Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis.

INTRODUCTION: Deterioration in mental health has been reported in a minority of individuals with cystic fibrosis starting elexacafor/tezacaftor/ivacaftor (ELX/TEZ/IVA). We report our experience of using sweat chloride and markers of clinical stability to titrate dose reduction with the aim of minimising adverse events and maintaining clinical stability. METHOD: Adults (n = 266) prescribed ELX/TEZ/IVA, were included. Adverse events, sweat chloride, lung function and clinical data were collected. RESULTS: Nineteen (7.1%) individuals reported anxiety, low mood, insomnia and "brain fog" with reduced attention and concentration span. Thirteen underwent dose reduction with sweat chloride remained normal (<30 mmol l-1) or borderline (30-60 mmol l-1) in six (46.2%) and seven (53.2%) cases respectively. Improvement or resolution of AEs occurring in 10 of the 13 cases. CONCLUSION: Dose adjustment of ELX/TEZ/IVA was associated with improvement in mental health AEs without significant clinical deterioration. Sweat chloride concentration may prove useful as a surrogate marker of CFTR function.

Requirements and access needs of patients with chronic disease to their hospital electronic health record: results of a cross-sectional questionnaire survey.

OBJECTIVES: To identify patient's views on the functionality required for personalised access to the secondary care electronic health record (EHR) and their priorities for development. DESIGN: Quantitative analysis of a cross-sectional self-complete survey of patient views on required EHR functionality from a secondary care EHR, including a patient ranking of functionality. SETTING: Secondary care patients attending a regional cystic fibrosis unit in the north of England. PARTICIPANTS: 201 adults (106 (52.7%) males), median age 29 years (range 17-58 years), entered and completed the study. Inclusion criteria are as follows: a confirmed diagnosis of cystic fibrosis, aged 16 years and over, at the time of clinical stability. OUTCOME MEASURES: Quantitative responses within 4 themes; (1) value placed on aspects of the EHR; (2) access requirements to functions of the EHR; (3) views on information sent to the EHR and (4) patient feedback entered into the EHR. A ranked score for 15 functions of the EHR was obtained. RESULTS: Highest ratings (% reporting item as very important/important) were reported for access to clinical measures (lung function (94%), C reactive protein (84%), sputum microbiology (81%) and blood results (80%)), medication changes (82%) and lists (83%) and sending repeat prescription (83%) and treatment requests (80%), while sending symptom diaries was less so (62%). Email contact with clinicians was the most valuable communication element of the EHR (84% very important/important). Of 15 features of the EHR (1=most desirable to 15=least desirable), patients identified 'clinical measures' (2.62 (CI 2.07 to 3.06)), and 'access to medication lists' (4.91 (CI 4.47 to 5.44)), as highest priority for development and the ability to comment on errors/omissions (11.0 (CI 10.6 to 11.5)) or experience of care (11.8 (CI 11.4 to 12.2)) as lowest. CONCLUSIONS: Patients want extensive personal access to their hospital EHR, placing high importance on the viewing of practical clinical measures and medication management. These influence routine day-to-day care and are priorities for development.