Psychosocial Impact of Inhibitors on Haemophilia Patients' Quality of Life

Remor, Eduardo; Arranz, Pilar; Miller, Robin E.

doi:10.1002/9780470757260.ch30

Cited by 9 publications

(10 citation statements)

References 20 publications

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“…This is consistent with previous studies , who reported that mothers are usually concerned with the control and management of the disease, and therefore they suffer more anxiety and depression states than the fathers. We think this is one aspect to consider, and try as far as possible, to make it work more shared by both parents, and thus, to promote family functioning and good adaptation to the situation.…”

Section: Discussionsupporting

confidence: 93%

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

2014

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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.

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Section: Discussionsupporting

confidence: 93%

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

2014

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“…To date, much of the psychosocial evaluation in haemophilia has been a component of quality of life questionnaire‐based studies initiated in the 1990s [1,7–9]. Qualitative research from the World Federation of Hemophilia and other sources has highlighted the importance of data collected as a result of individual or group therapy, or through other psychotherapeutic interventions [3,4,10,11]. Different approaches to offering psychosocial support in PWH have been reviewed in several reports from various institutions.…”

Section: Introductionmentioning

confidence: 99%

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

Cassis¹,

Querol²,

Forsyth³

et al. 2011

Haemophilia

103

155

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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.

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“…The presence of inhibitors may adversely affect health‐related quality of life (6, 34). However, despite observational data suggesting that ITI can increase the risk of complications and burden on patients, the panel advised that the available literature provides no empirical data on the impact of ITI on psychological health or well‐being.…”

Section: The Burden Of Iti Therapy: Complications and Their Managementmentioning

confidence: 99%

Immune tolerance induction in patients with severe hemophilia with inhibitors: expert panel views and recommendations for clinical practice

Benson¹,

Auerswald

Elezović

et al. 2012

European J of Haematology

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Patients with congenital hemophilia require lifelong replacement therapy with a clotting factor concentrate: factor (F) VIII in hemophilia A and FIX in hemophilia B (1). However, patients can develop inhibitors to these exogenous factors, resulting in the most serious treatment related complication in hemophilia (2, 3). Once inhibitors have developed, it is more challenging to achieve hemostasis than in non-inhibitor patients. Furthermore , the presence of inhibitors has a major impact on patients' physical functioning, quality of life, morbidity , and mortality (4-6). In patients with high-titer inhibitors [ ‡5 Bethesda units (BU)], immune tolerance induction (ITI) aims to eradicate anamnestic inhibitors and restore normal responses to replacement therapy. The process of ITI involves regular infusion of FVIII or FIX concentrate with the goal Abstract For hemophilia patients with inhibitors, immune tolerance induction (ITI) may help to restore clinical response to factor (F) VIII or FIX concentrates. Several ITI regimens and protocols exist; however, despite 30 yr of progressive investigation, the ITI evidence base relies mainly on observational data. Expert opinion, experience, and interpretation of the available evidence are therefore valuable to support clinical decision-making. At the Sixth Zü rich Haemophilia Forum, an expert panel considered recent data and consensus to distill key practice points relating to ITI. The panel supported current recommendations that, where feasible, ITI should be offered early to children and adults (ideally £5 yr of inhibitor detection) when inhibitor titers are <10 Bethesda units (BU) and should be stopped when successful tolerance is achieved. For hemophilia A inhibitor patients, ITI can be founded on recombinant FVIII at high doses. The panel considered that patients with a high bleeding frequency should be offered additional prophylaxis with a bypassing agent. For patients with hemophilia B, there may be a benefit of genetic testing to indicate the risk for inhibitors. ITI is often less effective and associated with a greater risk of side effects in these patients. For high-titer inhibitor (‡5 BU) hemophilia B patients, the panel advised that bypassing agents could be offered on demand in addition to ITI. Within future ITI regimens, there may be a role for additional immunosuppressant therapies. Participants agreed that research is needed to find alternatives to ITI therapy that offer durable and sustained effects and reduced rates of complications.

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Psychosocial Impact of Inhibitors on Haemophilia Patients' Quality of Life

Cited by 9 publications

References 20 publications

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

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

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

Immune tolerance induction in patients with severe hemophilia with inhibitors: expert panel views and recommendations for clinical practice

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