Predictors of quality of life among adolescents and young adults with a bleeding disorder

Abstract

Background: Health-related quality of life (HRQoL) in adolescents and young adults with bleeding disorders is under-researched. We aimed to describe factors related to HRQoL in adolescents and young adults with hemophilia A or B or von Willebrand disease.

Methods: A convenience sample of volunteers aged 13 to 25 years with hemophilia or von Willebrand disease completed a cross-sectional survey that assessed Physical (PCS) and Mental (MCS) Component Summary scores on the SF-36 questionnaire. Quantile regression models were used to assess factors associated with HRQoL.

Results: Of 108 respondents, 79, 7, and 14% had hemophilia A, hemophilia B, and von Willebrand disease, respectively. Most had severe disease (71%), had never developed an inhibitor (65%), and were treated prophylactically (68%). Half of patients were aged 13 to 17 years and most were white (80%) and non-Hispanic (89%). Chronic pain was reported as moderate to severe by 31% of respondents. Median PCS and MCS were 81.3 and 75.5, respectively. Quantile regression showed that the median PCS for women (61% with von Willebrand disease) was 13.1 (95% CI: 2.4, 23.8; p = 0.02) points lower than men. Ever developing an inhibitor (vs never) was associated with a 13.1-point (95% CI: 4.7, 21.5; p < 0.01) PCS reduction. MCS was 10.0 points (95% CI: 0.7, 19.3; p = 0.04) higher for prophylactic infusers versus those using on-demand treatment. Compared with patients with no to mild chronic pain, those with moderate to severe chronic pain had 25.5-point (95% CI: 17.2, 33.8; p < 0.001) and 10.0-point (95% CI: 0.8, 19.2; p = 0.03) reductions in median PCS and MCS, respectively.

Conclusions: Efforts should be made to prevent and manage chronic pain, which was strongly related to physical and mental HRQoL, in adolescents and young adults with hemophilia and von Willebrand disease. Previous research suggests that better clotting factor adherence may be associated with less chronic pain.

Keywords: Chronic pain; Hemophilia; Pain management; Patient adherence; Prophylaxis; von Willebrand disease.

Fig. 1

Distribution of SF-36 Physical Component Summary scores (n = 108). SF-36, 36-Item Short Form Health Survey; PCS, Physical Component Summary

Fig. 2

Distribution of SF-36 Mental Component Summary scores (n = 108). SF-36, 36-Item Short Form Health Survey; MCS, Mental Component Summary

Fig. 3

Box plots for SF-36 component and subscale scores (n = 108). The solid white line represents median value, and the white diamond represents the mean value. PCS, Physical Component Summary score; MCS, Mental Component Summary; BP, bodily pain; PF, physical function; RP, role limitations due to physical health problems; GH, general health; VT, vitality; SF, social functioning; RE, role limitations due to emotional problems; MH, emotional well-being/mental health