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When my son’s liver disease progressed due to Alagille syndrome (ALGS), his xanthomas worsened. The xanthomas were awful and incredibly painful for him. They seemed to quickly spread over his body and became physically disfiguring to him as they started to develop on his face, lips, and nose. 

After dealing with the xanthomas for a good amount of time, our medical team advised us there weren’t many treatment options for xanthomas since the type of cholesterol that causes the xanthomas is a product of liver disease and not the kind of cholesterol that can be treated with medication.

We were presented with a couple of possible treatment options. A liver transplant would treat the xanthomas, but we weren’t yet at the point in our journey where we were good candidates. The second option was trying a medication called cholestyramine. 

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Of course, once we got the prescription for the cholestyramine, I was excited to see if it could help my son. I wanted to do whatever I could to take xanthomas away from him or lessen their effect on his life. 

Read about experimental therapies for ALGS

The first concern we had before even administering cholestyramine was the difficulty of administering the medication. It had to be spaced out from medications, food, and drinks. It made the timing of administering the medication very difficult to get down. We had to be sure to stick to a strict schedule and it was stressful trying to look over our routines and schedules to see how we could make this work. 

After we made our first dose of the cholestyramine, we noticed how thick and full the medicine became. We were told from the beginning it isn’t a pleasant taste so we were hoping getting my son to take it would be easy. At that time, we were able to apply most of his medications by adding them to his bottle of formula, but this one could not be mixed in due to how much it would expand. 

Making him take the medicine was not easy. It was awful. He started gagging and vomiting and wouldn’t take anymore. At this point, we decided to try again, but we didn’t want to make him miserable by trying to force medication on him that wasn’t a necessity. We got some advice from our team on how to mix the medicine for optimal taste. They suggested mixing it in orange juice. 

The next time we tried giving the medicine we were out and decided that we would mix it with orange juice. We brought a small syringe with us so that once the medicine was mixed we could give it a little at a time. It was going great, and I thought we were going to have our first success until he yet again threw up all over the table in the restaurant we were at. Horrible! At that point, I decided the taste, application, and amount that had to be given was not worth the fight or making him sick. 

Cholestyramine didn’t work for us, but I’ve read that some other parents have better luck. It’s a very difficult medication to give especially with the taste and thickness. I’m glad medications like this are available to treat certain symptoms of rare diseases like ALGS.