1. What is the Cholangiocarcinoma Foundation?

Like many non-profit organizations, a tragedy was the driving force behind the launch of the Cholangiocarcinoma Foundation. Stacie Lindsey, president and founder of the organization, was caregiver to her brother, Mark Clements. He was diagnosed with cholangiocarcinoma, or bile duct cancer, at age 38. At the time of his diagnosis in 2005, very little information existed about this rare, complex cancer.

Mark’s final wish was to help the patients who came after him, so their journey would be easier than his. When Mark passed away, his family was determined that no one else should have to invest the time and effort they did to gather information about this disease and connect with experts. In 2006, they honored Mark’s wishes by starting the Cholangiocarcinoma Foundation, launching a website that consolidated the vital information they discovered while caring for Mark. Today the organization has grown to become the largest foundation that serves cholangiocarcinoma and the leading global resource for research, education and public awareness for this rare cancer.

The organization is dedicated to educating and supporting patients by providing the personal guidance and tools they need to navigate this complex disease. For a detailed description of the foundation, visit https://cholangiocarcinoma.org/.

2. What are the symptoms of cholangiocarcinoma that patients typically face? What is the long-term outlook for patients?

Cholangiocarcinoma arises from the tissues in the bile duct, which is a 4-to-5-inch-long tube that connects the liver and gallbladder to the small intestine. The bile duct allows bile, which is made in the liver and stored in the gallbladder, to flow into the small intestine. Bile is a liquid that breaks down fats found in foods and helps the body get rid of waste material filtered out of the bloodstream by the liver. Cancer can occur in any part of the bile duct, and roughly 8,000 new cases of bile duct cancer are diagnosed each year in the United States.

Unfortunately, the disease is usually discovered in later stages, and there is no proven treatment for it. It is a silent disease, and many of the symptoms that do stand out usually can be explained by other conditions, making it very hard to diagnose. Many people think they may have gallstones or heartburn issues, only to discover they have a tumor in their liver, or even worse, cancer that has spread outside the liver to other organs. Some of the late-stage symptoms that might lead a person to see a doctor include: jaundice, dark urine, pale stools, pain in the upper right abdomen that may radiate to the back, fever and itching, all of which may indicate the bile ducts are blocked with either a tumor or bile buildup.

The long-term outlook for patients today is much better than in the past, bringing much hope to those who are newly diagnosed. A decade ago, not a lot of options were available for patients, and the standard of care was palliative treatment only. Today there are roughly 160 clinical trials in progress for this disease, due to the high number of targetable mutations this cancer has. Many of these trials may be viable treatment options for some patients, depending on whether or not the patient has a specific type of mutation.

3. What are some of the biggest challenges patients and their families deal with who have this condition? Are there specific emotional, financial or physical challenges?

The biggest challenge patients face is getting an earlier diagnosis, as the disease is usually not found until late-stage. Qualifying for surgery is also a major hurdle for most patients. Surgery and transplant are the only options right now for a potential cure, and, even with those treatments, the recurrence rate is quite high. Only about 20% of patients qualify for surgery; for those patients, there is a 70–75% chance of recurrence.

Many patients face financial issues. Some are not able to work after diagnosis due to fatigue, if they have chemotherapy, or pain that sometimes goes along with the disease. While generally there are many financial resources to help cancer patients, those with cholangiocarcinoma do not always qualify for assistance because the disease is considered rare. The foundation website has a list of specific organizations that may be potential sources of information or financial assistance for patients.

Emotional distress is also very common, since the prognosis is often poor. Many patients experience sadness, anxiety and anger and may have difficulty expressing their feelings to loved ones. Family members and caregivers also face significant emotional challenges. Many feel helpless and very isolated. They want to do everything they can for their loved one, but they are often overwhelmed and do not know how to help. This is especially true for those who are not aware of the foundation and the assistance they offer. The foundation is working hard to spread the word so every newly diagnosed patient is made aware of the wide range of support that is available.

4. What resources does the foundation offer patients?

The foundation stresses the importance of getting a second opinion from an expert in this disease as soon as possible, and the organization helps patients do it. This is especially important for those diagnosed in a community setting, as physicians in these practices may not be familiar with cholangiocarcinoma because it is so rare. It is important to be under the care of a physician who is up to date on the research for this disease so they can provide the most optimal treatment choices. While physicians who specialize in cholangiocarcinoma are hard to find, the foundation provides a specialist map on the website that enables patients to click on a state and look for providers near them who treat a higher volume of cholangiocarcinoma patients.

Immediate biomarker testing is recommended for patients so they can have their tumor profiled to determine if they have any targetable mutations or immunotherapy biomarkers. If any are found, personalized treatment options through clinical trials may be available. It is especially encouraging today that about 50% of cholangiocarcinoma patients have a mutation that can be treated in a trial, so it is critical for biomarker testing to be done right away. The foundation has several animated videos on the website to help patients understand why biomarker testing is so important and why they need to ask their physician to have it done as soon as possible.

Online patient and caregiver support groups are another resource available to patients. One resource, CholangioConnect, is a mentorship program that enables one-on-one support among patients, survivors and caregivers. This peer-to-peer connection platform helps anyone touched by cholangiocarcinoma at any stage in their journey find a mentor to ask questions and get support from someone who has personal experience with the disease.

Many other resources are available from the foundation to guide patients and their families through diagnosis, treatment and survivorship. Complimentary books and fact sheets, webinars, discussion boards, blogs and a comprehensive resource guide can be found on the website, as well as many other helpful items. Personal guidance from a staff member who is a long-time cholangiocarcinoma survivor is also available.

Lastly, the foundation partners with other non-profit organizations, for-profit companies, healthcare institutions and various organizations worldwide to provide high-quality programs and services to the cholangiocarcinoma community. One important partnership is with a company called Ciitizen, a consumer-health technology company that helps patients collect, organize and share their medical records digitally, free of charge. The records are converted into a digital health profile shareable with doctors, family members and researchers. A clinical trial finder is also available from Ciitizen that gives patients a detailed report on clinical trial matches they could potentially qualify for. The trial report is very helpful for patients to take to their doctors to discuss treatment options, as trials are a critical part of treatment for this disease.

5. What outreach activities does the Cholangiocarcinoma Foundation use to connect with patients?

The foundation offers a variety of ways for patients to connect with the organization, in addition to the website. These include: Facebook, Instagram, LinkedIn, Twitter and YouTube.

There are also three support groups on Facebook: one for patients only, a caregiver’s group and a targeted immunotherapy group. Patients can connect with patients and caregivers in their area and ask questions about treatments and side effects and share experiences.

6. What volunteer opportunities are available with the foundation?

There are many volunteer opportunities for people who would like to assist the foundation. The website lists a variety of ways people can get involved with the organization, such as becoming a patient advocate or holding fundraisers to rally friends and family to support the foundation.

Patients can apply to be a volunteer in any of the fundraising areas, but one of the most important roles they can play is as a mentor to a newly diagnosed patient. Caregivers can also mentor other caregivers. There is always a high need for mentors, so everyone is encouraged to join this valuable program. Patients who enroll with Ciitizen to gather their health records also have the option to participate in cholangiocarcinoma research, enabling them to play a role in finding new treatment options.

7. Where can patients get the most up-to-date information on new advances in cholangiocarcinoma?

The foundation’s annual conference is the premier source for all up-to-date information and clinical trial results. It brings together patients, caregivers, scientists, researchers and healthcare providers from around the globe to discuss cutting-edge research, innovative therapies and patient education in the field of cholangiocarcinoma. The 2021 and 2020 conferences were held virtually due to COVID-19 restrictions, but the conference is normally an in-person event in Salt Lake City, UT. Patients can access all conference materials and recordings by registering on the website, which also has current information about the disease.

8. What closing thoughts should readers remember?         

The foundation is eager to connect with cholangiocarcinoma patients and their loved ones to offer assistance. Patients are encouraged to contact the foundation’s director of advocacy who can help them navigate the disease by assisting in getting a second opinion, finding an appropriate clinical trial, and connecting patients with resources and support so they know they are not alone.

Physicians who are treating cholangiocarcinoma patients are also welcome to contact the organization, especially those who seldom see the disease. The foundation has a wealth of resources and access to experts who can help them learn more about cholangiocarcinoma and clinical trials that may provide promising treatment options for their patients.

The Cholangiocarcinoma Foundation is dedicated to helping each and every patient navigate this disease and connect with other patients, as there is much strength and hope in numbers. No one has to go through this alone.