Imagine going to the doctor with complaints of fatigue and itchiness, only to have a blood test find that you have a chronic disease. Though their diagnoses with primary biliary cholangitis (PBC) — an autoimmune disease that causes chronic inflammation and can lead to irreversible scarring of the liver, with the potential to lead to liver failure — came almost 30 years apart, patient-advocates Kathy and Amber (last names withheld for privacy) share more than their common condition. Both are mothers of three who’ve gracefully adapted to life with PBC by making lifestyle adjustments, staying informed and embracing love and support from those around them.
Good Housekeeping spoke with them about this rare disease and how their friendship, founded on Kathy’s wisdom as a multidecadal PBC veteran and Amber’s zeal for knowledge after her more recent diagnosis, has helped them stay strong through life with it.
What was your life like before you were diagnosed with PBC?
Amber: I was diagnosed four years ago, in between the births of my eldest and my twin daughters. My life was very active. I nannied and worked as a youth minister. Line dancing is a favorite hobby of mine, so I would do that all the time.
Kathy: I also have three children. Prior to diagnosis, I had become a stay-at-home mom to spend more time with my children. My kids are adults now, but at the time my youngest had just turned 1. I got married in 1984 and was diagnosed in 1992.
Amber: The year I was born. (laughs)
Kathy: Yep! (laughs)
Amber: Having someone like Kathy when you’re first diagnosed is important — someone to tell you, “It’s okay, you're gonna get through this.” Because so few people know about this disease. You search online and read frightening sentences about 5-to-10-year life expectancies, but then there’s someone like Kathy, who’s lived with PBC for over 30 years, to tell you that doesn’t have to be your reality.
Had either of you heard of PBC before?
Kathy: Nope. In retrospect, I was extremely fatigued. But I thought that was just parenting.
Amber: Same! I’d never heard of PBC, but I remember feeling so tired after my first son was born. I kept asking people if my fatigue was normal. They’d just brush it off: “Well, yeah. You’re a mom.” But there were other symptoms, too. For example, my doctor asked if I had extreme itching. I looked at my husband: “Do I itch myself a lot?” He goes, “Honey, you’re itching yourself right now.”
Kathy: I’m blessed never to have had the itch. I was diagnosed through a routine blood test. It was shocking. I searched medical books trying to find PBC, but there was rarely any information there.
Amber: Yeah…I can’t imagine being diagnosed without the information that we have today. It’s only in the past 30 years that major advances in PBC research began happening. Realizing how much we’ve learned in such a short time gives me hope for the future. If this much progress happened in just 30 years, imagine where we’ll be in another 10.
How has your life changed since you were diagnosed with PBC?
Kathy: I changed my diet completely. I’m gluten-free and dairy-free now, and I exercise regularly. This helps me control the bloating and swelling that PBC can cause. It’s kind of funny — I’ve become healthier and sicker at the same time. But maybe that’s why I’m still here after 32 years.
Amber: And kicking butt, too!
Kathy: Trying… (laughs)
Amber: I keep a tight schedule to manage my fatigue. For example, Wednesday nights are when I go line dancing, so I’ll make sure not to schedule intensive activities beforehand. My family builds each day’s schedule around one shared activity, so we have energy to be present with each other. It’s all about planning now.
What would you tell someone who was just diagnosed with PBC or another rare disease?
Kathy: Don’t get stuck. You have to keep living. Yes, it’s important to give yourself permission to sit something out when you don’t feel good. But remember that it’s only one day. We’re in this for the long haul.
Amber: Give yourself to others and allow them to love you back. I remember telling one of my friends at the beginning of my journey with PBC that I didn’t want to be a burden. “That’s not your decision to make,” he said. “I get to decide if you’re a burden to me. You don’t get to make that decision for me.” Trusting these words helps me as a mother, spouse and friend. Allow yourself to be loved. Choose to be joyful.
Kathy: Amen.
Primary Biliary Cholangitis Basics
“Primary biliary cholangitis (PBC) is a chronic autoimmune disease that gradually damages and destroys bile ducts in the liver,” says Kris Kowdley, M.D., a hepatologist based in Seattle. “Chronic damage causes the organ to become scarred and unable to rid the body of toxins and may ultimately lead to liver failure or liver cancer if untreated.”
The most common symptoms are debilitating itching and fatigue, along with pain in the joints and upper-right abdomen. “As these symptoms are not unique to PBC, there can often be a delay in identifying them as being related to the disease,” Dr. Kowdley says. “It’s important to discuss obtaining liver-function tests with your healthcare provider if you’re experiencing these symptoms.” While most cases occur in women between 40 and 60 years of age, the disease can develop in patients as young as 18 years of age and of any ethnicity, race or sex.
This is the unique experience of Kathy and Amber and not representative of all people living with PBC. This is not medical advice. For questions about your health or the health of a loved one, please talk to your doctor.
©2024 Ipsen Biopharmaceuticals, Inc. All rights reserved. 1/2024 NON-US-003673