Pregnancy in Patients With Cystic Fibrosis.
Beth L Pineles1, Raksha Jain, Traci M Kazmerski
1Division of Maternal-Fetal Medicine and the Division of Pregnancy Loss and Comprehensive Family Planning, Department of Obstetrics and Gynecology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, and the Department of Pediatrics, University of Pittsburgh School of Medicine, and the Center for Innovative Research on Gender Health Equity (CONVERGE), University of Pittsburgh, Pittsburgh, Pennsylvania; the Division of Pulmonary and Critical Care, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas; the Division of Pulmonary Sciences and Critical Care Medicine and Pediatric Pulmonology, Department of Medicine and Pediatrics, National Jewish Health, Denver, and the Departments of Internal Medicine and Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, Colorado; the Department of Pediatrics, University of California, San Diego, San Diego, California; and the Division of Genetic Counseling and the Division of Pediatric Pulmonology, Allergy & Immunology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina.
Cystic fibrosis (CF) is increasingly affecting adults, with new CFTR modulators improving outcomes and increasing pregnancies. Careful family planning, including contraception and preconception counseling, is crucial for individuals with CF.
Area of Science:
- Medical Genetics
- Pulmonology
- Reproductive Health
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting all populations.
- Adults now represent the majority of CF patients, with increased life expectancy.
- Advances in CF care and CFTR modulators have improved outcomes and fertility.
Purpose of the Study:
- To review family planning considerations for individuals with CF in the era of CFTR modulators.
- To highlight the importance of preconception counseling and maternal-fetal medicine expertise.
- To discuss implications of new prenatal testing and transplacental drug transfer.
Main Methods:
- Literature review of recent advances in CF care, CFTR modulators, and reproductive health in CF.
- Synthesis of current recommendations for contraception, preconception, pregnancy, and breastfeeding in CF.
- Discussion of emerging technologies like noninvasive prenatal testing for fetal CF.
Main Results:
- CFTR modulators have significantly improved health outcomes and increased pregnancy rates in women with CF.
- Preconception consultation is vital due to increased risks during pregnancy for individuals with CF.
- Breastfeeding is encouraged, with infant monitoring for potential side effects.
- Noninvasive prenatal testing for fetal CF is emerging, with potential benefits from in-utero exposure to CFTR modulators.
Conclusions:
- Individuals with CF require comprehensive family planning strategies, including contraception and preconception care.
- The role of CFTR modulators in fetal development warrants further investigation.
- Ongoing research is essential to understand pregnancy and parenthood for individuals with CF in the modern therapeutic landscape.
More Related Videos
Related Concept Videos
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
Cystic Fibrosis: Management
Sinus disease and chronic...
Upper Respiratory Drugs: Antitussives, Expectorants, and Mucolytics
Antitussives include codeine, dextromethorphan (Robitussin), and benzonatate (Tessalon). Codeine and dextromethorphan exert their effects centrally by suppressing the cough reflex center in the medulla. Benzonatate operates peripherally within the respiratory tract by...
Oogenesis
Mitral Valve Prolapse III: Nursing Management
Chest Physiotherapy
Purpose
CPT is primarily used for patients with excessive bronchial secretions who have difficulty clearing...


