Objective quantification of adherence to prophylaxis in haemophilia patients aged 12 to 25years and its potential association with bleeding episodes

Pérez-Robles, Tamara; Romero-Garrido, José Antonio; Rodríguez-Merchán, E. Carlos; Herrero-Ambrosio, Alicia

doi:10.1016/j.thromres.2016.04.015

Cited by 18 publications

(41 citation statements)

References 22 publications

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“…This is a positive result because impairment would be expected due to the natural progression of the disease. However, the literature reports controversial data regarding the degree of association of adherence and joint health: from a higher number of hemarthrosis observed in less‐adherent patients, to no association of joint bleeding with reduced adherence to treatment …”

Section: Discussionmentioning

confidence: 99%

Effectiveness of the Medtep Hemophilia online platform for adherence to prophylactic treatment in haemophilia patients: Results from a 1‐year observational study

et al. 2018

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Section: Discussionmentioning

confidence: 99%

Effectiveness of the Medtep Hemophilia online platform for adherence to prophylactic treatment in haemophilia patients: Results from a 1‐year observational study

et al. 2018

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“…The main clinical (a) barriers to treatment adherence are high perceived burden of treatment and no or low burden of bleeds and symptoms that may reduce patients’ perception of needing PTX . Some patients have venous access difficulties and PTX is often seen as complicated and time‐consuming, with morning infusions being especially inconvenient . Historically, there were concerns about the risk of HIV or HCV infections, yet in later years concerns about adverse events are rarely reported a reason for non‐adherence to PTX.…”

Section: Resultsmentioning

confidence: 99%

“…Reportedly, adolescents frequently lack knowledge, self‐management skills and self‐efficacy, that is, the motivation and belief in their ability to make appropriate treatment choices . YWH often feel PTX interferes with their daily activities, have difficulties remembering infusions, dealing with sports restrictions and telling others about their disease . Their desire to fit in with healthy peers and live “normal” active lives may challenge disease acceptance .…”

Section: Resultsmentioning

confidence: 99%

“…Since the late 1980s, recombinant prophylactic treatment (PTX) has greatly improved the life of haemophilic patients. PTX is associated with reduced number of bleeds, less joint damage and pain and better health‐related quality of life (HRQoL) in severe haemophiliacs . Yet new challenges to treatment adherence have occurred as PTX requires frequent self‐infusions, typically two‐three mornings per week depending on the type and severity of haemophilia .…”

Section: Introductionmentioning

confidence: 99%

“…Yet new challenges to treatment adherence have occurred as PTX requires frequent self‐infusions, typically two‐three mornings per week depending on the type and severity of haemophilia . Non‐adherence has significant impact, however, as bleeds may reduce patients’ participation in school, work and daily activities lowering their psychosocial as well as physical QoL and functioning …”

Section: Introductionmentioning

confidence: 99%

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Adherence to prophylactic haemophilic treatment in young patients transitioning to adult care: A qualitative review

Mortensen¹,

Strand²,

Almén³

2018

Haemophilia

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Recombinant prophylactic treatment (PTX) has greatly improved morbidity, mortality and health‐related quality of life (HRQoL) in patients with severe haemophilia. Yet, treatment adherence appears suboptimal in adolescents and young adults with haemophilia (YWH). Young patients experience major biopsychosocial changes challenging their adherence through the transition from parental to self‐care, from paediatric to adult care. In clinical practice, a systematic approach to transition is rarely used and there is little evidence on best practices. This qualitative review was based on a systematic literature search including quantitative as well as qualitative research reports to examine all relevant factors influencing adherence to PTX in YWH. We aimed to gain comprehensive insight into main drivers and barriers to adherence by exploring them in the context of YWH’s disease perceptions, characteristics, HRQoL and needs. The outcome is an overview of the latest published recommendations to support treatment adherence in YWH during the transition from family‐oriented care to self‐care and from paediatric to adult care. The literature suggests that adherence to PTX is best supported when individual patient needs and preferences are taken into consideration when planning treatment. Preserving normality is a main priority in young patients making it crucial to support patients from early childhood in considering PTX as enabling rather than hindering a normal social and physically active life. Education in self‐management should include psychosocial support of patients as well as caregivers. This requires systematic transition planning including milestone assessments and ongoing multidisciplinary support until full self‐management is secured.

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Hemophilia prophylaxis adherence and bleeding using a tailored, frequency‐escalated approach: The Canadian Hemophilia Primary Prophylaxis Study

Dover

Blanchette

Wrathall

et al. 2020

Research and Practice in Thrombosis and Haemostasis

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Background Standard of care for persons with severe hemophilia A includes regular replacement of factor VIII (FVIII). Prophylaxis regimens using standard half‐life (SHL) FVIII concentrates, while effective, are costly and require frequent intravenous infusions. Aim This study evaluated the adherence of 56 boys with severe hemophilia A to tailored, frequency‐escalated prophylaxis with an SHL recombinant FVIII concentrate. Methods We reviewed the factor infusion and bleeding logs of study subjects. Adherence to the prescribed regimen was calculated on a weekly basis, and bleeding rates were determined from self/proxy‐reported bleeding logs. The primary outcome was adherence to the prescribed prophylaxis regimen. Results The median (range of values [ROV]) weekly adherence to prophylaxis was 85.7% (37.4%‐99.8%). The median (ROV) adherent weeks on steps 1 (weekly), 2 (twice weekly), and 3 (alternate‐day) were 92.9% (50%‐100%), 80.3 (32%‐96%), and 72.6% (14%‐98%); relative to step 1, subjects were less likely to be adherent on steps 2 and 3 (P < 0.00). On step 1, our cohort had higher adherence than previously reported rates. The median (ROV) adherence to the breakthrough bleeding protocol was 47.1% (0%‐100%). At any given time, bleeding risk was reduced by 15% for each 10% increase in adherence during the preceding 12 weeks (hazard ratio, 0.85; 95% confidence interval, 0.81‐0.90). Conclusion This cohort had high rates of adherence to the prescribed prophylaxis regimen. Initiating prophylaxis with once‐weekly infusions facilitated adherence to the prophylaxis regimen in this cohort of boys with severe hemophilia A started on primary prophylaxis at a very young age.

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Objective quantification of adherence to prophylaxis in haemophilia patients aged 12 to 25years and its potential association with bleeding episodes

Cited by 18 publications

References 22 publications

Effectiveness of the Medtep Hemophilia online platform for adherence to prophylactic treatment in haemophilia patients: Results from a 1‐year observational study

Effectiveness of the Medtep Hemophilia online platform for adherence to prophylactic treatment in haemophilia patients: Results from a 1‐year observational study

Adherence to prophylactic haemophilic treatment in young patients transitioning to adult care: A qualitative review

Hemophilia prophylaxis adherence and bleeding using a tailored, frequency‐escalated approach: The Canadian Hemophilia Primary Prophylaxis Study

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