I thought that it would be a good idea to explain what Primary Sclerosing Cholangitis is and how it affects Rick. Most of the time I will refer to it as PSC for short because it is really long, imagine the looks on people's faces when I say the whole thing. This is the definition of PSC that I copied from the American Liver Foundation website.
What is primary sclerosing cholangitis? Primary sclerosing cholangitis (PSC) is a chronic, or long-term, disease that slowly damages the bile ducts inside and outside the liver. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the gall bladder and the small intestine, where it helps digest fats and fatty vitamins. In patients with PSC, the bile ducts become blocked due to inflammation and scarring. This causes bile to accumulate in the liver, where it gradually damages liver cells and causes cirrhosis, or scarring of the liver. As cirrhosis progresses and the amount of scar tissue in the liver increases, the liver slowly loses its ability to function. The scar tissue may block drainage of the bile ducts leading to infection of the bile. PSC advances very slowly. Many patients may have the disease for years before symptoms develop. Symptoms may remain at a stable level, they may come and go, or they may progress gradually. Liver failure may occur 10-15 years after diagnosis, but this may take even longer for some PSC patients. Many people with PSC will ultimately need a liver transplant, typically about 10 years after being diagnosed with the disease. PSC may also lead to bile duct cancer. Endoscopy and MRI tests may be done to monitor the disease. What are the symptoms of PSC? Many people with PSC do not get symptoms, especially in the early stages of the disease. When symptoms do occur the most common are fatigue, pruritus, or intense itching of the skin, and jaundice, a yellowing of the skin and eyes. These symptoms may come and go, but they may worsen over time. As the disease continues, the bile ducts may become infected, which can lead to episodes of fever, chills and abdominal pain. What causes PSC? The cause of this disease is not known. About 70 percent of patients are men. It may be related to bacterial or viral infections, as well as problems in the immune system. Genetic factors may also play a role. PSC is considered an uncommon disease, but recent studies suggest that it may be more common than previously thought. How is PSC diagnosed? Because many PSC patients have no symptoms, the disease is often discovered through abnormal results on routine liver blood tests. Formal diagnosis is usually made by cholangiography, an X-ray test involving injection of dye into the bile ducts, or by an MRI. How is PSC treated? There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow.
When is a liver transplant necessary? Over time, many PSC patients will continue to suffer a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be necessary. The outcome for patients with PSC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent, with a good quality of life after recovery
Who is at risk for PSC? PSC is more common in men than women. It usually affects people between the ages of 30 and 60. As many as 75 percent of patients with PSC also have inflammatory bowel diseases, usually ulcerative colitis. What is being done to find a cure for PSC? PSC has been known for 100 years, but now doctors are able to diagnose it very early. This means that treatment can begin before the liver is severely damaged. Scientists are continuing to study the disease to find the cause and understand its development. In addition, drug therapy trials, involving a large number of patients around the world, are exploring the potential use of several additional medications to lessen the symptoms and control liver damage.
We were very worried after Rick got the PSC diagnosis. I googled it and what I found was that it is terminal and he would need a liver transplant. I have found others that have PSC and some of them were transplanted very early on in their disease and others have had it for several years. This disease is different for every person. Rick was "officially" diagnosed with it two years ago, but we believe he has had it since he was diagnosed with Crohn's disease four years ago. He has just recently begun showing the signs of being jaundiced. The whites of his eyes are yellow and his bilirubin is elevated. The thing about having a disease like this is that people that don't know he is sick would never really know by just glancing at him. And sometimes the people that do know don't realize how sick he really is because he continues to work and go about daily activities as if nothing is wrong. He has a great outlook for someone with his health.
If anyone has any questions just leave a comment under this post and I will try to answer them. I will make anonymous comments available for those that do not want to set up an account. But please if using anonymous please be sure to sign your name so I know who you are. Thanks for reading and for the support.
Hi - I was diagnosed with PSC and ulcerative colitis seven years ago, after several major cholangitis episodes. For several years I needed stent replacements in my main bile duct, because of biliary strictures, but this was resolved 1.5 years ago. I take UDCA (ursofalk) and mesalazine, as well as a large range of vitamins etc. After checking PSC on clinicaltrials.com, I have also been taking high doses of DHA. My liver readings are stable and my colonscopies, ultrasounds and biopsies show the diseases are making very slow progress. I rarely suffer from itching but find that phenergan helps. Would like to exchange information on Wellsphere or firstname.lastname@example.org
I was diagnosed with PCS at age 18 after going through severe infectious episodes and swelling of the abdomen for one year - it took them one year to diagnose me (in 1994) since at that time there were fewer than 25 cases diagnosed in the US in my age bracket - I ended up having esophogial verosis at age 21 (almost died a couple of times) and was shortly thereafter transplanted - it was an absolute miracle the way that it all worked out - I was given an escellent liver, no alcohol or drug use, not fatty, etc. from a 27 y/o man who died in a car accident (God bless him and his family!); it was great! I immediately felt soooo much better as soon as I woke up from the 12 hour surgery - my skin went from green/grey/yellow to a bright, brigth pink for about a week, then to normal color - I didn't feel like I had a basketball in my stomach - I remember telling the doctor I was worried (after transplant) because I couldn't feel my liver inside of me - he said that people normally don't feel their organs. Anyhow, God is great, and the transplant was wonderful. I also have UC, but it has been controlled, although I was hospitalized a few times for flare ups. I received the transplant in 1997 - in 2003, they said that it looked like PCS might be back, but I went on Ursodial and everything slowed down (I have taken 6 grams of Sulphasalazine daily for the last 15 years as well); things have progressed, unfortunately, and I started experiencing bad symptoms in 12/09. in 01/10 I had a MRCP and they noted that my main bile duct (extrahepatic) was strictured badly while the others looked good. Also located on the MRI/MRCP was a lession on my kidney. The lession ended up being cancerous. I was aslo "installed" with two internal/external biliary drains, which allow me to drain off excess bile - my kidney was also removed and they located thirteen tumors inside - the urologist believes that they were caused by long-term immunosuppression for transplant. My transplant team is now talking about doing a surgery to remove the damaged bile duct and "move up" the bowel. I'm not sure when it will be, but likely within a couple of months.
My comment is that Praise Jesus that I ended up having an MRI, because this caught the Papillary Renal Cell Carcenoma (which does not respond to radiation or chemo) before it spread out of the liver - this was the first time (after almost 13 years of having a transplant) that I had an MRI/MRCP. I later learned that some post-transplant patients have regular MRI's - I am not blaming anyone for the fact that I did not have regular MRI's since my transplant team, with God's help, has saved my life many times - BUT PLEASE PEOPLE, IF YOU ARE A POST LIVER TRANSPLANT PATIENT AND TAKE IMMUNOSUPPRESSANTS, ASK FOR, OR DEMAND REGULAR MRI'S -
Oh MountainBoy, you're very brave! I wish you a lot of luck and good health!!!
God is great and he will help you I'm sure.
My boyfriend was diagnosed with PSC 6 years ago. He's feeling ok so far. he also has UC and that is ok too. But who knows what might happen, I hope for the best and pray. Thank you for your positive spirit, it means a lot to me.
Wow, I am amazed at what you've gone through Mountain Boy... I wish and pray for your wellness.
Rick has a bright future I am sure!
My husband also has PSC along with UC. He should be on the transplant list by next week here in Tampa.. his meld has been 19, he is a somewhat young 40 year old... but severe ascities, fatigue, jaundice and all the usual chirrosis symptoms. in and out of hospital. We're positive. I know he'll be ok and hopefull he will get a transplant in the next year before status 1...
Stay positive and jump those daily hurdles Izis...