A novel cystic fibrosis-pregnancy service: Two-year review after introduction of elexacaftor/tezacaftor/ivacaftor

Background: As a quality service improvement response since elexacaftor/ tezacaftor/ivacaftor (ELX/TEZ/IVA) became available and the yearly average number of cystic fibrosis (CF) pregnancies (n = 7 pre-2020, n = 33 in 2021) increased significantly at an adult CF center (~600 people with CF), a monthly multidisciplinary CF-maternal health virtual clinic was established with antenatal virtual CF exercise classes dedicated to providing adaptive, specialist support to this cohort, aswell as outreach guidance and education to local obstetric teams. Method(s): This was a single-center retrospective reviewof Royal Brompton Hospital CF-Maternal Health multidisciplinary team clinic records and a patient survey from March 2020 to March 2022. Result(s): Of 47 pregnancies in 41 women (median age 30;) eligible for ELX/ TEZ/IVA at start of pregnancy, 40% (n = 19) were unplanned, and 19% (n = 9) used assisted conception. Three women with a history of infertility conceived naturally, having required assisted conception for previous pregnancies, and five women had multiple pregnancies during the study period. ELX/TEZ/IVA was continued in 60% (n = 28), delayed in 28% (n = 13), and stopped in 13% (n = 6) of pregnancies through maternal choice and careful clinical counselling. Pre-pregnancy pulmonary status was poorer in women who continued than in those who delayed or stopped (Table 1). Of those who stopped, 85% (n = 5) restarted because of pulmonary deterioration by the third trimester. Prenatal CF complications included at least one episode of minor hemoptysis in 21% (n = 9/41) of women, at least one infective exacerbation in 55% of pregnancies (n = 26/47), and noninvasive ventilation in one woman. Other pregnancy-associated complications included one case of ovarian hyperstimulation syndrome, one case of sub-segmental pulmonary embolism, and two cases of pregnancy-induced hypertension. Excluding 10 first trimester terminations, 10 current pregnancies, and one patient relocation, obstetric outcomes available for 26 pregnancies confirmed a live birth rate of 85% (n = 22/26) and a 15% first-trimester miscarriage rate (n = 4). Obstetric complications included preterm delivery rate of 23% (n = 6/26), including two cases of COVID infection resulting in two neonatal intensive care unit admissions, one case of endometritis after cesarean section, and a fourthdegree perineal tear. There were no ectopic pregnancies, maternal or neonatal deaths, or reports of infant cataracts or congenital malformations. Median gestational age was 37/40 weeks (range 29-40). Mode of delivery was via cesarean section in 45% (n = 10/22, of which twowere emergency) and vaginal in 55% (n = 12/22), of which 83% (n = 10/12) were via induction of labor for diabetes (CF or gestational) indication. Deliveries were supported and occurred equally at local obstetric units and in tertiarycare obstetric hospital settings (50%, n = 11/22). Patient-experience survey responses cited high levels of confidence in health optimization and prioritization during pregnancy and praised excellent inter-health care provider communication and peer-to-peer emotional support provided among expectant mothers in the virtual prenatal exercise groups. Table 1. Baseline demographic and clinical characteristics of elexacaftor/tezacaftor/ivacaftoreligible expectant mothers according to therapeutic decision (Table Presented) Conclusion(s): In the absence of clinical trial safety data, the novel approach of a dedicated CF-maternal health multidisciplinary team clinic with local obstetric outreach support has ensured regular specialist clinical and emotional peer-to-peer support for this cohort of women eligible for ELX/ TEZ/IVA to ensure optimal outcomes and experiences of their pregnancies, where appropriate, close to home.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved

Implementing tablet-based ototoxicity screening in adults with cystic fibrosis

Introduction: People with cystic fibrosis (PwCF) regularly receive antibiotics for treatment of lung infections, which can include intravenous aminoglycosides (IVAG) resulting in potential ototoxicity. Sound booth audiometry is costly, time-consuming, and requires further outpatient visits and audiologists. A quality improvement project delivered by specialist pharmacists to implement a tablet audiometry ototoxicity monitoring programme was launched in PwCF receiving IVAG therapy. Objectives: To implement a tablet ototoxicity screening programme in adults with CF. Methods: PwCF receiving IVAG completed tablet-based audiometry (0.25– 16 kHz) (Shoebox MD) alongside validated ototoxicity questionnaires at the beginning and end of treatment. Following a clinical pathway, clinicians undertook shared decision-making processes regarding continuation of AG if abnormality was detected, alongside referral for sound-booth audiometry. Results: Data were collected from April–Dec 2021. Thirty-eight patients (median [IQR] age 28.5 ([15.5] years;mean [SD] ppFEV1 62.3 [26.5]) were screenedwhowere on IVAG. Fifteen patients (39%)were referred for formal audiometry due to abnormal baseline results, of which 5 had symptoms of hearing loss identified through questionnaires. 3% (1/38) stopped AG therapy due to identified potential ototoxic risk. Twenty-two patients received screening at beginning and end of IVAG therapy: significant ototoxic effects were seen in 2 of these patients (9%);20 patients (91%) had no significant change from baseline audiometry. Conclusions: We present pilot results to show feasibility of tablet-based ototoxicity screening. More IV courses were completed at home than anticipated (due to Covid19) limiting end of IV testing. Further 3-month testing is planned to detect potential delayed ototoxic change. Nevertheless, our results show tablet audiometry to be an effective and practical screening tool used by non-audiologists for accurate, early identification of hearing loss and ototoxicity.

116 Implementing a digital clinic review service in a national lockdown

Background: In the United Kingdom, the national COVID lockdown was legally enforced on the March 26, 2020. As a result, people with CF were classed as clinically extremely vulnerable and were advised to shield at home. In this hospital outpatient clinics were halted;however, the adultCF service had started using the NuvoAir Home remote monitoring solution with 162 patients already established with home spirometry, video consultations, and data sharing with the clinical team. Our aim was to evaluate our experience of replacing all face-to-face clinical reviews with video consultations supported by self-monitoring, at pace and scale during the COVID-19 pandemic. Methods:FromApril2020, 2 members of the CF MDT started contacting all patients not on the NuvoAir Home platform (n=418)to discuss the virtual service and askiftheywere interested in joining. Onconsent, patients were given access to the platform, taught how to download the app, and told how to set up the spirometers (which were posted to them) and how often to perform spirometry. A dedicated email address and telephone line were set up for technical support. Patients were sent an SMS reminder via the platform to complete their spirometry prior to a video clinic appointment but also encouraged to monitor their spirometry at other times to build a personalized trend. Results: To date (March 31, 2021) 558 patients have been onboarded to the remote monitoring program. Pre-pandemic (month of March 2020), 417 spirometry sessions were recorded and 82 video consultations performed. Midway through (in the month of August 2020) n = 539 spirometry sessions had been recorded, with n = 325 video consultations, and in the month of March 2021, n = 609 spirometry sessions and n = 438 video consultations were recorded. Twenty-eight percent of spirometry sessions were unrelated to a video clinic. Total spirometry sessions for April 1, 2020, through March 31, 2021, were n = 6,969. Using ATS criteria n = 5,264 (76%) sessions were graded acceptable (A-D), and n = 1,705 (24%) sessions graded E+F. Reasons patients performed extra spirometry included: checking the effectiveness of treatment change, pre-clinic consultation, feeling unwell, and recovery after an exacerbation. Conclusion: We now have 558 adults with CF onboarded to the virtual platform. Although patients are reminded to do spirometry before an appointment, many also choose to self-monitor their health between clinic consultations. Clinician confidence in self-monitoring is supported by the grading of spirometry sessions using ATS criteria;the best is selected by the software. The platform is now also widely used by the CF MDT for one-to-one or small group support and education sessions. There are a small number of patients (n = 5) who do not wish to use this service for various reasons, including reluctance to change the status quo, hearing impairment, and access to technology. Alternatives are in place for those individuals. Moving the service forward we have added weighing scales and activity trackers, and organized postal, self-administered finger prick blood tests (e.g., liver function tests) and sputum samples. Patients have reported high satisfaction with the service as they are gaining more time for work, education, and family life, as well as saving money. However, they have also reported missing face-to-face contact. We are therefore working closely with patients to devise a hybrid virtual/face-to-face service for the future.