Survival after acute episodes of immune-mediated thrombotic thrombocytopenic purpura (iTTP) – cognitive functioning and health-related quality of life impact: a descriptive cross-sectional survey of adults living with iTTP in the United Kingdom. Issue 1 (1st January 2021)

• Record Type: Journal Article
• Authors: Holmes, Steve; Podger, Lauren; Bottomley, Catherine; Rzepa, Ewelina; Bailey, Karen M. A.; Chandler, Fleur
• Is Part Of: Hematology. Volume 26:Issue 1(2021)
• Page Start: 465
• Page End: 472
• Publication Date: 2021-01-01
• Subjects: Quality of life -- cognitive function -- psychological wellbeing -- thrombotic thrombocytopenic purpura -- cross-sectional survey -- patient engagement; Blood -- Diseases -- Periodicals; Hematology -- Periodicals; Blood -- Transfusion -- Periodicals; 616.15005
• DOI: 10.1080/16078454.2021.1945236
• Languages: English
• ISSNs: 1024-5332

Abstract

ABSTRACT: Objectives: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare life-threatening thrombotic microangiopathy affecting adults with unpredictable disease onset and acute presentation. This study aimed to describe the health-related quality of life (HRQoL), cognitive functioning and work productivity of survivors following acute episode(s) of iTTP in the United Kingdom (UK). Methods: An online survey was developed in collaboration with the TTPNetwork. Descriptive statistics were calculated for the health questionnaire Short Form Survey-36 Version 2 (SF-36v2), the Hospital Anxiety and Depression Score (HADS), the PROMIS Cognitive Function Abilities Subset – Short Form 6a (PROMIS CFAS – SF6a), and the Work Productivity and Activity Index: Specific Health Problem (WPAI-SHP), along with several iTTP-specific bespoke questions. Results: Fifty participants were recruited between July-November 2019. The mean (standard deviation [SD]) standardized SF-36v2 physical and mental component scores were 42.16 (9.59) and 33.61 (12.34), lower than population norms. The mean (SD) standardized PROMIS CFAS – SF6a score was 39.69 (7.86), lower than population norms. HADS mean (SD) scores of 12.18 (3.14) and 11.78 (2.36) indicated moderate levels of anxiety and depression, respectively. Of those employed (58%), approximately 42.73% of participants reported work productivity loss due to their iTTP. Participants also reported experiencing flashbacks, fatigue interference in family, social and intimate life, and fears of relapse. Discussion and conclusion: Regardless of recency of the last acute episode, participant scores signified impairments in all domains. Remission from an acute episode of disease does not signify the conclusion of care, but rather the requirement for long-term healthcare particularly focused on psychological support.