In late February 2016, while getting dressed for work, George Labonte — the deputy police chief of Wrentham, Massachusetts — noticed a lump under his Adam’s apple as he knotted the tie of his uniform.
Labonte’s primary care provider ordered imaging and referred the 35-year-old to an ear, nose, and throat specialist. A biopsy showed he had medullary thyroid cancer (MTC), a rare disease that accounts for only 3%-4% of all thyroid cancers.
He underwent surgery to remove his thyroid, followed by radiation and low doses of chemotherapy since the surgeon could not resect all of the cancer. After a stable period of a year and a half, imaging showed the cancer had spread to his spine. Another year of radiation bought more stability, but in January 2019, Labonte’s condition worsened; imaging showed metastasis to his liver, lower spine, ribs, and hips.
At that point, Labonte joined a clinical trial for LOXO-292, or selpercatinib capsules; he qualified based on a positive RET (rearranged during transfection) mutation. The human gene RET, which contains 21 exons, is localized on chromosome 10. After only 2 days on Retevmo™, as the therapy is now known, Labonte’s chronic diarrhea disappeared; within 6 months, his tumor shrank by 40%. His levels of both calcitonin and CEA (blood tumor markers) have dropped significantly, and he’s seen no further progression of his disease for the last year. LOXO-292 received US Food and Drug Administration approval in May 2020 and is marketed by Loxo Oncology, a subsidiary of Eli Lilly & Company.
“I am an active person, running and working out routinely,” said Labonte, now 40 and the father of 4 children. “I’m probably in the best shape of my life — except for still having cancer.”
Fellow thyroid cancer survivor Gary Bloom loves success stories like this.
A Network of Support for MTC Survivors
Bloom is executive director of the Thyroid Cancer Survivors’ Association (ThyCa), based in Olney, Maryland. Bloom said his nonprofit, which operates on a $550,000 annual budget, reaches about 125,000 people worldwide; that includes 4000 or so “meddies,” as people with MTC are known. About 1500 to 2000 new cases of MTC are diagnosed each year in the United States, he said.
“Back when we incorporated ThyCa in 1999, it was a lot harder for patients to find information,” said Bloom, 59, who had papillary thyroid cancer, the most common form of the disease. “In the case of MTC, the most important thing is a diagnosis. Too often, people are diagnosed with MTC as part of the pathology assessment after their thyroid is removed.”
By that point, he said, the cancer has often spread to distant organs. According to the American Society of Clinical Oncology, the 5-year survival rate for metastatic MTC is only 38%, compared with 76% for metastatic papillary thyroid cancer.
Among other things, ThyCa provides online education and support, as well as virtual meetups and a private Facebook group for about 3000 people with MTC. To date, the organization has raised $2.5 million for research through its collaborations with the American Thyroid Association and the American Association of Endocrine Surgeons.
According to Bloom, most people will live 10 to 20 years after a diagnosis of nonmetastatic MTC, a cancer that attacks the thyroid’s calcitonin-producing parafollicular C cells.
Sharing Information, Warnings, and Guidance
ThyCa board of directors member Bill McClain, 67, is one such “meddie.” The retired businessman, who ran a family-owned specialty chemical company, lives in Milford, Connecticut, with his wife Galina; he’s written 8 books, 3 of them on MTC.
“MTC is unlike any other cancer in what it looks like to the surgeon, how it spreads, and how it’s treated,” said McClain, who was diagnosed in 2008 at the age of 54 and has undergone 2 surgeries. “Oncologists as a group have not been trained in endocrinology. They’re trained in how to treat cancer, reduce the tumor mass, and debulk it by radiation or chemo before the surgery. With MTC, it’s entirely different. Surgery is the first line of defense; chemo is only the last resort.”
Before his diagnosis, McClain said he didn’t even know what a thyroid was. Thirteen years later, he’s an expert on the disease. His first 2 books are a compilation of 150 stories by 110 MTC patients and their caregivers. The first book, finished in February 2015, sold more than over 6000 copies. His latest book on MTC, The Butterfly Sings: After the Diagnosis, came out in August 2019.
“We’ve got meddies in the group who were diagnosed 40 years ago, and others who were diagnosed and died within a year,” he said. “A lot of it’s got to do with genes. Some people are more susceptible, and the first 4 TKIs [tyrosine kinase inhibitors] — there are 6 now — have very bad side effects. For a lot of people, they stop working after 18 months.”
McClain currently takes levothyroxine sodium tablets (SynthroidⓇ) to replace the hormones his thyroid used to produce. Other than a bout of interstitial lung disease that was misdiagnosed as COVID-19 earlier this year, his condition has been stable. He’s currently working with MD Anderson and the University of California-San Francisco on a Pentagon-funded rare disease project focused on patient advocacy.
‘I Started to Feel Human Again’
Software program manager Wendy Hull, 55, is a fellow “meddie.” Diagnosed in November 1998 while pregnant with her second child, the San Francisco resident eventually underwent 5 surgeries as her cancer spread to her neck, liver, and lungs. Since 2007, she’s taken three different TKIs, deeply affecting her quality of life.
“One benefit of a cancer with no effective treatments is that your doctors don’t assault you with toxic chemo and radiation,” Hull said. “For the first 9 years, my treatment was limited to surgeries, each of which only took about 2 weeks to recover from. My cancer was symptom-free during that ‘watch-and-wait’ period, and I now call them ‘the glory years.’ ”
She added: “The symptoms arrived with my first TKI, and I no longer know which are from the drugs and which are from the cancer.”
These include gastrointestinal problems; severe and painful acne; physical weakness; joint pain; hand-foot syndrome so severe she couldn’t walk or use her hands; renal insufficiency; difficulty breathing; and weight loss. At one point, Hull dropped to 85 pounds.
After one TKI kept Hull’s disease stable for 9years, kidney damage forced her to remain on such a low dose that it eventually lost its efficacy. She was later approved for compassionate use and joined the LOXO-292 trial at MD Anderson in Houston, Texas. After about 3 months, Hull said, “I started to feel human again.” Eventually, she was able to return to work part-time.
“Whenever I see an email with someone’s name as the subject line, my stomach tenses up because I expect it to be a death notice of another friend,” she said. “Financial planning is frustrating, because I could live a long life, or I could die next year. My kids are graduating from college this year, and I am thrilled to be here for that milestone.”
Despite everything she’s gone through in the last 23 years, Hull said she’s grateful to have lived long enough to see 3 therapies approved by the FDA for MTC.
“The most recent one has even allowed me to have a reasonable quality of life,” she said. “I don’t know how long it will be effective, but I now have hope that it will tide me over until the next drug comes along.”
Hull added that ThyCa “has made it possible” for people like her to stay updated on MTC.
“Hometown doctors often have lots of experience with the more common forms of thyroid cancer, but most have never seen an MTC patient. I was diagnosed before guidelines existed, and back then it wasn’t uncommon for doctors to mistakenly treat an MTC patient with radioactive iodine. Finding ThyCa connected me with the tiny community of ‘meddies’ to swap warnings and guidance,” she said. “Doctors always express surprise at how educated we MTC patients are about our disease.”
Meanwhile, Labonte — who’s rebounded from a low of 149 pounds to his current weight of 172 pounds — is training for the October 2021 Boston Marathon. He’ll be running to raise money for pediatric cancer research at Massachusetts General Hospital in Boston.
“When I was first diagnosed, the 10-year survival rate for MTC was 27%. But that was prior to LOXO or any other TKIs. With the way treatments have been advancing so quickly, better options are available now,” said Labonte, who is still in the trial. “If this disease is going to get me, I want it to get me at my best.”