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CFTR modulator therapy: transforming the landscape of clinical care in cystic fibrosis.
Taylor-Cousar JL, Robinson PD, Shteinberg M, Downey DG. Taylor-Cousar JL, et al. Lancet. 2023 Sep 30;402(10408):1171-1184. doi: 10.1016/S0140-6736(23)01609-4. Epub 2023 Sep 9. Lancet. 2023. PMID: 37699418 Review.
Following discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 and subsequent elucidation of the varied CFTR protein abnormalities that result, a new era of cystic fibrosis management has emerged-one in which scientific principles translated fr …
Following discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 and subsequent elucidation of the varied …
The modern landscape of fertility, pregnancy, and parenthood in people with cystic fibrosis.
Jain R, Kazmerski TM, Taylor-Cousar JL. Jain R, et al. Among authors: taylor cousar jl. Curr Opin Pulm Med. 2023 Nov 1;29(6):595-602. doi: 10.1097/MCP.0000000000001009. Epub 2023 Sep 1. Curr Opin Pulm Med. 2023. PMID: 37789771 Review.
PURPOSE OF REVIEW: With improved long-term survival and the expanding availability of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies that treat the underlying genetic defect in cystic fibrosis, more people are interested in parenthood. ...
PURPOSE OF REVIEW: With improved long-term survival and the expanding availability of cystic fibrosis transmembrane conductance regul …
Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study.
Daines CL, Tullis E, Costa S, Linnemann RW, Mall MA, McKone EF, Polineni D, Quon BS, Ringshausen FC, Rowe SM, Selvadurai H, Taylor-Cousar JL, Withers NJ, Ahluwalia N, Moskowitz SM, Prieto-Centurion V, Tan YV, Tian S, Weinstock T, Xuan F, Zhang Y, Ramsey B, Griese M; VX17-445-105 Study Group. Daines CL, et al. Among authors: taylor cousar jl. Eur Respir J. 2023 Dec 7;62(6):2202029. doi: 10.1183/13993003.02029-2022. Print 2023 Dec. Eur Respir J. 2023. PMID: 37945033 Free PMC article. Clinical Trial.
BACKGROUND: In two pivotal phase 3 trials, up to 24 weeks of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious and safe in patients with cystic fibrosis (CF) 12 years of age who have at least one F508del allele. The aim of this study is to assess long- …
BACKGROUND: In two pivotal phase 3 trials, up to 24 weeks of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious a …
Parenthood impacts short-term health outcomes in people with cystic fibrosis.
Kazmerski TM, Jain R, Lee M, Taylor-Cousar JL. Kazmerski TM, et al. Among authors: taylor cousar jl. J Cyst Fibros. 2022 Jul;21(4):662-668. doi: 10.1016/j.jcf.2022.02.006. Epub 2022 Feb 15. J Cyst Fibros. 2022. PMID: 35181269 Free article.
Females experienced significantly worse impacts on BMI from pre- to post-parenthood compared to males. CONCLUSIONS: Parenthood adversely impacts short-term health outcomes for people with CF and CFTR modulator use mitigates such effects. To better understand the impact of …
Females experienced significantly worse impacts on BMI from pre- to post-parenthood compared to males. CONCLUSIONS: Parenthood adversely imp …
Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world - opportunities and challenges.
Barry PJ, Taylor-Cousar JL. Barry PJ, et al. Among authors: taylor cousar jl. Curr Opin Pulm Med. 2021 Nov 1;27(6):554-566. doi: 10.1097/MCP.0000000000000819. Curr Opin Pulm Med. 2021. PMID: 34420018 Review.
PURPOSE OF REVIEW: The purpose of this review is to describe on-going and upcoming real-world studies that will aid the cystic fibrosis (CF) community in understanding the long-term efficacy, safety and challenges in utilizing this therapy and managing care. ...Treatment w …
PURPOSE OF REVIEW: The purpose of this review is to describe on-going and upcoming real-world studies that will aid the cystic fibrosis (CF) …
A survey: Understanding the health and perspectives of people with CF not benefiting from CFTR modulators.
Kramer-Golinkoff E, Camacho A, Kramer L, Taylor-Cousar JL. Kramer-Golinkoff E, et al. Among authors: taylor cousar jl. Pediatr Pulmonol. 2022 May;57(5):1253-1261. doi: 10.1002/ppul.25859. Epub 2022 Mar 28. Pediatr Pulmonol. 2022. PMID: 35170259 Free PMC article.
BACKGROUND: While the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulator use has improved daily life and long-term prognosis of CF for many with approved CFTR mutations, approximately 10% of people with CF (pwCF) have only symptomatic treatments …
BACKGROUND: While the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulator use has improved daily life and long- …
Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey.
Nash EF, Middleton PG, Taylor-Cousar JL. Nash EF, et al. Among authors: taylor cousar jl. J Cyst Fibros. 2020 Jul;19(4):521-526. doi: 10.1016/j.jcf.2020.02.018. Epub 2020 Mar 6. J Cyst Fibros. 2020. PMID: 32151568 Free article.
BACKGROUND: As their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR modulators in pregnancy and breastfeeding is currently unknown. ...Women stopping CFTR modulators in pregnancy may experience a decline in cl …
BACKGROUND: As their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR m …
Whole Blood Gene Expression Profiling Predicts Severe Morbidity and Mortality in Cystic Fibrosis: A 5-Year Follow-Up Study.
Saavedra MT, Quon BS, Faino A, Caceres SM, Poch KR, Sanders LA, Malcolm KC, Nichols DP, Sagel SD, Taylor-Cousar JL, Leach SM, Strand M, Nick JA. Saavedra MT, et al. Among authors: taylor cousar jl. Ann Am Thorac Soc. 2018 May;15(5):589-598. doi: 10.1513/AnnalsATS.201707-527OC. Ann Am Thorac Soc. 2018. PMID: 29425066
The robustness of gene clustering to categorize patients appropriately in terms of clinical characteristics, and short- and long-term clinical outcomes, remained consistent, even when adding in a secondary population with significantly different clinical outcomes. ...
The robustness of gene clustering to categorize patients appropriately in terms of clinical characteristics, and short- and long-term …
Decreases in Rhinology Care Utilization by People with Cystic Fibrosis on Highly Effective Modulator Therapy.
Han EJ, Beswick DM, Eshaghian PH, Turner GA, Lee JT, Li DA, Wang MB, Taylor-Cousar JL, Suh JD. Han EJ, et al. Among authors: taylor cousar jl. Ann Otol Rhinol Laryngol. 2024 Mar;133(3):340-344. doi: 10.1177/00034894231211626. Epub 2023 Nov 12. Ann Otol Rhinol Laryngol. 2024. PMID: 37953524
Additional studies are needed to determine rhinologic healthcare requirements for PwCF who remain on ETI for the long-term and to evaluate larger cohorts of PwCF on ETI....
Additional studies are needed to determine rhinologic healthcare requirements for PwCF who remain on ETI for the long-term and to eva …
Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis.
Nick JA, Chacon CS, Brayshaw SJ, Jones MC, Barboa CM, St Clair CG, Young RL, Nichols DP, Janssen JS, Huitt GA, Iseman MD, Daley CL, Taylor-Cousar JL, Accurso FJ, Saavedra MT, Sontag MK. Nick JA, et al. Among authors: taylor cousar jl. Am J Respir Crit Care Med. 2010 Sep 1;182(5):614-26. doi: 10.1164/rccm.201001-0092OC. Epub 2010 May 6. Am J Respir Crit Care Med. 2010. PMID: 20448091 Free PMC article.
RATIONALE: Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed with classic manifestations in childhood, and nonclassic phenotypes typically diagnosed as adults. ...OBJECTIVES: Examine effects of age at d …
RATIONALE: Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed w …