The digestive side of a respiratory disease
Cystic fibrosis is associated above all with the lungs, but it affects almost the whole digestive tract. With improved respiratory outlook and the arrival of CFTR modulators, digestive and liver problems have moved to the foreground in adult life.
What we monitor
- Exocrine pancreatic insufficiency. Adjusting the enzyme dose, which is almost always inadequate, and monitoring fat absorption
- Liver involvement. From isolated enzyme elevation to focal biliary cirrhosis. Follow-up with ultrasound and elastography
- Nutritional status. Weight, body composition, fat-soluble vitamins and bone density
- Distal intestinal obstruction syndrome. Recognising and treating it without ending up in theatre
- Gastro-oesophageal reflux, very common and with direct respiratory impact
- Cystic fibrosis-related diabetes
- Gallstones and their complications
Transition from paediatric care
This is one of the highest-risk moments in the whole condition. The patient moves from a team that has known them since childhood to an adult service that is often not set up for it, and some are lost along the way.
A stable digestive clinic, with the same person year after year, is one of the most valuable things at this stage.
Liver disease in adults has a better outlook than assumed
I contributed to a study published in the Revista Española de Enfermedades Digestivas analysing the course of liver involvement in adults with cystic fibrosis at a tertiary referral centre, which found a more benign course than expected. Also to an ultrasound study on the prevalence of biliary manifestations, presented at UEG Week.
My work in this area
I have been principal investigator at La Princesa University Hospital in several international clinical trials on exocrine pancreatic insufficiency in cystic fibrosis, including the SOLUTION, PANC 2002 and MS1819 studies, aimed at optimising enzyme replacement therapy.
Few gastroenterologists have specific experience of this condition in adults, which is why I work in close coordination with the respiratory units that follow these patients.