Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress

Abstract Health‐related quality of life (HRQOL) is reduced in Fabry disease (FD) and associated with clinical disease manifestations, but few have used Fabry‐specific severity scores to study how disease burden interferes with quality of life. We investigated how the Fabry DS3, consisting of four so...

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Autores principales:	Hege Kampen Pihlstrøm, Mina Susanne Weedon‐Fekjær, Birgitte Leisner Bjerkely, Charlotte von derLippe, Kristin Ørstavik, Per Mathisen, Ketil Heimdal, Trond Geir Jenssen, Dag Olav Dahle, Olga Karin Solberg, Solrun Sigurdardottir
Formato:	article
Lenguaje:	EN
Publicado:	Wiley 2021
Materias:	depression DS3 Fabry fatigue HRQOL pain Diseases of the endocrine glands. Clinical endocrinology RC648-665 Genetics QH426-470
Acceso en línea:	https://doaj.org/article/f083ac0f662c49bfbb2568975ffba424
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spelling	oai:doaj.org-article:f083ac0f662c49bfbb2568975ffba4242021-11-08T13:27:19ZHealth‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress2192-831210.1002/jmd2.12240https://doaj.org/article/f083ac0f662c49bfbb2568975ffba4242021-11-01T00:00:00Zhttps://doi.org/10.1002/jmd2.12240https://doaj.org/toc/2192-8312Abstract Health‐related quality of life (HRQOL) is reduced in Fabry disease (FD) and associated with clinical disease manifestations, but few have used Fabry‐specific severity scores to study how disease burden interferes with quality of life. We investigated how the Fabry DS3, consisting of four somatic domains and one patient‐reported item, associates with HRQOL, while also evaluating fatigue, pain and psychological distress as possible predictors. Thirty‐six adults with FD completed the Short‐form Health Survey (SF‐36), the hospital anxiety and depression scale (HADS), the brief pain inventory (BPI) and reported fatigue on a visual analog scale. Clinical data were collected from the last multidisciplinary hospital visit. Using correlation and hierarchical linear regression analyses, we examined associations between demographic, clinical and self‐reported predictors and the SF‐36 physical (PCS) and mental (MCS) component summary scores. Males scored lower than the general population in all SF‐36 domains (P < .05). General health and social functioning were reduced in females. Before including self‐reported symptom scores, DS3 showed associations with PCS (P = .009). Our fully adjusted model explained 66% of the variation in PCS, where education (P = .040) and fatigue (P = .002) retained significance. With HADS depression score (P = .001) as the sole significant factor, our regression model explained 56% of the variation in MCS. The DS3 score has implications for HRQOL in FD. Low education and fatigue represent major barriers to physical well‐being, while depression strongly influences mental quality of life. Fatigue should be recognized as an important endpoint in future FD trials. Increased efforts to diagnose and treat affective disorders are warranted.Hege Kampen PihlstrømMina Susanne Weedon‐FekjærBirgitte Leisner BjerkelyCharlotte von derLippeKristin ØrstavikPer MathisenKetil HeimdalTrond Geir JenssenDag Olav DahleOlga Karin SolbergSolrun SigurdardottirWileyarticledepressionDS3FabryfatigueHRQOLpainDiseases of the endocrine glands. Clinical endocrinologyRC648-665GeneticsQH426-470ENJIMD Reports, Vol 62, Iss 1, Pp 56-69 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	depression DS3 Fabry fatigue HRQOL pain Diseases of the endocrine glands. Clinical endocrinology RC648-665 Genetics QH426-470
spellingShingle	depression DS3 Fabry fatigue HRQOL pain Diseases of the endocrine glands. Clinical endocrinology RC648-665 Genetics QH426-470 Hege Kampen Pihlstrøm Mina Susanne Weedon‐Fekjær Birgitte Leisner Bjerkely Charlotte von derLippe Kristin Ørstavik Per Mathisen Ketil Heimdal Trond Geir Jenssen Dag Olav Dahle Olga Karin Solberg Solrun Sigurdardottir Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress
description	Abstract Health‐related quality of life (HRQOL) is reduced in Fabry disease (FD) and associated with clinical disease manifestations, but few have used Fabry‐specific severity scores to study how disease burden interferes with quality of life. We investigated how the Fabry DS3, consisting of four somatic domains and one patient‐reported item, associates with HRQOL, while also evaluating fatigue, pain and psychological distress as possible predictors. Thirty‐six adults with FD completed the Short‐form Health Survey (SF‐36), the hospital anxiety and depression scale (HADS), the brief pain inventory (BPI) and reported fatigue on a visual analog scale. Clinical data were collected from the last multidisciplinary hospital visit. Using correlation and hierarchical linear regression analyses, we examined associations between demographic, clinical and self‐reported predictors and the SF‐36 physical (PCS) and mental (MCS) component summary scores. Males scored lower than the general population in all SF‐36 domains (P < .05). General health and social functioning were reduced in females. Before including self‐reported symptom scores, DS3 showed associations with PCS (P = .009). Our fully adjusted model explained 66% of the variation in PCS, where education (P = .040) and fatigue (P = .002) retained significance. With HADS depression score (P = .001) as the sole significant factor, our regression model explained 56% of the variation in MCS. The DS3 score has implications for HRQOL in FD. Low education and fatigue represent major barriers to physical well‐being, while depression strongly influences mental quality of life. Fatigue should be recognized as an important endpoint in future FD trials. Increased efforts to diagnose and treat affective disorders are warranted.
format	article
author	Hege Kampen Pihlstrøm Mina Susanne Weedon‐Fekjær Birgitte Leisner Bjerkely Charlotte von derLippe Kristin Ørstavik Per Mathisen Ketil Heimdal Trond Geir Jenssen Dag Olav Dahle Olga Karin Solberg Solrun Sigurdardottir
author_facet	Hege Kampen Pihlstrøm Mina Susanne Weedon‐Fekjær Birgitte Leisner Bjerkely Charlotte von derLippe Kristin Ørstavik Per Mathisen Ketil Heimdal Trond Geir Jenssen Dag Olav Dahle Olga Karin Solberg Solrun Sigurdardottir
author_sort	Hege Kampen Pihlstrøm
title	Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress
title_short	Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress
title_full	Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress
title_fullStr	Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress
title_full_unstemmed	Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress
title_sort	health‐related quality of life in norwegian adults with fabry disease: disease severity, pain, fatigue and psychological distress
publisher	Wiley
publishDate	2021
url	https://doaj.org/article/f083ac0f662c49bfbb2568975ffba424
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Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress

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