From an early age, my son Jackson’s teeth started to deteriorate. Unfortunately, part of an Alagille syndrome (ALGS) diagnosis can involve issues with teeth.

The most common things I have seen have been extremely yellow teeth due to high jaundice levels, broken teeth, and cavities. My son’s vitamin D deficiency for a long period of time was a huge contributing factor to the stability of his teeth.  He was also on several medications (mostly compounded so they were extra “sugary”) that wore down his teeth. 

Around 1-year-old, my son started showing signs of bad teeth. We noticed a gray line across his front teeth.  Slowly, his front teeth began chipping and breaking into pieces. We took him to the pediatric dentist our daughters used. He told us he had several unstable teeth and lots of cavities, but with his health history, we should take him to a specialized dentist for children with complex medical diseases. 

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After visiting the new dentist’s office twice, it was decided that he would need to have dental surgery and they weren’t comfortable doing the surgery. They recommended another dentist who we visited with, but for some reason, I wasn’t comfortable proceeding. I spoke with our liver team and because my son was a current patient of theirs, he would be able to use the dental hospital associated with Children’s Hospital. This felt more comfortable to me as we were able to have all his medical records accessed on both sides. The dentist would be able to easily communicate with any current doctors regarding medical treatment and everyone would be on the same page.

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At our first visit, they said his front teeth were so damaged and dangerous they needed to be extracted. They said he would need a full dental workup, cleaning, and lots of work completed but would require general anesthesia which they were not comfortable with because of his age. However, the front 4 teeth needed to be extracted immediately, which typically requires surgery, but the waiting list was too long so we needed to do an emergency extraction in an office with a local anesthetic specialist.

My son, Jackson, following his first dental surgery.

It was awful. They put him in the chair and had this blanket type of device they wrapped around him and clamped shut with clips so that he couldn’t move. They injected the needle to numb him and started to extract the teeth.  What 1 year old would like this? It was horrible for his mom to watch, and I couldn’t wait for it to be over. 

After the initial extraction, we followed up every 3 months unless they needed to see us sooner. During these visits, they did extra fluoride treatments to try to strengthen the remaining teeth. They tried to treat him as much in the office as they could (such as treating certain teeth with a silver diamine fluoride treatment) to avoid putting him under general anesthesia.  After we found out my son was going to need a liver transplant, the dentist decided to go ahead and move forward with the surgery to complete other dental work.

My son was taken into the OR for over 4 hours and had several teeth extracted, baby root canals completed, and tooth-colored crowns placed on the teeth they were able to salvage. The aftermath wasn’t pleasant to deal with. Constant bleeding and swelling for several days as well as a kid who didn’t want to eat.

A few months later, he spit out one of the crowns, so I made an emergency appointment. To my surprise, none of the crowns were still in place. This was so disheartening. They were trying to replace the crowns in the office, but they did x-rays and noticed he needed some additional work completed. Now we were faced with another 4 hours or more of dental surgery. He had more teeth extracted and they decided it was best to use silver metal crowns instead of tooth-colored. While it was upsetting we had to put him under again, that surgery was the final one he has needed.  

In the end, after the dental work and surgeries his teeth, are finally in a good, stable place. Now we visit the dentist every 3 months for a check-up and cleaning. He is getting older and starting to lose his baby teeth and we just hope that as his adult teeth come in, they remain stable, and we don’t need any more dental surgeries. Dealing with dental issues on top of ALGS can be overwhelming and frustrating.