7 Questions About Your Liver, Answered

When Ellen began feeling more run-down than usual back in 2001, she didn’t give it much thought. After all, her job as a chef and hospitality director in southern Missouri could be demanding. “I worked long hours and was on my feet a lot,” she says. “I was active with my dogs, and I helped out with my first two grandchildren when they came along.”

But the results of a routine blood test that showed she had elevated liver enzymes did raise concerns for her doctor, who sent Ellen for additional tests. The diagnosis: a rare, progressive liver disease called primary biliary cholangitis (PBC). Affecting about 130,000 people in the U.S. — a majority of those diagnosed being middle-aged women — PBC destroys the small bile ducts in the liver. This can lead to gradual scarring in the liver, and subsequently cirrhosis and liver failure if left untreated.

There’s no cure for PBC, but there are treatment options available to help lower liver enzymes like alkaline phosphatase (ALP) or slow disease progression. There are also other treatments that may help manage commons symptoms, including chronic itch (pruritus) and complications. Talk to your doctor about what treatment options may be right for you — your care team will follow the latest clinical guidelines to help guide your care.

For many people, receiving a PBC diagnosis can feel overwhelming. “My doctor was knowledgeable about PBC, and knew where to direct me from that point,” says Ellen.

To provide some clarity on this vital organ and what can happen when it is affected by disease, we compiled some essential facts and tapped into Ellen’s invaluable experience of living with PBC.

1. What is PBC, and how does it affect the liver?

The liver is a critical organ in the human body, performing hundreds of functions necessary for good health. Among them: filtering waste and other toxins, metabolizing nutrients, aiding digestion through the production of bile — a fluid that aids in that process — and regulating blood clotting.

But when someone is living with PBC, their body’s own immune system mistakenly attacks and destroys those small bile ducts in the liver. This can cause bile to back up, leading to inflammation and permanent scarring. As scar tissue continues to replace healthy tissue, the liver loses its ability to perform all its vital tasks.

2. How will I know if I’m at risk?

While the exact cause of PBC is unknown, research suggests that genetics and environmental triggers, such as smoking, chemicals and infections, may increase the risk of developing the condition. PBC predominately affects women, though studies indicate that diagnoses among men are on the rise.

Both gender and age can be a factor, with the majority of newly diagnosed people in their 40s to 60s; however, it is important to monitor your liver health throughout your life. PBC often occurs alongside other autoimmune diseases, including rheumatoid arthritis and Sjögren's syndrome.

3. How is PBC diagnosed?

A blood test can identify PBC markers, such as elevated liver enzymes and antimitochondrial antibodies (or AMAs). Imaging exams like ultrasound and MRI can help rule out other potential sources of the bile obstruction, while some healthcare providers may recommend a biopsy. In addition to staying on top of screenings, it’s crucial to discuss symptoms and risk factors with your healthcare team.

4. What symptoms can occur?

Early symptoms may go unnoticed with PBC, as they may be mild or nonexistent. Many patients report experiencing fatigue as a symptom of PBC, along with itching (pruritus). This fatigue may be persistent and can interfere with daily life — often described as a kind overwhelming lack of energy. Other signs of a sick liver include dry eyes and mouth, abdominal and joint pain, yellowing of the skin or eyes, brain fog and high cholesterol levels.

“For the first 15 years, I suffered from debilitating fatigue,” Ellen says. “It’s not the same thing as just feeling exhausted or tired. In my experience, it feels like a lead weight, where you can’t actually move your body.”

Early on, Ellen also had severe itching. It is a symptom that can affect up to 70% of people living with PBC and tends to impact their daily lives. These sensations can last weeks, and moderate-to-severe nighttime itch can cause sleep deprivation, exhaustion, worsening fatigue and declining mental health.

“I thought it was a seasonal thing. It seemed to centralize around the palms of my hands, my feet and my ankles, and mostly occurred at night,” Ellen said.

5. How long does it take for PBC symptoms to escalate?

PBC progresses at different rates for each person. Regular monitoring with a healthcare provider is essential to track disease progression. For Ellen, more severe symptoms emerged about 16 years after her diagnosis. “I just didn’t feel right,” she says. “I drove my car to the closest hospital.”

Over time, Ellen began experiencing additional symptoms, including pain in the right upper quadrant of the abdomen, where the liver is located. “I also had an issue with my gallbladder, and my spleen became very enlarged,” she explains. “I also have what I call ‘fuzzy brain,’ or brain fog.”

6. How did PBC change your lifestyle?

Physically, she doesn’t have the endurance that she once had, she adds: “I’ve found that if I have a really full, busy day, I'll need the whole next day to recuperate. I can’t do a lot of the things I used to do with friends, or even go for long car rides.”

But she has been fortunate enough to have a group of close friends and has taken advantage of support groups. “I’ll go online if I have a specific question and feel I can benefit from others’ experience or opinions,” she says. “It’s also important to share what you’re going through with close friends and confidantes.”

7. What is your advice for someone who’s just been diagnosed with PBC?

“Be proactive. Read as much as you can about PBC,” Ellen advises. “If you don’t feel like you’re getting answers from your doctor, find a different one. As PBC progresses, you might have some really tough days. You have to be good to yourself. If your body is telling you not to do something, listen to it.”

This is a perspective from one patient and does not represent the opinions of all patients, caregivers or healthcare professionals. The information included is not a substitute for professional medical advice. Always talk to your healthcare provider.