Optimizing sexual and reproductive health across the lifespan in people with cystic fibrosis

West, Natalie E.; Kazmerski, Traci M.; Taylor‐Cousar, Jennifer L.; Tangpricha, Vin; Pearson, Kelsie; Aitken, Moira L.; Jain, Raksha

doi:10.1002/ppul.25703

Cited by 12 publications

(20 citation statements)

References 114 publications

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“…Additionally, 11 female participants were found to be pregnant during the study. Increased pregnancy rates associated with highly effective CFTR modulator use is an important consideration in the lives of PwCF, and we expect the need for further attention to reproductive and sexual health (45)(46)(47).…”

Section: Discussionmentioning

confidence: 99%

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial

Nichols

Paynter

Heltshe

et al. 2022

Am J Respir Crit Care Med

215

126

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Rationale:The cystic fibrosis (CF) modulator drug elexacaftor/tezacaftor/ivacaftor (ETI) proved highly effective in controlled clinical trials for individuals with ≥1 F508del allele, which occurs in at least 85% of people with CF (PwCF). Objective: PROMISE is a post-approval study to understand the broad effects of ETI through 30 months clinical use in a more diverse US patient population with planned analyses after 6 months. Methods: Prospective, observational study in 487 PwCF age ≥12 years with ≥1 F508del allele starting ETI for the first time. Assessments occurred before and 1, 3, and 6 months into ETI therapy. Outcomes included change in ppFEV 1 , sweat chloride concentration, body mass index, and selfreported respiratory symptoms. Results: average age was 25.1 years. 44.1% entered the study using tezacaftor/ivacaftor or lumacaftor/ivacaftor while 6.7% were using ivacaftor, consistent with F508del homozygosity and G551D allele, respectively. At 6 months into ETI therapy, ppFEV 1 improved 9.76 percentage points (95% CI 8.76, 10.76) from baseline, CFQ-R Respiratory Domain score improved 20.4 points (95% CI 18.3, 22.5), and sweat chloride decreased -41.7 mmol/L (95% ). BMI also significantly increased.Changes were larger in those naïve to modulators but substantial in all groups, including those treated with ivacaftor at baseline. Conclusions: ETI by clinical prescription provided large improvements in lung function, respiratory symptoms, and BMI in a diverse population naïve to modulator drug therapy, using existing two-drug combinations, or using ivacaftor alone. Each group also experienced significant reductions in sweat chloride concentration, which correlated with improved ppFEV 1 in the overall study population.

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Section: Discussionmentioning

confidence: 99%

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial

Nichols

Paynter

Heltshe

et al. 2022

Am J Respir Crit Care Med

215

126

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“…Deep disparities persist with respect to these characteristics not only in care access 26 and survival outcomes 27 among people living with CF, but also in research participation. 28 Each of us has focused our own activities with the national Foundation since that initial 2019 panel on amplifying the voices and contributions of scientists and advocates from intersectionally marginalized backgrounds who are living with CF.…”

Section: Discussionmentioning

confidence: 99%

Coming Full Circle: Reflections and Inspirations from a Cystic Fibrosis Patient Scientist Panel

Nowakowski

Balasa

Figueira

et al. 2022

INQUIRY

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Care for many progressive chronic diseases continues to improve, allowing patients to survive and thrive for longer periods of time1. People living with such conditions may now find themselves able to achieve long-term goals in education and career development2. Many people now occupy the dual roles of scientist and patient3. This commentary article synthesizes experiences of scientists and advocates with the progressive genetic disease cystic fibrosis (CF) who collaborated on a career development session for the Cystic Fibrosis Foundation’s inaugural ResearchCon event in 2019. It explores how such collaborations affirm and transform individual perspectives on patient science and its importance in broader scientific research agenda setting. We first share our own individual insights about the experience and impact of the ResearchCon panel session before progressing to discussion and future directions centering the shared insights from one another’s reflections.

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“…Key clinical considerations and research related to contraception are detailed in the companion paper in this issue. 17 …”

Section: Reproductive Decision‐making and Counselingmentioning

confidence: 99%

Family‐building and parenting considerations for people with cystic fibrosis

Kazmerski

West

Jain

et al. 2021

Pediatric Pulmonology

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As people with cystic fibrosis (CF) live longer and healthier lives, increasing numbers are considering the full range of reproductive options for their futures, including parenthood, pregnancy, or pregnancy prevention. As the face of CF changes, the CF care model must adapt to meet the reproductive health needs of both parents and nonparents with CF. This article summarizes the reproductive goals and familybuilding concerns faced by people with CF, including fertility, pregnancy, and alternative paths to parenthood, the impact of parenthood on mental and physical health, and important future research.

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Optimizing sexual and reproductive health across the lifespan in people with cystic fibrosis

Cited by 12 publications

References 114 publications

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial

Coming Full Circle: Reflections and Inspirations from a Cystic Fibrosis Patient Scientist Panel

Family‐building and parenting considerations for people with cystic fibrosis

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