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- เผยแพร่เมื่อ 16 ต.ค. 2024
- Gail Cawkwell, MD, PhD, Senior Vice President, Medical Affairs at Intercept, provides an overview of primary biliary cholangitis (PBC), a rare liver disease that is caused by an autoimmune reaction. Primary biliary cholangitis affects the bile ducts within the liver. The bile ducts carry a fluid called bile from the liver to the gallbladder, where it is stored. The bile ducts become inflamed and damaged, which causes bile to build up in the liver. This abnormal buildup destroys liver tissue and in later stages results in cirrhosis. Primary biliary cholangitis usually occurs between the ages of 40 and 60 and affects women more often than men. The cause of this condition is unknown, but research suggests that it is an autoimmune disease. Ursodiol (Actigall) and obeticholic acid (Ocaliva) are the only drugs approved by the U.S. Food and Drug Administration for the treatment of primary biliary cholangitis.
It is estimated that 90% of people who are diagnosed with PBC are women. Many people who are diagnosed with PBC are between 40-60 years of age. It is important to note, however, that men and young women can also develop PBC.
The most common symptoms of PBC are pruritus, or itching, and fatigue. Some people with PBC can also experience dry eyes and mouth. For people with more advanced PBC, serious liver damage can cause jaundice, or a yellowing of the skin and eyes.
Many people do not experience any symptoms at the beginning of their PBC. The only sign that they have PBC is that abnormalities appear in their liver function test results.
Doctors can test for PBC by taking blood samples and measuring certain chemicals related to liver function. The tests will show if there are elevated levels of alkaline phosphatase (ALP) and if antimitochondrial antibodies (AMAs) are present, which can be indicators of PBC.
In watching this video, I am struck with how “non-typical” my mother’s progression of PBC was.
My Mom’s lab tests were always normal, but she had significant swelling of her extremities, pockets of fat under her eyes. We didn’t know she had a liver problem until she ended up in a coma due to high ammonia level. She would go into the hospital with ammonia toxicity whenever she had a bladder infection, etc., and biopsies if her liver “indicated PBC”, but her labs continued to be in the normal range. We even took her to see a transplant team, but they only looked at her labs, said “everything looks good” and sent us on our way. Six months later, her labs FINALLY showed abnormalities, and eight days later she died.
How can a person’s liver continue to put out normal blood tests for YEARS, and suddenly fail?
I’m still trying to understand...
I’m soo sorry to hear that.,,
Same with me ,my gastrointestinal dr says if all this bloodwork is normal why did your primary run this test,referring to ama2 which showed positive ,I told him,still skeptical so I refer to my CT and he still skeptical so he says biopsy, I say no Fibroscan should be done first.The system is something screwy for sure,my family is as confused as you.I had my gallbladder removed so then they started taking me serious. Sorry for your loss wish they would've got it sooner, I dont think they will be able to save me either ,months in between appointments and tests due to this whole pandemic and broken health care system
40 year old man ,I was only diagnosed after getting my gallbladder removed,AMA2 test confirmed doctors suspicion, ultrasound confirmed how bad it is progressed. This is a very brutal disease and I really don't like how there is so little known .Good luck all of you that are diagnosed, hopefully they catch you sooner then me.
I had my gallbladder removed too! But I was 12 and diagnosed at 25.
@@EbonynIvory83 wow that long is insane, glad they got it figured out
How bad was it when it was diagnosed? I was hospitalized with biliary colic for a week last year and required emergency gallbladder surgery. They did tests for autoimmune hepatitis but not PBC
@@AnnaBreit the pain got a lot worse after gallbladder was removed.After my primary ran the blood test came back positive.So I had to have a Fibroscan done,and then a liver biopsy.The biopsy showed a more advanced and permanent liver disease N.A.S.H.,but no PBC. My blood tests still shows PBC,Dr says it's possible were they took samples hadn't been affected yet.So every 6 months I have all my levels tested,and unfortunately my liver is dying and I'm not candidate for transplant since it's autoimmune.Despite what "Specialists" say it is painful,the Liver has No pain receptors,BUT the capsule it sits in does ,so when the liver swells it pushes against it.I now eat once a day,drink more water then a camel,and no longer work.43 now and I've been told if I keep up what I'm doing I might get 5-10 years before cirrhosis.It wasn't easy and took a lot of appointments and testing.Be your own advocate and do your research,if your Dr won't run tests find another,if I hadn't I probably would be dead by 50.
@@AnnaBreit Pretty bad ,it was already in the N.A.S.H state,PBC still shows on Blood work but Liver Biopsy did not.My gallstone was the size of a toothpick ,they said my gallbladder took all it could and failed
I’m only 24 and have 2 tumors on my liver, at the hospital they told me they think it’s just Focal Nodular Hyperplasia so they just sent me home and told me to follow up with my primary doctor. Well, I did, my enzymes are staying elevated so I was referred to a GI, he tested me for SMA and AMA and both came back positive and I have an ultrasound & MRI in 2 weeks so I guess that’s not a good sign.
I am being tested for this
Good luck praying for ya its negative.
@@Troy-McClure81 it was positive 🥺 I am stage one though and the meds really seem to be helping! Thank you so much for the prayers!!🥰🥰🥰
@@welderprincess133 glad they caught you early,im transitioning from 3rd to 4th stage,hopefully u get many years to come.
@@Troy-McClure81 praying for you!! I have seen so many people in my support group say that they lived for many years at stage 3/4. Are you on Urso?
@@welderprincess133 im on nothing,its months in between appointments, sadly I live in one of the worse places in America for health care,Las Vegas lol
I got it at 25. 😭
I got it at 22 :/
@@AntiAChAse OMG ! I have never met anyone else who has got it young. How have you been coping with it ?
@@EbonynIvory83 Not well, tbh. I just found out after getting some blood tests for debilitating fatigue--what I presumed to be hypothyroidism. But my thyroid was fine and instead my ALP was elevated to 201 and a subsequent test showed I was positive for AMA. So any advice on how to deal with this would be great because I'm feeling pretty scared about this diagnosis.
@@AntiAChAse ya that happened me too. I started on the Ursodiol and have taken it for over a decade now. It lowered my enzymes drastically, it doesn't give any symptoms either and helped with the fatigue. I also try to eat foods that help the body with inflammation. And exercise.
@@EbonynIvory83 I'm glad you're doing better! How long did it take for Urso to help your fatigue?