Living With Primary Biliary Cirrhosis (Cholangitis)
Facts About Primary Biliary Cirrhosis (Cholangitis)
PBC (Primary Biliary Cirrhosis) is an autoimmune disease of the liver for which there is no known cause or cure.it is considered uncommon, but not rare. It affects It affects 40,000 of 100,000 adults. PBC occurs in women more than men by about 10:1. The diagnosis is usually made in middle age. According to the American Liver Foundation, most patients live 15-20 years before the disease reaches it's final stages.
Recently a name change has been adapted for this condition. It is know starting to be known as Primary Biliary Cholangitis. The name change better represents the majority of people who have this disease. Cholangitis reflects the inflammation of the bile ducts of the liver. This occurs from stages 1-3. At stage 4 cirrhosis occurs so that that stage it is then Primary Biliary Cirrhosis. The name change occurred in 2015 and is currently not used universally as yet.
It has been known to occur in 20 year olds. When it is diagnosed in earlier ages, the need for liver transplant becomes more likely earlier in life.
It is a disease of the biliary ducts of the liver which cause them to inflame and ultimately be destroyed. Many of us are not at the end stage of the disease which actually morphs into non alcoholic cirrhosis. At that stage of the disease, the only way to cure the damage is to have a liver transplant. This is a slow moving the disease and right now there is only one basic medication that helps slow the progression of the damage in our livers.
Presently, 4% of all the liver transplants in the USA are done for PBC. Since the demand for livers has increased with fatty liver disease and other liver problems increasing, this makes getting a transplant at times difficult.
Symptoms of PBC
While each of us vary in the symptoms of this disease that we experience, you might think that we were very healthy to look at us. For a great many of us, the disease is not physically visual until we start getting the end stage of the disease and we turn jaundiced. However for many of us, we suffer fatigue that is hard to explain unless you have experienced it. According to the PBC.org website, 80 % of us will suffer fatigue. It is a fatigue that can be debilitating to the extent that we are not able to continue our daily tasks. Learning to "pace " ourselves is important to allow ourselves to make the most of our day. The fatigue we experience is not linked to the stage of our disease. It can occur at any time. The best way I can describe it is to say that despite the amount of sleep, you still feel that you need more rest.
Another common symptom is an itching in our skin at various times that does drive us to distraction for sure. While there are some medications that may or may not work, research is working hard to get this debilitating condition under control. It can be very debilitating especially at night, causing the rest we need to be lacking.
Many of us suffer from things like dry mouth, joint stiffness, some aching in the area of our livers, dry eyes and more. Some of us have insomnia. Aches and pains in the joints is not uncommon. Not all patients suffer all symptoms and the amount of symptoms vary with each patient and the stage that they are in. The pain from the liver that we experience is mostly on the left side right under the ribs. It's not from the liver itself, but from an inflammation of the covering of it.
Vitamin difficiencies are not uncommon Vitamins A, D, E, and K are often not absorbed in our bodies. We often have to supplement them to keep going
A few of us have multiple autoimmune conditions which make life challenging, but not impossible.
More People Have Autoimmune Diseases Than You Realize
Do you have one of the hundred or more autoimmune diseases?
Many People With PBC Have Other Autoimmune Diseases
It is not uncommon for people with PBC to have other autoimmune diseases as well. No one knows why yet, but there is research being done to figure it all out. Sjogrens syndrome, Reynauds, thyroid, rheumatoid arthritis and fibromyalgia are some of the common ones. We also have a tendency to have osteopenia and osteoporosis as well. Not all PBC patients get them, but many do.
The Stages Of PBC
While many of us were diagnosed by several blood tests, there is one real way to know and stage PBC. That of course is a liver biopsy. At some point or another, most of us will have at least one liver biopsy. Once the diagnosis is confirmed and staged, most physicians do not recommend another biopsy. With a newer test called a fibroscan, more and more information is gathered without doing another invasive procedure. So many changes and new procedures are on the horizon that the future hopefully will provide our doctors with new tools to help us get through our lives with brighter outlooks.
As our disease progresses we get fibrosis (scarring ) which causes some stiffness of our livers. The fibroscan measures the stiffness of our livers and is very promising new technology. Although it has some limitations and is not totally or readily available, the future holds great promise for this technology
Stage I- Mostly shows inflammation, abnormal connective tissue or both. confined to the portal area of the liver
Stage 2-Inflammation, fibrosis, or both, confined to the portal and periportal areas
Stage 3-Bridging fibrosis
What PBC Looks Like-Biopsy Slide
What We Do Need Is Liver Donors
Most of us go through a lot of blood work, ultrasounds, fibroscans and MRI's to monitor our condition. Since this disease is slow going, you can live a reasonable life until you get to the stage where a liver transplant is necessary. The need for livers to replace damaged ones is high. Many people do die waiting for liver transplants. There are other conditions such as liver cancer, alcoholic cirrhosis, and more that need new livers too. The fact is that you simply cannot live without your liver. In most states and countries around the world, there are ways that you can become a donor when you get your driving license. You could give the gift of life when you no longer need your liver.
Some generous folks actually give live donations when they are a match. Since only a small part of the liver is needed, and the liver regenerates, it is a possibility. However it is a major surgeryAt this time the need for livers exceeds those available, so in behalf of all liver disease patients, we ask that you consider giving that gift of life
What Does The Future Hold For PBC?
There is continuing research that has made some strides, but there is so much more work that has to be done. Testing for PBC patients is improving and there are some medications that we might be able to look forward to in the near future. But of course, like anything else, all of this takes money.The most recent study that I could find shows 1 in 3–4,000 people have this disease. Since we are a limited group of people with this disease, funding has been limited.
Fibro scans are a new technology that when available are really helping PBC patients. It's a new technology and not as readily available as liver ultrasounds. But when available, it is a helpful tool in tracking liver disease
Currently there is one medication that hold promise. It is still in the testing stage. Primarily it is for patients that are not responsive to the other medication that is available. This , if apprioved by the FDA holds some hope to many patients.
Medications For PBC
Ursodiol has been the medication of choice for many years. While it does not cure PBC, for many, it keeps the progression of the disease at bay for many years.
A second medication has been approved by the FDA. It is for those patients who have had little or no response to the Ursodiol. We have been waiting for 20 years for a medication and now we have a new option.
The problem with these medications is that they are very pricey. There are no generics. The copays can be very pricey even with insurance.
PBC Medication INformation
- Oclavia-Facts ans inflrmation
Occalvia facts and information
- Ursodiol (Oral Route) Description and Brand Names - Mayo Clinic
My Personal PBC Journey
I was diagnosed with PBC at age 64 due to an abnormal blood test while having a workup for another condition. After several additional blood tests, the diagnosis was made. My stomach doctor was careful to explain everything that he could and in truth, at that moment I was glad it wasn't something worse. My journey with this chronic disease has been challenging, but my everyday life still has been fulfilling. Even though I am currently dealing with several other autoimmune conditions, I am focusing on my blessings rather than the challenges. I don't know what tomorrow may hold, but I am ok today.
Your PBC Medical Team
Once you have the diagnosis of PBC, it is time to set up the medical team to continue your specific care.
A lot of whom you will need in addition to your medical doctor will depend on the insurance coverage that you have. Some insurance plans will only cover specialists with a referral. So start with your medical doctor and discuss who needs to be on your team and how you will be referred.
It is also time to understand your insurance. Since there will be additional testing , blood work and office visits, it is important to understand what and how your insurance will cover your needs. Take time to read your policy. If you have any questions, make sure to write them down. Check with the online website for your insurance or call your insurance agent with any questions you may have.
Your Internist or regular doctor generally may only have a limited amount of knowledge about PBC so you will need additional care physicians to cover your needs.
A gastroenterologist is a doctor who specializes in disorders of the stomach and intestine. They are often involved in PBC management.
A hepatologist is a doctor who specializes in disorders of the liver, pancreas, gallbladder and biliary tree. They may be used in place of or in addition to the gastroenterologist.
A rheumotologist is a physician who specializes is diseases of the joints and bones. Because often PBC is associated with other autoimmune diseases, you may have consultations and care with this type of physician also.
Since you may have more than one physician on your team, it is important to make sure that there is good communication between your care team. You need to be proactive in making sure that each of them has the same information on any blood work, test results and changes in your medication.
The Human Liver
More Resources For PBC
- You May Be At Risk For Another Autoimmune Disease
- PBC Foundation - providing support for life
The PBC Foundation (UK) Ltd is a charity offering support and information to PBC sufferers, their families and friends.
- American Liver Foundation. Your Liver. Your Life.
The Web Site of the American Liver Foundation. Great resource for PBC and other liver diseases
Formed December 1, 1996, the PBCers is now the largest PBC online support group with almost 3,000 members worldwide. Family members and friends are encouraged to join and learn more about PBC. We also accept research donations
Things You Should Know About PBC Patients
If you were to look at me today at Stage 2 PBC with 4-5 other autoimmune diseases, you might not know what is going on. For the most part, until and if I get to the stage where my liver is permanently damaged, if you don't look closely, I might not look sick at all. But there is a lot going on when you look closely. My feet might be swollen from retaining fluids, I might walk slowly because of the fatigue that I am experiencing. You might see the scratches where I have been trying to relieve my itching. Some of my teeth have probably been replaced due to teeth loss. I might have what we refer to as brain fog. You might notice that I do things slowly and stop to rest frequently.
For most of us, we really don't want your sympathy, just your understanding and your help
Primary Biliary Cirrhosis Facebook Groups
- Primary Biliary Cirrhosis And Other Liver Diseases
- Primary Biliary Cirrhosis Awareness
Support group for anyone with PBC
This content is for informational purposes only and does not substitute for formal and individualized diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed medical professional. Do not stop or alter your current course of treatment. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.
© 2014 Linda F Correa