Prescribing issues experienced by people living with phenylketonuria in the UK

Article

Ford, S., O'Driscoll, M. and MacDonald, A. 2019. Prescribing issues experienced by people living with phenylketonuria in the UK. Molecular Genetics and Metabolism Reports. 21, pp. 1-7. https://doi.org/10.1016/j.ymgmr.2019.100527
TypeArticle
TitlePrescribing issues experienced by people living with phenylketonuria in the UK
AuthorsFord, S., O'Driscoll, M. and MacDonald, A.
Abstract

Background: Foods for Special Medical purposes (Phe-free protein substitutes [PS] and low protein foods [LPFs]) are central to successful dietary management of patients with phenylketonuria (PKU). In the UK, PS and LPFs are prescribed by primary care general practitioners (GPs) and dispensed by high street pharmacists or via home delivery companies. As patients and carers commonly reported access issues with PS and LPFs through the National Society for PKU (NSPKU) helpline, the NSPKU collected more information about the extent of prescriptions issues via a questionnaire.
Methods: A cross sectional online survey was carried out (consisting of 7 multiple choice and 3 open questions). The questionnaire was available via the NSPKU website over a nine-month period (15th May 2018 to 31st January 2019). Carers or patients with PKU were invited to participate if they had any problems accessing PS and LPF over a 9-month period.
Results: There were 252 responses. 65% for children and 35% for adults, aged ≥18y with PKU. 59% (n=146/246) of responses described difficulties accessing basic LPF (bread, pasta) and 33% (n=81/248) PS. 36% (n=88/248) said problems had re-occurred during >1y, and 40% (n=99/248) for < 3 months. 59% (n= 146/246) had supply issues with LPF and 33% (n= 81/246) with PS. 18% (n=42/234) reported that the local NHS authority had refused, restricted or had a policy to block treatment access; 27% (n=63/234) cited GPs declining requests or restricting prescription amounts. 21% (n=49/234) could not access all their products via a pharmacy, 40% (n=93/234) said their home delivery system failed, and 15% (n=34/234) of the dispensed prescribed products were out of date, incorrect, damaged or poor quality. For patients and carers these issues caused: stress (87%, n=210/241); they considered PKU was not taken seriously by professionals (66%, n=160/241); they reduced patient access to LPF and PS (53%, n=128/241); and affected the patients’ health (53%, n=128/241).

KeywordsPhenylketonuria; Phenylalanine; Diet; Low protein special foods; Protein substitutes
PublisherElsevier
JournalMolecular Genetics and Metabolism Reports
ISSN2214-4269
Electronic2214-4269
Publication dates
Online25 Oct 2019
Print01 Dec 2019
Publication process dates
Deposited28 Oct 2019
Accepted27 Sep 2019
Submitted31 Jul 2019
Output statusPublished
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Open
Accepted author manuscript
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Restricted
Copyright Statement

© 2019. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/

Digital Object Identifier (DOI)https://doi.org/10.1016/j.ymgmr.2019.100527
Web of Science identifierWOS:000500718300018
LanguageEnglish
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