Development of national consensus statements on food labelling interpretation and protein allocation in a low phenylalanine diet for PKU

British Inherited Metabolic Diseases Group (BIMDG) Dietitians Group,
Sharon EvansEmail author View ORCID ID profile,
Suzanne Ford,
Sarah Adam,
Sandra Adams,
Jane Ash,
Catherine Ashmore,
Gillian Caine,
Rachel Carruthers,
Sarah Cawtherley,
Satnam Chahal,
Anne Clark,
Barbara Cochrane,
Anne Daly,
Karen Dines,
Marjorie Dixon,
Carolyn Dunlop,
Charlotte Ellerton,
Moira French,
Lisa Gaff,
Cerys Gingell,
Diane Green,
Joanna Gribben,
Anne Grimsley,
Paula Hallam,
Una Hendroff,
Melanie Hill,
Rachel Hoban,
Sarah Howe,
Inderdip Hunjan,
Kit Kaalund,
Eimear Kelleher,
Farzana Khan,
Steve Kitchen,
Karen Lang,
Sharan Lowry,
Jo Males,
Georgina Martin,
Nicola McStravick,
Avril Micciche,
Camille Newby,
Claire Nicol,
Rachel Pereira,
Louise Robertson,
Kathleen Ross,
Emma Simpson,
Kath Singleton,
Rachel Skeath,
Jacqueline Stafford,
Allyson Terry,
Ruth Thom,
Alison Tooke,
Karen vanWyk,
Fiona White,
Lucy White and
Anita MacDonald

Orphanet Journal of Rare Diseases201914:2

https://doi.org/10.1186/s13023-018-0950-z

Received: 19 February 2018
Accepted: 2 November 2018
Published: 3 January 2019

Abstract

Background

In the treatment of phenylketonuria (PKU), there was disparity between UK dietitians regarding interpretation of how different foods should be allocated in a low phenylalanine diet (allowed without measurement, not allowed, or allowed as part of phenylalanine exchanges). This led to variable advice being given to patients.

Methodology

In 2015, British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 70) were sent a multiple-choice questionnaire on the interpretation of protein from food-labels and the allocation of different foods. Based on majority responses, 16 statements were developed. Over 18-months, using Delphi methodology, these statements were systematically reviewed and refined with a facilitator recording discussion until a clear majority was attained for each statement. In Phase 2 and 3 a further 7 statements were added.

Results

The statements incorporated controversial dietary topics including: a practical ‘scale’ for guiding calculation of protein from food-labels; a general definition for exchange-free foods; and guidance for specific foods. Responses were divided into paediatric and adult groups. Initially, there was majority consensus (≥86%) by paediatric dietitians (n = 29) for 14 of 16 statements; a further 2 structured discussions were required for 2 statements, with a final majority consensus of 72% (n = 26/36) and 64% (n = 16/25). In adult practice, 75% of dietitians agreed with all initial statements for adult patients and 40% advocated separate maternal-PKU guidelines. In Phase 2, 5 of 6 statements were agreed by ≥76% of respondents with one statement requiring a further round of discussion resulting in 2 agreed statements with a consensus of ≥71% by dietitians in both paediatric and adult practice. In Phase 3 one statement was added to elaborate further on an initial statement, and this received 94% acceptance by respondents. Statements were endorsed by the UK National Society for PKU.

Conclusions

The BIMDG dietitians group have developed consensus dietetic statements that aim to harmonise dietary advice given to patients with PKU across the UK, but monitoring of statement adherence by health professionals and patients is required.