The behaviour and perception of illness: modulating variables of adherence in patients with haemophilia.

BACKGROUND: The recommended treatment in patients with severe haemophilia is prophylaxis. This prevents bleedings and reduces or delays the haemophilic arthropathy. Adherence defined as the follow-up of the patient to the prescriptions of the health professional to his treatment, both pharmacological and modifications in habits or stylelifes. Adherence rates in haemophilia are better than in other chronic diseases, but there remain reasons for nonadherence. The aim was to see if disease behaviour and disease perception together with coping strategies modulate adherence to treatment. MATERIAL AND METHODS: Multicenter cross-sectional descriptive study. Patients recruited in Units of Hemophilia Hospitals and Regional Haemophilia Associations. Twenty-three patients participated The instruments used are as follows: data sheet, SF-36, A36 Hemophilia-Qol, Illness Perception Questionnaire-Revised (IPQ-R), Illness Behavior Questionnaire (IBQ) and Coping Strategies Inventory (CSI) and VERITAS-Pro. RESULTS: We found that 56% of patients with haemophilia are adherent. The most relevant clinical variables were hemarthrosis, diagnosis of arthropathy and presence of any coinfection, as well as family history, labour status and sociodemographic variables. Age was not a relevant variable in adherence. The worst adherence coincides with poorer quality of life and inadequate coping style. Nonadherent patients show greater conviction of disease, perception of cyclicity, more hypochondriacal behaviour towards the illness and perception concern about possible side effects of medication. CONCLUSION: Adherence in haemophilia requires a more experiential approach and individualized education. It is important that professionals take into account the patient's perception, behaviour and attitude towards the disease to achieve a better adaptation of their coping resources.

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

INTRODUCTION: Medtep Hemophilia platform is an online tool that allows patients with congenital coagulopathies to keep track of their daily condition-related events with the objective of ensuring successful adherence to therapy. AIM: To assess the effectiveness of Medtep Hemophilia in improving adherence to prophylactic treatment in haemophilia A and B patients in a 1-year prospective observational study, as well as its impact on the patient's disease status. METHODS: Patients (>13 years old) received support material to familiarize themselves with Medtep Hemophilia. Adherence to treatment, quality of life (QoL) and illness perception were assessed. Values at baseline, 1, 6 and 12 months, and changes from baseline value were analysed. The Hemophilia Joint Health Score (HJHS) test was applied at baseline and study completion. RESULTS: Forty-six patients were enrolled (43 evaluable). After 1 year, 56.4% patients showed continued use of the platform (100% compliance) whereas 25.6% were inactive. Treatment adherence increased both significantly (P < .001) and progressively during the study. Similarly, improved QoL and illness perception were observed with respect to baseline in most of the questionnaire components (P < .05 after 12 months). A patient's age had no influence on the results, whereas compliant patients (>80% of platform use) tended to score better than noncompliant. The HJHS test values remained similar during the study. CONCLUSION: The Medtep Hemophilia online platform helped the studied patients with haemophilia to improve their adherence to prophylactic treatment, while increasing their QoL and illness perception, as well as joint arthropathies stabilization.

Balance evaluation in haemophilic preadolescent patients using Nintendo Wii Balance Board®.

INTRODUCTION: Alterations in the musculoskeletal system, especially in the lower limbs, limit physical activity and affect balance and walking. Postural impairments in haemophilic preteens could increase the risk of bleeding events and deteriorate the physical condition, promoting the progression of haemophilic arthropathy. AIM: This study aims to evaluate static postural balance in haemophilic children, assessed by means of the Wii Balance Board® (WBB). METHODS: Nineteen children with haemophilia and 19 without haemophilia aged 9-10 years, have participated in this study. Postural balance was assessed by performing four tests, each one lasting 15 s: bipodal eyes open (BEO), bipodal eyes closed (BEC), monopodal dominant leg (MD) and monopodal non-dominant leg (MND). Two balance indices, standard deviation of amplitude (SDA) and standard deviation of velocity (SDV) were calculated in the anterior-posterior (AP) and medial-lateral (ML) directions. RESULTS: Index values were higher in haemophilic group and the differences were statistically significant (P < 0.05) in only six (SDAAP in BEO, BEC and MD conditions, SDAML in BEO, SDVAP in BEO and SDVML in MND condition) of 16 parameters analysed. CONCLUSION: Tests performed indicate a poorer static postural balance in the haemophilic cohort compared to the control group. Accordingly, physiotherapy programmes, physical activity and sports should be designed to improve the postural balance with the aim of preventing joint deterioration and improving quality of life.

Parents of children with haemophilia at an early age: assessment of perceived stress and family functioning.

Haemophilia is a chronic disease that requires a multidisciplinary approach for proper management and control of its clinical manifestations. The perception and management of parents of children with haemophilia can be affected by stressful situations as a result of treatment or disease progression. The aim of this study was to evaluate the perception of stress and family functioning in parents of children with haemophilia 1-7 years. This is an observational clinical study involving 49 parents of children with haemophilia 1-7 years who attended the VIII Workshop for Parents of Children with haemophilia, organized by the Spanish Federation of Hemophilia in La Charca, Murcia (Spain). After obtaining parental consent, the questionnaires was applied to them, FACES III (family functioning) and Pediatric Inventory for Parents (perceived stress), and a record of data on the clinical characteristics and treatment. Significant differences in the perception of stressors by gender of parents were found. A family history of haemophilia, the use of port-a-cath, inhibitor development and gender of the parents were the descriptive variables most correlated with dependents variables. These variables, together with the type of haemophilia affect significantly in the parental stress and family functioning. Parents have difficulty adjusting to disease management, perceiving many stressors. Gender and family history, can hinder the proper compliance with treatments, reducing its effectiveness.

Effectiveness of an educational intervention of physiotherapy in parents of children with haemophilia.

Haemophilia is a haematological disorder with an orthopaedic outcome. It requires not only medical but rather comprehensive care from infancy. The aim of this study was to assess the effectiveness of an educational intervention of Physiotherapy in parents of children with haemophilia under 4 years old. This is a non-randomized clinical trial, in which 22 parents participated children's with haemophilia under 4 years old. Half of them received an educational intervention of Physiotherapy. At the beginning and end of the study, a psychologist blinded to the assignment of subjects to each of the study groups, assessed the perceived quality of life, anxiety, perceived stress and family functioning of parents. A significant improvement was observed in the variables of perceived stress and family functioning of parents in the experimental group. The realization of an educational intervention in parents of children with haemophilia under 4 years old is effective. It reduces the stressors perceived by the parents and improves family cohesion and adaptability, as a consequence of the disease. It is necessary to carry out studies with follow-up periods to assess the effectiveness of educational programs of Physiotherapy for long term.

Effectiveness of two modalities of physiotherapy in the treatment of haemophilic arthropathy of the ankle: a randomized pilot study.

Although different techniques of physiotherapy have been described for the treatment of haemophilic arthropathy (HA) of ankle, hardly any studies have been applied manual therapy or educational physiotherapy and home exercises. The aim of this study was to assess the effectiveness of manual therapy and educational physiotherapy in the treatment of HA of the ankle. Thirty-one patients with HA of the ankle with a mean age of 35.29 (SD: 12.877) years randomized to manual therapy group (n = 11), educational group (n = 10) and a control group (n = 10). The two physiotherapy programmes were one with manual therapy articular traction, passive stretching of the gastrocnemius muscles, and exercises for muscle strength and proprioception (MT group) and the other with educational sessions and home exercises (E group). The study lasted for 12 weeks. The treatment with manual therapy improved the gastrocnemius muscle circumference, and the pain of ankle (P < 0.05). Six months later, MT group still enjoyed improvement. In the educational group there were improvements, but not significant, in the measured variables. No patient had ankle haemarthrosis during the study. The treatment with manual therapy improved the circumference of gastrocnemius and lessened pain in the patients with haemophilic arthropathy of the ankle.