General characteristics of the health-related quality of life in children with severe hemophilia A in Ukraine
Summary. Aim — to study the health-related quality of life in children with severe forms of hemophilia A and identify the main psychosocial predictors that affect it in children of all ages. Materials and methods. Assessment of quality of life (QOL) is conducted by the sociological survey using a validated Ukrainian version of the Haemo-QoL Questionnaire. Model of the survey: individual and electronic questionnaire. The study involved 105 children with severe hemophilia A. The patients were divided into three age groups according to the version of the questionnaire: I — 4–7, II — 8–12, III — 13–16 years. These surveys were analyzed, the general quality of life in each age group and the values of QOL for individual scales were identified; the correlations of the general QOL with individual determinants of psychosocial development were determined. Statistical analysis was performed using t-test, Mann — Whitney U-test and the Kruskal — Wallis H test, Spearman’s rank correlation coefficient R. Results. It has been established that QOL in children of the Ist age group is significantly the worst in the segment of family relations, with a value of the corresponding scale of 62.50 (62.50; 87.50), while in the group II the worst QOL was in the fields of activities in sport/school and family relationships with the values of the relevant scales of the questionnaire of 68.75 (56.25; 75.00) and 65.00 (50.00; 80.00), respectively. In group III, significantly the worst QOL was in the segment of attitude to physical health with an indicator of 75.00 (53.57; 83.92). At the same time, psychosocial predictors that best correlate with the general index of QOL in children is somewhat different from the worst determinants of psychosocial development in children of group I and in which the most significant predictor of QOL is determined to be relationships with friends and treatment, while in groups II and III such predictors correspond to the worst QOL factors. Conclusions. The QOL in children with hemophilia has age-related features, as well as psychosocial predictors that affect it, which is important to consider when designing and implementing psychosocial support and rehabilitation programs.
Key words: hemophilia, children, quality of life, questionnaire, psychosocial functioning.
- Kovtiuk N.I., Nechytailo Yu.M., Yukhymets I.O. (2012) Yakist zhyttia, poviazana iz zdoroviam u ditei z nevrolohichnoiu patolohiieiu ta mozhlyvosti yoho pokrashchannia. Klin. eksperyment. patolohiia, 1: 96–98.
- Kryvenko V.I., Kachan I.S., Pakhomova S.P. ta in. (2015) Yakist zhyttia ta prykhylnist do likuvannia v klinitsi vnutrishnikh khvorob. ZDMU, Zaporizhzhia, 80 s.
- Kryvenko V.I., Hrinenko T.Iu., Kachan I.C. (2011) Yakist zhyttia yak efektyvnyi obiektyvnyi kryterii diahnostyky ta likuvannia u suchasnii medytsyni. Zap. med. zhurn., 13(6): 91–96.
- Markin A.I., Dubei L.Ia., Khmiliarchuk L.I. (2018) Ukrainska versiia opytuvalnyka Haemo-QoL: linhvistychna ta kulturna adaptatsiia. Sovr. pedyatr., 1(89): 84–89.
- Markin A.I., Dubei L.Ia., Komendant Kh.M., Dubei N.V. (2017) Yakist zhyttia ditei z hemofiliieiu A, poviazana zi zdoroviam: mizhnarodnyi dosvid ta vitchyzniani realii. Perynatol. pedyatr., 4 (72): 125–133.
- Nechytailo Yu.M. (2013) Metodolohichni osnovy otsinky yakosti zhyttia, poviazanoi zi zdoroviam u ditei. zhurn. pedyatr. akusher. hynekol., 3: 5–13.
- Brown T.M., Lee W.C., Joshi A.V., Pashos C.L. (2009) Health-related quality of life and productivity impact in haemophilia patients with inhibitors. Haemophilia, 15(4): 911–917.
- Bullinger M., von Mackensen S. (2008) Psycho-social determinants of quality of life in children and adolescents with haemophilia-a cross-cultural approach. Clin. Psychol. Psychother., 15(3): 164–172.
- de Moerloose P., Fischer K., Lambert T. et al. (2012) Recommendations for assessment, monitoring and follow-up of patients with haemophilia. Haemophilia, 18(3): 319–325.
- Dekoven M., Wisniewski T., Petrilla A. et al. (2013) Health-related quality of life in haemophilia patients with inhibitors and their caregivers. Haemophilia, 19(2): 287–293.
- Deshbhratar D.P. (2016) Comparison of Quality of Life in Adults and Children with Haemophilia. Imper. J. Interdiscipl. Res., 2(7): 733–735.
- Espaldon A.M.D., Flerida G.H. (2014) Health â Related Quality of Life Assessment in Filipino Children with Hemophilia Aged 4 To 16 Years in a Tertiary Hospital. J. Hematol. Thromboembol. Dis., 2: 133.
- Ferreira A.A., Leite I.C., Bustamante-Teixeira M.T. et al. (2013) 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., 35(5): 314–318.
- Fischer K., van der Bom J.G., van den Berg H.M. (2003) Health-related quality of life as outcome parameter in haemophilia treatment. Haemophilia, 9(Suppl. 1): 75–81.
- Gringeri A., von Mackensen S., Auerswald G. et al. (2004) Health status and health-related quality of life of children with haemophilia from six West European countries. Haemophilia, 10(Suppl. 1): 26–33.
- Gusmão J.L., Mion D.Jr., Pierin A.M. (2009) Health-related quality of life and blood pressure control in hypertensive patients with and without complications. Clinics (Sao Paulo), 64(7): 619–628.
- Haemo-QoL Study Group (2018) The Quality of life assessment instrument for children and adolescents with haemophilia (http://haemoqol.de/scoring/manual/).
- Markin A., Dubey L. (2018) Test-retest reliability of Ukrainian version of the Haemo-QoL questionnaire. EUREKA: Health Sci., 3: 21–29.
- Mercan A., Sarper N., Inanir M. et al. (2010) Hemophilia-Specific Quality of Life Index (Haemo-QoL and Haem-A-QoL questionnaires) of children and adults: result of a single center from Turkey. Pediatr. Hematol. Oncol., 27(6): 449–461.
- Novik A., Salek S., Ionova T. (2012) Guidelines Patient-Reported outcomes in Hematology. The European Hematology Association, The Hague, The Netherlands.
- Salomon T., Chaves D.G., Brener S. et al. (2017) Determining the health-related quality of life in individuals with haemophilia in developing economies: results from the Brazilian population. Haemophilia, 23(1): 42–49.
- Srivastava A., Brewer A.K., Mauser-Bunschoten E.P. et al.; Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia (2013) Guidelines for the management of hemophilia. Haemophilia, 19(1): e1–e47.
- Tantawy A.A., Mackensen S.V., El-Laboudy M.A. et al. (2011) Health-related quality of life in Egyptian children and adolescents with hemophilia A. Pediatr. Oncol., 28(3): 222–229.