Medical co-morbidities of patients with haemophilia: pain, obesity and hepatitis C.

Clinical care of patients with haemophilia (PWH) has progressed rapidly over the past decade. Current therapy has allowed patients with haemophilia to live longer and many patients are now experiencing the co-morbidities of the general population. In this review article, we focus on three common diseases states that affect PWH: chronic pain, obesity and hepatitis C. Pain has been a co-morbidity for many years and PWH often have unusual needs for chronic pain relief compared to the general population. Obesity is not only increasing in the general population but also in patients with hereditary bleeding disorders. The co-morbidity of obesity not only causes increased pain progression and joint damage but also affects the dosing of factor concentrates. Finally, hepatitis C is known to have infected the majority of patients who received non-virally inactivated pooled factor concentrates in the past. New treatment regimens have been developed that allow the nearly uniform cure of chronic hepatitis C with a short course of oral medications.

Predictors of non-adherence to prescribed prophylactic clotting-factor treatment regimens among adolescent and young adults with a bleeding disorder.

INTRODUCTION: Adherence to clotting-factor treatment regimens, especially among adolescents and young adults (AYAs), is under-researched. AIM: We determined factors associated with better adherence to prophylaxis. METHODS: From April through December 2012, a convenience sample of AYA (aged 13-25 years) persons with haemophilia or von Willebrand disease (VWD) completed an online survey that assessed adherence to prescribed prophylactic treatment regimens [Validated Haemophilia Regimen Treatment Adherence Scale (VERITAS)-Pro]. Logistic regression analysis assessed demographic and clinical factors related to non-adherence (VERITAS-Pro≥57). RESULTS: Seventy-three prophylactically treating AYAs participated. Of which, 88%, 8% and 4% had haemophilia A, B and VWD respectively. Almost all (90%) had severe disease and 58% had never developed an inhibitor. Most were aged 13-17 years (56%), white (78%), non-Hispanic (88%), never married (94%) and had some type of health insurance (96%). Median VERITAS-Pro score was 48 (range = 25-78) and 22 (30%) participants were non-adherent to prophylaxis (VERITAS-Pro≥57). Final logistic regression modelling suggested that, compared to those aged 13-17 years, participants aged 18-25 years were 6.2 (95% CI: 1.8-21.0; P < 0.01) times more likely to be non-adherent. Compared to respondents whose mother had at least a Bachelor's degree, respondents whose mother did not were 3.8 (95% CI: 1.0-14.3; P = 0.05) times more likely to be non-adherent. CONCLUSIONS: Results suggest that adherence efforts should be especially targeted to young adults as they transition from adolescence (i.e. parental supervision) and assume primary responsibility for their bleeding disorder care. Healthcare providers should be mindful of AYAs whose mothers have less formal education and ensure that adequate time and resources are dedicated to family adherence education.

Better adherence to prescribed treatment regimen is related to less chronic pain among adolescents and young adults with moderate or severe haemophilia.

Little data exist, especially for adolescent and young adult (AYA) persons with haemophilia (PWH), about the relationship between adherence to prescribed treatment regimen and chronic pain. We examined this relationship among PWH (moderate or severe) aged 13-25 via cross-sectional survey. Adherence was assessed using the Validated Hemophilia Regimen Treatment Adherence Scale (VERITAS)-Pro and VERITAS-PRN for prophylactic and on-demand participants respectively. VERITAS scores range from 24 (most adherent) to 120 (least adherent). Chronic pain was measured using the FPS-R and was dichotomized as high for FPS-R scores ≥4 and low for <4. Logistic regression models were constructed to assess factors associated with having high (vs. low) chronic pain. Of 80 AYA respondents (79 men), most had severe disease (91%), infused prophylactically (86%) and had haemophilia A (91%). Fifty-one per cent were aged 13-17 and most were white (76%), non-Hispanic (88%) and never married (93%). Chronic pain was reported as high for 35% of respondents. Mean VERITAS-Pro scores for those with high and low chronic pain were 53.6 ± 12.3 vs. 47.4 ± 12.9, P = 0.05. VERITAS-PRN scores were similar across chronic pain status. Logistic regression revealed that for each 10-point reduction (i.e. increase in adherence) in the combined VERITAS (Pro and PRN) and VERITAS-Pro scores there was a 35% (OR = 0.65; 95% CI = 0.44, 0.96; P = 0.03) and 39% (OR = 0.61; 95%CI = 0.39, 0.96; P = 0.03) reduction in odds of having high chronic pain respectively. Among AYA PWHs, better adherence was associated with significantly lower odds of having high chronic pain. Moreover, non-whites were >4 times as likely as whites to report high chronic pain.