# Remarkable Response to Odevixibat in an Adult With Progressive Familial Intrahepatic Cholestasis Type 1 and Intractable Pruritus

## TL;DR

A 27-year-old with a rare liver disease experienced dramatic improvement in symptoms after starting odevixibat, avoiding the need for a liver transplant.

## Contribution

Demonstrates odevixibat's effectiveness in treating intractable pruritus in progressive familial intrahepatic cholestasis type 1.

## Key findings

- Odevixibat led to biochemical improvement and sustained resolution of pruritus.
- The patient resumed normal activities and achieved excellent quality of life.
- Liver transplantation was avoided due to preserved liver function and treatment response.

## Abstract

We report a case of significant clinical improvement after starting odevixibat in a 27-year-old patient with refractory pruritus due to progressive familial intrahepatic cholestasis type 1. He had poor response to multiple therapies and experienced profound decline in quality of life, describing his symptoms as emotionally exhausting and difficult to endure. He was evaluated for liver transplantation in March 2020 but was denied due to preserved liver function. Odevixibat was started in November 2021, resulting in biochemical improvement and dramatic sustained resolution of pruritus. He resumed normal activities, achieving excellent quality of life.