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40% (n=93/234) said their home delivery system failed, and 15% (n=34/234) of the dispensed prescribed products were out of date, incorrect, damaged or poor quality.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
Publisher's version
License
File Access Level
Open
Accepted author manuscript
License
File Access Level
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
Permalink -

https://repository.mdx.ac.uk/item/88818

Log in to edit

Download files

Publisher's version
1-s2.0-S2214426919301260-main.pdf
License: CC BY-NC-ND 4.0
File access level: Open

  • 40
    total views
  • 11
    total downloads
  • 0
    views this month
  • 1
    downloads this month

Export as

Related outputs

Evaluation of an online screen-based simulation initiative in the adult child midwifery department
O'Driscoll, M. and Traynor, M. 2021. Evaluation of an online screen-based simulation initiative in the adult child midwifery department. Middlesex University.
Hungry for change: the experiences of people with PKU, and their caregivers, when eating out
Poole, G., Pinto, A., Evans, S., Ford, S., O'Driscoll, M., Buckley, S., Ashmore, C., Daly, A. and MacDonald, A. 2022. Hungry for change: the experiences of people with PKU, and their caregivers, when eating out. Nutrients. 14 (3). https://doi.org/10.3390/nu14030626
Provision and supervision of food and protein substitute in school for children with PKU: parent experiences
Jones, H., Pinto, A., Evans, S., Ford, S., O'Driscoll, M., Buckley, S., Ashmore, C., Daly, A. and MacDonald, A. 2021. Provision and supervision of food and protein substitute in school for children with PKU: parent experiences. Nutrients. 13 (11), pp. 1-13. https://doi.org/10.3390/nu13113863
Exploring factors having an impact on attitudes and motivations towards volunteering in the undergraduate nursing student population − A comparative study of the UK and Ghana
Dyson, S., Korsah, K., Liu, L., O'Driscoll, M. and van den Akker, O. 2021. Exploring factors having an impact on attitudes and motivations towards volunteering in the undergraduate nursing student population − A comparative study of the UK and Ghana. Nurse Education in Practice. 53, pp. 1-10. https://doi.org/10.1016/j.nepr.2021.103050
Accidental consumption of aspartame in phenylketonuria: patient experiences
Newbould, E., Pinto, A., Evans, S., Ford, S., O'Driscoll, M., Ashmore, C., Daly, A. and MacDonald, A. 2021. Accidental consumption of aspartame in phenylketonuria: patient experiences. Nutrients. 13 (2), pp. 1-13. https://doi.org/10.3390/nu13020707
HOw patients view extended half‐life products: impressions from real‐world experience (The HOPE study)
Khair, K., Pollard, D., Harrison, C., Hook, S., O'Driscoll, M. and Holland, M. 2019. HOw patients view extended half‐life products: impressions from real‐world experience (The HOPE study). Haemophilia. 25 (5), pp. 814-820. https://doi.org/10.1111/hae.13803
The extent, variability and attitudes towards volunteering among nursing students: What are the implications for pedagogy in nurse education?
O'Driscoll, M., Dyson, S., van den Akker, O. and Mehta, N. 2017. The extent, variability and attitudes towards volunteering among nursing students: What are the implications for pedagogy in nurse education? National Council of Voluntary Organisations Conference 2017. Nottingham, UK 07 - 08 Sep 2017
The extent, variability and attitudes towards volunteering among nursing students: Implications for pedagogy in nurse education
O'Driscoll, M., Dyson, S., Liu, L., van den Akker, O. and Mehta, N. 2017. The extent, variability and attitudes towards volunteering among nursing students: Implications for pedagogy in nurse education. 11th Annual International Conference on Sociology. Athens, Greece 01 - 04 May 2017
The UK Health and Social Care Act (2012) and new public management – more of the wrong medicine?
O'Driscoll, M. 2018. The UK Health and Social Care Act (2012) and new public management – more of the wrong medicine? ATINER: 4th Annual International Conference on Public Health. Athens, Greece 24 - 28 Jun 2018
Do governing body and CSU nurses on clinical commissioning groups really lead a nursing agenda? Findings from a 2015 Survey of the Commissioning Nurse Leaders’ Network Membership
O'Driscoll, M., Allan, H., Lee, G., Savage, J., Tapson, C. and Dixon, R. 2018. Do governing body and CSU nurses on clinical commissioning groups really lead a nursing agenda? Findings from a 2015 Survey of the Commissioning Nurse Leaders’ Network Membership. Journal of Nursing Management. 26 (3), pp. 245-255. https://doi.org/10.1111/jonm.12485
Compassion in Practice – evaluating the awareness, involvement and perceived impact of a national nursing and midwifery strategy amongst health care professionals in NHS Trusts in England
O'Driscoll, M., Allan, H., Liu, L., Corbett, K. and Serrant, L. 2018. Compassion in Practice – evaluating the awareness, involvement and perceived impact of a national nursing and midwifery strategy amongst health care professionals in NHS Trusts in England. Journal of Clinical Nursing. 27 (5-6), pp. e1097-e1109. https://doi.org/10.1111/jocn.14176
Reproductive experience of women living with phenylketonuria
Ford, S., O'Driscoll, M. and MacDonald, A. 2018. Reproductive experience of women living with phenylketonuria. Molecular Genetics and Metabolism Reports. 17, pp. 64-68. https://doi.org/10.1016/j.ymgmr.2018.09.008
Living with Phenylketonuria: lessons from the PKU community
Ford, S., O'Driscoll, M. and McDonald, A. 2018. Living with Phenylketonuria: lessons from the PKU community. Molecular Genetics and Metabolism Reports. 17, pp. 57-63. https://doi.org/10.1016/j.ymgmr.2018.10.002
The extent, variability, and attitudes towards volunteering among undergraduate nursing students: implications for pedagogy in nurse education
Dyson, S., Liu, L., van den Akker, O. and O'Driscoll, M. 2017. The extent, variability, and attitudes towards volunteering among undergraduate nursing students: implications for pedagogy in nurse education. Nurse Education in Practice. 23, pp. 15-22. https://doi.org/10.1016/j.nepr.2017.01.004
A report of a North Central and East London-wide evaluation of the CEPN multi-professional/collaborative learning groups
O'Driscoll, M., Traynor, M. and Dyson, S. 2017. A report of a North Central and East London-wide evaluation of the CEPN multi-professional/collaborative learning groups. Presented to Health Education England.
Nurses’ experiences of clinical commissioning group boards
Allan, H., O'Driscoll, M., Savage, J., Lee, G. and Dixon, R. 2016. Nurses’ experiences of clinical commissioning group boards. Nursing Standard. 30 (42), pp. 46-55. https://doi.org/10.7748/ns.2016.e9633
Are senior nurses on clinical commissioning groups in England inadvertently supporting the devaluation of their profession?: A critical integrative review of the literature
Allan, H., Dixon, R., Lee, G., O'Driscoll, M., Savage, J. and Tapson, C. 2016. Are senior nurses on clinical commissioning groups in England inadvertently supporting the devaluation of their profession?: A critical integrative review of the literature. Nursing Inquiry. 23 (2), pp. 178-187. https://doi.org/10.1111/nin.12129
Survey of the Commissioning Nurse Leaders’ Network membership 2015: summary of results
Allan, H. and O'Driscoll, M. 2016. Survey of the Commissioning Nurse Leaders’ Network membership 2015: summary of results. online Middlesex University.
An evaluation of the impact of ‘Compassion in practice: nursing, midwifery and care staff - our vision and strategy’ on staff experience in NHS Trusts in England.
Allan, H., O'Driscoll, M., Liu, L., Corbett, K. and Department of Adult, Child and Midwifery, School of Health and Education, Middlesex University 2015. An evaluation of the impact of ‘Compassion in practice: nursing, midwifery and care staff - our vision and strategy’ on staff experience in NHS Trusts in England. School of Health and Education, Middlesex University.