Cystic Fibrosis Screening, Evaluation and Management of Hepatobiliary Disease

Publication Date: October 26, 2023
Last Updated: November 10, 2023

Screening

  • The CFF recommends that annual labs (total bilirubin, AST, ALT, alkaline phosphatase, GGT, Platelet Count) should be performed, at a time of clinical stability, for identification of hepatobiliary involvement in all persons with CF starting at CF diagnosis.
  • CFF recommends annual abdominal physical examination for hepatosplenomegaly for identification of advanced liver disease in all persons with CF starting at CF diagnosis.
  • CFF recommends abdominal ultrasound to assess the liver and spleen at least every two years in persons with CF from childhood until late adolescence, starting at age 3 or at diagnosis, if diagnosed after age 3.
  • In adults with CF, there is value in baseline screening for CFHBI, but the optimal frequency of subsequent imaging for those with normal baseline findings is unknown and a specific recommendation cannot currently be made.
  • CFF recommends that persons with CF who are found to have hepatobiliary involvement (abnormal physical exam, persistently elevated liver enzymes, or abnormal abdominal ultrasound findings), should undergo baseline liver elastography, when available, for the identification of advanced liver disease.
  • CFF recommends that persons with CF and hepatobiliary involvement should receive a standardized evaluation for other causes of liver disease.
  • CFF recommends that all persons with CF who have cholestatic liver test (Direct Bilirubin, GGT, and/or alkaline phosphatase) elevation, abdominal pain consistent with a biliary cause and inconclusive abdominal ultrasound should undergo magnetic resonance cholangiopancreatography (MRCP) to detect hepatolithiasis, choledocholithiasis, biliary strictures, and/or biliary obstruction.

Monitoring

  • CFF recommends that in persons with CF and liver involvement, a liver specific physical examination be performed at least annually and at each in-person visit to monitor for the progression of disease.
  • CFF recommends that for persons with CF and hepatobiliary involvement at least one liver fibrosis index be calculated and followed at least annually.
  • CFF recommends for persons with CF and hepatobiliary involvement an abdominal US be performed at least every 2 years to monitor for the progression of disease.
  • CFF recommends that persons with CF and hepatobiliary involvement or advanced liver disease undergo evaluation of liver stiffness with elastography at least annually, if available, to monitor for the progression of disease.
  • CFF recommends that persons with CF hepatobiliary involvement or advanced liver disease receive consultation with a pharmacist with experience in CF and hepatobiliary disease to identify high risk medications, polypharmacy, therapy modifications, and risk based on their hepatobiliary disease every 6 months, unless there have been no medication changes, to improve morbidity and mortality.
  • CFF recommends that persons with CF and advanced liver disease undergo liver specific physical examination and measurement of AST, ALT, GGT, alkaline phosphatase, conjugated (or direct) bilirubin, albumin, platelets, and international normalized ratio (INR) at least every 6 months to detect progression of liver disease.
  • CFF recommends that persons with CF and advanced liver disease, receive a nutritional assessment for malnutrition and liver specific macro- and micronutrient deficiencies every 6 months by a dietitian experienced in CF. When abnormalities are identified a treatment plan and monitoring of nutritional rehabilitation should be developed.
  • The CFF recommends, for persons with CF and advanced liver disease, beginning CF beginning CF beginning CF beginning CF beginning CF related diabetes (CFRD) screening at the diagnosis of advanced liver disease, even if earlier than age 10 and then annually thereafter, to provide early diagnosis and treatment of CFRD (using methodologies described in the most current CFRD Guidelines).
  • CFF recommends that persons with CF and advanced liver disease receive multidisciplinary care from pulmonology, gastroenterology/hepatology, endocrinology and when appropriate, a transplant center, to optimize liver outcomes.
  • CFF recommends that adults with CF and advanced liver disease with esophageal varices be managed according to the relevant most current published guidelines, such as those from the American Association for the Study of Liver Diseases or the Baveno group.
  • The CFF cannot provide a recommendation for or against endoscopic variceal surveillance in children with advanced liver disease, due to insufficient evidence.
  • CFF recommends that all CF children with cirrhosis or suspected cirrhosis, are screened annually for hepatocellular carcinoma with abdominal US and serum alphafetoprotein.
  • CFF recommends that all CF adults with cirrhosis or suspected cirrhosis are screened for hepatocellular carcinoma using the most current screening guidelines as per American Association for the Study of Liver Diseases or European Association for the Study of Liver Disease.

Overview

Title

Cystic Fibrosis Screening, Evaluation and Management of Hepatobiliary Disease

Authoring Organization