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Talking with Jennifer Weaver, today. Jennifer works with Cholangiocarcinoma Foundation (CCF). Before talking about the great work you’re doing with CCF. Let’s first talk about cholangiocarcinoma known as CCA or bile duct cancer (BDC). Tell Conversations, what is CCA?
Jennifer Weaver, Cholangiocarcinoma Foundation
Cholangiocarcinoma pronounced (koh-LAN-jee-oh-KAR-sih-NOH-muh) is a highly lethal and rare bile duct cancer of the liver with a poor prognosis. With approximately 10,000 cases a year being diagnosed in the United States, cholangiocarcinoma is the second most common primary liver cancer in the world. It is often diagnosed at advanced stages when treatment is only minimally effective, emphasizing the imminent need for novel therapies.
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Thanks for that, Jennifer. I infer that CCA is difficult to diagnose and that is why it is often dxd later stages. Why is that?
Jennifer Weaver, Cholangiocarcinoma Foundation
Cholangiocarcinoma symptoms often mimic other medical conditions, which is why people tend to ignore them or credit them to another condition. Those symptoms are:
Yellowing of your skin and the whites of your eyes (jaundice)
Intensely itchy skin.
White-colored stools.
Fatigue.
Abdominal pain on the right side, just below the ribs.
Losing weight without trying.
Fever.
Night sweats.
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Also which is the preferred term CCA or BDC or they are just used interchangeably?
Jennifer Weaver, Cholangiocarcinoma Foundation
CCA for cholangiocarcinoma.
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In HCC the most common form of liver cancer, there are certain underlying conditions like hep c, drinking that led to cirrhosis, etc. Are there such conditions in CCA/BDC?
Jennifer Weaver, Cholangiocarcinoma Foundation
The exact cause of bile duct cancer is unknown. However, some things may increase your chances of developing the condition. The most common include being over 65 years old or having a rare chronic liver disease called primary sclerosing cholangitis (PSC).
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And is there an ongoing effort to educate PCPs so that BDC can be identified quickly? What can be done on the physician side to make sure they are alert to any early signs?
Jennifer Weaver, Cholangiocarcinoma Foundation
Yes, the Foundation has a Scientific Medical Advisory Board and Nursing Advisory Board that is active in providing the most up-to-date information and guidelines to their colleagues. The Foundation also funds research, which to date has awarded $2.1 million in grant support for projects specifically on cholangiocarcinoma-specific research.
The Foundation also provides physicians with tear-off sheets and cell-line graphics on the website with additional opportunities to network by joining the International Cholangiocarcinoma Research Network (ICRN). This network is a global collaboration of research groups from renowned institutions who are working in concert to improve knowledge about cholangiocarcinoma etiology, prevention, early detection, treatment, and prognosis.
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Can you summarize the current treatment for CCA?
Jennifer Weaver, Cholangiocarcinoma Foundation
This is information from the Mayo Clinic about treatment for cholangiocarcinoma (CCA).
https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
Treatments for cholangiocarcinoma (bile duct cancer) may include:
Surgery. When possible, surgeons try to remove as much of cancer as they can. For very small bile duct cancers, this involves removing part of the bile duct and joining the cut ends. For more-advanced bile duct cancers, nearby liver tissue, pancreas tissue or lymph nodes may be removed as well.
Liver transplant. Surgery to remove your liver and replace it with one from a donor (liver transplant) may be an option in certain situations for people with hilar cholangiocarcinoma. For many, a liver transplant can be a cure for hilar cholangiocarcinoma, but there is a risk that the cancer will recur after a liver transplant.
Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be used before a liver transplant. It may also be an option for people with advanced cholangiocarcinoma to help slow the disease and relieve signs and symptoms. Chemotherapy drugs can be infused into a vein so that they travel throughout the body. Or the drugs can be administered in a way so that they are delivered directly to the cancer cells.
Radiation therapy. Radiation therapy uses high-powered energy beams from sources such as X-rays and protons to kill cancer cells. Radiation therapy can involve a machine that directs radiation beams at your body (external beam radiation). Or it can involve placing radioactive material inside your body near the site of your cancer (brachytherapy).
Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. Your doctor may test your cancer cells to see if targeted therapy may be effective against your cholangiocarcinoma.
Immunotherapy. Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide from the immune system cells. Immunotherapy works by interfering with that process. For cholangiocarcinoma, immunotherapy might be an option for advanced cancer when other treatments haven’t helped.
Heating cancer cells. Radiofrequency ablation uses electric current to heat and destroy cancer cells. Using an imaging test as a guide, such as ultrasound, the doctor inserts one or more thin needles into small incisions in your abdomen. When the needles reach the cancer, they’re heated with an electric current, destroying the cancer cells.
Photodynamic therapy. In photodynamic therapy, a light-sensitive chemical is injected into a vein and accumulates in the fast-growing cancer cells. Laser light directed at the cancer causes a chemical reaction in the cancer cells, killing them. You’ll typically need multiple treatments. Photodynamic therapy can help relieve your signs and symptoms, and it may also slow cancer growth. You’ll need to avoid sun exposure after treatments.
Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. It might involve placing a thin tube into the bile duct in order to drain the bile. Other strategies include bypass surgery to reroute the bile around the cancer and stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve signs and symptoms of cholangiocarcinoma.
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How big is the Patient Registry at this time and how many countries are represented?
And to that point, is the CCF a global non profit or just focused on the US?
I know you are the only non profit focused on CCA in the states and has a commanding presence in this condition.
Jennifer Weaver, Cholangiocarcinoma Foundation
The registry is global and the Foundation is global, too. I don’t have the specific number of countries but our international partners are the European Network for the Study of Cholangiocarcinoma (ENS-CCA), AMMF of the UK, Asia-Pacific Network, and the Cholangiocarcinoma Foundation of Thailand.
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CCF has a very strong online checklist for newly dxd patients. Can you highlight that section and include the link for it?
Jennifer Weaver, Cholangiocarcinoma Foundation
Yes, the new program was introduced on August 9, 2021, so it is just over 1 year old. Since it began, more than 1,200 newly diagnosed patients have been provided free care kits and information to help them navigate their cancer journey. The link is https://cholangiocarcinoma.org/newly-diagnosed/
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It seems very valuable. Nice work there.
Jennifer Weaver, Cholangiocarcinoma Foundation
We want to make sure patients know they are not alone. We connect them to support groups, provide educational webinars, a Specialist Map, how to get a second opinion and biomarker testing, and the opportunity to join CholangioConnect, a mentoring program. The mentoring program has matched 700 patients and caregivers and is still growing.
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Is CholangioConnect referenced on the same newly dxd tab on the site? I want to make sure newly dxd people can easily find it.
Jennifer Weaver, Cholangiocarcinoma Foundation
Yes – at the very top of the page.
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Noted. Thanks. You also seemed to have a strong TEAM CCF program that focuses on fundraising and community building. Can you highlight what you are doing there, Jennifer?
Jennifer Weaver, Cholangiocarcinoma Foundation
Yes, TeamCCF began in 2016 as a way to raise money through athletic events for the Cholangiocarcinoma Foundation. Since its inception, TeamCCF has participated in the Bank of America Chicago Marathon; the TCS New York City Marathon; The Berkeley Half Marathon; the AACR Philadelphia Marathon, Half Marathon and 8k; The CNO Financial Group Indianapolis Monumental Marathon, Half Marathon and 5k; and countless 5k runs and walks. Right now, Delorean Ostrom of Swanee, GA, is running a marathon each month to raise money for the Foundation and honor his 32-year-old wife, Mandy, who has cholangiocarcinoma. It’s called Racing for Hope. (racingforhope.org)
Jonathan is 60 years old
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That’s very inspirational. Thank you, Jonathan.
And at that age. Even more inspiring.
Clearly, you have many exciting initiatives at CCF, Do you have any others that you want to highlight?
Jennifer Weaver, Cholangiocarcinoma Foundation
Yes. We have a social media campaign that we launched at the 9th Annual Cholangiocarcinoma Foundation Conference in Feb. 2022 called, “Living with #CCAHope.” We have photos of various CCF community members – including researchers, clinicians and physicians – holding a sign with that statement to spread hope for this disease. When someone is newly diagnosed, they typically go to Google to research everything they can and are met with disheartening statistics and gloom and doom information. We want people to know there is always HOPE and the Foundation is a safe place they can come to get it. The Director of Advocacy, Melinda Bachini, is a beacon of hope. She will be a 13-year CCA survivor this December. She heads the Newly Diagnosed program and can relate to patient needs and desires. The more we can use the hashtag #CCAHope on social media, the more we can perpetuate HOPE.
Another initiative is educating the medical community about new billing codes specifically for CCA. We call it “Time 2C Billing Codes.” Cholangiocarcinoma is a bile duct cancer that can be divided into THREE subtypes. There is no ICD-10 code for perihilar CCA, which has led to the miscoding of perihilar CCA as intrahepatic CCA, affecting the accuracy of the reported incidence of CCA subtypes. Attached is a PDF that tells more from a Foundation’s trusted partner, Global Cholangiocarcinoma Alliance.
We also have the Turkey Trot coming up. This is an annual campaign in November where people choose to run, bike or walk while raising funds for cancer research. Participants choose the activity of their choice, set their own personal goals, recruit friends and family to sponsor, and complete it during the week of Thanksgiving.
Additionally, we are also encouraging donations with a program called, On Your Mark. This program allows donors to give monthly. There are nearly 200 donors enrolled currently and we’d like to see this program grow.
Lastly, we just published Nutrition & Cholangiocarcinoma. This 265-page book is available for download or can be ordered as a paperback from the CCF website. This book was two years in the making and is specific for CCA patients nutritional needs. The book is free after a brief survey is filled out at https://cholangiocarcinoma.org/nutrition-and-cholangiocarcinoma/
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This all wonderful and perfect way to close our conversation with all this great detail. You and your team are doing wonderful work at CCF and we’re very grateful for you highlighting this work. Thanks, Jennifer.
