Living with Primary Biliary Cholangitis
Menopause Is Complex. Is Your Healthcare Provider up for the Job?
January 15, 2025In late 2011, Dana Walker knew something was wrong when she kept getting winded walking up a small hill from the parking garage to her office. She was also getting unusually exhausted after sparring during tae kwon do practices.
“I would be completely out of breath, and my mouth would be very dry, so I was constantly running to get water,” Walker said. “My throat was just dry as a bone.”
Walker thought she might have diabetes because of her constant thirst. She shared her symptoms with her primary care provider and added that her palms had been itching as well.
Walker said her primary care provider suspected she might have primary biliary cholangitis, or PBC, and ordered a blood panel to check. When the panel came back with elevated liver enzymes, he referred Walker to a gastroenterologist, who ordered additional tests that confirmed the diagnosis.
It would be the start of a long and not always straightforward journey to manage her condition, which her doctor diagnosed as somewhere between stage 2 and stage 3 fibrosis. Walker learned that an early diagnosis and proper management were crucial to preventing severe liver damage that could lead to organ failure and even death.
What is PBC?
Once called primary biliary cirrhosis because the disease was often diagnosed at an advanced stage of liver disease, PBC is a rare autoimmune condition that mainly affects women over 40, but can also affect men. Researchers estimate that about 65 of every 100,000 women in the U.S. are living with PBC, and among people with the condition, 9 out of 10 are women.
Although PBC affects people from all racial backgrounds and ethnic groups, Hispanic people living with PBC are more likely than non-Hispanic white people living with PBC to be hospitalized, and Black people have higher odds of mortality from the disease.
PBC occurs when the cells that make up the small bile ducts within the liver are attacked or destroyed. When functioning normally, the ducts remove bile and move it into the intestines where it can be eliminated. When the ducts are destroyed, the bile builds up and has nowhere to go. Chronic itching, called pruritus, can be the way the blockage shows up in your body, but the bile buildup also damages the liver, which can lead to permanent scarring and cirrhosis, a disease where scarring takes over your liver tissue. Fatigue is another common symptom of PBC and many other autoimmune conditions.
“The majority of PBC patients have very mild or no symptoms and are only identified through abnormal blood tests,” said Christopher Bowlus, M.D., chief of the division of gastroenterology and hepatology at UC Davis Health. “The blood tests typically will show elevated liver enzymes.”
The tests look for a substance called alkaline phosphatase. People living with PBC typically have another blood marker called an antimitochondrial antibody. Most PBC cases are diagnosed if both factors are present.
People living with PBC should have clinical treatment. Without treatment, PBC can progress to liver damage.
“There are two aspects of PBC — quality of life related to severity of symptoms, and progression of liver disease, which can result in cirrhosis and need for liver transplantation,” Bowlus said. “They’re not necessarily related. People can have serious symptoms but mild liver disease and vice versa. Regardless of whether symptoms are present or not, all patients should be on treatment to prevent the progression of the liver disease.”
Steps you can take to prioritize your well-being while living with PBC
In addition to clinical treatment, Bowlus said minimizing alcohol use or avoiding it altogether is important for anyone with liver disease, particularly if their PBC is in an advanced stage.
Maintaining a healthy diet may help people living with PBC manage PBC symptoms, although nutritional needs can differ depending on their disease stage and seriousness of symptoms. Cutting back on food that requires bile in the digestion process, which includes foods that are high in saturated fats, sugar or sodium, may be helpful. Exercise may also help decrease fatigue and strengthen bones, which is important because 3 out 10 people living with PBC develop osteoporosis.
Good communication with your healthcare provider about how you’re feeling and your symptoms is another way to improve your experience living with PBC. For example, people living with PBC sometimes don’t make the connection between their disease and their chronic itching, so they don’t mention it to their healthcare provider, which leads to them not getting the appropriate care.
Increased awareness can lead to improved outcomes
The number of known PBC cases is increasing because of increased awareness of the disease, more accurate diagnoses and better treatment, Bowlus said, while noting that many people living with PBC remain undertreated. Healthcare providers (HCPs) have to be aware of the condition to make a correct diagnosis or refer patients to specialists, and even providers who know about PBC might not be aware of the latest treatment options available, including treatments that are useful for people who aren’t responding to the medicines that HCPs typically start with.
For people living with PBC who lack access to specialists, including those in rural areas or those experiencing economic instability that prevents them from seeing HCPs, undertreatment can turn a manageable diagnosis into a more serious one.
Walker said she feels fortunate to live in Baltimore, where she can visit world-class healthcare facilities with providers who are aware of PBC and can treat it early. In her social media support groups, however, Walker often reads stories from women who are frustrated that their providers don’t know much about the disease.
“They don’t feel they get the same care other people do because of their circumstances,” Walker said.
Walker said she tries to share as much information as she can to help others with PBC. She does it in honor of Shirley Bouloubassis, who befriended Walker when she was first diagnosed.
“Shirley was the first person I met that had PBC and she guided and checked in on me during a scary time in my life,” Walker said. “She called me and others her PBC sisters. She was a great advocate for promoting awareness, raising money for [research] and getting the PBC sisters together to remind us that we are not alone.”
Resources
This educational resource was created with support from Gilead.
