Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey
Autor(a) principal: | |
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Data de Publicação: | 2018 |
Outros Autores: | , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/1822/57927 |
Resumo: | Hemophilia is a rare genetic bleeding disorder associated with pain, impaired functionality, and decreased quality of life (QoL). Several studies have focused on patient-reported outcomes of people with hemophilia (PWH) worldwide, but no such data are available for Portugal. This survey aimed to describe sociodemographic, clinical, and psychosocial characteristics of PWH of all ages in Portugal. Questionnaires were answered by self-report or by parents of children with hemophilia (proxy version). Variables assessed were sociodemographic and clinical, physical activity patterns, pain, functionality (HAL/PedHAL), QoL (A36 Hemofilia-QoL/CHO-KLAT), anxiety and depression (PROMIS), and illness perceptions (IPQ-R). One-hundred and forty-six PWH answered the survey: 106 adults, 21 children/teenagers between 10 and 17 years, 11 children between 6 and 9 years, and 8 children between 1 and 5 years. Most participants had severe hemophilia (60.3%) and type A was most commonly reported (86.3%). Bleeding episodes, joint deterioration, and pain were very prevalent, with the ankles and knees being the most affected joints, as illustrated by HAL/PedHAL scores. The A36 Hemofilia-QoL assessment showed moderate QoL (96.45; 0–144 scale) and significant anxiety and depression symptoms were found in 36.7 and 27.2% of adults, respectively. CHO-KLAT global score (0–100 scale) was 75.63/76.32 (self-report/proxy). Concerning hemophilia-related illness beliefs, a perception of chronicity and symptoms unpredictability was particularly prominent among adults and children/teenagers. This survey provided a comprehensive characterization of Portuguese PWH, including the first report of psychosocial characteristics. The findings allow for a deeper understanding of life with hemophilia in Portugal and the identification of relevant health care and research needs |
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Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national surveyHemophiliaPainQuality of lifeFunctionalityPsychological factorsCiências Médicas::Medicina BásicaHemophilia is a rare genetic bleeding disorder associated with pain, impaired functionality, and decreased quality of life (QoL). Several studies have focused on patient-reported outcomes of people with hemophilia (PWH) worldwide, but no such data are available for Portugal. This survey aimed to describe sociodemographic, clinical, and psychosocial characteristics of PWH of all ages in Portugal. Questionnaires were answered by self-report or by parents of children with hemophilia (proxy version). Variables assessed were sociodemographic and clinical, physical activity patterns, pain, functionality (HAL/PedHAL), QoL (A36 Hemofilia-QoL/CHO-KLAT), anxiety and depression (PROMIS), and illness perceptions (IPQ-R). One-hundred and forty-six PWH answered the survey: 106 adults, 21 children/teenagers between 10 and 17 years, 11 children between 6 and 9 years, and 8 children between 1 and 5 years. Most participants had severe hemophilia (60.3%) and type A was most commonly reported (86.3%). Bleeding episodes, joint deterioration, and pain were very prevalent, with the ankles and knees being the most affected joints, as illustrated by HAL/PedHAL scores. The A36 Hemofilia-QoL assessment showed moderate QoL (96.45; 0–144 scale) and significant anxiety and depression symptoms were found in 36.7 and 27.2% of adults, respectively. CHO-KLAT global score (0–100 scale) was 75.63/76.32 (self-report/proxy). Concerning hemophilia-related illness beliefs, a perception of chronicity and symptoms unpredictability was particularly prominent among adults and children/teenagers. This survey provided a comprehensive characterization of Portuguese PWH, including the first report of psychosocial characteristics. The findings allow for a deeper understanding of life with hemophilia in Portugal and the identification of relevant health care and research needsNovo Nordisk HERO Research Grant 2015. P.R.P. has a grant (SFRH/BPD/103529/2014) from the Portuguese Foundation of Science and Technologyinfo:eu-repo/semantics/publishedVersionThieme Medical PublishersUniversidade do MinhoPinto, Patrícia Jesus RibeiroParedes, Ana Cristina MarquesPedras, SusanaCosta, Patrício SoaresCrato, MiguelFernandes, SusanaLopes, ManuelaCarvalho, ManuelaAlmeida, Armando20182018-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/1822/57927engPinto, P. R., Paredes, A. C., Pedras, S., Costa, P., et. al.(2018). Sociodemographic, Clinical, and Psychosocial Characteristics of People with Hemophilia in Portugal: Findings from the First National Survey. TH Open, 2(01), e54-e672512-946510.1055/s-0038-1624568https://www.thieme-connect.com/products/ejournals/abstract/10.1055/s-0038-1624568info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-07-21T12:49:35Zoai:repositorium.sdum.uminho.pt:1822/57927Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T19:48:04.396007Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey |
title |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey |
spellingShingle |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey Pinto, Patrícia Jesus Ribeiro Hemophilia Pain Quality of life Functionality Psychological factors Ciências Médicas::Medicina Básica |
title_short |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey |
title_full |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey |
title_fullStr |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey |
title_full_unstemmed |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey |
title_sort |
Sociodemographic, clinical, and psychosocial characteristics of people with hemophilia in Portugal: findings from the first national survey |
author |
Pinto, Patrícia Jesus Ribeiro |
author_facet |
Pinto, Patrícia Jesus Ribeiro Paredes, Ana Cristina Marques Pedras, Susana Costa, Patrício Soares Crato, Miguel Fernandes, Susana Lopes, Manuela Carvalho, Manuela Almeida, Armando |
author_role |
author |
author2 |
Paredes, Ana Cristina Marques Pedras, Susana Costa, Patrício Soares Crato, Miguel Fernandes, Susana Lopes, Manuela Carvalho, Manuela Almeida, Armando |
author2_role |
author author author author author author author author |
dc.contributor.none.fl_str_mv |
Universidade do Minho |
dc.contributor.author.fl_str_mv |
Pinto, Patrícia Jesus Ribeiro Paredes, Ana Cristina Marques Pedras, Susana Costa, Patrício Soares Crato, Miguel Fernandes, Susana Lopes, Manuela Carvalho, Manuela Almeida, Armando |
dc.subject.por.fl_str_mv |
Hemophilia Pain Quality of life Functionality Psychological factors Ciências Médicas::Medicina Básica |
topic |
Hemophilia Pain Quality of life Functionality Psychological factors Ciências Médicas::Medicina Básica |
description |
Hemophilia is a rare genetic bleeding disorder associated with pain, impaired functionality, and decreased quality of life (QoL). Several studies have focused on patient-reported outcomes of people with hemophilia (PWH) worldwide, but no such data are available for Portugal. This survey aimed to describe sociodemographic, clinical, and psychosocial characteristics of PWH of all ages in Portugal. Questionnaires were answered by self-report or by parents of children with hemophilia (proxy version). Variables assessed were sociodemographic and clinical, physical activity patterns, pain, functionality (HAL/PedHAL), QoL (A36 Hemofilia-QoL/CHO-KLAT), anxiety and depression (PROMIS), and illness perceptions (IPQ-R). One-hundred and forty-six PWH answered the survey: 106 adults, 21 children/teenagers between 10 and 17 years, 11 children between 6 and 9 years, and 8 children between 1 and 5 years. Most participants had severe hemophilia (60.3%) and type A was most commonly reported (86.3%). Bleeding episodes, joint deterioration, and pain were very prevalent, with the ankles and knees being the most affected joints, as illustrated by HAL/PedHAL scores. The A36 Hemofilia-QoL assessment showed moderate QoL (96.45; 0–144 scale) and significant anxiety and depression symptoms were found in 36.7 and 27.2% of adults, respectively. CHO-KLAT global score (0–100 scale) was 75.63/76.32 (self-report/proxy). Concerning hemophilia-related illness beliefs, a perception of chronicity and symptoms unpredictability was particularly prominent among adults and children/teenagers. This survey provided a comprehensive characterization of Portuguese PWH, including the first report of psychosocial characteristics. The findings allow for a deeper understanding of life with hemophilia in Portugal and the identification of relevant health care and research needs |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018 2018-01-01T00:00:00Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/1822/57927 |
url |
http://hdl.handle.net/1822/57927 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Pinto, P. R., Paredes, A. C., Pedras, S., Costa, P., et. al.(2018). Sociodemographic, Clinical, and Psychosocial Characteristics of People with Hemophilia in Portugal: Findings from the First National Survey. TH Open, 2(01), e54-e67 2512-9465 10.1055/s-0038-1624568 https://www.thieme-connect.com/products/ejournals/abstract/10.1055/s-0038-1624568 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Thieme Medical Publishers |
publisher.none.fl_str_mv |
Thieme Medical Publishers |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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