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Health-related quality of life among children with hereditary angioedema
Batya Engel-Yeger1, Henriette Farkas2, Shmuel Kivity3
1Department Faculty of Social Welfare and Health Sciences, University of Haifa Occupational Therapy, Haifa, Israel.
Insights
Children with symptomatic hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) experience significantly lower quality of life. Health-related quality of life is negatively impacted by attack frequency and location, particularly affecting school and physical well-being.
Area of Science:
- Pediatrics
- Immunology
- Genetics
Background:
- Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) significantly impacts patients' quality of life.
- Assessing health-related quality of life (HRQoL) in pediatric C1-INH-HAE patients is crucial for understanding disease burden.
Purpose of the Study:
- To evaluate the health-related quality of life (HRQoL) in children diagnosed with C1-INH-HAE.
- To compare HRQoL between pediatric C1-INH-HAE patients and healthy controls.
Main Methods:
- A cross-sectional study involving 34 children with C1-INH-HAE and 64 healthy controls aged 3-18 years.
- HRQoL was assessed using the Pediatric Quality of Life Inventory (PedsQL™) 4.0 Generic Core Scales.
- Disease activity and attack sites were recorded to correlate with HRQoL outcomes.
Main Results:
- Children with C1-INH-HAE attacks reported lower overall HRQoL, including school and psychosocial dimensions, compared to controls.
- A higher frequency of C1-INH-HAE attacks correlated negatively with total, school-related, and psychosocial HRQoL scores.
- Multisite attacks (laryngeal, abdominal, peripheral) resulted in significantly lower HRQoL across total, physical, and school domains compared to solely peripheral attacks.
Conclusions:
- Symptomatic pediatric C1-INH-HAE is associated with impaired health-related quality of life.
- The frequency and location of C1-INH-HAE attacks are key determinants of reduced HRQoL in children.
- School and physical domains are most affected by C1-INH-HAE attacks, highlighting the need for targeted interventions.
Background:
The clinical expressions of hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and its related burden may negatively affect patient quality of life. This study aimed to assess health-related quality of life (HRQoL) in children with C1-INH-HAE.
Methods:
Children (N = 98: 34 C1-INH-HAE patients, 64 healthy controls) aged 3-18 years were recruited in Israel and Hungary. All individuals completed a demographic questionnaire, a disease activity and site questionnaire, and the Pediatric Quality of Life Inventory (PedsQL™) 4.0 Generic Core Scales (child self-report and maternal proxy report) to assess HRQoL.
Results:
Among C1-INH-HAE patients, nine (26.5%) had 1-5 attacks/year, six (17.6%) had 6-18 attacks/year, eight (23.5%) had 25-60 attacks/year, and 11 (32.4%) were asymptomatic over the previous year. Children with C1-INH-HAE attacks demonstrated lower HRQoL than healthy control children across the total score, school, and psychosocial dimensions of the PedsQL™. The number of C1-INH-HAE attacks negatively correlated with the total HRQoL score (r = -0.48, p = 0.008), school-related HRQoL (r = -0.39, p = 0.02), and psychosocial HRQoL (r = -0.43, p = 0.01). Patients with multisite laryngeal, abdominal, and peripheral C1-INH-HAE attacks had a lower HRQoL compared with those who experienced solely peripheral attacks across the total score (p = 0.04), physical (p = 0.04), and school (p = 0.02) domains. There was no significant difference between asymptomatic C1-INH-HAE patients and healthy controls.
Conclusions:
Children with symptomatic C1-INH-HAE demonstrate impaired HRQoL compared with healthy controls. HRQoL was affected by the frequency and site of C1-INH-HAE attacks and mostly in the school and physical domains.
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