Center for Autoimmune Liver Disease

Patient Stories | Ciara and Autoimmune Hepatitis

Creative Treatment Helps Ciara and Other Patients Diagnosed with Autoimmune Hepatitis

The typical treatment for autoimmune hepatitis wasn’t working for now-14-year-old Ciara Woods. The outside-the-box therapy she received at the Center for Autoimmune Liver Disease is now standard of care for patients like her.

Sometimes a patient comes along who makes a doctor rewrite the rule book on how to treat a certain disease. For Amy Taylor, MD, that patient was Ciara Woods. 

Ciara was 9 years old when she was diagnosed with autoimmune hepatitis, a disease in which the body’s own immune system attacks the liver, causing it to become inflamed.

Because of the damage autoimmune hepatitis can cause to the liver, the goal of treating the disease is to stop the body from attacking itself by suppressing the immune system. That’s why the first-line therapy is typically a high dose of a steroid, like prednisone. Steroids act as an immune suppressant, but they also cause side effects when they’re used long term—side effects like mood swings, high blood pressure, sleeplessness, muscle weakness and weight gain.

“I knew the treatment guidelines for patients with autoimmune hepatitis, but working with Ciara allowed me to see the very real side effects that could result,” said Dr. Taylor, an assistant professor at the University of Cincinnati Department of Pediatrics. “I learned a lot from getting to work with her.”

Often, short-term use of a high-dose steroid will improve symptoms and lower liver enzymes. When this happens, the general rule of thumb is to lower the steroid dose and start a steroid-sparing medicine that suppresses the immune system, Dr. Taylor explained.

“But different kids respond differently to that combination of medicine,” she continued. And Ciara’s liver enzymes weren’t lowering significantly after two years on a steroid.  

Treating Outside the Lines

Ciara’s mother, Michelle Kendrick, first took her daughter to see Dr. Taylor for a second opinion in 2018, shortly after Ciara was diagnosed with autoimmune hepatitis and while she was being treated by another provider.

“Ciara’s doctor was trying to get her off steroids, but her liver kept getting worse,” Michelle said.

At that point, “I was on the earlier side of my hepatology career,” Dr. Taylor said. “But I had a clinical interest in autoimmune hepatitis, in particular, as well as outcomes around autoimmune disease.”

Dr. Taylor reviewed Ciara’s liver biopsy results and remembers feeling struck by how high of a steroid dose she’d been on, and for how long. 

“I sent recommendations to Ciara’s doctor and worked with her on medication,” Dr. Taylor said. “I knew Ciara was experiencing unwanted side effects from the long-term steroid use, so I focused my recommendations on how to lower the steroid dose while making sure the medication she was on was appropriate to her needs.”

When Ciara’s doctor went on maternity leave in 2019, Dr. Taylor took over her care. Initially, she tried Ciara on the steroid-sparing immunosuppressant azathioprine. “That was the typical, first-line medicine,” Dr. Taylor said. “What’s tricky about that one is that it doesn’t always work well right away.” 

Sure enough, a repeat liver biopsy confirmed Ciara's liver cells had ongoing inflammation due to her autoimmune liver disease. Dr. Taylor talked to Michelle about Ciara’s options. They could either try a second-line therapy or go straight to a third option that was stronger. 

“Given the degree of inflammation in Ciara’s liver, and after a long discussion with her mom, we elected to go with what is typically the third-line therapy,” Dr. Taylor said.

Dr. Taylor started Ciara on immediate-release Prograf, which is usually prescribed to transplant patients to prevent organ rejection. When Ciara suffered headaches as a side effect of the medication, Dr. Taylor helped Michelle get insurance approval for the longer-acting, slow-release Envarsus. 

It worked. Over the course of the next year and a half, Dr. Taylor was able to wean Ciara off steroids completely, while giving her as low of a dose of Envarsus as possible to suppress her immune system.

Due to her experience with Ciara and with other patients like her, Dr. Taylor and her colleagues now start with Envarsus for patients with autoimmune hepatitis who aren’t responding to high-dose steroids. 

“I’m hopeful that someday Ciara may be able to wean off it, but if not, my goal is to have her on as little medication as possible and for her to be able to do all the things she wants to do and live a really happy and healthy life with a well-functioning liver,” Dr. Taylor said.

Living a Full Life

Today, Ciara, now 14, has been off steroids for almost three years. Although she still gets tired sometimes and can have trouble eating greasy foods, she’s very active and her liver enzymes are normal, Michelle said. 

If she needs to lie down from time to time, “We just get through it,” Michelle continued. “Ciara makes sure to stay hydrated and takes breaks when she’s really tired. She plays tennis and softball. She’s able to live a normal life for the most part.”

Michelle believes sharing Ciara’s story will help other families like theirs.

“I think it’s important to help others know they’re not alone,” Michelle said. “Yes, you may have to take medicine for the rest of your life, but it’s manageable. Dr. Taylor has been an amazing doctor for Ciara. She cares about her patients and takes time to ask questions about how they’re feeling. She’s very thorough, and I appreciate that so much about her.”

Ciara and her family are a great example of not letting a disease dictate their lives, Dr. Taylor said.

“Even though autoimmune hepatitis is a rare disease, through our Center for Autoimmune Liver Disease, we have the privilege of seeing a lot of kids with it, and a lot of kids, like Ciara, who don’t respond how you would expect,” she said. “I think of Ciara’s story as encapsulating. It’s honestly shaped the way I think about autoimmune hepatitis and working with kids who have it. And I think it helps us to provide better care—not only to patients here but to patients everywhere.” 

(Published July 2023)