.9% had sickle cell anemia (HbSS disease), 33.3% had sickle beta thalassemia, and one patient (0.7%) had Sickle/D disease. A total of 205 medical visits of sickle cell disease were registered with 84 blood transfusion decisions. The most common indicated guideline reasons for transfusion were symptomatic anemia and acute hemolytic crisis with a drop of hemoglobin >2 g/dl below steady state hemoglobin and severe painful crisis only accounted for 38.1%.

Conclusion: In this study of patients with sickle cell disease, most blood transfusions were not indicated according to the transfusion guidelines (British Committee for Standards in Haematology – BCSH, 2017).

Metrics

Metrics Loading ...

References

Stuart MJ, Nagel RL. Sickle-cell disease. Lancet 2004; 364(9442):1343–6.

Ohene-Frempong K. Indications for red cell transfusion in sickle cell disease. J Hematol Oncol 2001; 38(S1):5–13.

Mehta S, Afenyi-Annan A, Byrns P, Lottenberg R. Opportunities to improve outcomes in sickle cell disease. Am Fam Physician 2006; 74:303–10.

Strouse J J, Heeney M M. Hydroxyurea for the treatment of sickle cell disease: efficacy, barriers, toxicity, and management in children. Pediatr Blood Cancer 2012; 59(2):365–71.

Eissa A, Markous R, Yahya N, Al-Allawi N. Hemoglobin F modulation in sickle cell disease: Experience in a single center in Iraqi Kurdistan. J Appl Hematol 2016; 7:85.

Halsey C, Roberts IAG. The role of hydroxyurea in sickle cell disease. Br J Haematol 2003; 120(2):177–86.

Kohne E. Hemoglobinopathies: clinical manifestations, diagnosis, and treatment. Dtsch Arztebl Int 2011; 108(31-32):532–40.

Davis BA, Allard S, Qureshi A, Porter JB, Pancham S, Win N, et al. Guidelines on red cell transfusion in sickle cell disease Part II: indications for transfusion. Br J Haematol 2017; 176(2):192–9.

Kaur M, Dangi CBS, Singh M. An overview on sickle cell disease profile. Asian J Pharm Clin Res 2013; 6(Suppl 1):25–37.

Bender MA, Hobbs W. Sickle cell disease. 2003 Sep 15 [updated 2012 May 17]. In: Pagon RA, Adam MP, Ardinger HH, Bird TD, Dolan CR, Fong CT, et al., editors. Gene Reviews. Seattle (WA): University of Washington, Seattle; 1993–2014.

Field JJ, Vichinsky E. Overview of the management and prognosis of sickle cell disease.31st ed. United States: Uptodate; 2014; 122–3.

Townes TM. Gene replacement therapy for sickle cell disease and other blood disorders. Hematology 2008; 2008(1):193–6.

Shenoy S. Hematopoietic stem cell transplantation for sickle cell disease: Current practice and emerging trends. Hematology 2011; 273–9.

Driss A, Asare KO, Hibbert JM, Gee BE, Adamkiewicz TV, Stiles JK. Sickle cell disease in the post genomic era: A monogenic disease with a polygenic phenotype. Genomics Insights 2009; 23–8.

Schnog J, Duits AJ, Muskiet F, Tencate H, Rojer RA, Brandjes D. Sickle cell disease a general overview. Neth J Med 2004; 62:364–74.

Elliott PV, Michael RD, Jennifer ST. Overview of the clinical manifestations of sickle cell diease. Uptodate 2014.

Ballas SK, Kesen MR, Goldberg MF, Lutty GA, Dampier C, Osunkwo I, et al. Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management. Scientific World Journal 2012; 949535.

Kelly S, Deng X, Hoppe C, Styles L. A pilot randomized trial of red blood cell transfusion for acute treatment of vaso-occlusive pain episodes in sickle cell anaemia. Br J Haematol 2015; 171(2):288–90.

Al Jaouni S, Almuhayawi M, Halawa T, Mehayawi M. Treatment adherence and quality of life outcomes in patients with sickle cell disease. Saudi Med J 2013; 34:261–5.

Josephson CD, Su LL, Hillyer KL, Hillyer CD. Transfusion in the Patient With Sickle Cell Disease: A Critical Review of the Literature and Transfusion Guidelines. Transfusion Med Rev 2007; 21(2):118–33.

Lionnet F, Arlet JB, Bartolucci P, Habibi A, Ribeil JA, Stankovic K. Recommandations pratiques de prise en charge de la drépanocytose de l’adulte. La Revu. de Méd Inte 2009; 30(S1):162–223.

Telen M. Principles and problems of transfusion in sickle cell disease. J Hematol Oncol 2001; 38:315–23.

Niscola P, Sorrentino F, Scaramucci L, De Fabritiis P, Cianciulli P. Pain syndromes in sickle cell disease: An update. Pain Medicine 2009; 10(3):470–8.

Houston-Yu P, Rana SR, Beyer B, Castro O. Frequent and prolonged hospitalizations: A risk factor for early mortality in sickle cell disease patients. Am J Hematol 2003; 72(3):201–3.

Ballas S K, Lusardi M. Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. Am J Hematol 2005; 79(1):17–25.

DeBaun MR, Gordon M, McKinstry R C, Noetzel MJ, White DA, Sarnaik SA, et al. Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med 2014; 371(8):699–710.

Steinberg MH, McCarthy WF, Castro O, Ballas SK, Armstrong FD, Smith W, et al. The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up. Am J Hematol 2010; 85(6):403–8.

Thornburg C D, Calatroni A, Telen M, Kemper A R. Adherence to hydroxyurea therapy in children with sickle cell anemia. J Pediatr 2010; 156 (3):415–9.

Al Hawsawi ZM, Ahmed Turkistani W. Effect of hydroxyurea in children with sickle cell disease in Saudi Arabia. J Taibah Univ Sci 2008; 3(2):129–34.

Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia risks and benefits up to 9 years of treatment. JAMA 2003; 289(13):1645–51.

Akinbami A, Dosunmu A, Adediran A, Oshinaike O, Phillip A, Vincent O, et al. Steady state hemoglobin concentration and packed cell volume in homozygous sickle cell disease patients in Lagos, Nigeria. Caspian J Intern Med 2012; 3(2):405–9.

Park DD, Jacob DG, Adedemola A, Mowatt K, Persaud T, Tipirneni A, et al. Sickle cell disease and the adherence to guidelines for use of blood transfusions. Blood 2017; 130:4652.

Downloads

Published

2021-04-27

How to Cite

Jameel, F. A., & Sadeeq, A. A. (2021). Sickle cell disease and the adherence to guidelines for the use of blood transfusions in Duhok, Kurdistan, Iraq. Zanco Journal of Medical Sciences (Zanco J Med Sci), 25(1), 486–492. https://doi.org/10.15218/zjms.2021.011

Issue

Section

Original Articles