ABOUT PBC
What is PBC?
Primary biliary cholangitis (PBC) is a rare autoimmune disease in which the body’s immune system attacks healthy cells in the liver by mistake. People with PBC experience liver inflammation and buildup of toxins, such as bile acids, causing liver damage and reduced liver function.
If left untreated, PBC can lead to liver complications, resulting in liver transplant or death. However, there are treatment options available to you.
Having a better understanding of PBC and discussing your treatment plan with your doctor can help manage disease progression.
What causes PBC?
Genetics and environmental triggers can increase the risk of developing PBC, but ultimately the cause is unknown. It’s important for you to know that your diagnosis was not caused by anything you did.
Did You Know?
PBC is:
Multifactorial (made up of genetic and environmental factors). While a cause is not fully understood, it is treatable
PBC is NOT:
Alcohol related
PBC isn’t my fault—this is just the way my liver works.
KATHY, LIVING WITH PBC
An invisible condition
Symptoms vary from person to person, but common symptoms include:
Fatigue
“It’s severe exhaustion that I can’t explain.”
Severe itching
"There is nothing that helps."
Brain fog
“My forgetfulness leads me to feeling angry and scared.”
Body pain
“It’s an ache that no one else can see.”
These are not the only symptoms of PBC. Beyond physical and mental symptoms, many people also experience an emotional burden of the disease.
It’s important to understand that PBC symptoms:
- Are unique to each person
- Can change over time
- Do not necessarily mean disease progression (or worsening)
Did You Know?
Up to 60%
of people with PBC do not have symptoms at diagnosis.
Ways to keep track of symptoms
There are scales that help measure how PBC is impacting your quality of life, from symptoms of persistent itch to the emotional aspects of the disease. Two of these scales are PBC-40 and 5-D itch, which are both used in clinical trials to monitor and track symptoms. Learn more about these scales below.
A 40-question survey to help measure the impact of PBC on your life, covering:
- Fatigue
- Itching
- Brain fog
- Emotional impact
- Social impact
Sample questions:
In the last 4 weeks…
- Has fatigue interfered with your daily routine?
- Has itching disturbed your sleep?
A 5-question survey used to measure persistent itching caused by PBC, including:
- Degree (intensity)
- Duration (for how long)
- Direction (better or worse over time)
- Disability (impact on daily activities)
- Distribution (where on body)
Sample questions:
- During the last 2 weeks, how many hours a day have you been itching?
- Over the past 2 weeks, has your itching gotten better or worse compared to the previous month?
Ask your doctor to discuss these scales with you at your next follow-up visit.
Life with PBC
Lifestyle and wellness information that may help
Discuss the following information with your doctor to ensure you are doing what’s best for you and your health.
Diet
While there aren’t specific dietary restrictions, it’s always recommended to eat a healthy, balanced diet!
Alcohol and smoking
Avoid excessive alcohol consumption and smoking.
Discuss drinking alcohol while on PBC treatment with your doctor, as the risks are not known.
Physical activity
While physical activity is important to a healthy lifestyle, fatigue from PBC may impact the way or how much you are able to exercise.
Discuss with your doctor what exercises may be right for you.
Interested in learning more about life with PBC?
Check out this interview in Good Housekeeping featuring two people with PBC—Kathy and Amber—and their stories.
Disease progression
Understanding liver lab test results
Liver function is measured with a series of laboratory (blood) tests. These tests help diagnose diseases and determine disease progression. Your doctor may have seen higher levels of some markers in a routine lab test, which resulted in a PBC diagnosis.
Levels to continue monitoring
Alkaline phosphatase (ALP)
A type of protein, called an enzyme, that is found in the liver and other parts of the body.
Bilirubin
A substance that is made when red blood cells are broken down; it passes through the liver to be removed from the body.
Elevated (higher) ALP and bilirubin levels may mean:
Liver damage or disease (such as PBC)
PBC treatment isn’t working well enough
Disease progression
These levels give you and your doctor a clearer view of if and how PBC is progressing.
Did You Know?
Did You Know?
The latest PBC guidance recommends getting lab tests done every 3-6 months.
If you would like to review your results with your doctor, ask to schedule an appointment.
Know your numbers
You can monitor your levels and see how they compare to your last test result. Refer to the scale below to see a range of normal to elevated ALP and bilirubin levels.
When discussing these levels with your doctor, you may hear the term “upper limit of normal,” or ULN. The ULN is the high end of a normal range for a lab result. In context of PBC, a high ALP level is usually defined as being at least 1.5 to 2 times the ULN.
You are your own best advocate for your health, and you have the power to be involved in your PBC care plan.
Schedule a follow-up visit with your doctor to review your latest liver lab test results.
Ready to learn about treatment response and monitoring disease progression?
