Browsing by Author "Elbert, Alexander"

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    Changes in Care during the COVID-19 Pandemic for People with Cystic Fibrosis
    (American Thoracic Society, 2022) Sanders, Don B.; Wu, Runyu; O’Neil, Tom; Elbert, Alexander; Petren, Kris; Jain, Raksha; Ren, Clement L.; Pediatrics, School of Medicine
    Rationale: Cystic fibrosis (CF) centers transitioned to telemedicine during the spring 2020 peak of the coronavirus disease (COVID-19) pandemic. Objectives: We hypothesized that people with CF (pwCF) with more severe disease would be more likely to be seen in-person. Methods: We used paired t tests to compare within-subject changes in body mass index (BMI) and percentage predicted forced expiratory volume in one second (FEV1) and calculated relative risk (RR) to compare pulmonary exacerbations (PEx) between pwCF enrolled in the CF Foundation Patient Registry with at least one in-person clinic visit after March 15 in both 2019 and 2020. Results: Overall, the proportion of clinical encounters that were in-person clinic visits decreased from 91% in 2019 to a low of 9% in April 2020. Among pwCF seen after March 15 in both 2019 and 2020, the mean (95% confidence interval [CI]) FEV1 percentage predicted was 1.3% (0.1-2.4) predicted higher in 2020 for children 6 to <12 years of age, and 7.5% (7.1-7.9) predicted higher in 2020 among pwCF ⩾12 years of age eligible for the highly effective CF transmembrane conductance regulator modulator, elexacaftor-tezacaftor-ivacaftor (ETI). There was no difference in FEV1 percentage predicted for pwCF ⩾12 years of age who were not eligible for ETI. Similarly, the mean (95% CI) BMI was 2.4 (2.0-2.8) percentile higher in 2020 for children 6 to <12 years of age and 5.2 (4.8-5.7) percentile higher in 2020 among children 12 to <18 years of age eligible for ETI. Mean (95% CI) BMI was 1.2 (1.2-1.3) (kg/m2) higher for pwCF ⩾18 years of age eligible for ETI, and 0.2 (0.1-0.3) (kg/m2) higher for pwCF ⩾18 years of age not eligible for ETI. The proportion of in-person clinic visits where any PEx was present was lower in 2020 compared with 2019, 25% compared with 38%, RR 0.82 (0.79-0.86). Conclusions: The care of pwCF was substantially changed during the spring 2020 peak of the COVID-19 pandemic. Among pwCF seen in-person in both 2019 and 2020 after the spring peak of the COVID-19 pandemic, lung function and BMI were higher in 2020 for children 6 to <12 years of age and pwCF eligible for ETI.
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    Quantity not sufficient rates and delays in sweat testing in US infants with cystic fibrosis
    (Wiley, 2020-11) McColley, Susanna; Elbert, Alexander; Wu, Runyu; Ren, Clement; Sontag, Marci; Legrys, Vicky A.; Pediatrics, School of Medicine
    Background Diagnostic sweat testing is required for infants with positive newborn-screening (NBS) tests for cystic fibrosis (CF). Infants have “quantity not sufficient” (QNS) sweat volumes more often than older children. A comprehensive study of QNS sweat volumes in infants has not previously been reported. Methods We surveyed US CF Centers to obtain QNS rates in all infants who had sweat testing at under 14 days and under 3 months of age. We then calculated QNS rates reported to the Cystic Fibrosis Foundation Patient Registry (CFFPR) 2010-2018 in 10-day increments from 1 to 60 days of life. We compared QNS sweat test rates in preterm (<37-weeks gestational age) vs term infants. We assessed age at sweat test and proportion of infants who did not have a sweat test reported by 60 days of age. Results Thirty-nine of 144 (27%) of CF Centers reported a mean QNS rate of 10.5% (range, 0-100) in infants 14-days-old or younger. CFFPR data showed the highest QNS rates in the youngest infants and in those born before 37 weeks of gestation. The median age at sweat testing decreased over time, but more than 22% of infants did not have a sweat test reported by 60 days. Conclusion Higher QNS rates are seen in the youngest infants with CF, but more than 80% of infants younger than 2 weeks of age have adequate sweat volumes. Sweat testing should not be delayed in infants with a positive CF NBS test.