We all have those well intentioned colleagues, friends, and family members. You try to explain to them what primary biliary cholangitis (PBC) is, what’s going on in your body, and what you’re feeling. But you’re usually met with a puzzled look and words that go like this: “But you look so good”; “You should just get more sleep”; “You’re doing too much”; or, “Isn’t there something you can take for that?”
I’ve had PBC since 2007, and honestly after eleven years it’s still difficult for me to get people to understand my experience with PBC. And, if you’re like me, you probably don’t tell a lot of people about your PBC because it’s almost impossible to explain it in a way that others understand. Heck, let’s face it, it’s almost impossible for those of us with PBC to truly understand it sometimes.
The following is information that may help you explain PBC when you’re ready to or have a need to share your diagnosis:
What is Primary Biliary Cholangitis (PBC)?
I have a rare, chronic liver disease that slowly destroys the bile ducts within my liver. Scarring of the liver occurs over years due to liver inflammation. Scarring caused by the inflammation can cause cirrhosis. Most of us with PBC will never reach the cirrhosis stage of the disease.
The cause of PBC is unknown, but it is not alcohol or drug induced and is not contagious. Recent studies show that it might be the result of autoimmunity, genetic predisposition, or infection. PBC is usually diagnosed in patients in my age group (between 35 and 60) and most commonly affects women (1 in 1000 are diagnosed each year) – 10 times more than men.
There are four stages to the disease, with Stage 4 being the most advanced. Most of us with PBC look healthy and show no signs of what is going on inside our bodies. Only in the advanced stages of the disease—Stage 4 Cirrhosis—are there any visible clues that PBC is present. One of those signs is a darker pigmentation in the skin or jaundice. My original diagnosis in 2007 showed Stage 0-1, and in 2017 I had progressed to Stage 1-2.
How is it Diagnosed?
PBC is diagnosed through a battery of blood tests, usually after routine blood tests show abnormally high liver enzymes called Alkaline Phosphatase. If blood tests alone are inconclusive, a liver biopsy will be done to confirm the diagnosis. My personal experience was an inconclusive diagnosis by blood tests and a confirmed diagnosis with liver biopsy.
What are the Symptoms?
Most of us may never have symptoms or won’t experience them for many years. There are many symptoms associated with PBC, but the two most common are fatigue and itching. Fatigue is often the first symptom most of us experience. Some report exhaustion to the point of getting a full 8-10 hours of sleep and still being too tired to function. Others report not getting enough sleep, including not being able to fall asleep or stay asleep.
A common misconception of well-intentioned family and friends is that your fatigue is caused by depression. Yet for those of us who suffer from fatigue, that’s not true. However, it is possible to become depressed as a result of dealing with long bouts fatigue. I find that my fatigue sets in around 3:00 pm and if I can get in my exercise and all of my important tasks done before then, I can make it through the day until bedtime.
Itching of the skin can be severe and unbearable. Some report pins and needles or crawling of the skin. The itching can become so bad and the scratching so intense that the skin bleeds. Some find comfort by applying heat, and others cold. Others turn to antihistamines such as Benadryl. There are lotions that can sometimes help. I have mild bouts of itching that come and go. I find that keeping my skin hydrated with lotion helps. I was also recently diagnosed with psoriasis, but have been able to keep it under control with steroid cream.
It’s important to note that the “stage” of the disease and the onset of symptoms are not related. You can be in Stage I with multiple and severe symptoms or in Stage IV with no symptoms at all.
Is There a Cure or Treatment?
There is no cure for PBC, but it can be treated. Currently, treatments include Ursodexycholic acid (ursodiol) and/or Obeticholic acid (ocaliva). Both medications are used to slow the progression of PBC. The original use for ursodiol was to help dissolve and prevent gallstones. Ursodiol for PBC is prescribed by the weight of the patient. For example, someone weighing 154 pounds would take 4 – 250mg tablets per day. Ocaliva is a fairly new treatment and is given in conjunction with Ursodiol to slow the progression of PBC. After 10 years of Ursodiol treatment I became unresponsive and my liver enzymes elevated. I was put on Ocaliva in addition to Ursodiol and my enzymes have stabilized.