After months of dealing with my son’s uncontrollable itch from Alagille syndrome (ALGS), we finally found a solution. My son has always been mildly itchy, but at the end of 2021, it started to increase. This was no surprise as the itch typically picks up around October because of the cooler, dry weather that comes in our area but after a month or two, things didn’t improve.
Initially, our team wanted to start the typical medications again, ursodiol and hydroxyzine, and run labs to check bile acids and lipids to see if there was a recent change that could explain the increase in itch. Once we got the results back, we noticed the ursodiol level was barely existent and the bile acids had increased quite a bit. Based on this information, we decided it would be best to add rifampin to the other medications he was taking.
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At first, the rifampin seemed to make a difference. We kept him on the same regimen and then in the summer of 2022 he got sick and we skipped the medications for a few days, but during this time he seemed to sleep well and not appear itchy. We stopped the medications and were successful with the itch being mild again for a few months. Then, it picked up with a vengeance.
The itching became so bad it would wake him up multiple times at night. He would just sit awake and itch and scratch his body for hours. I knew it was really bad when he would wake up in the morning with fresh scabs and dried blood not only on himself but on the sheets as well. Anytime he was restricted without distraction like in the car he would pick his feet to the point they would be raw and bleeding.
At our next visit with the liver team, I brought up all the concerns I had and how the itching increased, and even though we resumed the medications didn’t seem to make a dent to provide him some comfort. The team suggested we try Livmarli® (maralixibat). I had no experience with it but had read nothing but good things about it from other families who have used it for the itch.
It was a rather quick process for us to start the medication. I filled out some paperwork and the doctor’s office handled everything. From there, the team at Livmarli contacted me and went step by step through everything I needed to know. We had multiple phone calls for different things to discuss to make sure everyone was good to go. I can’t say enough good things about their service and what they’re doing in the ALGS world.
My son has been on Livmarli for about 3 weeks now and the difference in his itch is huge. He is now sleeping through the night, no longer itching himself to the point of bleeding, and no longer picking his feet raw. The change I see in him and his daily life has been huge and I am so thankful this medication was approved for use and we were eligible to try it. I can only hope as we continue to use Livmarli he continues to get amazing results.
