Table 3 Therapy management and satisfaction (single centre survey; n = 30; 17 females/13 males). A Clinic visits, laboratory controls and dietary treatment. B Transition satisfaction and coping with treatment
From: Ten years of specialized adult care for phenylketonuria – a single-centre experience
A: Clinic visits, laboratory controls and dietary treatment | ||
Frequency of laboratory controls/dried blood samples | n | % |
Monthly | 13 | 43.3 |
Every two month | 5 | 16.7 |
Quarterly | 5 | 16.7 |
Biannually | 3 | 10.0 |
Annually | 2 | 6.7 |
No answer | 2 | 6.7 |
Frequency of clinic visits | ||
Quarterly | 11 | 36.7 |
Three times a year | 2 | 6.7 |
Biannually | 12 | 40.0 |
Annually | 4 | 13.3 |
No visits | 1 | 3.3 |
Dietary treatment | ||
Phe/protein restricted diet | 22 | 73.3 |
Exact calculating Phe content | 4 | 12.3 |
Estimation of Phe content | 16 | 53.3 |
No strict diet regime/vegetarian diet | 2 | 6.6 |
Amino acid mixture (AAM) | 24 | 80.0 |
BH4 | 0 | 0 |
Discontinuation of the PKU treatment | ||
Never | 14 | 46.7 |
Once | 7 | 23.3 |
Several times | 5 | 16.7 |
No answer | 4 | 13.3 |
Reasons for discontinuation of the PKU treatment (n = 12) | ||
Stop of dietary treatment in adolescence according to former procedures | 3 | 25.0 |
Weariness to follow the diet/Wish for independence from treatment | 5 | 41.7 |
Good metabolic control following a vegetarian diet | 3 | 25.0 |
No answer | 1 | 8.3 |
Restart of the PKU treatment | 10 | 83.3 |
Reasons for restart of the PKU treatment (n = 10) | ||
Doctors recommendation | 2 | 20.0 |
More capability/long-term prevention | 2 | 20.0 |
Wish to become pregnant/pregnancy | 4 | 40.0 |
No answer | 2 | 20.0 |
B: Transition satisfaction and coping with treatment. | ||
Satisfaction with the actual individual metabolic control | n | % |
Very satisfied | 10 | 33.3 |
Satisfied | 14 | 46.7 |
Less satisfied | 5 | 16.7 |
Not satisfied | 0 | 0 |
No answer | 1 | 3.3 |
Personal importance of good metabolic control | ||
Very important | 12 | 40.0 |
Important | 14 | 46.7 |
Less important | 3 | 10.0 |
Not important | 0 | 0 |
No answer | 1 | 3.3 |
Current coping with dietary therapy | ||
Very well | 10 | 33.3 |
Well | 13 | 43.3 |
Moderately | 2 | 6.7 |
Badly | 1 | 3.3 |
No answer | 4 | 12.3 |
Coping with dietary treatment in adulthood in comparison to childhood | ||
Easier | 13 | 43.3 |
Indifferent | 8 | 26.7 |
More difficult | 5 | 16.7 |
No answer | 4 | 12.3 |
Satisfaction with transition process and adult care | ||
Very satisfied | 10 | 33.3 |
Satisfied | 15 | 50.0 |
Less satisfied | 0 | 0 |
Not satisfied | 3 | 10.0 |
No answer | 2 | 6.7 |