Cholestasis, Progressive Familial Intrahepatic 1

Byler Disease

Overview

Type of disease: Rare conditions

Progressive familial intrahepatic cholestasis 1, also known as Byler’s disease and Greenland-Eskimo familial cholestasis, is a rare condition and is one of the multiple forms of cholestasis. Cholestasis is a rare disease where a person’s liver can not move the bile it makes to the small intestine. The liver, an organ, is responsible for producing bile. Bile is a compound that helps people digest fats. Once the bile has been made, it is supposed to go to the small intestine, another organ, to digest the fats there. However, in people with cholestasis, the bile can not move to the small intestine because there is either a physical block or because the bile is stuck in the liver cells. People with progressive familial intrahepatic cholestasis 1 may have symptoms such as watery diarrhea, itchiness, and jaundice (yellowing of the skin). Talk with your doctor to find the best treatment for you if you have been diagnosed with progressive familial intrahepatic cholestasis 1.

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