Inside Pediatrics Winter 2018

CF Center in Santiago, Chile, providing care adhering to CF Foundation standards. Collaborative work has led to significant improvement in clinical outcomes and team expertise for a number of hospitals in Chile. In 2015, the HCSBA CF team was approached by five other Santiago-based hospitals that are now part of the project as affiliate hospitals. After the incorporation of the affiliate hospitals, the HCSBA CF Center renamed itself Regional CF Center Santiago. “This project started with one center, one hospital, and other hospitals in Santiago started to get interested,” Gutierrez said. “They joined by participating in our visits in Chile, and we provided similar information and feedback, but the first center

benefits, however, the structure of care deliveries, healthcare benefits, et cetera are different. Although we have started small, we feel we are making progress and hope to continue to do so in the years to come.” A mentorship by high-performing, U.S.-based CF teams, namely Children’s, UAB and the Baylor College of Medicine in Houston, plans to expand and train Latin America-based CF teams in Chile, Mexico and Uruguay. The mentorship would continue the development of an effective, adaptable and scalable training program for both mentors and trainees, and adapt transformative care management to local resources, culture, customers and healthcare delivery. ROSALIE’S STORY Rosalie Walker was only 2 weeks old when her parents, Rachel and Raven, received a call from her pediatrician telling them she may have cystic fibrosis (CF). Rosalie’s newborn screening test came back positive, indicating that Rosalie had inherited the disease. The preliminary results were confirmed by an immediate visit to Children’s of Alabama. “It was a shock for us to hear,” said Rachel, whose family lives in East Alabama. “Our baby was born healthy without any indication until the results of the screening test came back, so we went directly to Children’s in Birmingham for a follow-up sweat test.” A chloride sweat test helps diagnose CF, an inherited disorder that disrupts the normal function of epithelial cells that make up the sweat glands in the skin and also line passageways inside the lungs, liver, pancreas and digestive and reproductive systems. Rosalie’s hospital test results were confirmed. She inherited a double copy of the most common disease-causing gene mutation, delta F508, indicating that both of her parents were carriers. “When we learned that she had CF, the hospital staff was wonderful,” Rachel said. “It was very scary and an uncertain time for us, but they were very good about explaining everything about medications, breathing treatments and just the daily routine we needed to follow.” At 3 weeks old, Rosalie became ill with frequent vomiting and pneumonia. A bronchoscopy was performed to determine the type of bacteria in her lungs. “To facilitate her care during her hospital stay, Rosalie had an IV access placed [PICC line] and received antibiotics to treat the infection. She was discharged in good condition and has not been admitted since,” said Hector Gutierrez, M.D., director of the Cystic Fibrosis Center at Children’s. Now 3 years old, Rosalie visits Gutierrez and the Children’s CF team four times a year at its specialized CF outpatient clinic to maintain her health. She attends preschool and loves gymnastics, riding her bike, swimming and playing with her 7-year-old brother, Reed.

(HCSBA) that came to Birmingham became their leader and the point group for the others to learn from them.” Efforts thus far have shown a marked improvement in nutritional outcomes, lung function and microbiology among CF patients cared for by the participating hospitals in Chile, Gutierrez said. “We have been in discussion for two years with the government officials in Chile and other Latin American countries to work toward developing a solid CF network similar to what we have in the United States,” Gutierrez said. “We have explained the Pediatric pulmonologist Hector Gutierrez, M.D., is principal investigator of the Cystic Fibrosis Training Network for Latin America (CFTN-LA). For the past two years, Gutierrez and his team have worked with government officials in Chile and other Latin American countries to develop permanent CF networks there.

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