Congenital platelet disorders and health status–related quality of life

Abstract Background Patients with congenital blood platelet disorders (CPDs) demonstrate a predominantly mucocutaneous bleeding tendency. Repeated bleeds throughout life can have a significant impact on health status–related quality of life (HR‐QoL), but few studies have investigated HR‐QoL in patie...

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Autores principales:	Maaike W. Blaauwgeers, Marieke J. H. A. Kruip, Erik A. M. Beckers, Michiel Coppens, Jeroen Eikenboom, Karin P. M. vanGalen, Rienk Y. J. Tamminga, Rolf T. Urbanus, Roger E. G. Schutgens, the TiN study group
Formato:	article
Lenguaje:	EN
Publicado:	Wiley 2020
Materias:	bleeding tendency congenital blood platelet disorders health status quality of life SF‐36 Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/0c9d076f8a854bd88108a3b21255e944
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spelling	oai:doaj.org-article:0c9d076f8a854bd88108a3b21255e9442021-11-15T06:10:44ZCongenital platelet disorders and health status–related quality of life2475-037910.1002/rth2.12281https://doaj.org/article/0c9d076f8a854bd88108a3b21255e9442020-01-01T00:00:00Zhttps://doi.org/10.1002/rth2.12281https://doaj.org/toc/2475-0379Abstract Background Patients with congenital blood platelet disorders (CPDs) demonstrate a predominantly mucocutaneous bleeding tendency. Repeated bleeds throughout life can have a significant impact on health status–related quality of life (HR‐QoL), but few studies have investigated HR‐QoL in patients with CPDs. Objectives To determine HR‐QoL in patients with suspected or confirmed CPDs as compared with the general Dutch population and to assess the association between bleeding phenotype and HR‐QoL. Methods Data were derived from the Thrombocytopathy in the Netherlands (TiN) study, a cross‐sectional study of individuals suspected for a congenital platelet defect. TiN patients with an increased ISTH Bleeding Assessment Tool (ISTH‐BAT) score (>3 in men and > 5 in women) were included for analysis. HR‐QoL was assessed with the Short Form (SF)‐36 survey. Bleeding symptoms were evaluated with the ISTH‐BAT, resulting in a bleeding score. Results One hundred fifty‐six patients were analyzed, of whom 126 (81%) were women. Sixty‐two patients (40%) had a confirmed CPD. Compared to the general Dutch population, patients with a suspected or confirmed CPD reported decreased physical functioning, limitations in daily activities due to physical health problems, limitations in social activities, decreased energy levels and fatigue, pain, and lower general health status. HR‐QoL was not correlated with the ISTH‐BAT score and was similar in patients with a confirmed CPD and those in whom a CPD could not be diagnosed. Conclusion A bleeding tendency in patients with a suspected or confirmed CPD significantly impacts HR‐QoL, independent of a confirmed explanatory diagnosis.Maaike W. BlaauwgeersMarieke J. H. A. KruipErik A. M. BeckersMichiel CoppensJeroen EikenboomKarin P. M. vanGalenRienk Y. J. TammingaRolf T. UrbanusRoger E. G. Schutgensthe TiN study groupWileyarticlebleeding tendencycongenital blood platelet disordershealth statusquality of lifeSF‐36Diseases of the blood and blood-forming organsRC633-647.5ENResearch and Practice in Thrombosis and Haemostasis, Vol 4, Iss 1, Pp 100-105 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	bleeding tendency congenital blood platelet disorders health status quality of life SF‐36 Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	bleeding tendency congenital blood platelet disorders health status quality of life SF‐36 Diseases of the blood and blood-forming organs RC633-647.5 Maaike W. Blaauwgeers Marieke J. H. A. Kruip Erik A. M. Beckers Michiel Coppens Jeroen Eikenboom Karin P. M. vanGalen Rienk Y. J. Tamminga Rolf T. Urbanus Roger E. G. Schutgens the TiN study group Congenital platelet disorders and health status–related quality of life
description	Abstract Background Patients with congenital blood platelet disorders (CPDs) demonstrate a predominantly mucocutaneous bleeding tendency. Repeated bleeds throughout life can have a significant impact on health status–related quality of life (HR‐QoL), but few studies have investigated HR‐QoL in patients with CPDs. Objectives To determine HR‐QoL in patients with suspected or confirmed CPDs as compared with the general Dutch population and to assess the association between bleeding phenotype and HR‐QoL. Methods Data were derived from the Thrombocytopathy in the Netherlands (TiN) study, a cross‐sectional study of individuals suspected for a congenital platelet defect. TiN patients with an increased ISTH Bleeding Assessment Tool (ISTH‐BAT) score (>3 in men and > 5 in women) were included for analysis. HR‐QoL was assessed with the Short Form (SF)‐36 survey. Bleeding symptoms were evaluated with the ISTH‐BAT, resulting in a bleeding score. Results One hundred fifty‐six patients were analyzed, of whom 126 (81%) were women. Sixty‐two patients (40%) had a confirmed CPD. Compared to the general Dutch population, patients with a suspected or confirmed CPD reported decreased physical functioning, limitations in daily activities due to physical health problems, limitations in social activities, decreased energy levels and fatigue, pain, and lower general health status. HR‐QoL was not correlated with the ISTH‐BAT score and was similar in patients with a confirmed CPD and those in whom a CPD could not be diagnosed. Conclusion A bleeding tendency in patients with a suspected or confirmed CPD significantly impacts HR‐QoL, independent of a confirmed explanatory diagnosis.
format	article
author	Maaike W. Blaauwgeers Marieke J. H. A. Kruip Erik A. M. Beckers Michiel Coppens Jeroen Eikenboom Karin P. M. vanGalen Rienk Y. J. Tamminga Rolf T. Urbanus Roger E. G. Schutgens the TiN study group
author_facet	Maaike W. Blaauwgeers Marieke J. H. A. Kruip Erik A. M. Beckers Michiel Coppens Jeroen Eikenboom Karin P. M. vanGalen Rienk Y. J. Tamminga Rolf T. Urbanus Roger E. G. Schutgens the TiN study group
author_sort	Maaike W. Blaauwgeers
title	Congenital platelet disorders and health status–related quality of life
title_short	Congenital platelet disorders and health status–related quality of life
title_full	Congenital platelet disorders and health status–related quality of life
title_fullStr	Congenital platelet disorders and health status–related quality of life
title_full_unstemmed	Congenital platelet disorders and health status–related quality of life
title_sort	congenital platelet disorders and health status–related quality of life
publisher	Wiley
publishDate	2020
url	https://doaj.org/article/0c9d076f8a854bd88108a3b21255e944
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Congenital platelet disorders and health status–related quality of life

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