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

Abstract

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 6–7 years compared to 8–12 years and 13–16 years. The mean HRQoL in the domain of family were: 77.84 ± 23.12 among 6–7 years, 66.00 ± 17.34 among the 8–12 years and 60.38 ± 16.72 among 13–16 years. 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.

Quality of life in children and adolescents with hemophilia A: A systematic review and meta-analysis

“…Table 1 [ 6 , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , [37] , [38] , [39] , [40] , [41] , [42] , [43] , [44] , [45] , [46] , [47] , [48] , [49] , [50] , [51] , [52] , [53] , [54] ] provides a brief description of each included study and an outline of the characteristics of each study population. The 29 included studies reported on 6 distinct instruments that were used to measure HRQoL in 2346 children and adolescents with hemophilia A.…”

“… Dsouza et al. [ 35 ]/2020/India/Peer-reviewed journal article Cross-sectional, no treatment comparison 107 6-16 y Distribution according to socioeconomic class: 18.7% poor class, 67.3% lower middle class, and 14.0% upper middle class. 89.7% 100% 0.0% FVIII Proportion on prophylaxis not reported Haemo-QoL Long Form Kids II (8-12 y) and III (13-16 y).…”

“…Ten studies measured HRQoL with one of the following 4 generic instruments: PedsQL (5 studies) [ 28 , 30 , [40] , [41] , [42] ], EQ-5D-3L (3 studies) [ 27 , 31 , 32 ], KIDSCREEN-52 (1 study) [ 47 ], and KINDL (1 study) [ 54 ]. Twenty studies measured HRQoL with one of the following hemophilia-specific pediatric instruments: Haemo-QoL (18 studies) [ 6 , 31 , [33] , [34] , [35] , [36] , 38 , 39 , 43 , 44 , 46 , 48 , [50] , [51] , [52] , [53] , 55 ] and the Canadian Hemophilia Outcomes—Kids’ Life Assessment Tool (CHO-KLAT) (3 studies) [ 29 , 45 , 54 ].…”

“…The remaining studies reported HRQoL outcomes for children and adolescents in general (0-18 years of age). Ethnicity and sociocultural or socioeconomic information were reported in 9 studies ( Table 1 ) [ 30 , 33 , 35 , 38 , 39 , 41 , 44 , 47 , 51 ].…”

“…FVIII inhibitor status was reported in 20 of 29 studies. Two studies included only persons with hemophilia A with inhibitors [ 34 , 38 ], whereas 9 studies included only patients without inhibitors [ 6 , 35 , 36 , 42 , 43 , 45 , 50 , 51 , 54 ]. The remaining studies reported a median of 6.5% of patients with FVIII inhibitors (range, 3.3%-22.0%).…”