Everyday Life, Dietary Practices, and Health Conditions of Adult PKU Patients: A Multicenter, Cross-Sectional Study

• Published in: Annals of nutrition and metabolism 2020-12, Vol.76 (4), p.251-258
• Main Authors: Klimek, Annemarie, Baerwald, Christoph, Schwarz, Martin, Rutsch, Frank, Parhofer, Klaus G., Plöckinger, Ursula, Heddrich-Ellerbrok, Margret, vom Dahl, Stephan, Schöne, Klaus, Ott, Markus, Lang, Frauke, Hennermann, Julia B.
• Format: Article
• Language: English
• Subjects: Amino acids; Childbirth & labor; Cross-sectional studies; Diagnosis; Diet; Diet therapy; Health aspects; Hogs; Households; Low protein diet; Mental depression; Metabolism; Nutrient deficiency; Original Paper; Patients; Phenylalanine; Phenylketonuria; Proteins; Quality of life
• ISSN: 0250-6807; 1421-9697
• DOI: 10.1159/000510260

Background: Only few data on dietary management of adult phenylketonuria (PKU) patients are published. Objectives: This study aimed to assess living situation, dietary practices, and health conditions of early-treated adult PKU patients. Methods: A total of 183 early-treated PKU patients ≥18 years from 8 German metabolic centers received access to an online survey, containing 91 questions on sociodemographic data, dietary habits, and health conditions. Results: 144/183 patients (66% females) completed the questionnaire. Compared with German population, the proportion of single-person households was higher (22 vs. 47%), the rate of childbirth was lower (1.34 vs. 0.4%), but educational and professional status did not differ. 82% of the patients adhered to a low-protein diet, 45% consumed modified low-protein food almost daily, and 84% took amino acid mixtures regularly. 48% of the patients never interrupted diet, and 14% stopped diet permanently. 69% of the patients reported to feel better with diet, and 91% considered their quality of life at least as good. The prevalence of depressive symptoms was high (29%) and correlated significantly to phenylalanine blood concentrations (p = 0.046). However, depressive symptoms were only mild in the majority of patients. Conclusion: This group of early-treated adult German PKU patients is socially well integrated, reveals a surprisingly high adherence to diet and amino acid intake, and considers the restrictions of diet to their daily life as low.