As the mother of a child with Alagille syndrome (ALGS), being the primary caretaker became overwhelming quickly. There were lots of medications and appointments to manage as well as symptoms to monitor and things we had to keep our eyes open for such as the color/type of stools, if he was vomiting or having diarrhea, any random fevers, and much more. The only way to not feel so overwhelmed by the new diagnosis was to learn to take things day by day. 

ALGS for me is not something that I can think of in a long-term way. When I think of it in that way, it becomes very heavy, overwhelming,  especially with so much of the future remaining unknown. At any point, something new can arise, or the current situation can change. 

Ursodiol, AquADEKS, and liquid Vitamin D were my son’s main daily medications. Of course, over time we added more meds as needed to try to alleviate and manage different symptoms. I can recall AquADEKs being the worst medication. As soon as you opened the bottle, the smell would hit you. It was not a pleasant smell, and I cannot imagine that tasting it would be any better. AquADEKs is an orange-colored medication and it stained everything that it came into contact with. Administering AquaDEKS was a challenge. We would have to strip him down, make sure we had old towels or blankets around him in case it went anywhere but his mouth. He had a specialized bib for this specific medication. 

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We also had to monitor him closely afterward because if he vomited, it would also be orange and likely to stain everything. Of course, as he grew or as his lab work came in, changes were made to the medications. I would have to update my medication list with the new dosage amount. Sometimes, new medications were added.  We would have to learn the ins and outs of those medications and how to incorporate them into our routine. At one point there was a medication that he was prescribed that we had to time out from food, drinks, and other medications. Having our team help us figure out a routine for adding medications like these was the best thing because otherwise I drove myself crazy trying to figure out if I was handling it best

Read more about therapies for Alagille syndrome

During this “learning stage,” we had to come up with a new routine of giving medicines and figuring out the best way to give the medications to an almost 3-month-old baby. How do we ensure he gets the entire dose? What do we do if he throws up right after the medicine? What if he cries and does not want to take the medication? How do we make sure we do not miss a dose? 

It was important for us during this time to heavily rely on and learn from our medical team. They were able to present us with different ideas of best practices they had available from other families. Keeping detailed records of what medicine and what time they were given was key. 

Another important factor for us was learning how to administer the medications. We had to do trial and error to see which ways were the most effective for us to get him to take the medication.  In my experience, it was easiest for us to add them to a small bottle of formula. The medical team advised we give a very small amount of formula (we did 2 ounces) so that it was easy for him to finish to ensure he got the entire dose. As time went on and he got older, we would adjust the ways that we administer the meds. 

Finally, to help us learn to navigate our new journey, we learned the best way to manage multiple doctor visits with different specialists was to schedule the appointments back-to-back on the same day. This came as a suggestion from our medical team and has been an amazing “hack” to learn. The hospital went out of its way to help us set up the appointments consecutively so that we would not have to travel more than necessary. Even though it made for a long day, it was great to get 3 appointments done in one day rather than having to travel 3 different days or even weeks in a month to be seen.