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BackgroundIn pediatric hemophilia, caregivers are facing unique challenges to adherence and self-care in children and adolescents with hemophilia. Hemophilia treatment requires adequate prophylaxis and on-demand treatment, as well as a clear behavioral strategy to limit risk-taking in terms of physical exercise and diet. Medication adherence rates of hemophilia patients have been reported to decrease during late childhood and adolescence. In the developing child, moving safely from parent-care to self-care is one of the greatest challenges of integrative care within this domain. There is a clear need for initiatives designed to increase an individual’s motivation for treatment and self-care activities.DiscussionAmong motivational approaches, the self-determination perspective offers a useful framework to explain how the transition to self-care can be facilitated. We discuss how motivation regarding hemophilia treatment may be increased through parental autonomy support and we offer examples of applied communication techniques to facilitate autonomy-supportive caregiving. Although it has not yet been tested in the context of hemophilia, these communication techniques could potentially help caregivers promote adherence and self-care in children.SummaryConfronted by unique challenges to adherence and self-care, caregivers of children with hemophilia should move from an exclusive focus on illness-management education to an integrative strategy, including motivation-enhancing communication. The self-determination perspective provides important proximal objectives (e.g. autonomy support) to maintain optimal adherence in adolescents as they move from parent-care to self-care. Future research initiatives should address the practice of these communication techniques and evaluate them in the context of hemophilia.

Background: Hemophilia is a rare bleeding disorder in which illness perceptions have seldom been studied. Illness perceptions are important in predicting patients' behavior. Due to the risk of bleeding and joint damage, healthcare professionals often discourage some physical activities. Those restrictions can be difficult to follow for pediatric patients. This study investigates the relationship between illness perceptions, physical activity behavior, and intentions amongst this population. Methods: Twenty-four 6-18-year-old patients with severe hemophilia completed the Brief Illness Perception Questionnaire. A questionnaire assessing their level of physical activity and intentions toward safe and higher-risk physical activity was also administered. Clinical and sociodemographic data were collected. Associations were studied using hierarchical clustering of physical activity patterns, and Mann-Whitney U comparisons between clusters. Results: Perceptions ranged from slightly to moderately threatening, and 20-30% of participants had highly threatening perceptions on Consequences, Identity, Concern, and Emotional response. The subgroup who engaged in more high-risk physical activity and had stronger intentions to engage in this type of activity also held more concerns and perceived more symptoms. Conclusion: Patients at risk of non-adherence to recommendations concerning physical activity have more threatening illness beliefs that could be addressed during specific interventions or routine appointments. Addressing illness beliefs could be an element to behavior change. Strong emotional responses to hemophilia also stresses the need for psychosocial support strategies.

The objective of this study was to identify psychosocial factors to explain intentions of children and adolescents with hemophilia to adhere to recommendations for self-care. Twenty-four patients with hemophilia A and B, aged 6-18 years old, and their parents, completed a survey. Measures assessed factors from the theory of planned behavior, physical activity, and medical treatment adherence. The results indicate that past behaviors, attitudes, and subjective norms explained a large proportion of the intention to engage in future nonrecommended physical activity. This study supports the need to investigate motivational factors underlying behaviors in larger scale studies and identifies targets for future interventions.