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Clinical nutrition

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

European Journal of Clinical Nutrition volume 72, pages 87–92 (2018)Cite this article

Subjects

Abstract

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.

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Acknowledgements

We would like to thank NSPKU and all study participants for their willing cooperation, without which this study would not have been possible, and to Květoslava Mlčochová MSc. for her thoughtful revision of our manuscript. Pavel Ješina received support from general grant RVO VFN 64165.

Author contributions

TM, RP, ŠŠ, ML and TD designed and conducted research, and created the questionnaire for patients. TM, RP and TD wrote the manuscript and TM had primary responsibility for final content. TM and RP analyzed the data and TM performed statistical analysis. ŠŠ, ML and PJ conducted repeated and throughout revisions of the manuscript. PJ wrote medical parts of the manuscript and was concerned with medical aspects of the study.

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Author notes

  1. T Mlčoch and T Doležal: These authors contributed equally to this work.

Authors and Affiliations

  1. Institute of Health Economics and Technology Assessment, Prague, Czech Republic

    T Mlčoch & T Doležal

  2. Národní sdružení PKU a jiných DMP, z.s. (National Association of Patients with PKU and Other IMD (NSPKU)), Prague, Czech Republic,

    R Puda, M Lhotáková & Š Štěrbová

  3. Institute of Inherited Metabolic Disorders, Charles University—First Faculty of Medicine and General University Hospital in Prague, Prague, Czech Republic

    P Ješina

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Correspondence to T Doležal.

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Competing interests

RP and ŠŠ are members of NSPKU and ML is a NSPKU volunteer. NSPKU receives sponsorship from several producers of food for special medical purposes, including low-protein food. The remaining authors declare no conflict of interest.

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Mlčoch, T., Puda, R., Ješina, P. et al. Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic diseases. Eur J Clin Nutr 72, 87–92 (2018). https://doi.org/10.1038/ejcn.2017.102

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