Symptom 2: Cholestasis – The Result of Impaired Bile Flow in Alagille Syndrome
Another symptom of Alagille Syndrome is cholestasis, a condition that arises when the flow of bile from the liver slows down or stops. This condition can be visualized as a kind of internal traffic jam where the liver is the highway and bile, the vehicles.
Bile is essential for the body. Produced by the liver, it breaks down fats and eliminates waste products from the body. It is supposed to exit the liver and enter the small intestine. However, in Alagille Syndrome, there’s a blockade in this smooth transit, leading to cholestasis.
The consequences of cholestasis are not immediate but gradually unfold over time. The first sign is usually itchy skin, a rather subtle clue that something is amiss within. As bile and other substances accumulate in the liver, the itching intensifies. It may seem a mild nuisance at first, but don’t be fooled. The itch is a hint, an early whisper of the commotion within.
Cholestasis also leads to other notable changes. The bile’s absence from the digestive tract can lead to pale, fatty, and foul-smelling stools. Simultaneously, the urine turns darker due to the excess bilirubin excreted through the kidneys. These alterations in bodily excretions, while seemingly mundane, are signposts guiding towards the underlying condition.
The story of cholestasis is a tale of stagnation and disruption. It narrates the plight of the liver, an organ trying to carry out its duties despite the obstacles. And it’s this plight that paints the second stroke in the intricate picture of Alagille Syndrome. (2)