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Table 1 PKU-QOL scores in the study samples

From: Assessment of the impact of phenylketonuria and its treatment on quality of life of patients and parents from seven European countries

PKU-QOL domains – Median (Q1-Q3)

Child Sample

Adolescent sample

Adult sample

Parent sample

(N = 92)

(N = 110)

(N = 104)

(N = 253)

Symptoms

    

 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

    

 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

    

 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

    

 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)

-

  1. Note: lower PKU-QOL scores represent more positive outcome; 0 (no impact/no symptom) and 100 (extremely severe impact/very frequent symptom)
  2. aFemale patients only, n = 66