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Recently, my youngest son, diagnosed with Alagille syndrome (ALGS),  started to become so itchy that I scheduled an appointment with his GI doctor.  He was waking up several times a night and scratching himself for hours at a time. I would get him out of bed in the morning and find dried blood on him and his sheets. There was no place on his body that was safe from his itching.  We decided that we needed to have him seen for a check-up and have labs run. 

Thankfully, we were able to be seen within a week of requesting an appointment. The GI doctor looked over his body and asked questions about his itching “habits.” After taking one look at his extremities, he said, “These are way too many scabs, and we have to do something.” I also noticed a small “bump” near his elbow that concerned me, and I wanted to have it seen to see if we were dealing with a xanthoma. We decided it was best to run all the typical labs, plus throw in a lipid and bile acid panel so that we could see where we stood.

All his typical labs – CBC, hepatic function, basic metabolic, plus others looked good and stable, and nothing was out of the norm. We had to wait several days for the other two.  Once we got them back, our doctor reached out with the results. The lipid panel numbers were still roughly the same as the previous labs ran in 2020, so we decided that the “bump” was unlikely a xanthoma. We are going to keep an eye on it and watch the rest of his body.

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As for the bile acids, his total bile acid had more than doubled from the last time we ran the labs in 2020. This explained the itch. We decided that it was best to add a new medicine, rifampin, into his regimen to try to help control the itch.

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After going over all the details of adding the new compounded medication, the doctor sent the script over to our usual pharmacy. The pharmacy called the next day and told me that their distributor was unable to get the rifampin, so they would send the script to another compounding pharmacy in the area. The new pharmacy told me that they can get the medicine but they do not contract with my insurance, so the drug would be $100 out of pocket. I had fought for so long to get my son’s compounds covered that I was not willing to throw in the towel and pay this amount out of pocket when it should be a $0 amount owed.

Another compounding pharmacy in the area told me that not only do they not contract with my insurance, but they also didn’t have enough of a supply to make the dosage needed.  I contacted a fourth pharmacy that also did not have enough supply to make the medication. Finally, upon reaching out to the fifth pharmacy in another state that we use for other medications, it was able to run the prescription through insurance and were able to get the medicine needed to make the compound and they were able to mail it to us.

This is the first medication that I have run into problems with obtaining due to supply chain issues. It was very frustrating to call several places that didn’t have enough to make a month’s supply. On top of this, it was also frustrating to me that there were certain pharmacies that were not contracted with insurance, and everything would be out of pocket. 

I had no option but to have a compounded medication because my son is 3. I don’t understand why some compounding pharmacies work with insurance and some don’t. I think there need to be more options for people who have to use a different pharmacy because of the current supply chain issues.