PKU-QOL domains – Median (Q1-Q3) | Child Sample | Adolescent sample | Adult sample | Parent sample |
---|---|---|---|---|
(N = 92) | (N = 110) | (N = 104) | (N = 253) | |
Symptoms | Â | Â | Â | Â |
 Self-rated health status | - | 50.0 (25.0–50.0) | 25.0 (25.0–50.0) | - |
 Child health status | - | - | - | 25.0 (0.0–50.0) |
 Headaches | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) | 0.0 (0.0–25.0) |
 Stomach aches | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) |
 Tiredness | 25.0 (25.0–50.0) | 50.0 (25.0–50.0) | 50.0 (25.0–50.0) | 25.0 (0.0–50.0) |
 Trembling hands | - | - | 0.0 (0.0–25.0) | - |
 Irritability | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) | 25.0 (0.0–50.0) | 25.0 (25.0–50.0) |
 Aggressiveness | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) |
 Moodiness | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) | 25.0 (0.0–50.0) | 25.0 (0.0–50.0) |
 Sadness | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) | 25.0 (0.0–25.0) |
 Anxiety | 0.0 (0.0–0.0) | 0.0 (0.0–25.0) | 0.0 (0.0–50.0) | 0.0 (0.0–25.0) |
 Lack of concentration | 0.0 (0.0–25.0)) | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) | 25.0 (0.0–50.0) |
 Slow thinking | 25.0 (0.0–50.0) | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 0.0 (0.0–50.0) |
PKU in general | Â | Â | Â | Â |
 Emotional impact of PKU | 33.3 (16.7–50.0) | 30.0 (20.0–40.0) | 45.0 (25.0–60.0) | 37.5 (25.0–62.5) |
 Practical impact of PKU | 0.0 (0.0–12.5) | 8.3 (0.0–25.0) | 12.5 (6.3–25.0) | 12.5 (0.0–25.0) |
 Social impact of PKU | 16.7 (8.3–25.0) | 16.7 (8.3–25.0) | 16.7 (6.3–25.0) | 12.5 (5.0–25.0) |
 Overall impact of PKU | 18.8 (12.5–31.3) | 20.0 (12.5–30.0) | 27.1 (18.8–39.6) | 21.4 (11.5–35.0) |
 Anxiety – blood test | 6.3 (0.0–12.5) | 0.0 (0.0–12.5) | 0.0 (0.0–12.5) | 12.5 (0.0–37.5) |
 Impact of child anxiety – blood test | - | - | - | 12.5 (0.0–37.5) |
 Anxiety – blood Phe levels | 50.0 (25.0–75.0) | 25.0 (0.0–50.0) | 25.0 (25.0–50.0) | 50.0 (25.0–75.0) |
 Anxiety – blood Phe levels during pregnancy | - | - | 100.0 (75.0–100.0)a | - |
 Financial impact of PKU | - | - | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) |
 Information on PKU | - | - | 25.0 (25.0–50.0) | 25.0 (0.0–50.0) |
Phe-free amino acid supplement administration | Â | Â | Â | Â |
 Adherence to supplements | 0.0 (0.0–12.5) | 6.3 (0.0–25.0) | 16.7 (0.0–33.3) | 0.0 (0.0–25.0) |
 Management of supplements | - | - | - | 0.0 (0.0–25.0) |
 Practical impact of supplements | 0.0 (0.0–25.0) | 12.5 (0.0–25.0) | 18.8 (6.3–37.5) | 8.3 (0.0–33.3) |
 Guilt if poor adherence to supplements | 37.5 (0.0–75.0) | 25.0 (25.0–75.0) | 50.0 (25.0–75.0) | 50.0 (25.0–75.0) |
 Relationships within family because of supplements | 0.0 (0.0–0.0) | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) |
 Taste – supplements | 50.0 (0.0–50.0) | 50.0 (25.0–50.0) | 50.0 (25.0–50.0) | - |
Dietary protein restriction | Â | Â | Â | Â |
 Food temptations | 25.0 (0.0–50.0) | 25.0 (0.0–50.0) | 37.5 (12.5–50.0) | - |
 Adherence to dietary protein restriction | 0.0 (0.0–12.5) | 8.3 (0.0–18.8) | 18.8 (0.0–31.3) | 0.0 (0.0–25.0) |
 Management of dietary protein restriction | - | - | - | 20.8 (5.0–37.5) |
 Social impact of dietary protein restriction | 15.0 (5.0–30.0) | 5.0 (0.0–20.0) | 12.5 (4.2–25.0) | - |
 Practical impact of dietary protein restriction | - | 28.6 (14.3–32.1) | 35.0 (20.8–50.0) | 28.6 (14.3–46.4) |
 Overall impact of dietary protein restriction | - | 17.8 (9.1–25.0) | 25.0 (11.5–37.5) | - |
 Taste – specialty low protein food | 25.0 (0.0–25.0) | 25.0 (0.0–37.5) | 25.0 (25.0–50.0) | - |
Food enjoyment | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) | 25.0 (0.0–25.0) |
 Guilt if dietary protein restriction not followed | 50.0 (0.0–100.0) | 50.0 (25.0–75.0) | 50.0 (25.0–75.0) | 50.0 (25.0–75.0) |
 Overall difficulty following dietary protein restriction | 0.0 (0.0–25.0) | 0.0 (0.0–25.0) | 25.0 (0.0–50.0) | - |