Primary Sclerosing Cholangitis
In 1994, my husband Jim, was diagnosed with Primary Sclerosing Cholangitis (PSC). PSC is a Chronic liver disease where the biliary system of the liver suffer chronic inflammation. The cause of this condition is unknown, and over time liver damage will develop and eventually lead to the need of a liver transplant. At the time of diagnosis, Jim’s liver was still in good shape. Although there is no cure, the doctor explained to us that with the proper medicine and medical care the hope was to slow down the progression to a point where Jim may never need a transplant. Jim struggled with this initial diagnosis and after some time, came to accept it and started his course of medicine and regular doctor visits. Along the way, side effects did show themselves in the form of UC (ulcerative colitis).
70% of PSC patients develop UC and this proved to add another dimension to Jim’s illness.
As years passed, we continued to live normally. We married in 1997; welcomed our first child, a daughter in 1999 and our second child, a son in 2002. We had two beautiful, healthy children. Jim continued to work as a Union electrician and I was a stay at home mom. Things were, for the most part, wonderful and other than the occasional ulcerative colitis flare up, we had minimal reminders to the fact that Jim indeed had a liver disease. We lived a happy and blessed life. We did, however, remain uncertain as to what path this disease was going to lead us down. I thanked God for the beautiful things in my life and continually asked him to help us navigate through the uncertainty.
After 13 years of living with PSC, our worst fears came true. In November 2007, after two bouts of cholangitis (bile duct infections), 3 ERCP’s, and a lengthy round of tests, Jim was diagnosed with cholangiocarcinoma (bile duct cancer). The only cure was chemo, radiation, and a timely liver transplant. Since our Boston hospital did not transplant patients with bile duct cancer, we were sent to the Mayo Clinic in Rochester Minnesota.
Mayo Clinic pioneered a treatment plan for patients with bile duct cancer. In the past, to simply transplant a patient with bile duct cancer proved unsuccessful due to a high rate of cancer recurrence. With Mayo Clinic’s special pre-treatment protocol, Jim had a good chance of beating the cancer. Jim was put on the transplant list and we prepared ourselves for quite a journey.
Through it all, Jim put one foot in front of the other and continued to keep positive thoughts. At the end of November 2007, he traveled back to Mayo Clinic for the radiation and chemo treatments. Although he ended up hospitalized due to yet another bile duct infection, he managed to make it home just in time for Christmas. On our return visit to Mayo in January 08, he received the final stage of the radiation treatment. Due to the affects of the radiation and bile duct infection, Jim was looking pretty bad. He had lost a lot of weight and struggled with constant colitis flare ups. It was during this trip that the surgeon presented yet another option. Rather than wait for a cadaver liver, Jim was a candidate for a living donor transplant. A living donor would enable him to receive his transplant a lot sooner than waiting for a cadaver. But, how could we ask anyone to do this for us? How could we expect anyone to undergo such a major surgery…. A surgery with a recovery time of anywhere from 4-6 months. Not to mention, the whole process was to take place 2,000 miles away from our home. We instantly dismissed this option and traveled back to Massachusetts where we settled in for the long wait for a cadaver. The years of PSC had taken their toll on Jim’s liver; not only did he have the tumor, but he also had compromised liver function. Because of this, his colitis was not cooperating at all and Jim suffered immensely with intestinal issues. When he wasn’t in the hospital, he was on the couch, and through it all, his attitude never wavered. He was determined to win this battle with liver disease and remained grateful for the hard fact that he indeed had a shot to beat this.
In the meantime, family members and friends amazed us with their inquiries about the living donor program. In March of 08, an extended family member with no blood relation to Jimmy stepped forward and offered to donate. Jimmy, Rita (our donor), and myself traveled back to Mayo Clinic in February of 2008 where Rita underwent a weeklong series of tests.
In the end, her liver was a match and on April 22, 2008, Rita donated a little over half of her liver to Jim, ultimately saving his life. Rita was able to return home to her family 10 days after surgery to complete her long, hard recovery. Jim remained in Minnesota for two months. His recovery was a series of infections, feeding tubes, an episode of septic shock, and a blood clot, just to name a few. Even so, Jim continued to take things in stride and on June 12, 2008, he was able to return to Massachusetts to his home, his children, and his life!
The road was long and hard for all involved and I thank God every day for bringing Rita to us and her selfless gift that can never be re-paid. Jim is an inspiration; not only for his ability to remain strong, focused and determined, but in never giving up, never complaining, never asking “why me?” He had a job in front of him to get healthy and he did just that! It feels good to say that Jim no longer has PSC, cholangiocarcinoma, or cirrhosis. It is my hope that one day there will be a cure, not only for PSC, but for the numerous liver diseases afflicting so many people in our Country. With the endless efforts of the American Liver Foundation, someday this hope will be a reality.
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