“Cystic fibrosis is a story of success. Because of the achievements of medical science, medical technology, and family partners, so much has been accomplished in recent years,” says pediatric pulmonologist Hossein Sadeghi, MD, Director of the Sue and John L. Weinberg Cystic Fibrosis Center at NewYork-Presbyterian Morgan Stanley Children’s Hospital and Columbia. “Life expectancy in the 1980s was less than 20 years of age. Now life expectancy is around 50 in the U.S. aided by the increased availability of highly effective cystic fibrosis transmembrane conductance regulator modulator therapies that target specific genetic mutations. I project that life expectancy is going to even go higher and higher.”
Dr. Hossein Sadeghi
As pediatric patients with cystic fibrosis are living longer and healthier lives, Dr. Sadeghi and Columbia faculty at the Cystic Fibrosis Center, fully accredited by the Cystic Fibrosis Foundation, have developed a robust transition program that includes a CF transition coordinator, Victoria Piane, Nurse Practitioner, NewYork-Presbyterian/
However, studies have shown that the experience of healthcare transition from pediatric to adult care in cystic fibrosis remains poorly understood, particularly among racially and ethnically diverse adolescents and young adults (AYAs). To further understand the reasons for this, Dr. Sadeghi and colleagues in the Division of Pediatric Pulmonology and Sleep Medicine collaborated with the Columbia University School of Nursing recently in a qualitative descriptive study to explore the perspectives of a diverse sample of AYAs with cystic fibrosis regarding their healthcare transition. The results of this study were published in the March 2022 issue of the Journal of Pediatric Nursing.
One of our goals is to educate young people on basic knowledge about cystic fibrosis to help shift the responsibility from the families so that the patient takes ownership of his or her care.
— Dr. Hossein Sadeghi
The Columbia investigators identified 12 adolescents and young adults ages 15 to 24 years who had either begun to prepare for the transition to adult care, planned to transfer in the next three years, or had completed their transition in the past five years. A portion of the young people included in the study had experience with the cystic fibrosis web-based transition preparation program “Responsibility. Independence. Self-care. Education.”
Using an online survey, the investigators collected demographic information as well as information on responsibility for self-management behaviors, and conducted semi-structured video interviews.
The study comprised 25 percent female, 50 percent White, 33 percent Hispanic/Latino, and 25 percent Black. Three themes were identified from the researchers’ inductive analysis: independent care of the whole self; preparing for change and the unknown; and transition experiences vary.
Dr. Sadeghi and the research team concluded that not all participants experienced a smooth transition. “Participants shared mixed feelings about the transition preparation available to them at their CF center and offered constructive suggestions for improving the healthcare transition process,” noted the authors. “AYAs in our study desired adequate time to prepare and wanted additional information about adult care to ease their fear of the unknown.”
The participants expressed hesitation about transition when they felt little control over the process or lacked sufficient information about adult care. The investigators agreed that future research should focus on comprehensively assessing health care transition programs for patients with CF. This includes consideration of the parent perspective regarding transition preparation as well as other transition outcomes, such as lung function and adult CF clinic attendance.
The investigators also noted that pediatric nurses play an important role in the transition process and recommend that healthcare transition preparation should start early in adolescence with a gradual transfer of responsibility from parent to child. They added, “Nurses can support this transfer of responsibility by engaging adolescents as well as parents during care encounters and by offering support, education, and encouragement for independent self-management.”
“The transition study is just one example of our multiple research collaborations with colleagues in specialties throughout the hospital,” adds Dr. Sadeghi. Other studies include research with gastroenterologists looking at liver complications in patients with CF; endocrinologists focusing on bone health; and infectious disease specialists exploring the effects of cystic fibrosis transmembrane conductance regulator modulators on metabolites in the blood.
“This is a positive story of improvement and progress in every aspect of disease, life expectancy, and quality of life,” adds Dr. Sadeghi. “Whether it’s through clinical work or bench research or quality improvement initiatives, we are making great progress in this disease. We hope that with all the new therapies and interventions, patients with cystic fibrosis will have a normal life expectancy in the near future.”