Objective: This investigation is an attempt to describe coping with phenylketonuria (PKU) in order to understand some aspects underlying good compliance.
Methods: The coping concept was applied to PKU in two questionnaires. Self- and parental ratings were combined with assessments of phenylalanine levels and the severity of the disease. All Swedish patients with PKU born in 1980-91, a total of 53 children and youths with their parents, were invited to participate in the study and 41 (77%) of them did so.
Results: The patients turned out to have good compliance with the diet. The main result was that patients with separated or divorced parents were more likely to have higher phenylalanine levels and this association was not diminished by adjustment for the potential confounding factors.
Conclusion: Patients' need for support must be judged individually according to different family conditions.