Вопросы гематологии/онкологии и иммунопатологии в педиатрии

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Description of health related quality of life (HRQoL) of children and adolescents with hemophilia from India


Hemophilia is a genetic disease that impairs quality of life due to its chronicity of nature where the individual will experience spontaneous bleeding or bleeding after an injury which requires frequent visits to the hospital for treatment. The objective of this study was to find the Health -related Quality of Life (HRQoL) of children and adolescents with moderate and severe hemophilia between 6 years and 16 years using the Haemo-QoL questionnaire. A prospective survey was carried out among 107 children and adolescents from two hemophilia treatment centers in Karnataka state. Approval was taken from the ethical committee. The data was analyzed using SPSS version 16.0.

The mean age was 11.00±2.98 years. Among the 107 participants, 89.70% had hemophilia A and 10.30% had hemophilia B. Moderate hemophilia was found among 54.20% participants and 45.80% had severe hemophilia. Overall, the HRQoL scores (55.41) were higher in the age group of 6-7years compared to 8-12 years and 13-16 years. The mean HRQoL in the domain of family were: 77.84±23.12 among 6-7years, 66.00±17.34 among the 8-12 years and 60.38± 16.72 among 13-16years. Children demonstrated poor HRQoL in the domains of family and friends. The results indicate a need for continuous monitoring of QoL to identify better treatment methods.


Об авторах

Anjalin Dsouza
Manipal College of Nursing MAHE Manipal

Dinesh Nayak
Professor of Paediatrics, Dr. TMA Pai Hospital, Udupi, Manipal Melaka Medical College, Manipal Academy of Higher Education

Annamma Kurien
Professor of Pathology, Manipal Melaka Medical College, Academy of Higher Education, Manipal, India

Mamatha Pai
Professor, Department of child health nursing, Manipal Academy of Higher Education, Manipal,

Baby Nayak
Professor, Department of child health nursing, Manipal College of Nursing Manipal, Manipal Academy of Higher Education, Manipal,

Список литературы

1. Franchini M, Mannucci PM. Hemophilia A in the third millennium. Blood reviews. 2013Jul1;27(4):179-84..Available from: doi: 10.1016/j.blre.2013.06.002.

2. Broderick CR, Herbert RD, Latimer J, Curtin JA, Selvadurai HC. The effect of an exercise intervention on aerobic fitness, strength and quality of life in children with haemophilia (ACTRN012605000224628). BMC Hematology. 2006 Dec;6(1):2.Available from:

3. Bansal D, Totadri S. Comprehensive hemophilia management in India-Miles to go before we sleep. Indian Pediatr. 2018; 55: 559–560. Available from:

4. World federation of Hemophilia (2018) Report on annual global survey 2017. World federation of Hemophilia, Montreal. Available from :

5. Boehlen F, Graft L, Berntorp E. Outcome measures in haemophilia: A systematic review. European Journal of Haematology 2014; 93 (76):2–15. Available from :

6. Von Mackensen S, Harrington C, Tuddenham E, Littley A, Will A, Fareh M, et al. The impact of sport on health status, psychological well‐being and physical performance of adults with haemophilia.

7. Haemophilia. 2016 Jul;22(4):521-30. Available from:

8. Van den Berg HM, Feldman BM, Fischer K, Blanchette V, Poonnoose P, Srivastava A. Assessments of outcome in haemophilia–what is the added value of QoL tools?. Haemophilia. 2015 Jul;21(4):430-5. Available from:

9. 1111/hae.12731.

10. Whoqol Group. Development of the World Health Organization WHOQOL-BREF quality of life assessment. Psychological medicine. 1998 May;28(3):551-8. DOI:

11. Rentz A, Flood E, Altisent C, Bullinger M, Klamroth R, Garrido RP,et al MEMBERS OF THE HAEMO‐QoL‐A STEERING COMMITTEE. Cross‐cultural development and psychometric evaluation of a patient‐reported health‐related quality of life questionnaire for adults with haemophilia. Haemophilia. 2008 Sep;14(5):1023-34.Available from:

12. Oladapo AO, Epstein JD, Williams E, Ito D, Gringeri A, Valentino LA. Health‐related quality of life assessment in haemophilia patients on prophylaxis therapy: a systematic review of results from prospective clinical trials. Haemophilia. 2015 Sep;21(5):e344-58.Available from:

13. Aggarwal OP, Bhasin SK, Sharma AK, Chhabra P, Aggarwal K, Rajoura OP. A new instrument (scale) for measuring the socioeconomic status of a family: Preliminary study. Indian J Community Med. 2005 Oct;30(4):111-4.

14. Bullinger MV, Von Mackensen S, Fischer K, Khair K, Petersen C, Ravens‐Sieberer U, et al. Pilot testing of the ‘Haemo‐QoL’quality of life questionnaire for haemophiliac children in six European countries. Haemophilia. 2002 Mar; 8:47-54. Available from:

15. Von Mackensen S, Bullinger M, Haemo‐QoL Group. Development and testing of an instrument to assess the Quality of Life of Children with Haemophilia in Europe (Haemo‐QoL). Haemophilia. 2004 Mar;10:17-25. Available from

16. Taha MY, Hassan MK. Health-related quality of life in children and adolescents with hemophilia in Basra, Southern Iraq. Journal of pediatric hematology/oncology. 2014 Apr 1;36(3):179-84. Available from: DOI: 10.1097/MPH.0000000000000117.

17. Tantawy AA, Mackensen SV, El-Laboudy MA, Labib JH, Moftah F, El-Telbany MA, et al. Health-related quality of life in Egyptian children and adolescents with hemophilia A. Pediatric hematology and oncology. 2011 Mar 21;28(3):222-9..Available from:

18. St-Louis J, Urajnik DJ, Menard F, Cloutier S, Klaassen RJ, Ritchie B, et al. Generic and disease specific quality of life among youth and young men with hemophilia in Canada. BMC Hematology 2016; 16(1):13.Available from: 10.1186/s12878-016-0052-x.

19. Ferreira AA, Leite IC, Bustamante-Teixeira MT, Correa CS, da Cruz DT, Rodrigues Dde O, et al. Health-related quality of life in hemophilia: results of the Hemophilia-Specific Quality of Life Index (Haem-a-Qol) at a Brazilian blood center. Rev Bras Hematol Hemoter 2013; 35(5): 314–18.Available from:

20. Minners AH, Sabin CA, Tolley KH, Jenkinson C, Kind P, Lee CA. Assessing health-related quality-of-life in individuals with haemophilia. Haemophilia 1999; 5: 378-85.

Для цитирования:

., ., ., ., . . Вопросы гематологии/онкологии и иммунопатологии в педиатрии. 2020;19(3).

For citation:

Dsouza A., Nayak D., Kurien A., Pai M.S., Nayak B.S. Description of health related quality of life (HRQoL) of children and adolescents with hemophilia from India. Pediatric Hematology/Oncology and Immunopathology. 2020;19(3). (In Russ.)

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