Hemoglobin F as a predictor of health-related quality of life in children with sickle cell anemia
As treatment options for children with sickle cell anemia (SCA) continue to expand survival, evaluation of factors associated with health-related quality of life (HRQoL) is becoming an important aspect for further improving clinical management. Although the general features of SCA are similar, factors influencing HRQoL within a country may differ from those of other countries, therefore this study aimed to explore factors affecting HRQoL in children with SCA living in the Sultanate of Oman.
This was a cross-sectional study in which the PedsQL™ Sickle Cell Disease Module was used to evaluate the overall HRQoL in children with SCA. The socio-demographic data, clinical, and treatment outcomes were collected. Univariate and multivariate linear regression analyses were used to identify predictors of HRQoL.
A total of 123 children with SCA, aged from 2 to 16 years were enrolled. The mean total HRQoL score was 52 ± 15% (9–94), where Worry II scale recorded the highest score. The multiple regression analysis revealed that the only predictors of total HRQoL score were hemoglobin F (B = 0.64, 95% confidence interval [CI] 0.149–1.118, P = 0.009) and to a lesser degree white blood cell count (B = − 0.99, 95% CI − 1.761 to − 0.198, P = 0.01), independently of other study parameters such as age, gender, spleen status, and hydroxyurea therapy.
Collectively, these findings indicated that hemoglobin F out-weighted white blood cell count in predicting HRQoL in Omani children with SCA. Recognition of these factors could help health professionals to develop effective strategies to improve the overall HRQoL in these young patients.
KeywordsHemoglobin F Predictor Quality of life Sickle cell anemia
We are thankful to all participants and their families and also to the Hospital Information System staff of the SQUH for providing technical assistance. This work was supported in part by grants from The Oman Research Council (#ORG/HSS/13/002) and the Sultan Qaboos University (IG/MED/HAEM/14/01).
MRB designed, performed, analyzed data, and wrote the manuscript. AB and GH participated in study deign, data collection, and analysis. ME, MS, AB, ZQ, HK, RQ, and YW participated in data collection, reviewed, and critically revised the manuscript.
Compliance with ethical standards
Conflict of interest
The authors stated that they had no interests which might be perceived as posing a conflict or bias.
- 1.Ware, R. E., de Montalembert, M., Tshilolo, L., & Abboud, M. R. (2017). Sickle cell disease. The Lancet, 390(10091), 311–323.Google Scholar
- 2.Yawn, B. P., Buchanan, G. R., Afenyi-Annan, A. N., Ballas, S. K., Hassell, K. L., James, A. H., et al. (2014). Management of sickle cell disease: Summary of the 2014 evidence-based report by expert panel members. Journal of the American Medical Association, 312(10), 1033–1048.Google Scholar
- 3.Sharef, S. W., Al-Hajri, M., Beshlawi, I., Al-Shahrabally, A., Elshinawy, M., Zachariah, M., et al. (2013). Optimizing hydroxyurea use in children with sickle cell disease: Low dose regimen is effective. European Journal of Haematology, 90(6), 519–524.Google Scholar
- 4.Panepinto, J. A. (2012). Health-related quality of life in patients with hemoglobinopathies. Hematology. American Society of Hematology Education Program, 2012, 284–289.Google Scholar
- 5.Beverung, L. M., Varni, J. W., & Panepinto, J. A. (2015). Clinically meaningful interpretation of pediatric health-related quality of life in sickle cell disease. Journal of Pediatric Hematology/Oncology, 37(2), 128–133.Google Scholar
- 6.Menezes, A. S., Len, C. A., Hilário, M. O., Terreri, M. T., & Braga, J. A. (2013). Quality of life in patients with sickle cell disease. Revista Paulista de Pediatria, 31(1), 24–29.Google Scholar
- 7.Panepinto, J. A., & Bonner, M. (2012). Health-related quality of life in sickle cell disease: Past, present, and future. Pediatric Blood & Cancer, 59(2), 377–385.Google Scholar
- 8.Badawy, S. M., Thompson, A. A., Lai, J. S., Penedo, F. J., Rychlik, K., & Liem, R. I. (2017). Health-related quality of life and adherence to hydroxyurea in adolescents and young adults with sickle cell disease. Pediatric Blood & Cancer, 64(6), e26369.Google Scholar
- 9.Thornburg, C. D., Calatroni, A., & Panepinto, J. A. (2011). Differences in health-related quality of life in children with sickle cell disease receiving hydroxyurea. Journal of Pediatric Hematology/Oncology, 33(4), 251–254.Google Scholar
- 10.Beverung, L. M., Strouse, J. J., Hulbert, M. L., Neville, K., Liem, R. I., Inusa, B., et al. (2015). Health-related quality of life in children with sickle cell anemia: Impact of blood transfusion therapy. American Journal of Hematology, 90(2), 139–143.Google Scholar
- 11.Bhatia, M., Kolva, E., Cimini, L., Jin, Z., Satwani, P., Savone, M., et al. (2015). Health-related quality of life after allogeneic hematopoietic stem cell transplantation for sickle cell disease. Biology of Blood and Marrow Transplantation, 21(4), 666–672.Google Scholar
- 12.Psihogios, A. M., Daniel, L. C., Tarazi, R., Smith-Whitley, K., Patterson, C. A., & Barakat, L. P. (2018). Family functioning, medical self-management, and health outcomes among school-aged children with sickle cell disease: A mediation model. Journal of Pediatric Psychology, 43(4), 423–433.Google Scholar
- 13.Panepinto, J. A., Pajewski, N. M., Foerster, L. M., Sabnis, S., & Hoffmann, R. G. (2009). Impact of family income and sickle cell disease on the health-related quality of life of children. Quality of Life Research, 18(1), 5–13.Google Scholar
- 14.Sehlo, M. G., & Kamfar, H. Z. (2015). Depression and quality of life in children with sickle cell disease: The effect of social support. BMC Psychiatry, 15, 78.Google Scholar
- 15.Barakat, L. P., Daniel, L. C., Smith, K., Renée Robinson, M., & Patterson, C. A. (2014). Parental problem-solving abilities and the association of sickle cell disease complications with health-related quality of life for school-age children. Journal of Clinical Psychology in Medical Settings, 21(1), 56–65.Google Scholar
- 16.McClish., D. K., Smith, W. R., Levenson, J. L., Aisiku, I. P., Roberts, J. D., Roseff, S. D., et al. (2017). Comorbidity, pain, utilization, and psychosocial outcomes in older versus younger sickle cell adults: The PiSCES project. BioMed Research International, 2017, 4070547.Google Scholar
- 17.Trzepacz, A. M., Vannatta, K., Gerhardt, C. A., Ramey, C., & Noll, R. B. (2004). Emotional, social, and behavioral functioning of children with sickle cell disease and comparison peers. Journal of Pediatric Hematology/Oncology., 26(10), 642–648.Google Scholar
- 18.Rizvi, M. A. K., & Hossain, M. Z. (2017). Relationship between religious belief and happiness: A systematic literature review. Journal of Religion and Health, 56(5), 1561–1582.Google Scholar
- 19.Gharaibeh, H. F., & Gharaibeh, M. K. (2012). Factors influencing health-related quality of life of thalassaemic Jordanian children. Child Care Health and Development, 38(2), 211–218.Google Scholar
- 20.Mevada, S. T., Al Saadoon, M., Zachariah, M., Rawas, A., Wali, A. H., Y (2016). Impact of burden of thalassemia major on health-related quality of life in Omani children. Journal of Pediatric Hematology/Oncology, 38(5), 384–388.Google Scholar
- 21.Dale, J. C., Cochran, C. J., Roy, L., Jernigan, E., & Buchanan, G. R. (2011). Health-related quality of life in children and adolescents with sickle cell disease. Journal of Pediatric Health Care, 25(4), 208–215.Google Scholar
- 22.Bhagat, V. M., Baviskar, S. R., Mudey, A. B., & Goyal, R. C. (2014). Poor health related quality of life among patients of sickle cell disease. Indian Journal of Palliative Care, 20(2), 107–111.Google Scholar
- 23.Brandow, A. M., Brousseau, D. C., Pajewski, N. M., & Panepinto, J. A. (2010). Vaso-occlusive painful events in sickle cell disease: Impact on child well-being. Pediatric Blood & Cancer, 54(1), 92–97.Google Scholar
- 24.Ingerski, L. M., Modi, A. C., Hood, K. K., Pai, A. L., Zeller, M., Piazza-Waggoner, C., et al. (2010). Health-related quality of life across pediatric chronic conditions. Journal of Pediatrics, 156(4), 639–644.Google Scholar
- 25.Alkindi, S., Al Zadjali, S., Al Madhani, A., Daar, S., Al Haddabi, A., Al Abri, H., et al (2010). Forecasting hemoglobinopathy burden through neonatal screening in Omani neonates. Hemoglobin, 34(2), 135–144.Google Scholar
- 26.Panepinto, J. A., Paul Scott, J., Badaki-Makun, O., Darbari, D. S., Chumpitazi, C. E., Airewele, G. E., et al (2017). Determining the longitudinal validity and meaningful differences in HRQL of the PedsQL™ sickle cell disease module. Health and Quality of Life Outcomes, 15(1), 124.Google Scholar
- 27.Thornburg, C. D., Dixon, N., Burgett, S., Mortier, N. A., Schultz, W. H., Zimmerman, S. A., et al. (2009). A pilot study of hydroxyurea to prevent chronic organ damage in young children with sickle cell anemia. Pediatric Blood & Cancer, 52(5), 609–615.Google Scholar
- 28.Steinberg, M. H., Chui, D. H., Dover, G. J., Sebastiani, P., & Alsultan, A. (2014). Fetal hemoglobin in sickle cell anemia: A glass half full? Blood, 123(4), 481–485.Google Scholar
- 29.Charache, S. (1997). Mechanism of action of hydroxyurea in the management of sickle cell anemia in adults. Seminars in Hematology, 34(3 Suppl 3), 15–21.Google Scholar
- 30.Murray, N., Serjeant, B. E., & Serjeant, G. R. (1988). Sickle cell-hereditary persistence of fetal haemoglobin and its differentiation from other sickle cell syndromes. British Journal of Haematology, 69(1), 89–92.Google Scholar
- 31.Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., et al. (1994). Mortality in sickle cell disease. Life expectancy and risk factors for early death. New England Journal of Medicine, 330(23), 1639–1644.Google Scholar
- 32.Okpala, I. (2004). The intriguing contribution of white blood cells to sickle cell disease—a red cell disorder. Blood Reviews, 18(1), 65–73.Google Scholar
- 33.Litos, M., Sarris, I., Bewley, S., Seed, P., Okpala, I., & Oteng-Ntim, E. (2007). White blood cell count as a predictor of the severity of sickle cell disease during pregnancy. European Journal of Obstetrics & Gynecology and Reproductive Biology, 133(2), 169–172.Google Scholar
- 34.Calia, R., Lai, C., Aceto, P., Luciani, M., Camardese, G., Lai, S., et al. (2015). Emotional self-efficacy and alexithymia may affect compliance, renal function and quality of life in kidney transplant recipients: Results from a preliminary cross-sectional study. Physiology & Behavior, 142, 152–154.Google Scholar
- 35.Calia, R., Lai, C., Aceto, P., Luciani, M., Camardese, G., Lai, S., et al. (2015). Attachment style predict compliance, quality of life and renal function in adult patients after kidney transplant: Preliminary results. Renal Failure, 37(4), 678–680.Google Scholar