This website is made possible by an educational grant from Vertex Pharmaceuticals Incorporated.To the Editor, It’s been almost 10 years since the Cystic Fibrosis Foundation began the Developing Innovative Gastroenterology Specialty Training (DIGEST) program. In total, 68 faculty have completed or are currently enrolled in this unique program, which provides…
Latest Issue: September 2024
Remaining Barriers to Normalcy in Cystic Fibrosis: Advances in Gastroenterology, Hepatology, and Nutrition
Nutritional status in the era of highly effective CFTR modulators
Advances in cystic fibrosis (CF) diagnostics and therapeutics have led to improved health and longevity, including increased body weight and decreased malnutrition in people with CF. Highly effective CFTR modulator therapies (HEMT) are associated with increased weight through…
Improving growth in infants with CF
Cystic fibrosis (CF) results in chronic pulmonary infections, inflammation, pancreatic insufficiency, and multiple gastrointestinal manifestations. Malnutrition and poor growth are hallmarks of CF, and strongly associated with poor outcomes. Through newborn screening, many infants can be diagnosed within…
Overnutrition in persons with cystic fibrosis on modulator therapy and the relationship to obstructive sleep apnea
Cystic fibrosis (CF) care is evolving with the ubiquitous use of modulator therapy and resultant increase in lifespan. It is important for CF clinicians to monitor the pathologic weight gain that is concomitantly being seen as obesity is…
Nutrition and behavioral health in cystic fibrosis: Eating and body image
People with CF (pwCF) are at high risk for malnutrition, making nutritional management a critical aspect of CF care. Over the past several decades, optimal nutritional status for pwCF has been defined by body mass index (BMI) based…
Pancreas and pancreatitis: Exocrine pancreatic insufficiency
Exocrine pancreatic insufficiency (EPI) is highly prevalent among individuals with cystic fibrosis (CF). Individuals diagnosed with EPI are often labeled as having “pancreas insufficient cystic fibrosis (PI-CF)” while those with normal exocrine function are labeled as “pancreas sufficient…
Pancreatitis in cystic fibrosis: Presentation, medical and surgical management, and the impact of modulator therapies
Patients with Cystic Fibrosis (CF) are at increased risk of acute (AP) and chronic (CP) pancreatitis, and their complications. The extent of remaining healthy pancreatic parenchyma determines the risk of developing future episodes of pancreatitis, as well as…
The foregut in cystic fibrosis
The aerodigestive organs share a kindred embryologic origin that allows for a more complete explanation as to how the foregut can remain a barrier to normalcy in people with cystic fibrosis (pwCF). The structures of the aerodigestive tract…
The gastrointestinal microbiome, small bowel bacterial overgrowth, and microbiome modulators in cystic fibrosis
People with cystic fibrosis (pwCF) have an altered gastrointestinal microbiome. These individuals also demonstrate propensity toward developing small intestinal bacterial overgrowth (SIBO). The dysbiosis present has intestinal and extraintestinal implications, including potential links with the higher rates of…
Constipation and DIOS: Diagnosis, differential diagnosis, and management
Cystic Fibrosis (CF) is a complex disorder that requires multidisciplinary expertise for effective management. The GALAXY study estimated the prevalence of constipation to be about 25% among People with Cystic Fibrosis (PwCF), identifying it as one of the…
Evolving data on risk and current screening recommendations for colorectal cancer in cystic fibrosis: Pre- and posttransplant
Advances in treatment for cystic fibrosis (CF), including cystic fibrosis transmembrane conductor regulator (CFTR) modulators, have ushered in an era where patients with CF have much longer life expectancies. This shift in life expectancy demands increased attention to…
Pathophysiology of Cystic Fibrosis Liver Disease
Hepatobiliary complications of Cystic Fibrosis (CF) constitute a significant burden for persons with CF of all ages, with advanced CF liver disease in particular representing a leading cause of mortality. The causes of the heterogeneity of clinical manifestations,…