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Survivorship in acquired thrombotic thrombocytopenic purpura (aTTP) – impacts on quality of life (QoL) and cognitive functioning in patients from the UK
Background
Acquired or immune-mediated TTP is a life-threatening illness affecting adults aged in their mid-40s. Disease onset is unpredictable, with many of those affected otherwise healthy before their first acute admission. Anecdotal reports describe significant difficulties returning to normal life following discharge from hospital, however evidence is limited, especially in the UK.
Aims
Assess quality of life in aTTP survivors
Methods
Patient group representatives were interviewed to identify relevant QoL concepts and health outcomes for TTP, and to validate a set of bespoke questions. An online survey was then developed to investigate QoL using the generic 36-item Short Form Survey (SF-36), anxiety and depression using the Hospital Anxiety and Depression Score (HADS), cognitive function using the PRO Measurement Information System (PROMIS) – Cognitive Function Abilities – Short Form 6a (PROMIS CFASF-6a), and workplace productivity according to the Work Productivity and Activity index (WPAI). The survey was publicised through the UK TTP Network social media, with a target of between 30 and 50 adult TTP patients. It was also opened to carers of TTP patients.
Results
Between July and November 2019, 50 patients and 10 carers completed the survey. Carer group data are not described here.
Demographic data are available for 35 patients. The majority were female, aged 30-60 years. Participants had experienced a median of 2 acute episodes of disease since diagnosis, with half reporting an acute admission in the previous 12 months.
Mean SF-36 scores were markedly lower compared with UK population norms across all domains, suggesting worse overall QoL. Mean (SD) physical component score was 42.16 (9.59), UK norm 53.64 (5.88) – mean (SD) mental component score was 33.61 (12.34), UK norm 51.28 (9.01).
Participants who experienced an acute episode in the last 12 months scored lower on each domain than those who did not.
Mean (SD) cognitive function T-score was 39.89 (7.89) points by PROMIS CFASF-6a, which is at least one standard deviation below that for the US general population (mean 50, SD 10), indicating relevant cognitive dysfunction. Normative UK data are not available.
Moderate levels of anxiety (median=12, IQR 4) and depressive symptoms (median=12, IQR 2.75) were reported across all patients, as measured by the HADS (scale ranges from 0-21: 0-7 is normal, 8-10 mild, 11-14 moderate, 15-21 severe).
29 of 50 respondents were employed at the time of completing the survey. A mean of 51% work productivity loss was reported in those employed (including time off for health reasons, lost productivity while at work). Across all 50 respondents the general activity impairment was 56%.
Conclusion
QoL of aTTP survivors is reduced compared to the UK population norm. Relevant cognitive impairment is seen compared to population norms. Survivors suffer from anxiety and depressive symptoms, and report reduced participation in work and normal daily activities. Health service provision for survivors of acute TTP should allocate specific resources to psychological rehabilitation efforts.
Authors
S Holmes, L Podger, J Bernarde, C Bottomley, F Chandler
Journal
Therapeutic Area
Hematology
Center of Excellence
Patient-Centered outcomes
Year
2020
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