What I’ve Learned by Raising a Child With a Rare Liver Condition

Amanda Cota and child

In 2021, 14-month-old Cooper was diagnosed with Alagille syndrome. Here’s how his mom Amanda manages his most challenging symptoms.

I’ve had Alagille syndrome (ALGS), a rare condition caused by bile duct abnormalities, for as long as I can remember. For those unfamiliar, bile is a liquid that helps remove waste and breaks down fat. When you eat, the bile goes into tubes called bile ducts that carry it to the small intestine.

Alagille syndrome can affect the liver, heart, and other parts of the body and leads to a range of symptoms. Two of the most common are itchy skin and jaundice, which causes yellowing of the skin or whites of the eyes. Itchy skin is a symptom I’ve always struggled with. I’m now 29, and it’s become less uncomfortable, and I’ve found remedies that help a bit. But I still deal with it. 

Starting a Family 

Alagille syndrome is a genetic condition that can be passed down through the generations, and I have a family history of it. My sister Nicole also had the condition and died when she was just a year old. She had complications with her liver and died after a liver transplant that was just too much for her little body. I once visited a genetic counselor who told me that there was a 50/50 chance that I would pass Alagille syndrome to my children. 

When I became pregnant with my son, Cooper—who is now 14 months—I knew that he could develop the condition. Because some symptoms of Alagille syndrome can show up in utero, I had a bunch of fetal echocardiograms during my pregnancy. The scans suggested that Cooper had some heart abnormalities, which indicated that he could have Alagille syndrome. 

I was very scared when he started showing signs. I was wondering how I’d be able to handle his diagnosis. Would it be as severe as Nicole’s? Will he need a liver transplant? Is he going to need surgery? So many questions were running through my mind. I never grew up around other babies, so I was learning how to care for a baby in general, and now with his diagnosis, I knew I would face many challenges. You might think growing up with this disease would make it a little easier to understand, but it didn’t. 

Cooper didn’t have any symptoms until he was about a month old. At that time, he started to become jaundiced, so I took him to several specialists. His blood work showed some abnormalities, including potential liver damage, and he was officially diagnosed with Alagille syndrome about a month later.

Helping Cooper with His Symptoms 

About two months after his diagnosis, Cooper started to become itchy. He would rub his face and head, and he would wiggle on his back because he didn’t know how to scratch himself. I began to feel very guilty as Cooper started to show signs of itching. I felt like his discomfort was all my fault. He was put on an oral medication to ease the itch, but it hasn’t totally stopped his symptoms. I also tried to use oatmeal-based creams and lotions designed for people with eczema, but they didn’t seem to help. 

When he was really young, the only way I could get him to go to sleep was to swaddle him, so he couldn’t use his arms to scratch himself. He won’t let me swaddle him anymore, so I bought ScratchSleeves, which have a satiny material that covers his hands. They prevent him from scratching his skin and ears. 

More recently, he’s started scooting his butt along his crib and banging his head into the rails to itch. It was terrifying when I saw him do this for the first time. My pediatrician recommended that I cut and place squishy pool noodles on his crib rails so he doesn’t hurt himself.* Even with the added protection, it’s still unsettling that he does this.

*Editor’s note: Do not try this at home before speaking with your healthcare provider.

Cooper also scratches his ears during the daytime. It used to be constant—all day, every day—but with his current medication, he’s now scratching once or twice daily. When we’re home, I try to be playful with his hands and keep them away from his ears instead of repeatedly telling him he can’t itch. 

For parents who are just beginning this journey, my best advice is to take it one day, one step at a time.

Car rides present a different challenge. Since I can’t redirect his hands while I’m driving, he’s managed to scratch his ears until they’ve bled. Now I put mittens on him in the car to prevent that. (The ScratchSleeves are a little pricey, so we reserve them for bedtime.)

Since Cooper is 14 months old, he’s too young to communicate with me about his symptoms. I also started thinking about how I get relief from my Alagille syndrome challenges to help him more effectively. For example, I get itchier when I get hot, and a nice, cool shower can help. I also tend to feel itchier at night. Because of this, I give Cooper a lukewarm bath every night before bed to help him cool off. I also set his room at a comfortable temperature and keep a fan continuously running for good air circulation. It’s heartbreaking to watch your baby sit there and itch. I just try to keep him cool and comfortable, and I give him lots of love and hugs.

Finding The Silver Lining 

I am endlessly grateful that I can draw from my experiences living with Alagille syndrome. Having a child with this disease has given me an entirely new perspective on myself, the condition, my life, and my son. I’m learning new things every day. And I’m learning new ways to take better care of myself and Cooper. 

 I feel so blessed to be his mother, he is becoming such a sweet little man, and I could not be more honored to be a part of his journey. 

For parents who are just beginning this journey, my best advice is to take it one day, one step at a time. This is a challenging disease for both the parents and the children to navigate. Just give all your love and comfort to your little one. Our little warriors are strong and resilient and will face many challenges, and as parents, we will be by their side every step of the way. 

3 Sources
Verywell Family uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. Medline Plus.  Bile duct diseases.

  2. Medline Plus. Alagille syndrome.

  3. Morton A, Kumar S. Alagille syndrome and pregnancyObstet Med. 2021;14(1):39-41. doi:10.1177/1753495X19849738

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