Primary billiary cirrhosis :(

[andre79]

So, i received today the latest test results, and turns out that i have a positive AMA (Antimitochondria Antibodies) The ASMA and LKM are negative, so that rules out autoinmune hepatitis, but the positive AMA is indicative, and very specific of Primary Billiary Cirrhosis.

So, i am screwed for several reasons:

1) This illness has no cure, and there is only one treatment aproved is acid Ursodesoxicólico which might slow down the progression but has not effect on the symptoms. The other cure would be a liver trasplant. Sigh.

2) I have tons of symptoms that cannot be explained by that illness. Maybe the gastrointestinal symptoms, the high cholesterol, the fatigue could be related to it, but what about the swollen joints, the night sweats, the dilated pupils, the stiff neck, the chest pain, the pain over my body? And specially the very virus like onset that i had?

3) in the case i should find an infectious pathogen that is causing my symptoms, could i tolerate the harsh and long term antibiotics treatment with my liver in so bad condition?

Until today i had the hope that i would find an infectious reason for my illness, and a treatment for it. Now i feel hopeless.

Sorry, i don't want to sound negative but now i don't know what to do.

 

[Jonathan Edwards]

So, i received today the latest test results, and turns out that i have a positive AMA (Antimitochondria Antibodies) The ASMA and LKM are negative, so that rules out autoinmune hepatitis, but the positive AMA is indicative, and very specific of Primary Billiary Cirrhosis.

So, i am screwed for several reasons:

1) This illness has no cure, and there is only one treatment aproved is acid Ursodesoxicólico which might slow down the progression but has not effect on the symptoms. The other cure would be a liver trasplant. Sigh.

2) I have tons of symptoms that cannot be explained by that illness. Maybe the gastrointestinal symptoms, the high cholesterol, the fatigue could be related to it, but what about the swollen joints, the night sweats, the dilated pupils, the stiff neck, the chest pain, the pain over my body? And specially the very virus like onset that i had?

3) in the case i should find an infectious pathogen that is causing my symptoms, could i tolerate the harsh and long term antibiotics treatment with my liver in so bad condition?

Until today i had the hope that i would find an infectious reason for my illness, and a treatment for it. Now i feel hopeless.

Sorry, i don't want to sound negative but now i don't know what to do.

My memory for the specificity and sensitivity of these antibodies is not that good but I do not think an AMA on its own is diagnostic of PBC. Most of these antibodies turn up from time to time in people with no relevant symptoms. Do you have itching? Itching is usual early on in PBC. Also do you have any evidence of obstructive liver changes like raised alkaline phosphatase? If not I would begin to wonder if this is a red herring.

PBC is not a good condition to have for sure but we now know it is an autoimmune disease with a known antigen and although benefit from rituximab is not yet established there are trials in progress and it is quite likely to at least halt disease. Moreover, in the not too distant future there is a reasonable chance of a more definitive treatment.

So I would not be too pessimistic on the PBC front as yet.

You mentioned swollen joints, which does not sound like PBC or CFS/ME (or infection).

 

[Jonathan Edwards]

I see from another thread that you do have abnormal liver tests but AST and ALT point to hepatitis rather than established cirrhosis. Hopefully your physician can give you a clear answer and maybe advise something more than just symptomatic therapy.

 

[andre79]

Thank you for your reply Dr. Edwards, and thank you specially for giving your expert help in the forum. It is very much appreciated.

I do have itching, even though in the onset of my illness was way worse than now. I had related the itching with food allergies or histaminosis but now with this results i guess is part of the PBC.

About my liver enzymes they were a little bit high, but got normal in the tests i did last month.I doubled the milk thistle dosis, maybe that's why.

Two months ago:

ALT 68 (0-41)
AST 38 (0-40)
Gamma Glutamil Transpeptidasa 113 ( 5-36)

One month ago (after raising the milk thistle dosis)

ALT 32 (0-41)
AST 26 (0-40)
GGT 41 (5-36)

My billirubin is always normal, so i guess that's a good sign, isn't it? At least of slow progression.

I do have other symptoms of PBC like osteopenia (i am 36 years old woman, too young to have osteopenia), and i wake up every morning with a hangover like i had drank alcohol. I was never a heavy drinker, only in social ocassions and i don't drink alcohol since onset of the illness.

About the swollen joints, they are not huge swollen, just a little red inside my knees, when i press them together, and sometimes it hurts a little when i walk or take the stairs.

I have an appointment next thursday with my doctor, i sure hope she can give me options. At the moment i am thinking about starting the therapy with acid ursodesixilcolico and see what happens.

I edit to add that i did Hep B and Hep C tests are they were negative.

 

[ahmo]

@andre79 Sorry to hear of your results. Just 2 suggestions you might want to follow up. 1) coffee enemas for cleansing liver; 2) low dose naltrexone for autoimmune issues. there's a long low dose naltrexone thread on pr, and I'm linking some info. Best to you

http://www.sawilsons.com/library.htm Many sources of info re CE

http://www.mygutsy.com/the-ultimate-liver-detox-coffee-enemas/
[url]http://www.lowdosenaltrexone.org/index.htm

https://sites.google.com/site/dudleyslowdosenaltrexonesites/[/URL]

 

[Jonathan Edwards]

That does sound reasonably consistent with early PBC, although your doctor will be in a much better position than me to make a diagnosis.

I have not been following the use of rituximab in PBC but the few trials that have been done look to me to be as positive as one could reasonably expect. E.g.

Am J Gastroenterol. 2013 Jun;108(6):933-41. doi: 10.1038/ajg.2013.51. Epub 2013 May 7.
B-cell depletion with rituximab in patients with primary biliary cirrhosis refractory to ursodeoxycholic acid.
Myers RP1, Swain MG, Lee SS, Shaheen AA, Burak KW.

I think what one would hope for with rituximab is some modest reduction in enzyme abnormalities but chiefly a halting of any deterioration. It looks as if there may be some symptomatic benefit as well. What worries me a bit is it looks as if researchers have just studied single courses of rituximab and to make any sense I think one would need to make a decision to use it regularly for maybe 2-5 years. It would seem sensible to think about using it at an early stage when there is no established cirrhosis. The problem for routine care would of course be getting approval for funding a treatment of this sort but maybe some longer term trials are being planned. Just a thought.

 

[andre79]

After spending the whole morning in bed crying over my diagnosis i pulled myself together and started my research. I won't give up that easily. I usually do my research before going to the doctor, because i am not willing to take the psychosomatic nonsense again from them and i need them to take me seriously.

Anyway, i came up with a plan to discuss with the doctor next thursday:

900 mg of Ursodeoxycholic acid, starting with 300 mg once a day, and increasing up to 900 mg in two weeks. That's the primary treatment.

Also, i have been reading about hepatic encephalopaty which has some symptoms in common with the episode that i call the onset. It is produced by acummulation of ammonia in the brain, because of the lack of billis that degrades it.

The treatment for that is to produce three or four bowel movements a day, with lactulose, which trappes the ammonia and expel it. Also, i will combine with rifaximin to kill the bacteria that produces ammonia. Of course, i would take a strong probiotic with that.

Lower the protein in the diet seems to be recommended.

And finally i found this very interesting study that tries to relate manganese level in blood with fatigue in PBC.

http://www.ncbi.nlm.nih.gov/pubmed/15016756

It would make sense to take an manganese antagonist like zinc (i already take 50 mg daily, but raise it to 100 mg) to see if it helps with the fatigue.

Also, i have been reading about PBC and its relation with neurotransmitters and HPA axis dysfunction. Very interesting but i am overload with information right now and i need to settle down to process it.

 

[andre79]

This is fatigue related in PBC:

"The etiology of fatigue is unclear; although some evidence suggests that abnormalities of the hypothalamic-pituitary-adrenal axis, decreased release of serotonin, and increased production of proinflammatory cytokines (ie, interleukin-1 [IL-1], interleukin-6 [IL-6], tumor necrosis factor-α [TNF-α] ) may be responsible.”

So, what would be the treatment for that?

 

[Jonathan Edwards]

The logical thing is to get rid of the antibodies causing the liver, and perhaps other tissue, injury. Rituximab will reduce the antibodies but it doesn't look as if information is available about long term benefit. Dr Julia Newton is currently studying the effects of rituximab on fatigue in PBC in Newcastle, UK.

 

[andre79]

So, i went to the internist doctor and showed her the tests. She said that my symptoms were consistent with PBC but i have to repeat the tests in a more specialized laboratory and the only way to get a final diagnosis is through a liver biopsy. She said that in case it's positive, i would be in a very early stage and with much possibility of slow down the progression. She has seen patients with this disease and said i was nothing like them, neither my tests results.

Also, she was very enphatic and firm when told me that I did not have hepatic encephalopaty. She said to forget about it, no no no and no. Ok then.

Then i mentioned her the possibility of Rituximab, and she said that rituximab has shown good results in some autoinmune diseases but it was not "holy water" because it has many adverse efffect (as any meds, by the way) And then told me that it was pointless discussing treatments without a diagnosis.

Finally she refered me to the best hepatologist in the country, and now i have to ask for an appointment with him and see what happens from now on.

Today i am seeing things in a very differente way. On the good side, at least i could have a diagnosis that is adknowlegde by doctors, and treatments options. If the treatments fail In the worst case escenary, that i should need a liver transplant (unlikely but possible) that's one of the easiest transplant with very high rates of survival. The chance of relapse with a new liver is 30% at ten years, but in that case the process starts over and i could have a normal life expectancy.

One anecdotic experience: the nurse that was taking my blood asked why i was doing those tests, and i said to see if i have PBC. And she said: oh i have that, antimitochondrial antibodies positive and i am taking urso acid. She said it like it was not big deal. I wonder how "rare" this disease really is.

 

[Jonathan Edwards]

So, i went to the internist doctor and showed her the tests. She said that my symptoms were consistent with PBC but i have to repeat the tests in a more specialized laboratory and the only way to get a final diagnosis is through a liver biopsy. She said that in case it's positive, i would be in a very early stage and with much possibility of slow down the progression. She has seen patients with this disease and said i was nothing like them, neither my tests results.

Also, she was very enphatic and firm when told me that I did not have hepatic encephalopaty. She said to forget about it, no no no and no. Ok then.

That sounds like good advice to me. (And no you cannot have encephalopathy at this point!) If you see the top liver person then you should get access to the range of current options and very likely trial options. PBC is uncommon but not really 'rare'. It is uncommon enough that if you have it you are quite likely to be considered for new trial options if you see someone with a special interest.

It does sound as if you might be what one could call 'diagnosable but clinically borderline', which is not uncommon for autoimmune disease. Moreover, people who are borderline for the characteristic features of a specific autoimmune disease may still get quite a lot of trouble with the less tangible symptoms that get called 'fatigue' and suchlike. My understanding is that people with PBC can have as much trouble with fatigue as anything else. And although the condition can be progressive, in a lot of autoimmune diseases there are cases that never really progress much. Some patients with RA have a few small joints swollen but not painful for years without anything changing. My suspicion is that your prognosis as of now is not bad. Moreover, you may be lucky in that before too long a treatment may get established that can get you back to normal. That might seem pie in the sky, but then it seemed pie in the sky for RA in 1991. By 2001 we had lots of people completely well.

 

[andre79]

@Jonathan Edwards thank you Dr Edwards, your post made my eyes watery

I must keep a positive attitude, i don't want to get sicker. And I must trust the doctors, I know they would do the best for me. I am willing to try anything that gets me better.

By the way, i just found out that the Social Security Institute in my country gives Rituximab for free, so could be an option if my doctor allows it.

 

[Jammy88]

Good luck @andre79 !! don't lose the hope.

 

[NK17]

@Jonathan Edwards thank you Dr Edwards, your post made my eyes watery

I must keep a positive attitude, i don't want to get sicker. And I must trust the doctors, I know they would do the best for me. I am willing to try anything that gets me better.

By the way, i just found out that the Social Security Institute in my country gives Rituximab for free, so could be an option if my doctor allows it.

May I ask in which country you live?

 

[andre79]

Sent you a private message @NK17

 

[andre79]

Hello all!

I wanted your opinion on something. I have a real possibility of getting Naltrexone (50 mgs x 30 tablets) and i have been reading about Low Dose Naltrexone and its good results on autoinmunity. Apparently you can get it at any drugstore in my country without recipe (the wonders of living in a latin country) It's a little bit expensive but i can make the effort.

Since i have a diagnosis of fibromyalgia, i wonder if i could benefit from it.

I have an appointment with the hepatologist on december 2nd and he would probably order more tests related to PBC but i need to start working on my wellbeing, specially because i want to start a third post grade course on january 15 and i need energy and motivation, which i am currently lacking.

I would start with 1 mg during the nights for the first month, and raise it slowly.

My questions are:

Is LDN addictive?

I am currently taking 25 mgs of seroquel at night to sleep and 30 mgs of cymbalta daily. Both affect neurotransmitters, should i discontinue the use of these two medications during the LDn experiment?

How long does it usually takes to see the effect? I know this is variable from person to person, but an average figure would be nice, and what should i expect? Increase of symptoms first, reactions, etc.

If i should have an underlaying infection, could the LDN make it worse?

I have been trying to find studies about PBC and LDN but i didn't. I don't want to make my condition worse.

So, what do you think?

Thank you!

 

[Jonathan Edwards]

Hello all!

I wanted your opinion on something. I have a real possibility of getting Naltrexone (50 mgs x 30 tablets) and i have been reading about Low Dose Naltrexone and its good results on autoinmunity. Apparently you can get it at any drugstore in my country without recipe (the wonders of living in a latin country) It's a little bit expensive but i can make the effort.

Since i have a diagnosis of fibromyalgia, i wonder if i could benefit from it.

I have an appointment with the hepatologist on december 2nd and he would probably order more tests related to PBC but i need to start working on my wellbeing, specially because i want to start a third post grade course on january 15 and i need energy and motivation, which i am currently lacking.

I would start with 1 mg during the nights for the first month, and raise it slowly.

My questions are:

Is LDN addictive?

I am currently taking 25 mgs of seroquel at night to sleep and 30 mgs of cymbalta daily. Both affect neurotransmitters, should i discontinue the use of these two medications during the LDn experiment?

How long does it usually takes to see the effect? I know this is variable from person to person, but an average figure would be nice, and what should i expect? Increase of symptoms first, reactions, etc.

If i should have an underlaying infection, could the LDN make it worse?

I have been trying to find studies about PBC and LDN but i didn't. I don't want to make my condition worse.

So, what do you think?

Thank you!

Dear Andre79,
If you have early PBC then you should ask your hepatologist before taking any prescription medication in case there as a change in liver metabolism of the drug.

Naltrexone has no effect on autoimmunity. I am interested in the fact that it seems to help people with ME (which maybe you do not have after all?). I guess that it is possible that it counteracts whatever is upsetting the lower parts of the brain in ME. That could be very interesting. It might possibly have the same effect in fatigue associated with PBC but it might be completely different. I think you need to discuss it with your hepatologist. PBC is not an easy disease to manage and your hepatologist is not likely to thank you for making it more difficult without them knowing!!

 

[andre79]

Thank you Dr Edwards for your advice and i will follow it. I will wait until my appointment with the hepatologist and ask him if i can take the LDN.

At least I know that i have the chance of getting the LDN, i already found out where to buy it jejeje some times i get desperate to get better, specially the fatigue symptom which is unbearable, and since i used to be such an active person, it makes me want to jump on anything that could help me. But as you said, i should consult first with the doctor. I will let you know what he tells me.

 

[andre79]

OMG! I am so confused. I received the tests i did in the specialized laboratory and i am AMA negative? This is the best laboratory for inmune test in my country.

The only finding is that i have low C3, and i have tested this three times and is always the same but at least is going up very slowly. (From 55 to 66)

I don't know what to think. Dr Edwards i don't want to be annoying but i would appreciate your opinion on these test results. Can a person have AMA positive, and negative intermitentely without any treatment? I still might have pbc?

When i did the second test i was taking antibiotics for an abcess i had in one finger. Does that affect the results? I asked in the laboratory and they said no, but i am not sure.

 

[Jonathan Edwards]

OMG! I am so confused. I received the tests i did in the specialized laboratory and i am AMA negative? This is the best laboratory for inmune test in my country.

I am not going to be able to give you reliable advice here because there are too many unknown factors for me to judge. However, autoantibody levels do go up and down and it is not particularly unusual for them to go from positive to negative. One obvious possibility is that you were just 'on the edge' of full blown PBC but your immune system has sorted itself out. I cannot see why that should be impossible. Another possibility is that some unknown factor is affecting the test result and you may find another time it is positive again. Whatever the antibody test it sounds as if a liver specialist should keep an eye on your liver function. I guess the antibody going negative has to be a good sign. The only downside is that you may feel that you end up with no diagnosis and still feeling ill - you may be in the same boat as everyone with ME in other words. I don't want to give an opinion on what I think the real answer is but maybe the thing is just to see how things pan out a bit more and maybe it will become clear?