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

Health and Quality of Life Outcomes

Research

Angela Lambing¹  Michelle L. Witkop²  Terry L. Anderson³  John M. McLaughlin³  Bartholomew Tortella³  James E. Munn⁴ 

[1] Henry Ford Hemophilia & Thrombosis Treatment Center, 2799 W Grand Blvd, Detroit, MI, USA;Currently: Bayer HealthCare, Whippany, NJ, USA;Northern Regional Bleeding Disorder Center, 1105 Sixth St, Traverse City, MI, USA;Pfizer Inc, Global Innovative Pharma, 500 Arcola Rd, 19426, Collegeville, PA, USA;University of Michigan Hemophilia Treatment Center, 1500 E. Medical Center Drive, Ann Arbor, MI, USA;

关键词: Chronic pain;    Hemophilia;    Pain management;    Patient adherence;    Prophylaxis;    von Willebrand disease;   

DOI  :  10.1186/s12955-017-0643-7

 received in 2016-11-03, accepted in 2017-03-29,  发布年份 2017

来源: Springer

【 摘 要 】

BackgroundHealth-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.MethodsA 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.ResultsOf 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.ConclusionsEfforts 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.

【 授权许可】

CC BY   
© The Author(s). 2017