Congenital platelet disorders and health status-related quality of life

Maaike W. Blaauwgeers; Marieke J. H. A. Kruip; Erik A. M. Beckers; Michiel Coppens; Jeroen Eikenboom; Karin P. M. van Galen; Rienk Y. J. Tamminga; Rolf T. Urbanus; Roger E. G. Schutgens; TiN Study Group

doi:10.1002/rth2.12281

Congenital platelet disorders and health status-related quality of life

Maaike W. Blaauwgeers, Marieke J. H. A. Kruip, Erik A. M. Beckers, Michiel Coppens, Jeroen Eikenboom, Karin P. M. van Galen, Rienk Y. J. Tamminga, Rolf T. Urbanus, Roger E. G. Schutgens^*, TiN Study Group

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

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.

Original language	English
Pages (from-to)	100-105
Number of pages	6
Journal	Research and practice in thrombosis and haemostasis
Volume	4
Issue number	1
DOIs	https://doi.org/10.1002/rth2.12281
Publication status	Published - Jan 2020

Keywords

bleeding tendency
congenital blood platelet disorders
health status
quality of life
SF-36
ILLNESS PERCEPTION
BLEEDING SCORES
DISEASE
INDIVIDUALS
ADULT
WOMEN

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Cite this

@article{3fa9146a69a94f6f8c51b3c0e6b2a46a,

title = "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 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.",

keywords = "bleeding tendency, congenital blood platelet disorders, health status, quality of life, SF-36, ILLNESS PERCEPTION, BLEEDING SCORES, DISEASE, INDIVIDUALS, ADULT, WOMEN",

author = "Blaauwgeers, {Maaike W.} and Kruip, {Marieke J. H. A.} and Beckers, {Erik A. M.} and Michiel Coppens and Jeroen Eikenboom and {van Galen}, {Karin P. M.} and Tamminga, {Rienk Y. J.} and Urbanus, {Rolf T.} and Schutgens, {Roger E. G.} and {TiN Study Group}",

note = "Funding Information: The authors thank all patients for participating in the study. Publisher Copyright: {\textcopyright} 2019 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals, Inc on behalf of International Society on Thrombosis and Haemostasis.",

year = "2020",

month = jan,

doi = "10.1002/rth2.12281",

language = "English",

volume = "4",

pages = "100--105",

journal = "Research and practice in thrombosis and haemostasis",

issn = "2475-0379",

publisher = "Wiley",

number = "1",

}

TY - JOUR

T1 - Congenital platelet disorders and health status-related quality of life

AU - Blaauwgeers, Maaike W.

AU - Kruip, Marieke J. H. A.

AU - Beckers, Erik A. M.

AU - Coppens, Michiel

AU - Eikenboom, Jeroen

AU - van Galen, Karin P. M.

AU - Tamminga, Rienk Y. J.

AU - Urbanus, Rolf T.

AU - Schutgens, Roger E. G.

AU - TiN Study Group

N1 - Funding Information: The authors thank all patients for participating in the study. Publisher Copyright: © 2019 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals, Inc on behalf of International Society on Thrombosis and Haemostasis.

PY - 2020/1

Y1 - 2020/1

N2 - 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.

AB - 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.

KW - bleeding tendency

KW - congenital blood platelet disorders

KW - health status

KW - quality of life

KW - SF-36

KW - ILLNESS PERCEPTION

KW - BLEEDING SCORES

KW - DISEASE

KW - INDIVIDUALS

KW - ADULT

KW - WOMEN

U2 - 10.1002/rth2.12281

DO - 10.1002/rth2.12281

M3 - Article

C2 - 31989090

SN - 2475-0379

VL - 4

SP - 100

EP - 105

JO - Research and practice in thrombosis and haemostasis

JF - Research and practice in thrombosis and haemostasis

IS - 1

ER -