The Mission of the PBC Alliance is to Care for the needs of PBC patients by Advocating for new technology and treatment,
funding Research, and to provide Education for PBC patients, medical professionals and the community.
We will soon post information on upcoming PBC Alliance support group meetings – please visit us again for updates!
April 25, 2012 – Rare Disease Caucus meeting in Washington D.C.
In April, Dr. Bruce Bacon of the Saint Louis University Liver Center, Dr Mark Green of Northwestern University, Dr. Mark Pruzinski, of Intercept Pharmacuticals as well as Deborah Sobel, a PBC Patient presented information to the Rare Disease Caucus concerning PBC, including education about the disease, current treatment, potential for future solutions as well as the challenges of treating patients with PBC.
The Rare Disease Caucus was founded in 2009 and is a bi partisan and bi cameral (congressmen and senators). The focus of the caucus is to provide FDA oversight and design legislative solutions to the problems facing those suffering from rare diseases.
I was diagnosed with PBC in 1999 (stage 1), biopsy in 2005 showed progression to stage 2, third biopsy in 2010 showed stage 3/4 with overlap of AIH. Endoscopy on 1/2013 showed I have portal hypertensive gastropathy with cherry spots in gastric area. My liver labs are stable. Can you tell me what is the prognosis in terms of how fast it will continue to progress, develop complications, or listed for transplant?
“We agree that your situation is very worrisome since you have well documented progression of you liver fibrosis since 1999. It is possible that treating the autoimmune hepatitis component of your liver disease will slow or even stop the progression, but this can vary from person to person. Without such a response, you could start to develop complications of cirrhosis over the next 1-5 years. Unfortunately, we cannot review your MRI images without seeing you in clinic. We hope that you understand” Dr. Brent A. Tetri
I’m hoping someone can help me out. I’m a 22 yr old seemingly healthy male. About a year ago I had abnormal lfts. I was referred to a G.I. and tested for pretty much everything from what he told me. All which were negative test. I’ve also had ultrasounds done. CT and MRI. As well as a hyda scan. All showed nothing. I was then ordered to have a liver biopsy. The nurse called me and said they are puzzled and they want to send my biopsy to a “better” lab and want me to be tested for ANA or AMA. I guess to rule out PBC? I’m just lost and scared through all this. I can’t seem to get a straight answer and they act like they really don’t know what’s going on. I respect their work and like my doctor. Can you help me out? Maybe I’m ok? Thanks for any help you can provide.
“Although we have a lot of good blood tests to identify specific causes of liver disease, sorting out the causes of elevated liver enzymes can be difficult at times. It sounds like you are in one of those situations. It is always a good idea to get a second opinion on the interpretation of a liver biopsy when the answer is not clear. Diseases such as Wilson’s disease can be particularly difficult to diagnose and may require a number of different tests such as blood tests, a liver biopsy, a 24 hour urine collection and even an eye examination by an ophthalmologist.” Dr. Brent A. Tetri
My wife has liver PBC stage 2. diagnosed in 2007. She is 34 yrs old, we have 2 children ages 13 and 5 and I’ve noticed within the past 2 years that she has become withdrawn. She seems lost in her own mind? She seems not to care much about me or our kids. She’s kind of heartless and has no feelings anymore. I don’t know what to do anymore. Is this part of her PBC condition? I help her out around the house I’m good to the kids, and work hard to support her in so many ways. I never get a thank you. She’s still very itchy. She takes her meds. She also no longer wants to be touched. Any information would help me understand. Thanks.
“For most people, living with PBC is as simple as taking ursodiol and having regular checkups. However, for some people living with a disease that can cause fatigue and itching with a risk of progressive liver disease takes an emotional toll leading to depression and other difficulties. She should see her liver specialist and have a frank discussion about these problems. She may benefit from seeking additional help. You certainly seem to be on the right track in trying to be supportive.” Dr. Brent A. Tetri
I was diagnosed with Stage 2 PBC in 2008 from an AMA (52) and a liver biopsy. Started Urso on the day of dx. Weeks later, my slides were sent to Baylor Liver Center. Their labs (neg AMA) along with the review of my biopsy slides led them to believe I have NASH, but they were scratching their heads over the PBC. They said “on paper,” I have PBC, but the damage evidenced in the biopsy indicated NASH. They have flip-flopped numerous times over whether or not I have PBC, even asking me to stop taking URSO, over my own Dr’s objections. I stopped for 3 months. AMA was back up in the 40s. LFT were more elevated, too. I went back on URSO. 3 months later, AMA was negative again. Baylor Drs are stating they have NEVER SEEN ANY PBC patient who (1) also has NASH; and (2) EVER has a negative AMA. EVER. Therefore, they now say they don’t think I have PBC. I stopped the URSO 4 months ago. I am awaiting my labs from last week to see what my AMA and ANA counts are now. ANA has fluctuated between 1:320 with a speckled pattern, to 1:80 over the past 15 years.
My condition has bewildered several doctors for a long time. They felt certain I had Lupus… then possibly a pituitary tumor… then possibly sjogren’s… and a host of other auto-immune glandular diseases. Each time, they chased down those rabbit holes and came back with nothing. (Besides Hashimoto’s – dx ’97, and Diabetes – dx ’00). My gastro, my infectious disease specialist, and my internist are all in agreement that I definitely have PBC and should take URSO for the remainder of my life. They were all distressed over the fact that I stopped it again, just to see what would happen to my count (based on Baylor’s findings). They also have been quite vocal in their opinion that I have been given BLATENTLY misleading information from Baylor. Frankly, they think Baylor is “full of it,” where my diagnosis is concerned. I don’t know what to believe! I trust my personal caregivers completely. I value their opinions. They have repeatedly saved my severely handicapped sister’s life and we have a mutual respect for each other’s medical knowledge. Baylor, on the other hand, is focused on learning “for the greater good” of the general population, and are considered “liver experts.” I want to do what is best for my health, but am troubled by the advice given by valued physicians. Based on what I’ve shared here, if you were me, what would you believe?? And what would you do next?
“This is clearly a complicated situation and you were right to seek the opinion of others. Without seeing you and evaluating your records and biopsy slides, we are not in a position to help sort this out for you. Since there are clearly very different interpretations of what your diagnosis and treatment should be, we can only suggest that you seek yet another expert opinion. The hepatologists at the SLU Liver Center would be happy to see you if you would like.” Dr. Brent A. Tetri
My mother passed away on 8/10/2012 and while in the hospital they said she had PBC or maybe NASH. I test postive for both the ama and ana antibodies but the doctor does not think I have PBC because my alkaline levels are normal – but so were my mothers. I’ve also been tested for syrogns syndrome… neg… I’ve had dry mouth and dry eyes for 2-3 years and also have a lot of itchy skin with nothing there. What do you think I should do?
“Yours is a complex situation and we would recommend that you see a hepatologist (liver specialist) to sort this out.” Dr. Brent A. Tetri
With treatment (Urso) my LFT’s are in the normal range. My doctor thinks my fatigue should be gone, but it has not improved at all. I still have a period of several hours in the afternoon where I absolutely must go to bed, with some improvement in the evening. Is the fatigue of PBC expected to go away when LFT reach normal? This conflict in expectation/actuality has been very stressful to me.
“Fatigue is the most common symptom seen in patients with Primary Biliary Cirrhosis (PBC) and generally does not respond to any medications available. I frequently have patients with normal liver tests while being on Ursodiol who still have significant fatigue. I find no correlation with normalization of abnormal liver tests and improvement in fatigue.”. Dr. Bruce R Bacon
