Children’s Hospital Colorado (Children’s Hospital Colorado) is celebrating Cystic Fibrosis Awareness Month by highlighting growing changes in care that start treatment for cystic fibrosis before birth to improve lifelong health outcomes and reduce length of stay. Through a highly coordinated multidisciplinary approach, teams at the Colorado Children’s Respiratory Institute and the Colorado Fetal Care Center have cared for 10 families through prenatal therapy for patients who showed signs of cystic fibrosis in utero. To date, no other U.S. health system cares for so many patients from birth, making Children’s Colorado one of the most experienced health systems of its kind in the world.
Ten babies born with cystic fibrosis (CF) who received prenatal treatment at Children’s Colorado had early symptoms of meconium ileus. Meconium ileus is a severe intestinal obstruction that often requires surgery soon after birth. Patients who received early prenatal intervention resolved this obstruction before birth and showed significant signs of improved pancreatic function. As a result, the length of stay in the neonatal intensive care unit (NICU) was significantly reduced compared to patients born with intestinal obstruction without prenatal treatment.
CF is a genetic disease that affects the lungs, digestive system, and other organs. The body produces thick mucus that clogs the lungs and pancreas, making it difficult to breathe and absorb nutrients. Twenty years ago, the average life expectancy for people with CF was 36 years. Children born with CF are now expected to live into their 60s, reflecting steady progress with early intervention and improved treatments such as ETI therapy.
ETI therapy is a three-drug transmembrane conductance regulator modulator for cystic fibrosis. The combination of drugs improves the movement of salts and water within cells, reducing the buildup of thick mucus. At Children’s Colorado, our care teams are expanding access to this therapy during pregnancy, allowing you to begin treatment before your baby is born.
Prenatal treatment gives families unprecedented options to take action during pregnancy. Rather than waiting to address symptoms after birth, we can start supporting your baby’s health early and work with your family every step of the way. ”
Michael Zaretsky, MD, Director of Research, Colorado Fetal Care Center
Additionally, researchers and clinicians have found that treatment administered prenatally and during early infancy supports healthier cell function during a critical period of lung development. Infants exposed during this period showed lower than expected sweat chloride levels. This is an established marker associated with lung health. Continuing treatment after birth may help maintain these increases during infancy, when the lungs are particularly vulnerable to inflammation and infection.
“With cystic fibrosis, lung damage often begins very early in life,” says Jordana Hoppe, M.D., medical director of Children’s Colorado’s respiratory clinic. “ETI therapy gives patients the option of moving their child’s CF care from reactive to preventive, offering incredible potential for lifelong respiratory benefits.”
Prenatal treatment allows treatment to continue seamlessly after birth. ETI therapy can be administered through breast milk until the infant is able to take the medication independently.
Approximately 1,000 people are diagnosed with CF each year, 75% of whom are diagnosed by age 2. Because the severity of CF increases over time, early intervention is key to helping infants and young children manage symptoms into adulthood. The results of prenatal ETI therapy demonstrate a new and innovative care model available to families at risk for CF. Children’s Colorado remains dedicated to this multidisciplinary approach and research collaborations at the regional and national levels to improve clinical experiences and patient outcomes.
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