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

Abstract

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.

Male gender, school attendance and sports participation are positively associated with health‐related quality of life in children and adolescents with congenital bleeding disorders

“…Variables found to be significantly associated with HRQOL are gender, participation in sports and school attendance. A recent study in the United States on predictors of HRQOL in adolescents with CBD also found gender associated with HRQOL . Boys reporting higher HRQOL than girls have been demonstrated in (healthy) populations and partially explain the influence of gender on HRQOL.…”

“…Boys reporting higher HRQOL than girls have been demonstrated in (healthy) populations and partially explain the influence of gender on HRQOL. In addition, as mentioned earlier, the HRQOL of girls with CBD may be affected by heavy menstruations, and it is possible that this has a more immediate detrimental effect on HRQOL than the long‐term effects (eg arthropathy) associated with haemophilia, which may have not yet fully manifested in this population …”

Calidad de vida: un aspecto olvidado en el paciente con hemofilia

“…La intensidad del dolor es el principal factor influyente en el nivel de calidad de vida física y psicológica de los pacientes con hemofilia, razón por la que el alivio clínico del mismo y el mantenimiento del bienestar físico es crucial para el mantenimiento de una buena CVRS [12,21]. Este síntoma se presenta en mayor proporción en los pacientes con hemofilia severa, sin embargo, también es un hallazgo de importancia en la hemofilia leve a moderada que se debe tener en cuenta durante el abordaje.…”

“…La calidad de vida de los pacientes con hemofilia presenta variación con la edad, ya que a medida que se envejece cambian las preferencias y necesidades de los pacientes [16,21]. Se ha descrito que, para la población pediátrica con hemofilia, el principal determinante para una buena calidad de vida se compone de las amistades, familia y el tratamiento, mientras que, para la población adolescente son la actividad física, el apoyo percibido y la escuela.…”

“…La medición de la calidad de vida se realiza a través de la evaluación de la percepción del paciente sobre su bienestar general, las experiencias relacionadas con tener un trastorno hemorrágico y la satisfacción con el tratamiento del mismo; además, un punto importante de la medición de la calidad de vida es que permite medir el grado de funcionalidad de un paciente [9,21]. En la Tabla 2 se pueden observar distintos instrumentos descritos en la literatura para la recolección de datos relacionados con la calidad de vida en hemofilia y los resultados de sus respectivos estudios.…”