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Open Access
CC-BY 4.0 · TH Open 2018; 02(01): e54-e67
DOI: 10.1055/s-0038-1624568
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Sociodemographic, Clinical, and Psychosocial Characteristics of People with Hemophilia in Portugal: Findings from the First National Survey

Patrícia Ribeiro Pinto
1   Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal
2   ICVS/3B's – PT Government Associate Laboratory, Braga/Guimarães, Portugal
,
Ana Cristina Paredes
1   Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal
2   ICVS/3B's – PT Government Associate Laboratory, Braga/Guimarães, Portugal
,
Susana Pedras
3   School of Psychology, University of Minho, Braga, Portugal
,
Patrício Costa
1   Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal
2   ICVS/3B's – PT Government Associate Laboratory, Braga/Guimarães, Portugal
4   Faculty of Psychology and Education Sciences, University of Porto, Porto, Portugal
,
Miguel Crato
5   Portuguese Haemophilia Association, Lisbon, Portugal
6   European Haemophilia Consortium, Brussels, Belgium
,
Susana Fernandes
7   Department of Transfusion Medicine and Blood Bank, Centre of Hemophilia, Centro Hospitalar São João, Porto, Portugal
,
Manuela Lopes
7   Department of Transfusion Medicine and Blood Bank, Centre of Hemophilia, Centro Hospitalar São João, Porto, Portugal
,
Manuela Carvalho
7   Department of Transfusion Medicine and Blood Bank, Centre of Hemophilia, Centro Hospitalar São João, Porto, Portugal
,
Armando Almeida
1   Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal
2   ICVS/3B's – PT Government Associate Laboratory, Braga/Guimarães, Portugal
› Institutsangaben
Funding This work is supported by a grant from the Novo Nordisk HERO Research Grant 2015. P.R.P. has a grant (SFRH/BPD/103529/2014) from the Portuguese Foundation of Science and Technology.
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Publikationsverlauf

04. Oktober 2017

04. Dezember 2017

Publikationsdatum:
14. Februar 2018 (online)

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Abstract

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.

Keywords

hemophilia - pain - quality of life - functionality - psychological factors

Authors' Contributions

All authors had full access do data and participated in the preparation of this manuscript. All authors approved the final version and are accountable for any issue regarding the accuracy and integrity of the work.