Newly diagnosed with PBC

[Pawsandchoc]

Hi there!

I just got my official diagnosis today and was started on Ursodiol. I have been feeling fatigued for about 13 years and no one could find a reason. In the last 3 years, my liver enzymes went up and I started getting even weirder symptoms. Neurological impairments, heart pain, muscle pain. All sorts.

I saw a note on my most recent labs, under a negative ANA test, that said "possible anti-mitochondrial antibodies". And mentioned it to my PCP. He said yeah we can test for that. So indirectly I found out I had this. Doctors had attributed my high liver enzymes to my gallstones and did not think of this.

Thankfully I am at F0-1. So hopefully no real damage yet. I do have diarrhea after taking the first dose of the Urso! But I hope that will get better.

I've been very uncomfortable, so I'm thankful to have a reason, and hopefully, things will improve now.

[Flid]

Welcome! This is a great place to chat when you are new. I am almost a month in and very similar. I also had diarrhoea once or twice in the first week but have settled in now and don’t notice any effects to my daily life from the Urso. I have noticed I have more energy - some of the exhaustion has eased and I think the brain fog is also lessening but it’s early days and that could be in my head!

Did you join the PBC foundation? They have all the up to date info and can provide accurate information and support.

[Pawsandchoc in reply to Flid]

Thank you! Yeah I'm glad I found this site! It's nice to know a bit of what to expect. It was a bit scary not knowing how bad my liver was/is. But a relief that I caught it before a lot of damage was done. And grateful for the Urso even with the diarrhea! lol But good to hear it should be temporary.

I just joined the PBC Foundation. Thank you for letting me know about that!

And glad to hear your energy and brain fog is getting better. That's awesome. My other neuro symptoms have gotten better with B & D vitamins, but my body and brain are still tired.

[OdinsMom]

I wish you the best of luck and hope you feel better. That being said keep in mind Ursodiol doesnt' treat symptoms but slows the disease process.

[Pawsandchoc in reply to OdinsMom]

Thank you!

[Lu11]

I’m glad you got diagnosed and now you’re taking Urso. I wish doctors checked AMA sooner. I researched and asked for an AMA test and that’s how I found out I’m positive. Try to exercise even if it’s walking and eat a clean diet. That always helps!😊💓

[Pawsandchoc in reply to Lu11]

Yes I wish they did too! They couldn't see past my gallbladder being removed. I will start working on the exercise. I have avoided regular exercise due to it bringing on fatigue. But maybe I can get over that hump. I've been working on my diet as well.

[MamaOnTheRun in reply to Pawsandchoc]

I tried the Whole30. I found Gluten, soy and refined sugar caused a lot of inflammatory issues for me. I eliminated them from my diet and a lot of brain fog went away. Inflammation in joints (I also have RA) also diminished drastically.

[swinstan]

For info about the longer term, I was found to have AMA around 2003 and back then URSO was beginning to be prescribed but research results were still uncertain so I declined it. I had no symptoms at all. Just the antibodies. A couple of years later, it was shown that URSO did work for some people but not everyone. Some were responders - others not. As already stated here, URSO doesn't really impact upon symptoms but does limit or maybe even improve blood results. At this stage, I started taking URSO with no side effects, which has kept my test results all within normal bounds. I also remained asymptomatic for many years. Latterly, I do suffer a bit with fatigue and memory problems but that might just be because I'm 70 soon and not the PBC. I think what I want to say is that for many of us, PBC shows just as an anomaly in a blood test and we're otherwise unaffected - and it can stay that way for many years. As many PBCers say, you are more likely to die with PBC than of PBC, so please take heart.

[Pawsandchoc in reply to swinstan]

Thank you! I've definitely had fatigue & brain fog for years. I guess there's no real treatment for that part...yet.... But I'm glad to stop any progression. Hopefully! I did better with the Urso today. Not as sick feeling.

[Michi1 in reply to Pawsandchoc]

I’m a health coach (because of PBC) and have reversed my fibroscan from Stage 2 to Stage 0. I’d be happy to get on a Zoom call with you and share the basics. DM me if you want to schedule a call.

[Lilydoll10 in reply to Michi1]

When you say you’ve reversed your fibroscan how did you do that? I have been told I will have to wait 49 weeks to get my first one on NHS and have been anxious about what state my liver is in and what I can do to help it. My bloods are back in the normal/ acceptable range after 3 months on Urso and though I am trying to be healthy and have stopped drinking I wonder what else I can do?

[AMAgirl in reply to swinstan]

Can I ask please, how long after testing positive for AMA did you get PBC? Also, how long have you lived symptom free with PBC? I am new here and absolutely terrified! You post has helped. Thank you. ps Do you know, if you only have AMA from NAFLD, when your liver goes back to normal from diet and exercise, why don't the AMAs go?

[swinstan in reply to AMAgirl]

Hi AMA girl. Happy New Year.

Just to give you a timeline:

Diagnosed in 2003 after a range of blood tests for something unrelated showed AMAs to be present. I think just having the AMAs is enough for a diagnosis in the UK. In the US, I think they do more invasive tests like biopsies.

A few more tests and scans showed no liver damage so far and I had no symptoms - so at this stage I was diagnosed with PBC but there were no adverse effects in any way. I was offered Urso but the actual published research at this time showed very mixed results so I personally wasn't convinced I needed daily medication for the rest of my life. With the agreement of my consultant I chose not to take it at this time.

I had annual liver function tests thereafter - that's how slowly it tends to progress. It wasn't until 2014, over 10 years later, that the tests showed a deterioration in liver function. ALP and GGT were very much raised; ALT was a little over the threshold. The mixed results for Urso research were clearer now. Some people responded to it and others not at all, so for some people it was the first real option to help minimise liver damage. That said, it doesn't do much to reduce any overt symptoms. I started on Urso and it worked for me. LFTs returned to normal although it did take over 18 months.

Since then (Nov 2016), back on annual blood tests, with good results. Also, still no symptoms. Any tiredness or forgetting is more likely my age

So that's over 20 years since my diagnosis and apart from taking Urso there has been no impact whatsoever - so there is every reason to be hopeful and remember that many of the people posting here are the unfortunate ones who are either further along than we are or haven't responded to the meds. Us luckier ones tend not to post the positive outcomes as much as we perhaps should.

And sorry, I don't know anything about NFALD.

I hope you are still feeling okay despite the AMAs and that you stay that way.

[MamaOnTheRun]

pbc-society.ca/ I would also check here! They have great conferences with lots of great info. And there may be a local support group for you. I am in Chicago, Illinois and we have the Illinois PBCers.

[Pawsandchoc in reply to MamaOnTheRun]

Thank you! I will! I live in Lincoln, Nebraska, so I may be one of a very few here. lol. Pretty small town.

[MamaOnTheRun]

I couldn’t tolerate ursodial at first. I was told to try another manufacturer. There are like 8. I tried 4 and found one that didn’t cause the nausea and diarrhea for me. Now I take smaller doses multiple times a day and am fine.

[Pawsandchoc in reply to MamaOnTheRun]

I seem to be getting used to it thankfully!

[Mimisboysx3]

I have taken Urso for 2+ years. It has brought my liver enzymes down very little. I'm now taking 600 mg daily and will be having labs in October. I cannot tolerate a higher dose of Urso. Diarrhea and abdominal pain is very common for me.

[Pawsandchoc in reply to Mimisboysx3]

I hope your enzymes are better in October! I'm on 1200 a day to start. I seem to be doing better on it thankfully.

[Michi1 in reply to Mimisboysx3]

Urso is prescribed by weight and unless you are REALLY tiny 600 mg would not be enough for an adult. Maybe there are tricks to tolerating a higher dose? Have you tried hard core nutritional intervention with the Urso?

[Blueberries1970 in reply to Michi1]

Hello.. my heptologist believes that I have PBC with a neg michondrial antibody test. My ALP has been upper high normal to the highest of 154 dating back to 2017. My internist believed I had fatty liver and urged me to lose weight. I insisted on an ultrasound which found very mild fatty liver. He gave me the option to see a hepologist. That hepologist ordered additional lab tests including GGT (which was one point over normal) a liver panel (ALP was 138) and an antibody test which was negative. I had a fibroscan which was totally normal and no fatty liver. The dr says at this point he believes without doing a biopsy that I have PBC with neg AB. He said I could start taking URSO but it was my choice since my numbers are on the upper limit normal lower high side. I asked what he would do if he were me and he said wait 6 months redo labs and ultrasound and go from there. He was willing to do a biopsy but didn’t not at this point recommend it. I’m just torn.. should I jump on the meds at this point? Thank you to anyone for your input. By the way my husband and I are both very scared at this point as this was not on our radar. I was just hoping by losing 25 pounds that I had reversed my fatty liver.

[Michi1 in reply to Blueberries1970]

I replied to you, but the reply did not go under your post here. Look for it separately. It should be below.

[Michi1]

OK. First, in most of us with PBC, our alkaline phosphatase is not slightly elevated without medication. For diagnosis it is typically two times or more the upper limit. Also, ALT and AST are also elevated. There is a particular combination of elevations in the AST, ALT and ALP that forms one of three points they look at in the diagnosis. The second is the test for anti-mitochondrial antibodies. As I recall from our patient symposium here in Miami, the test for GGT is not specific to PBC and could diagnose other types of auto immune diseases as well. Yours is negative. The third can be a biopsy. A biopsy is no longer the standard of care in most places, and is generally only used in certain limited circumstances. A lot of places now use a fibroscan, which can measure the rigidity of your liver tissue and determine whether you have fibrosis or cirrhosis. From what you said, your fibroscan came out fine. They do not diagnose PBC without two out of the three.

Based on what you have said here it does not sound like you have PBC, but I am not a doctor.

if I were you, I would follow my doctor‘s recommendation to wait and see. Why would you want to “ have a disease“ diagnosed or start taking a drug you will need to take for the rest of your life if the conditions are not there?

PBC is very rare, and there are not a lot of hepatologists with experience treating it. You might want to find someone who has a focus on autoimmune liver diseases. If you are experiencing symptoms, it is possible you have something else that would be treated differently. If that is the case, you really need to continue to pursue the situation and get down to the actual problem so you get the treatment you need.

[Michi1 in reply to Michi1]

this was intended to respond to the post above by Blueberries1970

[Blueberries1970 in reply to Michi1]

Thank you so much for your quick response. My husband and I are in a tailspin. The liver Doctor that I saw is based out of a San Francisco Liver Transplant Center and hospital and he specializes in the liver and autoimmune diseases. I do need to say that I have had hashimotos for 25 years. I have no symptoms of that either but have been monitored for years by an endocrinologist who says I do not require treatment because no symptoms and the lab reads are not that off. I get them tested every 6 months. Based on the history of hashimotos, the ALP that’s never been over 158 on any lab, and I’ve had lots, over 7 years, the ggt which was only tested 1 time 2 months ago it was 61, the liver dr is saying that I most likely have PBC. I am going back in 6 months as he suggested because he says I am in the very early stage of the disease. I think in 6 months I will just come to terms and get a biopsy to put this thing to bed! I was just so taken aback when he said PBC because I tested negative for the antibodies two months ago. He says that 5 to 10 percent of the cases are ab neg. All my other liver labs are completely normal. Any info helps!

[Michi1 in reply to Blueberries1970]

Have you considered a second opinion?

It sounds like he's just suggesting monitoring, which makes sense, but it is possible it could be something not related to your liver at all.

I'm a big believer in not looking for problems where they don't exist, but that's a personal decision.

When you lost 25 pounds, how did you lose the weight? Could the #s be related to your diet or lifestyle in some way? Have you seen a nutritionist or a naturopath or functional medicine doc who could look at things in a less organ-focused way?

So many of these things can be addressed with overall lifestyle changes.

[Blueberries1970 in reply to Michi1]

The liver dr says that the liver test numbers for Alp and ggt suggest a bile flow problem Cholasis I think that the spelling. There is a bile duct flow issue albeit not bad but not normal either. It has caused zero liver damage at this point. From my understanding ALP is caused by inflammation in the intestines, bones and liver. I had a test to identify the various places the inflammation and the test pointed to liver. The liver dr ordered a ggt test which was one number above normal and he said that helps to isolate that the inflammation is coming from the liver. I am considering a second opinion or just a biopsy as I don’t need the mental stress this causes. I lost weight in a good way because the internist believed that I had fatty liver based on my bmi which was 30 but now is 26. He said to eat a Mediterranean diet, eat smaller portions, cut out sugars, and exercise more. I did all that and so far have lost 25 lbs in 7 months. He also said to lose gradually as losing it fast is hard on the liver and some people actually gets bad liver lab results. Plus, it’s a lifestyle change and I want it to be permanent. Thank you for all your info!

[Michi1 in reply to Blueberries1970]

I don't know if this helps at all but I had a hospital visit before they diagnosed me because of pain which the ER doc thought might be gallstones. Apparently the same liver enzyme profile you see in PBC can show up in gallstones, but they couldn't see anything in an ultrasound of my gallbladder. The ER doc told me sometimes you can see the formation of early gallstones with labs before you can see it on the ultrasound. I changed my diet and the pain went away and never came back. I lost 35 pounds then thinking if it's gallstones I could deal with that with diet, and then a year later I went to the doctor for a UTI and he ran "checkup" labs and found my ALP was double what it was a year before.

"Cholestatic" liver diseases are the ones that involve reduced flow of bile. PBC and PSC.

[Blueberries1970 in reply to Michi1]

Well it’s something to consider. I have no pain. I have had two ultrasounds that included the gallbladder. Both (6 months apart) showed a very small polyp that the dr is not concerned about but no gallstones. But as you said they may not show up on the ultrasound. The first ultrasound was for the ALP labs to rule out fatty liver and they found the gallbladder polyp during the ultrasound. The second ultrasound was to recheck the size of the polyp which has stayed the same size.

[ninjagirlwebb in reply to Blueberries1970]

FYI. It wouldn’t hurt to get a 2nd opinion. Just bring all your labs etc to your appointment & have another liver specialist confirm the pbc.

The diagnosis of PBC is based on a comprehensive assessment of clinical features, laboratory tests, imaging examinations, and histological findings. The diagnosis of PBC is based on the presence of two of the three criteria: (1) biochemical evidence of cholestasis (ALP and GGT elevation), and exclusion of extrahepatic cholestasis by imaging examinations; (2) presence of AMA/AMA-M2 or other PBC-specific autoantibodies (such as anti-spl00 or anti-gp210); and (3) histologic evidence of non-suppurative destructive cholangitis and destruction of the interlobular bile ducts.

There is no need for a biopsy these days for diagnosis unless it can’t be assessed using the above.

[Blueberries1970 in reply to ninjagirlwebb]

Thank you for your input as well as others! The only thing that checks your info that you listed is the slightly elevated ggt and slightly elevated ALP .. zero evidence of liver damage and no symptoms. The liver dr seems to think it’s very early diagnosis. I am going to ask for a second opinion and more testing as the liver dr said a Ct scan or mri wouldn’t help with diagnosis. I just have to wonder what else could it be.. why the slow bile duct flow as indicated with the ALP tests for 7 yrs and the raised GGT

[ninjagirlwebb in reply to Blueberries1970]

It will take a while to see exactly what it is. If pbc, don’t worry too much because it was caught really early. As long as urso normalizes your alk phos, your lifespan is similar to the general population. This is what my hepatologist told me. You will be just fine. Just make sure you do whatever your doctor tells you.

What is a gamma-glutamyl transferase (GGT) test?

Here’s some ggt info:

A gamma-glutamyl transferase (GGT) test measures the amount of GGT in your blood GGT is an enzyme. Enzymes are proteins that speed up certain chemical reactions in your body. Thousands of enzymes play an important role in all of your body functions. The GGT enzyme is found throughout your body, but it is mainly found in your liver.

If your liver or bile duct is damaged, GGT may leak into your bloodstream. So high levels of GGT in your blood may be a sign of liver disease or damage to the bile ducts. Bile ducts are tubes that carry bile in and out of the liver. Bile is a fluid made by the liver that is important for digestion. A GGT test can't diagnose the specific cause of liver disease. The test can only indicate that your liver is being damaged. This test is usually done with or after other liver function tests, most often an alkaline phosphatase (ALP) test. ALP is another type of liver enzyme. While both your GGT and ALP may be elevated in diseases that affect your bile ducts and liver, only ALP will be elevated in bone disease. This helps your health care provider to understand if your issue may be due to a bone disorder instead of your liver.

[ninjagirlwebb in reply to Blueberries1970]

Did the doctor test for the below? It is one of those things that can help diagnose.

(2) presence of AMA/AMA-M2 or other PBC-specific autoantibodies (such as anti-spl00 or anti-gp210)

Yes I know a lot about pbc since I have it and my doctor discusses it with me.

[Blueberries1970 in reply to ninjagirlwebb]

I believe he tested for ama.. he suspects ama negative Pbc thank you for your quick response.. my husband can’t believe how helpful this group is!

[Blueberries1970 in reply to Blueberries1970]

I believe the test was michondrial AB test and it is negative

[ninjagirlwebb in reply to Blueberries1970]

We all went through what you did and understand how nerve wrecking this is. Feel free to reach out anytime. We need to support each other!

[Blueberries1970 in reply to ninjagirlwebb]

Thank you so much! This is so hard to navigate. I’ve been trying to figure out for over a year now what is going on with my ALP level. I’m going to my internist tomorrow so hopefully he can explain some of this to me or possibly order more tests himself or another referral.

[ninjagirlwebb in reply to Blueberries1970]

Here is another test that my hepatologist did to delve into the alk phos:

Alkaline phosphatase (ALP) is an enzyme found in many body tissues such as liver, bile ducts, bone, and intestine. There are several different structural forms of ALP called isoenzymes. The structure of the enzyme depends on where in the body it is produced. This test is most often used to test ALP made in the tissues of the liver and bones.

The ALP isoenzyme test is a lab test that measures the amounts of different types of ALP in the blood.

[ninjagirlwebb in reply to Blueberries1970]

My hepatologist said if ama negative, then test for the below to confirm pbc:

other PBC-specific autoantibodies (such as anti-spl00 or anti-gp210)

[Blueberries1970 in reply to ninjagirlwebb]

Thank you I took a screenshot of the tests your dr recommended. These are the antibody tests ordered. Mitochondrial AB (m2) it was negative. Immunoglobulin M, QN, serum Normal range 26-217 Tested at 52 normal

[ninjagirlwebb in reply to Blueberries1970]

Good luck. Just use your scientific mind, and ask your doctor questions until you feel you are educated about what is going on.

[Blueberries1970 in reply to ninjagirlwebb]

Thanks again for all your info, support and reassuring words. I know either way I’m going to be ok now!

[ChoiceM]

Welcome. A very similar thing happened to me. The fatigue is rough sometimes. For 15 years I had elevated enzymes and fatigue. In Idaho they did an ultrasound of my liver but didn't find any blockage so they said they would watch my enzymes. When they remained elevated I was told "some people just have a higher normal" and not to be concerned. So I continued my status quo and let it go. Fast forward 10 years in Washington state, I was getting sores all over my chest and forearms that itched like crazy. One doctor said I was allergic to the sun, even though I tanned at a salon and no breakout anywhere else. One doctor said flea bites which we didn't have in our house. Finally they did a biopsy of one of the sores and that lead me down the path of autoimmune tests, which lead me down more tests and more tests. By the time I was officially diagnosed with pbc, I had interstitial lung disease and a small coronary blockage from the cholesterol, rare side effects of undiagnosed pbc. I was last tested at F1-2. I've made big dietary changes to reduce my cholesterol, stopped alcohol altogether, and now have normal enzymes. Still fatigued, unfortunately, and the sores are gone. Still itchy sometimes but not like before. Urso makes me nauseous in the am if I don't eat first, which was new to me but I've gotten used to it. It's expensive (urso) but cheaper than a liver transplant.

I was angry at first at all the misdiagnoses that could have saved me the damage that has happened and the other consequences of doctors that just aren't familiar with this rare autoimmune condition. I am thankful for Urso even with the hair loss and weight gain. Good on you for advocating for your own health!!! I feel like we are educating the medical industry. I am glad you found out earlier than I did because of your courage and hope the very best for you. Keep us posted.