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Table 3 Mean AE-QoL and HAE-QoL scores during the 88-week extension (US only), all C1-INH(SC) doses combined

From: Long-term health-related quality of life in patients treated with subcutaneous C1-inhibitor replacement therapy for the prevention of hereditary angioedema attacks: findings from the COMPACT open-label extension study

Domain (range of possible scores)

Extension week 6

(n = 38)

Extension week 22

(n = 45)

Extension week 46

(n = 43)

Extension week 70

(n = 30)

AE-QoL scoresa, mean (SD) (higher scores = greater impairment)

 Total score (0–100)

13.39 (13.701)

17.68 (17.097)

16.45 (15.761)

17.89 (18.936)

 Functioning (0–100)

5.10 (11.881)

7.08 (18.396)

5.96 (15.607)

7.08 (20.351)

 Fatigue/mood (0–100)

20.66 (19.734)

26.11 (22.102)

23.14 (20.296)

23.83 (22.116)

 Fears/shame (0–100)

15.35 (18.787)

20.09 (22.355)

19.77 (21.499)

22.64 (26.526)

 Nutrition (0–100)

5.92 (10.372)

10.56 (18.262)

10.76 (18.416)

10.42 (21.795)

Domain (range of possible scores)

Extension week 26 (n = 40)

Extension week 50 (n = 40)

Extension week 74 (n = 16)

HAE-QoL scoresb, mean (SD) global score range: 25 to 135 (higher scores = less impairment)

 Global score (25–135)

120.2 (19.15)

122.3 (19.72)

115.7 (26.54)

 Physical functioning and health (4–23)

21.0 (3.00)

21.3 (3.05)

20.9 (3.70)

 Disease related stigma (3–15)

13.8 (2.24)

13.7 (2.37)

13.6 (2.66)

 Emotional role and social functioning (4–20)

18.4 (2.81)

18.5 (2.86)

18.3 (3.18)

 Concern about offspring (2–10)

7.9 (2.91)

8.3 (2.66)

7.8 (2.65)

 Perceived control over Illness (4–20)

16.7 (4.23)

17.4 (4.12)

15.5 (6.03)

 Mental health (4–24)

21.2 (3.92)

21.9 (3.49)

20.3 (4.84)

 Treatment difficulties (4–23)

21.4 (2.87)

21.3 (3.26)

19.4 (5.40)

  1. These assessments were not performed at baseline
  2. AE-QoL Angioedema Quality of Life Questionnaire, HAE hereditary angieodema, HAE-QoL hereditary angeiodema quality of life questionnaire, HRQoL health-related quality of life, C1-INH(SC) subcutaneous C1-inhibitor, US United States, SD standard deviation
  3. aThe AE-QoL [31, 35] is a validated angioedema-specific instrument comprised of 17 questions based on a recall period of 4 weeks. For the total and each domain, scores can range from 0 to 100 (higher scores indicate greater impairment)
  4. bThe HAE-QoL [32] is a HRQoL questionnaire specifically designed for studying the impact of HAE due to C1-INH(SC) deficiency on adult patients’ quality of life. It consists of 25 items assigned to 7 dimensions (3 or 4 items per dimension); each item scored from 1 to 5 or 1 to 6. The global score reflects the sum of all 25 individual item scores (higher scores indicate better outcomes)
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172