Table 4 Information on treatment of the patients

Treatment for phenylalanine control *

66

44

72

 None

1 (1.5)

2 (4.5)

2 (2.8)

 Phenylalanine-restricted diet

56 (84.8)

37 (84.1)

61 (84.7)

 Medical nutrition products

41 (62.1)

33 (75.0)

50 (69.4)

 Large neutral amino acid (LNAA)

4 (6.1)

1 (2.3)

12 (16.7)

 Sapropterin

-

1 (2.3)

2 (2.8)

Difficulties in implementing phenylalanine-restricted diet *

66

44

72

 None

5 (7.6)

2 (4.5)

4 (5.6)

 The products are expensive

57 (86.4)

41 (93.2)

54 (75.0)

 Options are limited

43 (65.2)

27 (61.4)

49 (68.1)

 Difficulty in implementation at school

37 (56.1)

21 (47.7)

30 (41.7)

 Difficulties in implementation due to work life /education/trip, etc.

34 (51.5)

24 (54.5)

40 (55.6)

 Lack of taste/flavor

30 (45.5)

17 (38.6)

29 (40.3)

 It is difficult to prepare/no recipe

24 (36.4)

16 (36.4)

27 (37.5)

 Low/limited efficacy in lowering phenylalanine levels

22 (33.3)

10 (22.7)

23 (31.9)

 Reaction of the people around because of diet type

23 (34.8)

13 (29.5)

26 (36.1)

Difficulties in using medical nutrition products *

65

41

72

 None

8 (12.3)

3 (7.3)

12 (16.7)

 Products are expensive

47 (72.3)

37 (90.2)

53 (73.6)

 Limited options

33 (50.8)

27 (65.9)

42 (58.3)

 Difficulties in implementation due to work life /education/trip, etc.

24 (36.9)

19 (46.3)

37 (51.4)

 Lack of taste/flavor

31 (47.7)

20 (48.8)

28 (38.9)

 Low/limited efficacy in lowering phenylalanine levels

19 (29.2)

15 (36.6)

16 (22.2)

 Other

5 (7.7)

3 (7.3)

3 (4.2)

Difficulties in using amino acid or LNAA *

66

44

72

 None

45 (68.2)

32 (72.7)

58 (80.6)

 Poor taste

19 (28.8)

11 (25)

9 (12.5)

 Low/limited efficacy in lowering phenylalanine levels

5 (7.6)

5 (11.4)

2 (2.8)

 Difficulty in using due to high number of tablets

5 (7.6)

4 (9.1)

2 (2.8)

Those who underwent Sapropterin response testing

63

5 (7.9)

39

2 (5.1)

68

5 (7.4)