How Bile Duct Cancer Begins...

My mother's mother in 1954. She died from liver complications in 1969. Did my mother inherit mutated genes from her mother? Doubtful, but mom did inherit some issues that complicated her disease.

Now that my frantic caregiving chores for mom are over, I've been concentrating on how her cholangiocarcinoma occurred. My mother's age contributed to her risk factor. More than two out of three patients diagnosed with cholangiocarcinoma are older than age 65, and my mother was 78 when she was diagnosed. But lately I've been viewing more news on younger deaths from this cancer.

Cancer usually results from gene mutation, and sometimes this proclivity to certain mutations is inherited. Gene mutations related to bile duct cancers, however, usually are acquired during a lifetime rather than inherited. What changes during a lifetime could contribute to bile duct cancer?

• Liver fluke (parasitic flatworm) infections that settle in the bile duct and cause irritation is a major cause of bile duct cancer in Asia, but very rare in the U.S. It can affect people who travel to Asia and who eat raw or poorly cooked fish in that region.
• Bile duct infections or inflammation (cholangitis) can lead to scar tissue (sclerosis), which can create a risk for cholangiocarcinoma. Many people who have this disease (Primary sclerosing cholangitis or PSC) also may have issues with ulcerative colitis. Risk factors for progression of any of the issues to bile duct cancer can be as high as 30 percent.
• Bile-filled sacs connected to the bile duct are called choledochal cysts. The cells in these cysts, as with cysts in other parts of the body, can contain areas of pre-cancerous changes.
• People who are born with, or who develop, abnormalities where the bile and pancreatic ducts meet can be at higher risk for bile duct cancer. This abnormality can prevent bile from exiting the ducts normally, and that condition can prove deadly.
• Cirrhosis of the liver, caused by alcohol excess or diseases such as hepatitis, can create scar tissue that can affect the bile duct and lead to #2. Liver diseases and subsequent scar tissues can contribute up to 15 percent of bile duct cancers.
• Exposure to certain industry chemicals and PCBs can also lead to possible bile duct cancer.
• Thorotrast, a contrast agent that radiologists no longer used in x-rays, is a huge culprit in cholangiocarcinoma cases in individuals who are old enough to have had x-rays through the mid-1950s. The connection between the ingestion of Thorotrast and bile duct cancer can be up to 100 percent.

Some medical facilities and cancer institutions list other causes, including obesity, exposure to asbestos, smoking, exposure to radon, and diabetes. All these factors often contribute to liver damage and then scarring and damage to bile ducts. Is there a way to determine if you are at risk for bile duct cancer? Yes -- if you know that you are subject to any of the conditions listed above.

Can this cancer be detected early? According to the American Cancer Society, the answer is no.

The bile duct is located deep inside the body, so early tumors cannot be seen or felt by health care providers during routine physical exams. There are currently no blood tests or other tests that can reliably detect bile duct cancers early enough to be useful as screening tests. Without effective screening tests, most bile duct cancers are found only when the cancer has grown enough to cause symptoms. The most common symptom is jaundice, a yellowing of the skin and eyes, which is caused by a blocked bile duct.

From experience, I can tell you that the jaundice is a symptom, but it's also an alarm. It's the alarm that starts the race to the end of the cancer patient's life. How much time will that patient have? Anywhere from three months to 18 months, depending upon a number of factors including new procedures that can extend that patient's life.

But, that's another story...

Testing the Waters, Finding a Cause

Mom at Kure Beach, NC, 1953

I'm not sure who took this photograph of mom when she was at Kure Beach, NC, in 1953, but this photo marks a transition time in her life. She had already met dad and they would marry the day after Christmas that same year. This is one time they were apart during that year between his discharge from the Air Force and their wedding.

Mom already had several surgeries by 1953, including a broken wrist which was set wrong. The surgeons had to re-break her wrist and re-set it. She also had one ovary removed for some reason (precancerous?). Other than these issues, mom was rarely ill.

After mom died, I finally had the breathing room to learn more about cholangiocarcinoma and the causes behind this cancer. What I learned was vague at first. This type of cancer is more prevalent in Asian countries, where it is mainly caused by a common parasitic infection of the bile duct. These parasites, or liver flukes, infect people who eat food that contains this parasite. But, this risk factor is small outside Asian countries.

Other factors that can contribute to the 2,000-3,000 cases per year in the U.S. today include bile duct stones, choledochal cysts, or an ulcerative colitis that affects that entire large bowel creating what is known as primary sclerosing cholangitis (PSC, or type of inflammation of the bile duct). All of these conditions are rare, which can account for the rareness of the disease in this country. But, the numbers of people who are diagnosed with cholangiocarcinoma are rising. Why?

The main culprit, in my opinion, is Thorotrast. Thorotrast is a highly radioactive contrasting dye that was used in x-rays in the 1930s, 1940s, and -- in the U.S. -- through the 1950s. Most other countries stopped using this highly radioactive agent by 1950. Patients in the U.S., however, were possibly exposed to this contrasting agent every time they had x-rays through at least the mid-1950s.

The harmful part about Thorotrast is its half-life. This radioactive chemical stays in the body for about 22 years or more. As it decays, it emits harmful alpha radiation. Interestingly enough, this chemical gravitates to the patient's liver and bile duct. Patients who ingested Thorotrast are 100% more likely than the general population to develop bile duct cancer.

There's no way to track how many patients ingested Thorotrast during its time on the market, but one source states that between two to ten million people worldwide were treated with this radioactive compound. What researchers have discovered recently is that a disproportionate number of people who have developed bile duct cancer were once treated with Thorotrast. These cancers occur, usually, decades after the initial treatment.

This delay in cancer development could easily explain the age factor in developing cholangiocarcinoma. More than two out of three patients who develop bile duct cancer are older than age 65.

Mom was 78-years-old when she was diagnosed with cholangiocarcinoma. She had x-rays, at least two to three of them, before this photo was taken at Kure Beach in 1953. These thoughts are disconcerting for me for a number of reasons, but mainly because she looks like she deserved better. We all deserve better.