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

The Impact of a Community Tour on Incoming Residents During Orientation at an Inner-City Hospital

Study Objective: Healthcare providers at inner-city hospitals face the unique challenge of learning how to care for disproportionately impoverished patient populations. We investigate the impact of an institution-wide community tour on incoming residents’ sense of preparedness to provide care to impoverished patients and their knowledge of social determinants of health. Methods: A two-hour voluntary bus tour of an inner-city hospital’s catchment area was offered to incoming residents during orientations in June 2016 and 2017. Residents visited a local school, healthcare clinic, needle-exchange program, police precinct, homeless shelter, and community park, among other locations. Pre-tour surveys were collected from individuals present at orientation, and post-tour surveys were collected from those who attended. A 5-point Likert scale questionnaire was used to gauge preparedness to work with impoverished patients and subjective knowledge of social determinants of health. Numerical values of 1 to 5 were designated as strongly disagree, disagree, neutral, agree, and strongly agree, respectively. Numerical values of 4 and 5 were deemed as “high.” A 9-option multiple choice question assessed objective knowledge of the hospital’s catchment area poverty rate. Fisher’s exact test was used to determine statistical significance. Results: 219 incoming residents completed the pre-tour survey, 91 demonstrated interest in attending the tour (41.6%), and 76 completed the post-tour survey. Of the 219 residents who completed the pre-tour survey, majority specialized in internal medicine (89, 40.6%), pediatrics (32, 14.6%), OMFS (25, 11.4%), transitional year (25, 11.4%), and emergency medicine (19, 8.7%);the remainder were psychiatry, OB-GYN, and general surgery. The majority of participants endorsed previous experience working with underserved populations (196 of 219, 89.5%). A significantly larger percentage of participants rated their preparedness to work with impoverished patients as “high” after the tour (63/76, 82.9%) than before (157/219, 71.7%) (Fisher’s exact test p = 0.0021). Similarly, a large fraction rated their knowledge of social determinants of health as “high” after the tour (64/76, 84.2%) than before (85/219, 38.8%) (Fisher’s exact test p < 0.0001). The percent of participants reporting the correct poverty rate significantly increased from 13/219 (5.9%) to 19/76 (25%) (Fisher’s exact test p < 0.0001). Conclusion: This study demonstrates that a community bus-tour of an inner-city hospital’s catchment area augmented incoming residents’ preparedness to work with an impoverished population and their knowledge of social determinants of health. As most participants demonstrated similar levels of preparedness and knowledge prior to the tour, it is likely that new physicians would benefit from direct exposure to their hospitals’ surrounding communities at the start of residency. As COVID-19 health measures are mitigated, residency programs should strongly consider tours or other community immersion experiences to assist with new residents’ knowledge and preparedness to care for their respective patient communities.

Rollout of Kaftrio® to adult cystic fibrosis patients at the Royal Brompton Hospital during a global pandemic

Objectives: To review the rollout process for Kaftrio® at this large adult CF Centre following approval by the European Medicines Agency (EMA) in June 2020, EMA marketing authorisation and commissioning by NHS England on 21st August 2020, taking into consideration the limitations imposed on clinical services due to COVID-19. Methods: Recent rollout of Symkevi® meant that this adult CF service had already developed processes for the identification of suitable patients and medication distribution. Due to COVID-19 restrictions, innovative methods for clinical review, patient discussion, testing for liver function and occasionally genotype were developed. Furthermore, contract extensions and new paperwork for Homecare companies had to be produced as medication was delivered to patients’ homes. This established process involving multidisciplinary digital consultations, Bluetooth spirometry and self-administered postal blood tests was therefore replicated. Additionally, to ensure access for as many patients as possible we re-checked some genetic mutation results, especially where diagnosis pre-dated current testing technology. Results: This table shows the rate at which our patients were able to access Kaftrio®. Two thirds of patients had access to Kaftrio® a month after it was commissioned and 265 of the 348 eligible patients were taking Kaftrio® within 2 months. [Table presented] Conclusion: Using our experience from the launch of Symkevi®, we were able to adapt to this much larger rollout to despite the limitations imposed by the pandemic. To ensure that patients would benefit from early access we started putting processes in place before Kaftrio® was licensed by the EMA. As soon as commissioning was announced by NHS England, we were ready to start prescribing. This allowed our patients to access Kaftrio® very quickly with 144 out of 348 eligible patients taking their initial dose just over 2 weeks later and 286 patients on treatment after just over 2 months.

TENSION PNEUMOMEDIASTINUM IN A PATIENT WITH COVID-19 PNEUMONIA

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Mechanical ventilation can commonly cause mediastinal emphysema which in rare cases can convert to tension pneumomediastinum. This complication can result in cardiopulmonary compromise and decompression may be lifesaving. CASE PRESENTATION: A 53 year old woman with a history of ulcerative colitis, obstructive sleep apnea, chronic kidney disease and diabetes developed renal failure requiring dialysis and severe ARDS due to COVID 19. She was ventilated with 6cc/kg tidal volumes and a PEEP of 12cm/H2O to maintain a plateau pressure less than 30cm/H2O and a driving pressure under 15cm/H2O. She developed extensive subcutaneous emphysema and bilateral subcutaneous angiocatheters were placed. After initial clinical improvement sedation was decreased but with any awakening our patient became dyssynchronous and unstable. CT scan of the head and chest showed large volume pneumomediastinum with minimal pneumoperitoneum and pneumocephalus and no pneumothorax. Tension pneumomediastinum was assumed to be contributing to her hemodynamic instability and decompression of her mediastinum was pursued. A 10F pigtail catheter was inserted into the anterior mediastinum under CT guidance. Approximately 100 cc of air was drained during the procedure with immediate improvement in heart rate, oxygen saturation and ventilator pressures. The pigtail was attached to a pleurovac at 20 cc/H2O suction. Ventilator settings were reduced and subsequent reduction in sedation was accompanied by ventilator synchrony and she was able to follow commands. Unfortunately, despite this improvement, this patient eventually succumbed to COVID related illness. DISCUSSION: Pneumomediastinum may be spontaneous or secondary. Symptoms may include dyspnea, chest pain, and cough. Clinical signs are nonspecific and include tachycardia, ECG changes, and rarely tension physiology from direct cardiac and lung compression. This impedes venous return and increases ventilator pressures(1,3). Chest radiograph is a common diagnostic tool for pneumomediastinum but is limited(2). CT chest is important(2) particularly in cases with no improvement. Several techniques have been indicated in the relief of the tension physiology(1,2,3). We placed a pigtail catheter in the most common site: the anterior mediastinum. Rarely, mediastinotomy and sternotomy(1) may be done in resistant cases. If missed, more severe complications like pneumopericardium may result causing tamponade. Early diagnosis is key. CONCLUSIONS: Tension Pneumomediastinum is a life-threatening and early diagnosis and intervention is key. Reference #1: Clancy DJ, Lane AS, Flynn PW, Seppelt IM. Tension pneumomediastinum: A literal form of chest tightness. J Intensive Care Soc. 2017;18(1):52-56. doi:10.1177/1751143716662665 Reference #2: Beckett A, Tien H, Engels P, Paton-Gay JD, Rizoli S. Tension pneumomediastinum. J Trauma. 2011;71(4):1089. doi:10.1097/TA.0b013e31820edd2a Reference #3: Wolfe MW, Meltzer JS. Delayed Tension Pneumomediastinum after Cardiac Surgery. Anesthesiology. 2018;129(4):809. doi:10.1097/ALN.0000000000002257 DISCLOSURES: No relevant relationships by Corrielle Caldwell, source=Web Response No relevant relationships by Aditi Desai, source=Web Response No relevant relationships by David Hirschl, source=Web Response no disclosure on file for Seth Koenig;