Pregnancy in cystic fibrosis: Review of the literature and expert recommendations

Jain, Raksha; Kazmerski, Traci M.; Zuckerwise, Lisa C.; West, Natalie E.; Montemayor, Kristina; Aitken, Moira L.; Cheng, Edith; Roe, Andrea H.; Wilson, Alexandra; Mann, Caitlin; Ladores, Sigrid; Sjoberg, Jacqui; Poranski, Madeline; Taylor‐Cousar, Jennifer L.

doi:10.1016/j.jcf.2021.07.019

Cited by 50 publications

(37 citation statements)

References 95 publications

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“…People who have chronic medical conditions may have reproductive health concerns that are both general and disease-specific. Our team developed two patient-directed tools to support family planning care for women with rheumatic and musculoskeletal diseases (RMDs) and cystic fibrosis (CF); these conditions are independently associated with pregnancy-related concerns, including potentially higher rates of maternal morbidity and mortality and/or adverse neonatal outcomes [52]. Approximately 7 million women in the United States have RMDs, including autoimmune and connective tissue diseases, and many of these diseases are diagnosed during reproductive age [53].…”

Section: Tools To Address Considerations Related To Chronic Medical C...mentioning

confidence: 99%

A Framework for Femtech: Guiding Principles for Developing Digital Reproductive Health Tools in the United States

et al. 2022

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The United States has abysmal reproductive health indices that, in part, reflect stark inequities experienced by people of color and those with preexisting medical conditions. The growth of “femtech,” or technology-based solutions to women’s health issues, in the public and private sectors is promising, yet these solutions are often geared toward health-literate, socioeconomically privileged, and/or relatively healthy white cis-women. In this viewpoint, we propose a set of guiding principles for building technologies that proactively identify and address these critical gaps in health care for people from socially and economically marginalized populations that are capable of pregnancy, as well as people with serious chronic medical conditions. These guiding principles require that such technologies: (1) include community stakeholders in the design, development, and deployment of the technology; (2) are grounded in person-centered frameworks; and (3) address health disparities as a strategy to advance health equity and improve health outcomes.

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Section: Tools To Address Considerations Related To Chronic Medical C...mentioning

confidence: 99%

A Framework for Femtech: Guiding Principles for Developing Digital Reproductive Health Tools in the United States

et al. 2022

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“…Vitamin A supplementation should continue at <10,000 IU/day. 43,44 Also, clinicians should ensure patients are taking adequate amounts of folic acid, iron, calcium, and phosphorus.…”

Section: Pregnancymentioning

confidence: 99%

Update on the management of vitamins and minerals in cystic fibrosis

2022

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Advancements in respiratory and nutrition management have significantly improved the survival of patients with cystic fibrosis (CF). With the availability of several nutrition interventions such as oral/enteral nutrition supplements, enteric‐coated pancreatic enzymes, and water‐miscible CF‐specific vitamin supplements, frank vitamin deficiencies—with the exception of vitamin D—are rarely encountered in current clinical practice. Whereas they were previously considered as micronutrients, our current understanding of fat‐soluble vitamins and minerals as antioxidants, immunomodulators, and disease biomarkers has been evolving. The impact of highly effective modulators on the micronutrient status of patients with CF remains elusive. This narrative review focuses on the updates on the management of fat‐soluble vitamins and other micronutrients in CF in the current era and identifies the gaps in our knowledge.

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“…With the development of CFTR modulator therapies, improved function of the CFTR protein is made possible. Widespread use of CFTR modulators has resulted in improved lung function, nutritional status, pulmonary exacerbations, need for hospitalisation secondary to CF-related complications and overall survival [3][4][5][6][7]. Individuals ⩾18 years of age now make up more than half of the global CF population [2,8,9], and more people with CF are considering pregnancy than ever before.…”

Section: Introductionmentioning

confidence: 99%

Management of pregnancy in cystic fibrosis

Montemayor

Tullis

Jain

et al. 2022

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With recent therapeutic advances in care, people with cystic fibrosis (CF) are living longer and healthier lives. Development of the cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies has led to improved function of the CFTR protein resulting in improved lung function, decreased rates of pulmonary exacerbations and improved nutritional status for the majority of people with CF. Given improved quality and quantity of life, more people with CF are considering becoming pregnant than ever before. Since the first reported pregnancy in a woman with CF in 1960, the management of pregnancy in CF has been of increased interest and is an active field of research.In this review, we aim to discuss the management of pregnancy in CF. We discuss the optimisation of preconception health, management of maintenance CF therapies, and use of CFTR modulators during pregnancy and lactation. We also describe the management of pulmonary exacerbations during pregnancy as well as the unique management of pregnancy in a post-transplant patient with CF.Educational aimsTo describe considerations for optimisation of preconception health.To describe the management of chronic CF therapies including CFTR modulators during pregnancy and lactation.To describe treatment of an acute pulmonary exacerbation during pregnancy.To describe the management of pregnancy in individuals with CF following organ transplantation.

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Pregnancy in cystic fibrosis: Review of the literature and expert recommendations

Cited by 50 publications

References 95 publications

A Framework for Femtech: Guiding Principles for Developing Digital Reproductive Health Tools in the United States

A Framework for Femtech: Guiding Principles for Developing Digital Reproductive Health Tools in the United States

Update on the management of vitamins and minerals in cystic fibrosis

Management of pregnancy in cystic fibrosis

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