Hep B Blog

Slaying the Fibrolamellar Beast

 

Fibrolamellar Chimera- Slay the Beast
The chimera mutation is common to all fibrolamellar tissue. The mythological chimera is part lion, part goat, and part serpent. Original Chimera illustration by Jessica Guercia Design by Deborah Corrigan Visual Arts | DeborahCorriganVA.com.

February 28 is Rare Disease Day.

Last year, we featured the story of Gail Trecosta, who described how her teenage son Matthew was bravely battling a rare form of liver cancer called fibrolamellar hepatocellular carcinoma (FHC).

This year we are featuring FHC again but with a renewed sense of urgency and purpose. 

 

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“Matthew fought his fight with grace and courage. He will be greatly missed every second of every day.”

We at Liver Cancer Connect were heartbroken to learn that Matthew passed away on June 24, 2014. He was 15 years old.

Our deepest condolences and thoughts are with Matthew’s family as they try to cope with their immeasurable loss.

To honor Matthew and his family, we are highlighting FHC again this year and explaining how you can help slay this dreadful beast of a disease.

What is fibrolamellar hepatocellular carcinoma (FHC)?

FHC is a  rare form of liver cancer that primarily affects teens and young adults. Unlike typical hepatocellular carcinoma (HCC), FHC is not associated with chronic liver disease, cirrhosis or a virus, and is not more prevalent in a particular ethnicity or gender.

FHC is diagnosed in approximately 200 people a year worldwide, but is often diagnosed at a late stage because of its vague symptoms and lack of diagnostic markers. Surgical removal of the tumor (resection) is the only effective treatment option. But even with surgery, FHC has a high rate of coming back (recurrence) and spreading (metastasis). Researchers are working hard to figure out why the treatments for HCC are less successful against FHC.

What do we know about FHC?

Because FHC is so rare, most clinical trials exclude patients with FHC. So, almost all questions about FHC remain unanswered:

    • What environmental factors can cause FHC?
    • Are there any genetic factors that lead to a predisposition?
    • Who is at risk and how can it be prevented?
    • How does a tumor develop and how can you keep it from spreading?
    • How can it be diagnosed accurately and earlier?
    • What are the best treatment options when surgical resection is not possible?
    • How can it be cured?

Although FHC was first thought to be less deadly than other HCCs, researchers now believe FHC is as aggressive as HCC and people with FHC do not have significantly better 5-year survival rates than with other types of HCC.

How you can help slay the fibrolamellar beast

You can do something about this! Here’s how:

Fund cancer research or an organization that is fighting FHC. Your donation to the following organizations will support much-needed research, fund clinical trials, and help build the FHC registry.

The Fibrolamellar Cancer Foundation : Nonprofit organization dedicated to raising awareness about FHC and supporting research to find better treatments and a cure!

The Tucker Davis Fibrolamellar Research Facility at The Rockefeller University maintains the Fibrolamellar Tissue Bank, which stores tissue samples and supports the development of cell lines and animal models for FHC and has an active screening program for drugs to treat FHC.

The Fibrolamellar Registry, a website run by individuals with FHC and their families, will serve as a primary source of shared information on FHC and is partnered with the Fibrolamellar Tissue Bank. A health survey database and place to share medical records are currently under development.

Fibrolamellars of the World Unite!”: A private Facebook resource for families facing FHC.

Fibrolamellar patients can help too!

  • Donate fibrolamellar tissue samples for research purposes.
    • Note: Individuals may make donations at any hospital, but the Fibrolamellar Tissue Repository was created at The Tucker Davis Fibrolamellar Research Facility at The Rockefeller University to serve as a central repository.
  • Donate medical information to the FHC registry. The combined information from shared medical records can be used to learn more about the causes of FHC and how to treat it. At this time, theFibrolamellar Registry is developing the capability/database to serve this purpose.  

Why is research so important?

Scientists still need to learn more about the genetic changes that cause FHC, and they are getting closer to finding some of the answers. Recently a research group identified a genetic alteration that may cause tumor growth in people with FHC.

This is encouraging progress, but we still need to do so much more to find the best treatments or a cure. Currently, there is no single institution or research group collecting information on different treatments for FHC. This lack of knowledge is holding back the progress in fighting this disease. We need to build on the momentum generated by the recent discoveries and continue vital research efforts.

Editor’s Note: Join Us on the Hill!

The Hepatitis B Foundation (and its dedicated liver cancer program, Liver Cancer Connect) is a member of the Deadliest Cancers Coalition. Along with our coalition partners, we are urging Congress to safeguard funding for cancer research. Please join us on Tuesday, March 10, from 10 -11:30 am in 122 Cannon House Office Building for a congressional briefing on the importance of cancer research.

 

Comments on this blog are closed. If you have questions about hepatitis B or this blog post, please email info@hepb.org or call 215-489-4900.

6 thoughts on “Slaying the Fibrolamellar Beast”

  1. Thank you for your post featuring Matthew Lynch, my nephew, and the continued need to Slay the Beast, fibrolamellar hepatocellular carcinoma (FHC), for Rare Disease Day. Our family collective continues to be in shock and pain over the loss of Matthew. Matthew was someone who cared very much about giving and helping others. I look forward to the day doctors can tell young people diagnosed with this illness — and their parents — “Yes, we have a cure.” Then, I imagine, we will turn our attention to other ways to carry his light forward in a joyful, giving capacity.

  2. Thank you for the post about this “Beast”, FHC. We too lost our son, Austin, last June to this nasty disease. Dr. Sandy Simon was a great help to us in our battle and his group, including his daughter, Elana, have done remarkable research that will aid in developing drugs to attack this chimera kinase. We send our condolences to the Trecosta family. Losing a child is a heart breaking loss but we all hope and pray for the discovery of an effective treatment and even a cure for this deadly Cancer.
    Cathy & Peyton Green

  3. Thank you to all of Matthew’s family and friends, and to the FHC community, for sharing your stories and for your outpouring of support. We are honored to share the story of Matthew and his loving family. We at the Hepatitis B Foundation and Liver Cancer Connect fervently hope that there will soon be effective treatments and a cure for this dreadful disease, so that no other families will have to endure the loss of a precious child.

  4. Our 14 year old Daughter, Nicole was also diagnosed with this horrible Cancer. She passed away 4 months to the day of her diagnosis. She didn’t have a fighting chance. It happened so quickly. She went from feeling fine and nomal in Decemner 2013. Then January 2014 for a few weeks was experiencing light headed and nausea. Our world changed January 29th when they performed an ultrasound. She was stage 4 and it had already metasized to her abdomen and lungs. Our family has forever been changed.

  5. Amanda was diagnosed Feb 2014. Stage 4. She was a conductor for the railroad, a strong energetic beautiful young woman not afraid of a challenge. She had a degree in auto body repair. So much to live for and so loving! She loved her family, friends, her horses and her dog. She was so full of life before. She turned 25 in August and went to live with Jesus on October 27th. Nine months! She had been throwing up, not able to eat she was having heat flashes, Drs removed a tumor the size of a soccer ball in April. While she is not physically with us she is in my heart and ever on my mind. Today marks 4 months since she left. Fibrolamellar took her away…and Jesus gained wonderful woman in Heaven! Our family is forever changed and missing her till we see her again… Tears will flow !

  6. Thank you, Gail, for sharing this important information with the broader liver cancer community. Matthew, Nicole, Amanda, Austin inspire us every day to keep fighting for a cure.
    Rachael
    Fibrolamellar Registry

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