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

Figures & data

Table 1. Participant demographics and characteristics.

	Total (n = 35)	‘≤12months_group’ group (n = 10)	‘>12months_group’ (n = 25)
Gender, n (%)
Male	1 (3)	1 (10)	0 (0)
Female	34 (97)	9 (90)	25 (100)
Age (years), n (%)
18–30	3 (9)	1 (10)	2 (8)
31–40	9 (26)	2 (20)	7 (28)
41–50	10 (29)	5 (50)	5 (20)
51–60	9 (26)	2 (20)	7 (28)
61–70	4 (11)	0 (0)	4 (16)
Year of aTTP diagnosis, n (%)
1995–2000	3 (9)	0 (0)	3 (12)
2001–2005	5 (14)	1(10)	4 (16)
2006–2010	3 (9)	0 (0)	3 (12)
2011–2016	12 (34)	2 (20)	10 (40)
2017–2019	12 (34)	7 (70)	5 (20)
Total number of acute aTTP episodes
Mean (SD)	2.7 (3.4)	3.5 (5.9)	2.4 (1.8)
Median (range)	2.0 (1–20)	1.5 (1–20)	2.0 (1–8)

Figure 1. SF-36v2 domains, stratified by recency of last acute episode.

Note: The data represents the mean norm-based scores with the 95% CIs for each of the eight domains of the SF-36 and also for the two component summary measures. Data for each measurement are compared to the mean value for the US population that has been normalized to a mean score of 50, designated by the line.

PF=Physical Functioning; RP=Role Physical; BP=Bodily Pain; GH=General Health; V=Vitality; SF=Social Functioning; RE=Role Emotional; MH=Mental Health; PCS=Physical Component Score; and MCS=mental Component Score.

Table 2. Scores for PROMs.

	Total sample (n = 50)	‘≤12months_group’ (n = 25)	‘>12months_group’ (n = 25)
	Mean scores (SD)	Mean scores (SD)	Mean scores (SD)
SF-36v2 Physical Component Score	42.16 (9.59)	40.20 (5.19)	44.12 (12.36)
SF-36v2 Mental Component Score	33.61 (12.34)	30.59 (11.80)	36.64 (12.36)
PROMIS CFAS – SF6a (Cognitive Function)	39.69 (7.86)	41.78 (6.97)	37.59 (8.28)
HADS – Anxiety Scale	12.18 (3.14)	12.52 (3.32)	11.84 (2.98)
HADS – Depression Scale	11.78 (2.36)	10.92 (2.69)	12.64 (1.60)
	Median % (IQR)	Median % (IQR)	Median % (IQR)
	n = 29	n = 13	n = 16
WPAI – SHP Presenteeism ^1,3	10.00 (0.00–40.00)	0.00 (0.00–20.00)	30.00 (0.00–42.50)
	n = 27	n = 13	n = 14
WPAI-SHP Absenteeism ^1,3	0.00 (0.00­ – 58.38)	0.00 (0.00–20.00)	0.00 (0.00–3.41)
WPAI-SHP Work Productivity Loss ^1,3	42.73 (20.00–96.00)	78.38 (20.00–100.00)	35.00 (22.50–65.00)
	n = 50	n = 25	n = 25
WPAI-SHP Activity Impairment^2,3	70.00 (30.00–90.00)	80.00 (40.00–100.00)	41.00 (0.00–70.00)

¹ Scores for those who are in work. ²Scores for total sample regardless of employment status. ³See supplemental information for definitions.

Figure 2. Bespoke questions – worry about having another iTTP episode.

Figure 3. Bespoke questions – isolation.

Figure 4. Bespoke questions – flashback severity among those reporting ever having flashbacks.

Table 3. Bespoke questions – activities of daily living, n = 50.

	Not at all	A little bit	Somewhat	Quite a bit	Very much	I don’t wish to answer
How much has iTTP affected the following? n (%)
Ability to travel in daily life	18 (36)	13 (26)	8 (16)	5 (10)	6 (12)	0 (0)
Ability to go on holidays	16 (32)	15 (30)	4 (8)	9 (18)	6 (12)	0 (0)
Ability to be independent	15 (30)	11 (22)	11 (22)	7 (14)	6 (12)	0 (0)
Your finances	18 (36)	11 (22)	11 (22)	5 (10)	4 (8)	1 (2)
Desire/ability to have sex	16 (32)	12 (24)	8 (16)	6 (12)	7 (14)	1 (2)
I have been able to put my thoughts into words without extra effort n (%)	6 (12)	22 (44)	15 (30)	4 (8)	3 (6)	0 (0)

Table 4. Bespoke questions – fatigue interference, n = 50.

	Never	Rarely	Sometimes	Often	Always	I don’t wish to answer
In the past 4 weeks how often did fatigue interfere with … n (%)
Your family life?	5 (10%)	2 (4%)	10 (20%)	20 (40%)	13 (26%)	0 (0%)
Your social activities?	2 (4%)	3 (6%)	9 (18%)	18 (36%)	18 (36%)	0 (0%)
Your work?	2 (4%)	6 (12%)	5 (10%)	14 (28%)	21 (42%)	2 (4%)

Data availability statement

Qualified researchers may request access to patient-level data and related study documents including the clinical study report, study protocol with any amendments, blank case report form, statistical analysis plan, and dataset specifications. Patient-level data will be anonymized and study documents will be redacted to protect the privacy of our trial participants. Further details on Sanofi’s data-sharing criteria, eligible studies, and process for requesting access can be found at: https://www.clinicalstudydatarequest.com/.