Background and aims Metabolic decompensation may occur in patients with disorders of intermediary metabolism during intercurrent illness. Early intervention strategies are crucial in order to halt decline. This poses a particular challenge in emergency departments (EDs), particularly at peak times. Impending deterioration may not be clinically apparent, and so may not result in prioritisation for initial assessment or subsequent triage categorisation. It is therefore crucial to ensure appropriate care pathways are in place, yet there are no existing national guidelines regarding timeliness of ED assessment for these patients. We therefore aimed to assess current practice to inform service development.

Methods Retrospective electronic database and medical chart review over a 3 month period. Nine standards were set through consensus between the ED and inherited metabolic disease (IMD) teams, relating to timeliness, notifications and assessment criteria (grade of clinician and assessment performed).

Results Of 38 IMD presentations, 30 were deemed at risk of decompensation due to their condition. 33 (92%) had an electronic diagnosis alert, 28 (83%) had a specific electronic ED management plan. 21 (54%) were triaged within 15 min of arrival, and following triage 8 (21%) and 19 (50%) were seen within 10 and 30 min respectively. There was no apparent correlation with triage category. 8 were discharged prior to senior review 6 of which were at risk of decompensation. 13 of the 30 patients at risk of decompensation received IV/NG fluids 5 of which were within an hour.

Conclusions Patients at risk of decompensation due to an inborn error of intermediary metabolism are difficult to identify, and subsequently may not fulfil criteria for urgent assessment. Use of electronic alerts, in conjunction with training, departmental policies, and tailored management plans are likely to improve care, and have been implemented. Clarity of communication is key, specifically in identifying those most likely to deteriorate unexpectedly. However, this study was not designed to identify whether or how quickly any patient deteriorated. Further multicentre work is therefore crucial to delineate optimal assessment and intervention timelines, and will result in the development of national standards that will improve both short and long term outcomes for this vulnerable group.