Abstract
Patients with phenylketonuria (PKU) are reliant on special low protein foods (SLPFs) as part of their dietary treatment. In England, several issues regarding the accessibility of SLPFs through the national prescribing system have been highlighted. Therefore, prescribing patterns and expenditure on all SLPFs available on prescription in England (n = 142) were examined. Their costs in comparison to regular protein-containing (n = 182) and ‘free-from’ products (n = 135) were also analysed. Similar foods were grouped into subgroups (n = 40). The number of units and costs of SLPFs prescribed in total and per subgroup from January to December 2020 were calculated using National Health Service (NHS) Business Service Authority (NHSBSA) ePACT2 (electronic Prescribing Analysis and Cost Tool) for England. Monthly patient SLPF units prescribed were calculated using patient numbers with PKU and non-PKU inherited metabolic disorders (IMD) consuming SLPFs. This was compared to the National Society for PKU (NSPKU) prescribing guidance. Ninety-eight percent of SLPF subgroups (n = 39/40) were more expensive than regular and ‘free-from’ food subgroups. However, costs to prescribe SLPFs are significantly less than theoretical calculations. From January to December 2020, 208,932 units of SLPFs were prescribed (excluding milk replacers), costing the NHS £2,151,973 (including milk replacers). This equates to £962 per patient annually, and prescribed amounts are well below the upper limits suggested by the NSPKU, indicating under prescribing of SLPFs. It is recommended that a simpler and improved system should be implemented. Ideally, specialist metabolic dietitians should have responsibility for prescribing SLPFs. This would ensure that patients with PKU have the necessary access to their essential dietary treatment, which, in turn, should help promote dietary adherence and improve metabolic control.
Highlights
Phenylketonuria (PKU), an inborn error of amino acid metabolism, is caused by phenylalanine hydroxylase deficiency, an enzyme that converts phenylalanine to tyrosine [1]
This is the first study to examine the cost of all special low protein foods (SLPFs) available on prescription in England compared to regular and ‘free-from’ foods available in supermarkets
It is the first study to examine the number and type of low protein items prescribed and expenditure on individual SLPFs and total SLPFs prescribed by the National Health Service (NHS) in England over 1 year
Summary
Phenylketonuria (PKU), an inborn error of amino acid metabolism, is caused by phenylalanine hydroxylase deficiency, an enzyme that converts phenylalanine to tyrosine [1] This leads to neurotoxicity, causing severe intellectual disability if untreated [2]. Regular protein containing foods e.g., bread, flour and pasta, are replaced with special low protein foods (SLPFs) that contain minimal protein [2,3] These deliver a substantial source of energy, providing up to 50% of daily energy intake [4,5,6], fibre [7], they offer essential bulk, add variety and so help to sustain dietary adherence and aid metabolic control [8,9,10]
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