Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Cystic Fibrosis (CF) is an inherited condition that impacts multiple organ systems, one of which is the liver. The presentation and manifestation of liver disease in CF are varied, and there is ongoing research to better understand its etiology and clinical implications. Some studies have shown that liver disease in CF impacts 10% of people with CF (PwCF) by age 10 and 30% by age 30. Screening and monitoring for hepatic involvement in CF continue to evolve as new guidelines and therapies are created. In 2023, new consensus guidelines were released regarding the evaluation, nomenclature, and monitoring of liver disease in CF. Two new terms define the impact of CF on the liver: CF hepatobiliary involvement (CFHBI) and advanced cystic fibrosis-associated liver disease (aCFLD). Monitoring and evaluation of the progression of liver disease in CF requires close laboratory monitoring in coordination with imaging. A mainstay of evaluating PwCF for CFHBI includes elastography scanning to determine liver stiffness measurements to assess for possible fibrosis. An affordable, portable, and non-invasive approach to complete elastography evaluations is through point-of-care transient elastography (POCTE). This article will cover the use and utility of POCTE as it pertains to the evaluation of CFHBI and aCFLD in the pediatric CF population. More specifically, this article will provide background information regarding guidelines for when and how to use POCTE in the care of PwCF. It will also provide detailed instructions on how to perform POCTE, how to interpret results, the next steps in care, limitations of POCTE, as well as other considerations in its use. Lastly, this article will describe a quality improvement project implementing POCTE for the first time in a large pediatric CF clinic.