Survey Reveals Barriers to Gastroenterology Care in Cystic Fibrosis Clinics

By Senthilkumar Sankararaman, MD

Cystic fibrosis (CF) is a multisystem disorder that affects the lungs, pancreas, gut, liver, and sinuses. It is the most common lethal genetic disorder among white populations worldwide, and currently, there are approximately 30,000 people living with the disorder in the United States. According to the 2023 Cystic Fibrosis Foundation (CFF) registry, the median predicted survival of individuals born between 2019 and 2023 is 61 years. Just two decades ago, it was approximately 33 years.

Improvements and opportunities in CF management

Management of respiratory manifestations and nutrition therapy are the mainstay of CF care. With this improvement in respiratory and nutrition management, people with CF are living longer and healthier lives. Further, the recent introduction of CFTR-directed therapies, such as elexacaftor-tezacaftor-ivacaftor (ETI), has drastically improved respiratory symptoms in people with CF, but improvements in extrapulmonary manifestations, such as gastrointestinal disorders, remain modest.

People with CF are frequently seen by gastroenterologists for multiple reasons, such as abdominal pain, bloating, constipation, gastroenterology reflux disease, elevated liver enzymes, pancreatitis, management of refractory malabsorption (steatorrhea), CF-related liver disease, tube (enteral) feeding and colonoscopy screening for colon cancer.

The importance of gastroenterology support in CF care

There is significant variability in the provision of gastroenterology services for people of all ages with CF across the globe. In some centers, gastroenterologists are well-integrated as essential members of the multidisciplinary team and may see patients regularly within the CF clinics. This setup is ideal and creates many opportunities for successful interdisciplinary collaboration between gastroenterologists, registered dietitians and other members of the CF team.

On the other hand, in many CF centers, because of a lack of availability, gastroenterologists may not participate in the CF center clinics and may see people with CF in their general gastroenterology clinics on an ad hoc basis.

A strong collaboration between registered dietitians and gastroenterologists in CF could enhance patient outcomes in multiple ways, such as the development of mutually accepted treatment plans, improved coordinated delivery of high-standard patient care, development of patient educational materials, and enhanced adherence to clinical practice guidelines. Also, this interdisciplinary collaboration could enhance the production of quality improvement projects, research and other scholarly activities.

Developing and distributing an international survey

To evaluate the current status of this collaboration, our team surveyed registered dietitians and gastroenterologists regarding the current participation of gastroenterologists in CF centers and identified possible areas to enhance partnership between the two disciplines.

We distributed an anonymous online survey targeting registered dietitians and gastroenterologists involved in CF care through three international listservs (CF Nutrition, CF DIGEST and PEDGI). A total of 131 respondents participated in this survey, including 80 registered dietitians and 51 gastroenterologists (41 pediatric and 10 adult gastroenterologists) who manage people with CF. Most respondents (82%) were from the United States, and two-thirds had ≥5 years of experience in CF.

Responses reveal a perception discrepancy

This cross-sectional survey provided a snapshot of the current participation of gastroenterologists in CF centers and the extent of registered dietitian–gastroenterologist collaboration in CF. The results demonstrated significant variability in responses between registered dietitians and gastroenterologists regarding the ongoing collaborative efforts.

A significantly higher number of gastroenterologists perceived that they work collaboratively with registered dietitians in CF care (both in direct and indirect clinic-related activities), whereas a significant number of registered dietitians reported a lack of availability of gastroenterologists for partnership. A significant number of registered dietitians reported the nonavailability of gastroenterologists for collaboration and increased availability of gastroenterologists in pediatric centers.

However, both disciplines had mutual respect and interest in further collaborative efforts.

Barriers to collaboration

Barriers to interdisciplinary collaboration included a lack of CF expertise and dedicated time among the gastroenterologists and difficulties in coordinating the gastroenterology clinics. More gastroenterologists than registered dietitians perceived that they worked collaboratively with the other disciplines in various domains (clinical care, quality improvement, research, presentations and publications). There is an increased need for gastroenterologist participation and collaboration (particularly in adult centers) in CF alongside registered dietitians to enhance comprehensive patient care.

Putting it all together—and looking ahead

This is the first study that systematically evaluates the extent of, and barriers to, the collaboration between these two disciplines.Our survey results highlight the wide variability in the involvement of gastroenterologist providers in CF care. As life expectancy among people with CF improves, having gastroenterologists with expertise in the care of adults with CF will be increasingly important for managing gastrointestinal symptoms, hepatobiliary involvement, and colonoscopic cancer screening.

As the CF landscape continues to change in the era of CFTR-directed therapies, we recommend that future efforts focus on CF-based training for gastroenterologists, including bidirectional learning collaborations between CF-registered dietitians and gastroenterologists. Finally, we recommend improved access to gastroenterologists for people with CF by more thoughtful integration of gastroenterology specialists in CF clinics.