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Quality of Life Research

, Volume 28, Issue 2, pp 473–479 | Cite as

Hemoglobin F as a predictor of health-related quality of life in children with sickle cell anemia

  • Mohamed-Rachid BoulasselEmail author
  • Amira Al-Badi
  • Mohamed Elshinawy
  • Juhaina Al-Hinai
  • Muna Al-Saadoon
  • Zahra Al-Qarni
  • Hammad Khan
  • Rizwan Nabi Qureshi
  • Yasser Wali
Article

Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusions

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.

Keywords

Hemoglobin F Predictor Quality of life Sickle cell anemia 

Notes

Acknowledgements

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).

Author contributions

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.

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Copyright information

© Springer Nature Switzerland AG 2018

Authors and Affiliations

  • Mohamed-Rachid Boulassel
    • 1
    • 2
    • 5
    Email author
  • Amira Al-Badi
    • 1
  • Mohamed Elshinawy
    • 3
    • 4
  • Juhaina Al-Hinai
    • 1
  • Muna Al-Saadoon
    • 3
  • Zahra Al-Qarni
    • 1
  • Hammad Khan
    • 2
  • Rizwan Nabi Qureshi
    • 2
  • Yasser Wali
    • 3
    • 4
  1. 1.Department of Allied Health Sciences, College of Medicine and Health SciencesSultan Qaboos UniversityMuscatOman
  2. 2.Department of Haematology, College of Medicine and Health SciencesSultan Qaboos UniversityMuscatOman
  3. 3.Department of Child Health, College of Medicine and Health SciencesSultan Qaboos UniversityMuscatOman
  4. 4.Pediatric Department, Faculty of MedicineAlexandria UniversityAlexandriaEgypt
  5. 5.Department of Allied Health Sciences, College of Medicine and Health SciencesSultan Qaboos UniversityAl-Khoudh, MuscatOman

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