Improving comprehensive care in the haemophilia community: building on the <scp>HERO</scp> Study

Buzzi, A.; Kelley, L. A.; Gregory, Maggie; Skinner, Mark W; Kalnins, W.

doi:10.1111/hae.12945

Cited by 3 publications

(2 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The psychosocial question battery was based largely on those developed for the HERO study by an international expert panel, informed initially by a literature review13 and qualitative interactive survey14,15 that resulted in the 10 country qualitative survey instruments 8. Additional clarifying questions were added based upon issues raised by expert panel and authors (eg, impact of prior relationships and disclosure)16 and feedback from global and regional HERO summit 17. Additionally, five PRO instruments were administered: EQ-5D-5L with visual analog scale (VAS), BPI, HAL, PHQ-9 (completed by both adults with hemophilia B and caregivers of children with hemophilia B), and GAD-7 (completed by caregivers only).…”

Section: Methodsmentioning

confidence: 99%

<p>Correlations between patient-reported outcomes and self-reported characteristics in adults with hemophilia B and caregivers of children with hemophilia B: analysis of the B-HERO-S study</p>

Buckner¹,

Sidonio²,

Witkop³

et al. 2019

PROM

View full text Add to dashboard Cite

PurposePain, anxiety, depression, and other aspects of health-related quality of life (HRQoL) are important issues for people with hemophilia and caregivers of children with hemophilia. Patient-reported outcome (PRO) instruments may be used to assess aspects of HRQoL; however, the use of PROs in clinical management of patients with hemophilia is limited and inconsistent. The Bridging Hemophilia B Experiences, Results and Opportunities Into Solutions (B-HERO-S) study evaluated the impact of hemophilia B on HRQoL and other psychosocial aspects in affected adults and caregivers of children with hemophilia B. This post hoc analysis assessed correlations between PRO scores and psychosocial questions commonly asked in comprehensive care settings among B-HERO-S respondents.Patients and methodsB-HERO-S consisted of two online surveys, one administered to adults with hemophilia B (n=299) and one administered to caregivers of children with hemophilia B (n=150). The adult survey included EQ-5D-5L with visual analog scale, BPI, HAL, and PHQ-9. The caregiver survey included PHQ-9 and GAD-7. Questions related to demographics, hemophilia treatment, and psychosocial questions asked in comprehensive care visits were also included in the surveys. A post hoc analysis was performed to assess correlations between responses to selected psychosocial questions with PRO scores.ResultsFor adults with hemophilia B, greater pain severity and pain interference scores were associated with work-related problems, functional limitations, and relationship, psychological, and treatment issues. Significant correlations were also noted between some of these psychosocial outcomes and depressive symptoms. For caregivers, greater depression and anxiety were associated with employment issues, their child’s functional, relationship, and psychological issues, having had difficulty or concerns with treatment/factor availability or affordability, and having less frequent HTC visits.ConclusionHigh correlations were observed between PRO scores measuring pain, depression, and anxiety and questions commonly used in the comprehensive care setting to assess the psychosocial impact of hemophilia.

show abstract

Section: Methodsmentioning

confidence: 99%

<p>Correlations between patient-reported outcomes and self-reported characteristics in adults with hemophilia B and caregivers of children with hemophilia B: analysis of the B-HERO-S study</p>

Buckner¹,

Sidonio²,

Witkop³

et al. 2019

PROM

View full text Add to dashboard Cite

show abstract

“…While psychosocial issues and the need for comprehensive care in congenital hemophilia have been extensively studied, 24 – 30 the need for comprehensive management of patients with congenital FVII deficiency is no less important. Even within the HTC networks, experience with moderate-to-severe FVII deficiency is variable.…”

Section: Discussionmentioning

confidence: 99%

Phenotypical variability in congenital FVII deficiency follows the ISTH-SSC severity classification guidelines: a review with illustrative examples from the clinic

Jain¹,

Donkin²,

Frey³

et al. 2018

JBM

View full text Add to dashboard Cite

BackgroundOne of the most common rare inherited bleeding disorders, congenital factor VII (FVII) deficiency typically has a milder bleeding phenotype than other rare bleeding disorders. Categorizing severity in terms of factor activity associated with hemophilia (severe <1%, moderate 1%–5%, mild 6%–40%) has led to the observation that bleeding phenotype does not follow closely with FVII activity. Over the past decade, large-scale global registries have investigated bleeding phenotype more thoroughly. The International Society on Thrombosis and Haemostasis has reclassified FVII deficiency as follows: severe, FVII <10%, risk of spontaneous major bleeding; moderate, FVII 10%–20%, risk of mild spontaneous or triggered bleeding; mild, FVII 20%–50%, mostly asymptomatic disease.Case reportsEleven illustrative cases of congenital FVII deficiency adapted from clinical practice are described to demonstrate the variability in presentation and in relation to FVII activity levels. Severe FVII deficiency usually presents at a young age and carries the risk of intracranial hemorrhage, hemarthrosis, and other major bleeds. Moderate FVII deficiency tends to present later, often in adolescence and particularly in girls as they reach menarche. Milder disease may not be apparent until found incidentally on preoperative testing, during pregnancy/childbirth, or following unexplained bleeding when faced with hemostatic challenges.ConclusionIt is important for health care professionals to be aware of the new definitions of severity and typical presentations of congenital FVII deficiency. Failure to appreciate the risks of major bleeding, including intracerebral hemorrhage in those with FVII activity <10%, may put particularly young children at risk.

show abstract

Comprehensive care for haemophilia: A literature review for improving institutional cooperation

Brondi

Palareti

Mazzetti

2019

International Journal of Healthcare Management

View full text Add to dashboard Cite

This paper investigates how the notion of comprehensive care (CC) in haemophilia is proposed in institutional guidelines and specialised understandings. A literature review of the articles published in Haemophilia was carried. The main features which authors refer to when mentioning CC were outlined and compared with the principles, assumptions and recommendations set by the World Federation of Haemophilia in relation to this care model.The results show a fragmented scenario. First, very few articles are devoted or explicitly refer to CC. Second, only a few of them completely adhere to the notion of CC as proposed in institutional guidelines. In contrast, most articles stress a single feature, providing a partial view of the model. The focus is mainly on promoting physical health, which is however frequently associated with psychosocial health. Multidisciplinarity has great emphasis, too.Third, some key issues for CC emerge. They are implicitly present in institutional guidelines, but they become the very focus of some articles, which reflect on three challenges to be dealt 2 with: care for all, transition care and systemic care. Such challenges offer the opportunity to clarify and expand the notion of haemophilia CC, and to propose potential future developments in research designs and interventions.

show abstract

Improving comprehensive care in the haemophilia community: building on the HERO Study

Abstract: References

Cited by 3 publications

References 4 publications

<p>Correlations between patient-reported outcomes and self-reported characteristics in adults with hemophilia B and caregivers of children with hemophilia B: analysis of the B-HERO-S study</p>

<p>Correlations between patient-reported outcomes and self-reported characteristics in adults with hemophilia B and caregivers of children with hemophilia B: analysis of the B-HERO-S study</p>

Phenotypical variability in congenital FVII deficiency follows the ISTH-SSC severity classification guidelines: a review with illustrative examples from the clinic

Comprehensive care for haemophilia: A literature review for improving institutional cooperation

Contact Info

Product

Resources

About