Issues in the measurement of quality of life in hemophilia

Feldman, Brian M.

doi:10.5581/1516-8484.20130118

Cited by 5 publications

(6 citation statements)

References 19 publications

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“…In addition, the averaging of whole integers results in numerical scores that cannot distinguish between a patient who always has a problem on one item and a patient who occasionally has problems across many items, limiting the potential to analyze individual domains from a patient’s overall scores. 28 …”

Section: Discussionmentioning

confidence: 99%

Recombinant factor IX Fc prophylaxis reduces pain and increases levels of physical activity, with sustained, long-term improvements in patients with hemophilia B: post hoc analysis of phase III trials using patient-reported outcomes

Astermark

Hermans

Ezzalfani³

et al. 2023

Therapeutic Advances in Hematology

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Background: Pain is a common symptom of hemophilia that may adversely affect patients’ quality of life (QoL). Previous post hoc analyses of prophylaxis with recombinant factor IX Fc fusion protein (rFIXFc) have been published for adults and adolescents, demonstrating improvements in health-related QoL (HRQoL) when assessed by the haemophilia-specific QoL (HaemAQoL) questionnaire. Objective: To describe in depth the evolution of QoL, pain- and activity-related domains and questions for pediatric, adolescent, and adult patients with hemophilia B treated with rFIXFc prophylaxis. Design: A post hoc analysis of data from a series of clinical trials. Methods: This post hoc, long-term analysis assessed patient-reported outcomes (PROs) from the Kids B-LONG (NCT01440946: pediatric) and B-LONG (NCT01027364: adults and adolescents) parent studies and the B-YOND (NCT01425723: all age groups) extension study. Results: Ninety-two adult and adolescent patients that started in the B-LONG study were assessed, with a median (range) duration of follow-up of 58.9 (0.0–78.4) months. The Haem-A-QoL total score was significantly reduced from baseline by 4.45 ( p ⩽ 0.01), as were the subdomains ‘physical health’ (9.10; p = 0.001), ‘sports and leisure’ (11.25; p ⩽ 0.01), ‘treatment’ (2.69; p = 0.05), and ‘view of self’ (5.81; p = 0.002). Thirty pediatric patients that started in the Kids B-LONG study were assessed, with a median (min–max) duration of follow-up of 36.7 (9.0–59.9) months. The high level of satisfaction demonstrated by the PROs at baseline was maintained. Conclusion: rFIXFc prophylaxis reduced perceived pain and increased levels of physical activity with sustained, long-term improvements in QoL in adult and adolescent patients with hemophilia B and maintained high QoL scores in pediatric patients.

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Section: Discussionmentioning

confidence: 99%

Recombinant factor IX Fc prophylaxis reduces pain and increases levels of physical activity, with sustained, long-term improvements in patients with hemophilia B: post hoc analysis of phase III trials using patient-reported outcomes

Astermark

Hermans

Ezzalfani³

et al. 2023

Therapeutic Advances in Hematology

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“…Although the participation scale modified for this study was untested before our research, our tests for internal consistency met minimum recommended cutoffs. The modified scale addresses the personal and subjective nature of quality of life 28 and this feature was the primary reason why this scale was selected. Future work may include determining the psychometric properties of the scale for children.…”

Section: Discussionmentioning

confidence: 99%

“…Two questions relevant to North America were added (to explore if participants play games and use social networking sites as compared with their classmates). Grades of participation restriction were proportionately redefined as: no significant restriction (0-15), mild restriction (16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27), moderate restriction (28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40), severe restriction (41-66), and extreme restriction (67-115).…”

Section: With Permission From the Developers Of The Adult Participationmentioning

confidence: 99%

Social participation and hemophilia: Self‐perception, social support, and their influence on boys in Canada

Chiu

Blanchette

Barrera

et al. 2021

Research and Practice in Thrombosis and Haemostasis

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Background Patients with hemophilia may experience joint damage, which can impair participation, yet few studies have examined the impact hemophilia may have on social participation and quality of life. Objectives The aims of this study are to assess the relationship between patient social participation and self‐perception, social support, and impact on the family. Patients/Methods A random representative sample of 50 boys with hemophilia from The Hospital for Sick Children, Toronto, Canada, completed measures of social participation (Participation Scale for kids), self‐perception (Self‐Perception Profile for children and adolescents), and social support (Social Support Scale for children). Participants' parents completed Family Impact Module of the Pediatric Quality of Life Inventory. Data were analyzed using Pearson product‐moment correlations. Results Social participation was strongly correlated with self‐perception subscales Social Acceptance ( r = −0.5, p = <0.001) and Global Self‐Worth ( r = −0.6, p = <0.001) for all participants. The Athletic Competence subscale was strongly correlated for adolescents only ( r = −0.6, p = <0.01). There were strong correlations between social participation and social support from parents ( r = −0.6, p = <0.001), teachers ( r = −0.5, p = <0.001), and classmates ( r = −0.6, p = <0.001) and moderate correlations for support from close friends ( r = −0.4, p = <0.01). There were no significant correlations with family impact. Conclusion In the context of a country with unlimited access to safe clotting factor concentrates, boys with hemophilia have few social participation restrictions. Although correlational findings do not represent causality, they suggest that encouragement of social participation may be beneficial in boys with hemophilia to increase self‐perception as well as strengthen their social support network.

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“…Un adecuado conocimiento y entendimiento de que un niño con una condición como la hemofilia A tiene derecho a una buena calidad de vida es primordial. La atención de estos niños en la consulta de estomatología pediátrica debe estar orientada hacia un cuidado minucioso, profesional e interdisciplinario que no genere complicaciones médicas ni dolor innecesarios (1)(2)(3). Durante su ciclo vital, los niños con hemofilia A viven experiencias naturales, como la "exfoliación" de un diente temporal o el gateo, que deben transcurrir normalmente.…”

Section: Introductionunclassified

“…El cuidado de la salud de estos niños debe extremarse en la atención primaria (5), pues una hemorragia posterior a una intervención odontológica invasiva podría conducir a complicaciones graves, incluso a la muerte (6,7). De allí que alcanzar y mantener la salud bucal por medio de la prevención de enfermedades orales y adecuada nutrición es de suma importancia, no solo para la calidad de vida (1,2), sino también para evitar intervenciones odontológicas que conlleven riesgos inherentes que muchas veces podrían ser prevenibles (4). Con respecto al manejo interdisciplinario, debe existir una estrecha relación entre el oncohematólogo pediatra y el estomatólogo pediatra (4), a fin de proporcionar cuidados orales seguros e integrales, tanto en su atención ambulatoria como hospitalaria en los casos de hemofilia A moderada y grave, en el marco de los sistemas de seguridad social en salud (8,9).…”

Section: Introductionunclassified

Niños con hemofilia y su atención odontológica por estomatología pediátrica. Revisión de la literatura / Children with Hemophilia and their Dental Care by Pediatric Stomatology. Review of Literature

Ramírez

2016

Univ Odontol

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ABSTRACT. Purpose: To carry out systematic review of literature about diagnostics, severity, and dental treatment considerations of children with Hemophilia A. Methods: The search for articles and other document was conducted through the databases available at the National Library System of the National University of Colombia. The English and Spanish terms used in the search were “pediatric dentistry,” “hemophilia A,” dental care,” “coagulation factors,” and/or “coagulation disorders.” Results: The sample consisted of 29 articles that met the inclusion criteria. Hemophilia A is a congenital disorder caused by a deficiency of coagulation factor VIII. Treatment of hemophilia depends on its severity and focuses on avoiding risking life of children due to preventable bleeding by timely applying local therapies such as tranexamic acid or factor VIII, depending on dental needs. Dental treatment of hemophilia A should be interdisciplinary and must emphasize primary health care, health promotion, and prevention in order to avoid invasive dental treatments that could cause bleeding. In addition, treatments seek to maintain oral health and to provide quality of life to children living with hemophilia A.

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Issues in the measurement of quality of life in hemophilia

Cited by 5 publications

References 19 publications

Recombinant factor IX Fc prophylaxis reduces pain and increases levels of physical activity, with sustained, long-term improvements in patients with hemophilia B: post hoc analysis of phase III trials using patient-reported outcomes

Recombinant factor IX Fc prophylaxis reduces pain and increases levels of physical activity, with sustained, long-term improvements in patients with hemophilia B: post hoc analysis of phase III trials using patient-reported outcomes

Social participation and hemophilia: Self‐perception, social support, and their influence on boys in Canada

Niños con hemofilia y su atención odontológica por estomatología pediátrica. Revisión de la literatura / Children with Hemophilia and their Dental Care by Pediatric Stomatology. Review of Literature

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