You have PBC…..now what?
With the diagnosis of PBC (primary biliary cholangitis), you have a lot of questions. Where do you start? How do you start? What do you do first? Who do you ask? When do you start? Then the questions about how this disease progresses, what it means to you and your family, what you can expect going forward and many other questions yet to be realized.
PBC is a chronic disease which means you will have PBC for the rest of your life, or until a cure is developed and you are cured. PBC is a progressive disease which means the disease continues to slowly destroy the small bile ducts of your liver. PBC is an autoimmune disease – your body is attacking your liver cells as if they were an infection or foreign cells in your body. PBC is not considered a heredity disease but there are cases of familiar disease in female relatives such as mothers, daughters, sisters, and aunts.
What is your journey going to look like? What will you experience? Each PBC patient has their own journey and yet many of us find similarities.
What happens next?
First, let’s start at the beginning. You may be one of the lucky ones that was diagnosed “quickly” due to an astute medical professional recognizing your intense itching as pruritus rather than allergies and tested you for the AMA2 antibody in your blood. You may have stumbled upon a diagnosis because your liver enzymes were elevated and this was caught in a routine blood test for your annual check up. You may have been on a journey of doctor visits and tests trying to figure out the puzzle of your diagnosis for many months, or even years!
However you came to be at this point, you now have a diagnosis of PBC. What now?
Your doctor will likely prescribe medication and monitor your liver enzymes annually. Because PBC is a slowly progressing disease when medication is taken as prescribed, you may only see your liver specialist every year or every other year as your situation requires. Doctor’s often consider us as “an easy patient” because all they require is routine blood testing and prescription renewals. This is something of a misconception as there may be symptoms of the disease that require additional treatment. Pruritus (itching) and fatigue are the most common symptoms reported by PBC patients. Both can be treated with some behavior modifications, diet and exercise and if required, medication.
What else do I need to know?
Here is where the PBC patient typically falls off the planet for the medical profession. Unfortunately, many health care providers miss the opportunity to discuss the need for support for this chronic illness. As a PBC patient and an advocate, I work to engage the medical community on the importance of support for the PBC patient. A patient that knows the important facts about their disease and has a good support system is more likely to be compliant with taking medication, which in turn means the disease progresses slower than without medication. Identifying complications of advancing liver damage also helps promote a healthier patient and minimizes the impact of severe problems late in the disease stage.
Also, it is important for the healthcare community to understand why the PBC community requested that the disease name be changed from Primary Biliary Cirrhosis to Primary Biliary Cholangitis. The word cirrhosis means scarring in the liver. PBC slowly destroys the small bile ducts which eventually leads to liver failure if not slowed adequately. When liver failure occurs, the only treatment is a transplant. Since that stage of the disease only happens at the last stage (stage 4), cholangitis (meaning inflammation of the liver) is a more accurate word describe the majority of people with PBC. The stigma of the word cirrhosis caused confusion and discrimination for patients. There will be more on this topic in a future blog post.
For now, you need to know you are not alone in this journey with PBC. There are excellent resources available for patients to gain support, either in person or in the online community. PBCers Organization (http://pbcers.org) is the largest PBC patient support group in the US and has a number of support venues for PBC patients both through their social media channels as well as in person conferences and local meetings. If you are in Canada, the PBC Society (pbc-society.ca/) is there for you and in the UK, PBC Foundation (https://www.pbcfoundation.org.uk/) helps those in need of support. Other groups and organizations are available around the world to aid in education and support of the PBC Community.
PBC Advocacy is a personal passion of mine. Not only is it a way to “pay it forward”, it is a way to express myself in a positive way as I continue my own journey with this disease. Learn more about advocacy activities at Advocacy Arenas on the PBC Advocate website.