Psychosocial aspects of haemophilia: a systematic review of methodologies and findings

Abstract

Psychosocial factors have a significant impact on quality of life for patients with chronic diseases such as haemophilia. Interventions to support the psychosocial needs of patients and their families, such as offering information and assistance, clarifying doubts, and teaching coping strategies to minimize the impact of disabilities, may help to maximize patient outcomes and improve quality of life for their families. The aim of this study was to evaluate the current literature on psychosocial aspects of haemophilia. Literature searches were performed using the PubMed database to identify studies evaluating psychosocial stressors in persons with haemophilia. Articles pertaining to the HIV epidemic were excluded from the analysis, as were those published before 1997. The literature reviews identified 24 studies, covering a range of different populations, generally with small cohorts (n < 100). Most studies were questionnaire based, with almost no overlap in terms of the instruments used. Only one study combined questionnaire techniques with qualitative methods. Except for two European studies, all publications reported data from a single country. Overall, studies tended to show that quality of life is reduced in persons with haemophilia, with a potential impact on education and employment, particularly when prophylactic treatment is not available. Carrier status in women may have a psychosocial impact and affect reproductive choices. Data on psychosocial aspects of the haemophilia life cycle are lacking in the published literature, along with data from developing countries. There is a need for more international, multifaceted research to explore and quantify the social and psychological aspects of life with haemophilia.

Prophylaxis usage, bleeding rates, and joint outcomes of hemophilia, 1999 to 2010: a surveillance project

“…In addition to the enormous expense of replacement factor concentrate, hospitalizations, and joint replacements, the human cost of hemophilic arthropathy is loss of employment opportunities, less favorable insurance access, decreased social participation, and a high prevalence of depression. 3,4 Prophylaxis, a therapy that seeks to prevent bleeding events in hemophilia by routine replacement of deficient clotting factor, has been shown in a randomized clinical trial to prevent both joint hemorrhage and arthropathy when started prior to 30 months of age and prior to the occurrence of 3 hemorrhages into any single joint. 5 To date, clinical trial evidence supports the efficacy of initiation of prophylaxis after exceeding these thresholds in reducing the rate of joint hemorrhage in adolescents and adults with preexisting arthropathy, but has not provided definitive data on joint outcomes other than bleeding in patients beyond early childhood.…”

Calidad de vida: un aspecto olvidado en el paciente con hemofilia

“…• El último nivel es el desarrollo de estos sentimientos por un diagnóstico tardío, que lleva a mayores complicaciones y, por lo tanto, peor CVRS [9,29].…”

Joint Mobility and Physical Function of Danish Hemophilia Patients: A Three-Wave Panel Study Spanning 24 Years

“…Hence, the follow-up data on PWH ≥40 years are limited [17, 19]. A strong Dutch study evaluated trends in medical and social outcomes over 30 years (1972–2001) using 5 postal surveys and including 90% of the population of PWH [20, 21].…”