Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic diseases

• Published in: European journal of clinical nutrition Vol. 72; no. 1; pp. 87 - 92
• Main Authors: Mlčoch, T, Puda, R, Ješina, P, Lhotáková, M, Štěrbová, Š, Doležal, T
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.01.2018; Nature Publishing Group
• Subjects: 692/700/2814; 692/700/478; Adolescent; Adolescent Nutritional Physiological Phenomena - ethnology; Adult; Carbohydrates; Caregivers; Child; Child Nutritional Physiological Phenomena - ethnology; Child, Preschool; Clinical Nutrition; Complications and side effects; Cost of Illness; Costs and Cost Analysis; Czech Republic; Diet, Protein-Restricted - economics; Diet, Protein-Restricted - ethnology; Economic aspects; Economics; Energy intake; Energy value; Epidemiology; Female; Food; Food Supply - economics; Health aspects; Humans; Intellectual Disability - economics; Intellectual Disability - ethnology; Intellectual Disability - etiology; Intellectual Disability - prevention & control; Internal Medicine; Low protein diet; Male; Medicine; Medicine & Public Health; Metabolic Diseases; Metabolic disorders; Metabolism, Inborn Errors - diet therapy; Metabolism, Inborn Errors - economics; Metabolism, Inborn Errors - ethnology; Metabolism, Inborn Errors - physiopathology; Nutrition research; original-article; Patient Compliance - ethnology; Patients; Phenylketonuria; Phenylketonurias - diet therapy; Phenylketonurias - economics; Phenylketonurias - ethnology; Phenylketonurias - physiopathology; Protein restricted diet; Proteins; Public Health; Rare Diseases - diet therapy; Rare Diseases - economics; Rare Diseases - ethnology; Rare Diseases - physiopathology; Self Report; Young Adult; Czech Republic
• ISSN: 0954-3007; 1476-5640; 1476-5640
• DOI: 10.1038/ejcn.2017.102

Background/Objectives: Phenylketonuria (PKU) and several other inherited metabolic diseases (IMD) require a lifelong low-protein diet (LPD), otherwise they lead to many health complications. LPDs, however, carry a significant economic burden for patients and their families. The objective of this study was to explore the costs of low-protein foods (LPFs) necessary for LPD as well as dietary patterns and compliance towards an LPD. Subjects/Methods: A detailed questionnaire was created in cooperation with National Association of PKU and other IMD (NSPKU), and consequently sent to all NSPKU members treated with an LPD ( n =303). A total of 184 respondents from the Czech Republic were included in the study (174 had PKU, 10 had other IMD). Results: The average daily consumption of LPF was equal to 411.7 g (PKU) and 345.6 g (other IMD), which corresponds to energy value of 5558 kJ and 4438 kJ, respectively, per patient per day. Patients mostly consumed low-protein flour (≈30% of energy intake), pasta (≈18%), basic pastry (≈15%) and sweets (≈10%). The average monthly costs of LPDs were equal to €130 (PKU) and €129 (other IMD) per patient per month. The compliance with LPD was decreasing with increasing age ( P <0.0001). Conclusions: This is the largest study examining costs and dietary patterns of LPDs in patients with PKU and the first study of this kind in other IMD patients requiring an LPD. The study clearly showed that an LPD carries a very high economic burden for families, which may lead to less LPD compliance and potential severe health consequences.