AimTo investigate whether the current practice of liver function test (LFT) monitoring within a large tertiary paediatric cystic fibrosis centre adheres to local and national guidelines with respect to baseline monitoring, frequency of monitoring in the first year of treatment and action taken in the event of abnormal results for patients newly started on Kaftrio® treatment.MethodAll patients currently receiving Kaftrio® treatment were identified. Patients were excluded if they had not been on treatment for one year, if they had prolonged gaps in treatment or if they had bloods taken at outreach clinics with results not accessible to the investigator. Data was retrieved from each patient’s electronic laboratory reports and recorded on a data collection form. Data collected included: date Kaftrio® started; did the patient have baseline LFTs within one year before starting; did the patient have their first LFTs done 3 months after starting; in the first year did the patient have four sets of LFTs at 3-month intervals; were LFTs in range; if LFTs were abnormal was appropriate action taken. A 2-week tolerance was permitted. The data was analysed to assess compliance to guidelines. Assessments of transaminases (ALT and AST) and total bilirubin are recommended prior to initiating treatment then every 3 months during the first year of treatment.1–3Elevated ALT/AST two to three times the upper limit of normal (ULN) with normal serum bilirubin should prompt repeat LFTs within 8 weeks.1ALT/AST greater than five times ULN, or greater than three times ULN with high bilirubin warrants treatment being interrupted.1–3Results31 patients were reviewed. 30 patients (97%) had baseline LFTs. 10 patients (32%) had LFTs at 3 months. Of the 21 patients who did not, 15 of those had their first set of LFTs taken too early at 1 or 2 months and 6 were taken late at 4,5 or 6 months. Zero patients had four sets of LFT results taken at the correct 3-monthly intervals in the first year. 4 patients (13%) had at least one set of abnormal LFTs. 50% of abnormal results were acted upon in accordance with guidelines.ConclusionThe results showed that, other than baseline monitoring, current practice is not in accordance with guidelines both in relation to frequency of LFT monitoring and appropriate action being taken on the finding of abnormal results. Further study is required to investigate the reasons for poor outcomes and how compliance with the guidelines can be improved. Encouragingly, the rate of liver impairment as an adverse drug reaction was low. Trial data should be reviewed to assess the significance of waiting until month 3 to start LFT monitoring (i.e. when do deranged LFTs typically first manifest?) and therefore whether early testing is problematic (in patients with no history of liver impairment). Guideline development should be an area of focus to put measures in place to improve guideline compliance. However, with MDT agreement, consideration should be given to adapting local practice to deviate from national guidance to better fit real-world practice.ReferencesTrust guideline. Management of children with Cystic Fibrosis. (local access only).Vertex Pharmaceuticals (Europe) Ltd (2022) Kaftrio 75 mg 50 mg 100 mg film coated tablets. Available at: https://www.medicines.org.uk/emc/product/11724/smpc [Accessed June 2022]MHRA (2022) Drug Safety Update Ivacaftor, tezacaftor, elexacaftor (Kaftrio▾) in combination with ivacaftor (Kalydeco): risk of serious liver injury; updated advice on liver function testing. Available at: https://www.gov.uk/drug-safety-update/ivacaftor-tezacaftor-elexacaftor-kaftriov-in-combination-with-ivacaftor-kalydeco-risk-of-serious-liver-injury-updated-advice-on-liver-function-testing [Accessed June 2022]

Children admitted to our hospital with cystic fibrosis had frequent medication errors due to polypharmacy and addition of specialist and high-risk medications despite an electronic prescribing and medicines administration system in place. We describe a multidisciplinary quality improvement project that combined a computerised order entry system (CPOE) with human factor process changes. Over 12 months, our run chart showed a 43% reduction in prescription errors. For medications prescribable via the CPOE, errors reaching the patient reduced from 50% to 29%. Electronic prescribing can be seen by clinicians as a fixed unalterable system contributing to rather than ameliorating errors. Improving safety requires whole team engagement and working closely with programmers to adapt function and influence human factors.

AimAssess the incidence, type and severity of prescribing errorsAssess the use and efficacy of a pre–existing prescribing aidReduce the number and severity profile of prescribing errors through increased use of the prescribing aid.MethodTwenty cystic fibrosis (CF) patients, admitted consecutively to a tertiary paediatric centre, were included in the study. Use of the pre-existing CF prescribing aid and prescribing errors were detected prospectively by daily pharmacist review of electronic drug charts. A doctor and a pharmacist then retrospectively assigned a severity score (A-I), using a standardised tool.1 Ten patients were evaluated initially. Following this, paediatric CF patients were tagged on the electronic prescribing system, creating a pop-up alert to direct prescribers to the CF prescribing aid when those patients’ electronic medicine records were opened.ResultsTen patients (163 medication orders) were evaluated in phase I pre-intervention, and ten patients (157 medication orders) were evaluated in phase II post-intervention.In total 127 prescribing errors were recorded. The most common types of prescribing error were: failing to prescribe a patient’s regular medicine (27%); prescribing the wrong formulation (19%); prescribing the wrong dose (14%).No errors, in either phase of the study, caused patient harm. The highest severity score in this study was awarded to prescribing errors that reached the patient, did not cause harm but required intervention to preclude harm or required extra monitoring (category D).1 Throughout both phases, 32% of error containing orders were related to medicines that were not included in the CF prescribing aid, the error rate was comparable in both phases (30%; 35%). These medicines were non-CF specific drugs therefore not appropriate to be included in the prescribing aid. These medication orders were excluded from further pre- and post- intervention analysis.In the pre-intervention group, 100 CF medication orders were prescribed. The prescribing aid was used in 42% of orders. 43% of medication orders had at least one prescribing error. 27% of errors were of severity category D.In the post-intervention group, 104 CF medication orders were prescribed. The prescribing aid was used in 70% of orders. 27% of medication orders had at least one prescribing error. 15% of errors were of severity category D.ConclusionIntroducing a paediatric CF alert system successfully increased prescriber utilisation of the CF prescribing aid, with subsequent reduction in error rate and severity of CF specific medicines. We are confident that this improvement reflects our intervention, although without in-depth statistical analysis we cannot attribute causality. However, errors still occurred despite the use of the prescribing aid and errors occurred in non-CF specific medicines. A continuous quality improvement approach is being adopted to explore alternative causes for error and further interventions that can be made, with focus on dovetailing inpatient and outpatient prescribing and medication reco...