Psychometrics of patient-reported outcomes measurement information system in von Willebrand disease, inherited platelet function disorders, and rare bleeding disorders

Evelien S. van Hoorn*, Sterre P.E. Willems, SYMPHONY consortium and the Dutch research group for PROMIS implementation in inherited bleeding disorders, Wala Al Arashi, Annick S. de Moor, Calvin B. van Kwawegen, Lorynn Teela, Martijn A.H. Oude Voshaar, Idske C.L. Kremer Hovinga, Roger E.G. Schutgens, Saskia E.M. Schols, Frank W.G. Leebeek, Lotte Haverman, Marjon H. Cnossen, Samantha C. Gouw, Hester F. Lingsma

*Corresponding author for this work

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Abstract

Background: 

Patient-reported outcomes measurement information system (PROMIS) measures can be used to measure patient-reported outcomes. PROMIS measures, including computer adaptive tests (CATs) and short forms, have demonstrated the ability to adequately assess outcomes in patients with hemophilia. It is, however, unclear if PROMIS measures are suitable for patients with von Willebrand disease (VWD), inherited platelet function disorders (IPFDs), and rare bleeding disorders (RBDs). 

Objectives: 

To evaluate the feasibility, measurement properties, and relevance of PROMIS measures in adults with VWD, IPFDs, and RBDs. Methods: In this cross-sectional multicenter study, adults with VWD, IPFDs, and RBDs completed 9 PROMIS measures and the Short Form-36 version 2 (SF-36v2) electronically. Feasibility was determined by the number of completed items and floor/ceiling effects. Measurement properties included construct validity based on a multitrait–multimethod analysis and reliability using the reliability coefficient and greatest lower bound. Relevance was evaluated based on comparison with the Dutch general population. 

Results: 

In total, 111 patients (median age, 57 years [IQR, 44-67]; 60% VWD, 16% IPFD, 24% RBD) participated. Mean number of items answered varied from 5.3 to 8.7 (range, 4-12) per PROMIS CAT in patients with VWD. Construct validity was supported for all CATs and all instruments had a good reliability (≥0.70). The PROMIS measures had less ceiling effects than the SF-36v2. 

Conclusion: 

The PROMIS measures are a feasible, valid, and reliable alternative for the SF-36v2 in patients with primarily nonsevere forms of VWD. The relevance of the selected measures was limited. Additional research is necessary to evaluate the PROMIS measures in adults with IPFDs and RBDs.

Original languageEnglish
Article number102474
JournalResearch and Practice in Thrombosis and Haemostasis
Volume8
Issue number4
DOIs
Publication statusPublished - May 2024

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