Treatment adherence in adolescents with chronic diseases is around 50%, and failure is more common in preventive therapy. In haemophilia, contradictory results are reported by the published studies. The objective of this study was to evaluate adherence with factor VIII (FVIII) prophylaxis in Spanish patients with severe haemophilia A between age 6 and 20 years. Data were collected retrosp-ectively in the previous 2 years. The primary endpoint was the absolute adherence index (AAI), and the endpoints were related to clinical status, age, prophylaxis regimen, responsibility for factor administration and quality of life (QoL), assessed by the Haemo-QoL questionnaires. A total of 78 patients from 14 Spanish hospitals were recruited. Adherence ranged between -64.4 and 66.7 (mean -3.08). No differences were observed between children and adolescents (7.11 vs. 6.39; P = 0.809). A statistically significant association (P < 0.010) between infra adherent group and target joint was found, as was a statistically significant difference (P < 0.010) between the number of bleeding episodes experienced by the adherent group (mean 1.4) and by infra adherents (mean 4.5). There was no significant difference between AAI and prophylactic regimen (6.35 vs. 6.96, P = 0.848), neither between AAI and the person responsible for factor administration (5.57 vs. 8.79, P = 0.326). The Haemo-QoL scores (8-12 years) were related to adherence level (P < 0.05). Adherence was approximately ideal and patients perceived a high QoL. Because of the repercussions for compliance, it is essential to work during puberty on emotional and self-acceptance aspects of the disease, as well as coping, and the patient's family, school and health team relationships.

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Persons with haemophilia, living with their condition from infancy, require attention from a biopsychosocial approach, in which both the biological and the biographical dimension are addressed. These patients and their environment may benefit greatly from having professionals to help them manage, pre-emptively if possible, to adapt to the disease, cope with the experience of suffering and overcome the difficulties caused by chronicity. The ultimate goal of the interventions was to achieve the best quality of life possible with tailored objectives throughout the patient's life, including disease control, addressing the particular difficulties, and achieving optimal empowerment. This article aims to describe the role of Health Psychology and its professionals in supporting the young patient with haemophilia and provide a brief guide that might be useful for health professionals involved in his care. From the psychological perspective, this paper focuses on communication of diagnosis, the role and support of the family, issues during infancy, childhood and adolescence and how the healthcare team can address them to provide successful support.

Introduction More than half of adult patients with severe haemophilia (PWH) suffer pain daily, with chronic pain (CP) in more than 15% of cases, thereby reducing their quality of life (QoL). However, there are no evidence‐based therapeutic guidelines for pain management. Aim To evaluate the effectiveness of a combined protocol based on psychology and physiotherapy in the improvement of CP self‐efficacy in PWH with CP. Secondary outcomes are changes in QoL, emotional status, pain and kinesiophobia. Methods In this prospective controlled trial study, recruited patients were allocated either to an experimental group (EG, n = 10) or to a control group (CG, n = 9). EG received interventions over four months: one cognitive‐behavioural therapy (CBT) session per month and three home exercise sessions per week. Self‐efficacy (Chronic Pain Self‐Efficacy Scale), QoL (A36 Hemophilia‐QoL), emotional status (Hospital Anxiety and Depression Scale and Rosenberg's Self‐esteem Scale), pain (Visual Analogue Scale) and kinesiophobia (Tampa Scale for Kinesiophobia) were assessed at three time points (Week 0, Month 4 and Month 7). The intervention effects were determined with mixed 2‐factor ANOVAs. Results The EG showed a significant improvement (p < .05) in the control of symptoms and pain management scores on the Self‐Efficacy Scale, QoL, self‐esteem emotional status, pain and kinesiophobia. The intervention effects remained significant (p < .05) over time for pain management, QoL, pain and kinesiophobia. Conclusion The non‐pharmacological treatment applied based on CBT and physiotherapy showed to be effective in improving CP self‐efficacy, QoL and emotional status, while reducing pain and kinesiophobia in PWH with CP.