HEALTH-RELATED QUALITY OF LIFE IN SICKLE CELL DISEASE PATIENTS IN AKRE, IRAQI KURDISTAN
Keywords:
Health-related Quality of Life, HRQoL, Iraq, Sickle cell disease, SF-36, PedsQL
Abstract
https://doi.org/10.31386/dmj.2019.12.1.4
Background: Sickle cell disease is an important health problem in Akre- Iraqi Kurdistan, and this study aimed to assess health-related quality of life (HRQoL) in this condition.
Patients and Methods: A total of 63 patients registered as sickle cell disease at Akre thalassemia center in Kurdistan, and an equivalent number of age and sex matched healthy individuals, were recruited. The patient records were retrieved and their clinical and laboratory findings reviewed. Children and adolescent had their HRQoL assessed using PedsQL 4.0 generic score, while the Medical outcome study 36 item short form (SF-36) was used for adults.
Results: The mean age ± SD of the enrolled patients was 15.43 ± 8.79 years, and included 44 females and 19 males. The overall mean HRQoL in both children and adults were significantly worse than respective healthy counterparts, and adults fared worse than children. Among the predictors of low quality life were the occurrence of painful episodes, transfusion frequency, S. Ferritin ≥ 2500 ng/ml , LDH ≥ 700 U/L, and avascular necrosis of femoral head (P= 0.02 , 0.007 , 0.012 , 0.025 and 0.002 respectively).
Conclusions: Both children and adults with sickle cell disease at Akre thalassemia center had worse quality of life compared to their healthy counterparts. Several predictors for a worse HRQoL were identified, in the studied cohort. Further studies from other centers are necessary to have a more comprehensive view of the HRQoL in Iraqi Kurdistan.
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References
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24. Panepinto JA, Beverung LM., Varni JW. Clinically meaningful interpretation of pediatric healthrelated quality of life in sickle cell disease. J Pediatr Hematol/Oncol. 2015; 37(2): 128-133.
25. Bhatia M, Kolva E, Cimini L, Jin Z, Satwani P, Savone M, et al. Health-related quality of life after allogeneic hematopoietic stem cell transplantation for sickle cell disease. Biol Blood Marrow Transplant. 2015; 21(4): 666-672.
26. McClish DK, Penberthy LT, Bovbjerg VE, Roberts JD; Aisiku IP, Levenson JL, et al. Health related quality of life in sickle cell patients: The PiSCES project. Health Qual Life Outcomes. 2005; 3(50):1-7.
27. Schlenz AM, Schatz J, McClellan CB, Roberts CW. Responsiveness of the PedsQL to pain-related changes in health-related quality of life in pediatric sickle cell disease. J Pediatr Psychol. 2012; 37(7): 798-807.
28. Anie KA, Steptoe A, Bevan D. Sickle cell disease: Pain, coping and quality of life in a study of adults in the UK. Br J of Health Psychol. 2002; 7(3): 331-344.
29. Martínez-Triana R, MartínezRodríguez M, Guerra-Romero C, Guerra-González EM, MachadoAlmeida T, Machín-García S, et al. Cuestionario para evaluar la calidad de vida relacionada con la salud de adultos con drepanocitosis. Rev Cuba Hematol Inmunol Hemoter. 2012; 28:385-97. [abst]
30. van Tuijn CF, van Beers EJ, Schnog JJ, Biemond BJ. Pain rate and social circumstances rather than cumulative organ damage determine the quality of life in adults with sickle cell disease. Am J Hematol. 2010;85(7):532-5.
31. Pereira SAS, Brener S, Cardoso CS, Proietti ABFC. Sickle Cell Disease: quality of life in patients with hemoglobin SS and SC disorders. Rev Bras Hematol Hemoter. 2013; 35(5): 325-31.
32. Al-Allawi N, Qadir SMA ,Puehringer H, Chui DHK, Farrell JJ, Oberkanins C. The association of HBG2, BCL11A, and HMIP polymorphisms with fetal hemoglobin and clinical phenotype in Iraqi Kurds with sickle cell disease. Int. J Lab Hematol. 2019 ;41(1):87-93
2. Sehlo MG, Kamfar HZ. Depression and quality of life in children with sickle cell disease: effect of social support. BMC Psychiatry. 2015; 15: 78.
3. Hays RD, Morales LS. The RAND-36 measure of health related quality of life. Ann Med. 2001; 33:350-357.
4. Varni JW, Seid M, Kurtin PS. PedsQL™ 4.0: Reliability and validity of the Pediatric Quality of Life Inventory™ Version 4.0 Generic Core Scales in healthy and patient populations. Med Care. 2001; 39(8):800-812.
5. Panepinto JA. Health-related quality of life in patients with hemoglobinopathies. ASH Education Program Book. 2012; 2012(1): 284289.
6. https:// www.rand.org/healthcare/surveys_tools/mos-F36-itemshort-form accessed on 23rd December 2018.
7. Ware JE Jr, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. conceptual framework and item selection. Med Care. 1992; 30:473-483.
8. Mikael NA, Al-Allawi NAS. Factors affecting the quality of life in children and adolescents with thalassemia in Iraqi Kurdistan. Saudi Med J. 2018; 39 (8): 799-807
9. Menezes AS, Len CA, Hilário MO, Terreri MT, Braga JA. Quality of life
in patients with sickle cell disease. Rev Paul Pediatr. 2013; 31(1): 24-29.
10. Panepinto JA, O'mahar KM, DeBaun MR, Loberiza FR, Scott JP. Healthrelated quality of life in children with sickle cell disease: child and parent perception. Brit J Haematol. 2005; 130(3): 437-444.
11. Varni JW, Burwinkle TM, Seid M, Skarr D. The PedsQL™* 4.0 as a pediatric population health measure: Feasibility, reliability, and validity. Ambul Pediatr. 2003; 3(6): 329–341.
12. Dale JC, Cochran CJ, Roy L, Jernigan E, Buchanan GR. Health-related quality of life in children and adolescents with sickle cell disease. J Pediatr Health Care. 2011; 25(4): 208215.
13. Rijken M, van Kerkhof M, Dekker J, Schellevis FG. Comorbidity of chronic diseases: effect of disease pairs on physical and mental functioning. Qual Life Res. 2005; 14:45-55.
14. Alonso J, Ferrer M, Gandek B, Ware JE Jr, Aaronson K, Mosconi P, et al. Health-related quality of life associated with chronic conditions in eight countries: results from the international quality of life assessment (IQOLA) project. Qual Life Res. 2004; 13: 283-298.
15. Amr MA, Amin TT, Al-Omair OA. Health related quality of life among adolescents with sickle cell disease in Saudi Arabia. Pan African Medical Journal. 2011; 8:10.
16. Matos MA, Malheiros CD, Matos SSR. Health Related Quality of Life of Patients .with Sickle Cell Disease. J
Blood Disord Transfus. 2016; 7(2): 345-348.
17. Ojelabi AO, Graham Y, Ling J. Health-related Quality of Life Predictors in Children and Adolescents with Sickle Cell Disease: A Systematic Review. Int. J Trop Dis Hlth. 2017; 22(2): 1-14.
18. Dampier C, LeBeau P, Rhee S, Lieff S, Kesler K, Ballas S, et al. Healthrelated quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium. Am J Hematol. 2011;86(2):203-5.
19. Ahmed AE, Alaskar AS, Al-Suliman AM, Jazieh AR, McClish DK, Al Salamah M, et al. Health-related quality of life in patients with sickle cell disease in Saudi Arabia. Health Qual Life Outcomes. 2015;13:183.
20. Ahmed AE, Alaskar AS, McClish DK, Ali YZ, Malhan HM, Aldughither MH, et al. SCD in Saudi children, teens, and adults: A study in healthrelated quality of life. J blood Disord. 2016; 3 (1):1036.
21. Freitas SLF, Ivo ML, Figueiredo MS, Gerk MAS, Nunes CB, Monteiro FF. Quality of life in adults with sickle cell disease: an integrative review of the literature. Revista Brasileira de Enfermagem. 2018; 71(1), 195-205
22. Nwogoh B, Ofovwe CE, Omati CE. Health-related quality of life in Sickle cell disease subjects in Benin city, Nigeria. Afr. J. Med Health Sci. 2016; 15: 80-85.
23. Hassan MK. Health Related Quality of Life (HRQoL) of Patients with Thalassemia and Sickle Cell Disease and the Impact of these Diseases on Families. Proceedings of the Academy for sickle cell and thalassaemia conference (ASCAT), 2016 Oct 5-7; London.
24. Panepinto JA, Beverung LM., Varni JW. Clinically meaningful interpretation of pediatric healthrelated quality of life in sickle cell disease. J Pediatr Hematol/Oncol. 2015; 37(2): 128-133.
25. Bhatia M, Kolva E, Cimini L, Jin Z, Satwani P, Savone M, et al. Health-related quality of life after allogeneic hematopoietic stem cell transplantation for sickle cell disease. Biol Blood Marrow Transplant. 2015; 21(4): 666-672.
26. McClish DK, Penberthy LT, Bovbjerg VE, Roberts JD; Aisiku IP, Levenson JL, et al. Health related quality of life in sickle cell patients: The PiSCES project. Health Qual Life Outcomes. 2005; 3(50):1-7.
27. Schlenz AM, Schatz J, McClellan CB, Roberts CW. Responsiveness of the PedsQL to pain-related changes in health-related quality of life in pediatric sickle cell disease. J Pediatr Psychol. 2012; 37(7): 798-807.
28. Anie KA, Steptoe A, Bevan D. Sickle cell disease: Pain, coping and quality of life in a study of adults in the UK. Br J of Health Psychol. 2002; 7(3): 331-344.
29. Martínez-Triana R, MartínezRodríguez M, Guerra-Romero C, Guerra-González EM, MachadoAlmeida T, Machín-García S, et al. Cuestionario para evaluar la calidad de vida relacionada con la salud de adultos con drepanocitosis. Rev Cuba Hematol Inmunol Hemoter. 2012; 28:385-97. [abst]
30. van Tuijn CF, van Beers EJ, Schnog JJ, Biemond BJ. Pain rate and social circumstances rather than cumulative organ damage determine the quality of life in adults with sickle cell disease. Am J Hematol. 2010;85(7):532-5.
31. Pereira SAS, Brener S, Cardoso CS, Proietti ABFC. Sickle Cell Disease: quality of life in patients with hemoglobin SS and SC disorders. Rev Bras Hematol Hemoter. 2013; 35(5): 325-31.
32. Al-Allawi N, Qadir SMA ,Puehringer H, Chui DHK, Farrell JJ, Oberkanins C. The association of HBG2, BCL11A, and HMIP polymorphisms with fetal hemoglobin and clinical phenotype in Iraqi Kurds with sickle cell disease. Int. J Lab Hematol. 2019 ;41(1):87-93
Published
2019-12-01
How to Cite
AMEER TAHIR, D., AL-ALLAWI, N., MANSOUR KHADUM, V., & JASIM MOHAMMED, A. (2019). HEALTH-RELATED QUALITY OF LIFE IN SICKLE CELL DISEASE PATIENTS IN AKRE, IRAQI KURDISTAN. Duhok Medical Journal, 13(2), 31-43. Retrieved from http://dmj.uod.ac/index.php/dmj/article/view/89
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