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UK Patient Access to Low-Protein Prescription Foods in Phenylketonuria (PKU): An Uneasy Path

2025; Multidisciplinary Digital Publishing Institute; Volume: 17; Issue: 3 Linguagem: Inglês

10.3390/nu17030392

2072-6643

Sharon Evans, Cameron Arbuckle, Catherine Ashmore, Sarah Bailey, Giana De Sousa, Wahid Chaudhry, Clare Dale, Anne Daly, B.R. Downey, Jane Dundas, Charlotte Ellerton, Suzanne Ford, Lisa Gaff, Joanna Gribben, Anne Grimsley, Melanie Hill, Laura Murphy, Camille Newby, Natalia Oxley, Rachel Pereira, Alex Pinto, Rachel Skeath, Alexander Sparks, Simon Tapley, Allyson Terry, Georgina Wood, Alison Woodall, Katie Yeung, Anita MacDonald,

Diet and metabolism studies

Background: Special low-protein foods are essential in the dietary treatment of phenylketonuria (PKU). In the UK, these are available on prescription through the General Practitioners (GPs) and distributed via nutritional home delivery companies or pharmacies. Methods: A 58-item online non-validated semi-structured questionnaire was emailed to British Inherited Metabolic Disease Group (BIMDG) dietitians and dietetic support workers (DSW)/administrators working in PKU to ascertain the main system issues and errors with the supply of low-protein prescription foods (LPPF). Results: 73% (n = 53/73) of dietitians and 72% (n = 18/25) of DSW/administrators responded. A total of 80 questionnaires (representing 44 paediatric and 36 adult PKU centres) were completed. A total of 50% (n = 40/80) of respondents reported patient/caregiver problems accessing LPPF at least weekly. The most common problems were unavailable products (82%), missing LPPF in deliveries (79%), and delayed deliveries (66%). For 64% of respondents, >25% of their patients had recurring problems accessing LPPF, and 69% of respondents spent ≥1 h/week and 11% >5 h/week correcting LPPF patient supply issues. The most common foods patients experienced supply issues with were bread (96%), pasta/rice (41%) and milk replacements (35%). This was associated with GP prescription errors (65%), LPPF prescriptions sent to incorrect dispensers/suppliers (60%), and manufacturer supply issues (54%). Problems with patients/caregivers included not ordering LPPF in a timely way (81%), not responding to messages from home delivery companies (73%) and poor understanding of the ordering process (70%). The majority (93%) of respondents reported that prescription issues impacted their patients’ blood Phe control. Suggestions for improving access to LPPF included centralisation of the system to one supplier (76%) and apps for ordering LPPF (69%). Conclusions: The supply of LPPF for PKU in the UK is problematic; it may adversely affect the ability of patients to adhere to dietary management, and a review investigating patient access to LPPF is urgently required.