Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency
Skye Peltier,1 Angela Kellum,2 Janet Brewer,3 Alexander Duncan,4 David L Cooper,5 Hossam Saad5 1Center for Bleeding and Clotting Disorders, University of Minnesota Medical Center - Fairview, Minneapolis, MN, USA; 2Louisiana Center for Bleeding and Clotting Disorders, Tulane University, New Orleans,...
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| Format: | Article |
| Language: | English |
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Dove Medical Press
2020-09-01
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| Series: | Journal of Blood Medicine |
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| Online Access: | https://www.dovepress.com/psychosocial-impact-and-disease-management-in-patients-with-congenital-peer-reviewed-article-JBM |
| _version_ | 1818159134514610176 |
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| author | Peltier S Kellum A Brewer J Duncan A Cooper DL Saad H |
| author_facet | Peltier S Kellum A Brewer J Duncan A Cooper DL Saad H |
| author_sort | Peltier S |
| collection | DOAJ |
| description | Skye Peltier,1 Angela Kellum,2 Janet Brewer,3 Alexander Duncan,4 David L Cooper,5 Hossam Saad5 1Center for Bleeding and Clotting Disorders, University of Minnesota Medical Center - Fairview, Minneapolis, MN, USA; 2Louisiana Center for Bleeding and Clotting Disorders, Tulane University, New Orleans, LA, USA; 3Comprehensive Health Education Services, Hanson, MA, USA; 4Department of Pathology & Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA; 5Novo Nordisk Inc., Plainsboro, NJ, USACorrespondence: Skye PeltierCenter for Bleeding and Clotting Disorders, University of Minnesota Medical Center – Fairview, Minneapolis, MN, USATel +1 612-273-5047Fax +1 612-273-5018Email speltie1@fairview.orgPurpose: Congenital factor VII (FVII) deficiency is a rare bleeding disorder of variable phenotype with predominantly mucocutaneous bleeding. The aim of this study was to identify the burden of FVII deficiency on patients and caregivers through a better understanding of the management and psychosocial impact of this disease.Materials and Methods: A rare disease specialty recruiter from Comprehensive Health Education Services recruited participants for this online survey, which was conducted from January 31 to March 12, 2019. A moderator-assisted questionnaire was used to collect data on demographics, diagnosis, treatment, and psychosocial impact.Results: Of the 45 respondents (25 patients and 20 caregivers), the majority were female (56%). Respondents reported a wide variety of initial bleeding symptoms, including bruising (58%), epistaxis (56%), and menorrhagia (36% of females). Because symptoms varied between individuals and were not always severe, diagnosis was often delayed. Mean time to obtain a diagnosis was 6.5 years and mean age at first diagnosis was 12.9 years. One-quarter (24%) of the respondents reported more than 100 bleeds of any severity over the previous year. When treating bleeds, 44% of patients reported using antifibrinolytics, and 42% reported using recombinant activated factor VII. Almost 31% of respondents reported missing schooldays as children, and 16% reported losing or resigning from a job in adulthood as a direct result of their disease. Notably, 29% of caregivers and 10% of their partners had also experienced issues with employment. Forty percent of respondents reported not participating in contact sports during childhood, and 22% continued to avoid contact sports in adulthood.Conclusion: Overall, FVII deficiency has a substantial psychosocial impact, but most patients are satisfied with their disease management and are optimistic about their future. Patients desire additional educational, social, and financial support.Keywords: bleeding disorder, psychosocial impact, recombinant activated factor VII, survey |
| first_indexed | 2024-12-11T15:41:09Z |
| format | Article |
| id | doaj.art-53c80aae28914f70872ba27d38dc3991 |
| institution | Directory Open Access Journal |
| issn | 1179-2736 |
| language | English |
| last_indexed | 2024-12-11T15:41:09Z |
| publishDate | 2020-09-01 |
| publisher | Dove Medical Press |
| record_format | Article |
| series | Journal of Blood Medicine |
| spelling | doaj.art-53c80aae28914f70872ba27d38dc39912022-12-22T00:59:48ZengDove Medical PressJournal of Blood Medicine1179-27362020-09-01Volume 1129730356967Psychosocial Impact and Disease Management in Patients with Congenital Factor VII DeficiencyPeltier SKellum ABrewer JDuncan ACooper DLSaad HSkye Peltier,1 Angela Kellum,2 Janet Brewer,3 Alexander Duncan,4 David L Cooper,5 Hossam Saad5 1Center for Bleeding and Clotting Disorders, University of Minnesota Medical Center - Fairview, Minneapolis, MN, USA; 2Louisiana Center for Bleeding and Clotting Disorders, Tulane University, New Orleans, LA, USA; 3Comprehensive Health Education Services, Hanson, MA, USA; 4Department of Pathology & Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA; 5Novo Nordisk Inc., Plainsboro, NJ, USACorrespondence: Skye PeltierCenter for Bleeding and Clotting Disorders, University of Minnesota Medical Center – Fairview, Minneapolis, MN, USATel +1 612-273-5047Fax +1 612-273-5018Email speltie1@fairview.orgPurpose: Congenital factor VII (FVII) deficiency is a rare bleeding disorder of variable phenotype with predominantly mucocutaneous bleeding. The aim of this study was to identify the burden of FVII deficiency on patients and caregivers through a better understanding of the management and psychosocial impact of this disease.Materials and Methods: A rare disease specialty recruiter from Comprehensive Health Education Services recruited participants for this online survey, which was conducted from January 31 to March 12, 2019. A moderator-assisted questionnaire was used to collect data on demographics, diagnosis, treatment, and psychosocial impact.Results: Of the 45 respondents (25 patients and 20 caregivers), the majority were female (56%). Respondents reported a wide variety of initial bleeding symptoms, including bruising (58%), epistaxis (56%), and menorrhagia (36% of females). Because symptoms varied between individuals and were not always severe, diagnosis was often delayed. Mean time to obtain a diagnosis was 6.5 years and mean age at first diagnosis was 12.9 years. One-quarter (24%) of the respondents reported more than 100 bleeds of any severity over the previous year. When treating bleeds, 44% of patients reported using antifibrinolytics, and 42% reported using recombinant activated factor VII. Almost 31% of respondents reported missing schooldays as children, and 16% reported losing or resigning from a job in adulthood as a direct result of their disease. Notably, 29% of caregivers and 10% of their partners had also experienced issues with employment. Forty percent of respondents reported not participating in contact sports during childhood, and 22% continued to avoid contact sports in adulthood.Conclusion: Overall, FVII deficiency has a substantial psychosocial impact, but most patients are satisfied with their disease management and are optimistic about their future. Patients desire additional educational, social, and financial support.Keywords: bleeding disorder, psychosocial impact, recombinant activated factor VII, surveyhttps://www.dovepress.com/psychosocial-impact-and-disease-management-in-patients-with-congenital-peer-reviewed-article-JBMbleeding disorderpsychosocial impactrecombinant activated factor viisurvey |
| spellingShingle | Peltier S Kellum A Brewer J Duncan A Cooper DL Saad H Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency Journal of Blood Medicine bleeding disorder psychosocial impact recombinant activated factor vii survey |
| title | Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency |
| title_full | Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency |
| title_fullStr | Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency |
| title_full_unstemmed | Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency |
| title_short | Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency |
| title_sort | psychosocial impact and disease management in patients with congenital factor vii deficiency |
| topic | bleeding disorder psychosocial impact recombinant activated factor vii survey |
| url | https://www.dovepress.com/psychosocial-impact-and-disease-management-in-patients-with-congenital-peer-reviewed-article-JBM |
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