When you’re diagnosed with a rare cancer

With few medical options, patients take their lives into their own hands

In New York City, indoor cycling is a contact sport. A few minutes before Spinning class at a midtown gym, most women are already on bikes and pedaling hard to warm up; those who didn’t sign up in advance jostle anxiously at the door, hoping for a no-show.

Amid this sea of black-clad intensity, Jennifer Goodman Linn stands out like a burst of sunlight. She has wrapped her hair with an orange bandanna emblazoned with the words Cycle for Survival and sports a bright lemon-yellow jersey. She breezes past the jockeying around her, stopping to give the instructor a hug, then smiles quietly as she settles on a bike and sets the resistance. Arms poised, she’s calm and happy as the first strains of Kanye West’s “Heartless” pulse, a woman exactly where she wants to be.

That she is here at all is some kind of a miracle. Five years ago, at age 33, the marketing executive learned she had soft tissue sarcoma, a type of cancer that attacks body tissues such as nerves and muscles and strikes only 10,000 Americans each year (compared with 200,000 breast cancer diagnoses). She has slogged through three heartbreaking recurrences, three courses of chemotherapy to shrink her tumors and four grueling surgeries to remove them. Cycling has been the one constant: She begged her doctors to set up a stationary bike in her hospital room, and when she returned to class after her first remission, bald and weakened, her instructor and fellow riders welcomed her with applause. Moved by how her sport had helped her heal, in 2007 she launched Cycle for Survival, an event in which teams of riders hit the gym and pedal for hours to raise money for rare cancers. “I realized that I could either admit defeat or use the cancer to do a good thing,” she says. “The choice seemed clear.” 

Linn’s medical journey, on the other hand, has been anything but. Her first symptoms were odd but not particularly alarming: night sweats, a chronic cough and a sudden, sharp pang in her abdomen one afternoon while she was playing tennis with her husband, Dave. “I was losing weight,” she recalls, “but my pants were tight.” Was she pregnant? No, although the newlyweds were trying. And when Linn began taking her temperature to see if she was ovulating, she found she had a fever. With her worry building after several weeks of symptoms, Linn saw her doctor, who ordered blood tests, followed by a series of scans.

When the radiologist saw the results, he bluntly informed her, “You have a big tumor in your abdomen.” Referred to a surgeon who would remove the mass — which was the size of a cantaloupe — Linn learned it could be sarcoma. Yet, like many people, she had never heard of the word sarcoma and didn’t grasp what it meant. “I know it sounds strange, but because the surgeon never used the word cancer, and because I was so young, fit and healthy, I assumed it wasn’t even a possibility. My main concern was that they take [the growth] out without doing a hysterectomy, so I could still have a baby someday,” she says. “I was in deep denial.”

Watching and waitingEven as she was wheeled into surgery, she still hoped the mass would turn out to be a uterine fibroid. Only in recovery did Linn learn that it was indeed cancer; the medical team had removed it as best they could, along with part of her colon, appendix and abdominal tissue. When she and Dave arrived for her first oncologist appointment a few weeks later, reality finally hit full-force. It was, Linn says, “the first time I had an out-of-body-scared moment.” That doctor told Linn and her husband that her odds of surviving five years were 50 percent — no better than a coin flip. Worse, there was no medical protocol for how to proceed.

“When Dave and I found out I had a rare cancer, we immediately wanted as much information as possible,” Linn says. But facts were in short supply. As with all orphan cancers, the term used for any type that affects fewer than 200,000 Americans at one time, research was limited. The “big four” cancers — breast, lung, colon and prostate, which together account for about half of all new cancer cases in the United States — receive the lion’s share of funding and attention. “With common cancers, there is more data, along with collective experience, to develop guidelines for treating these diseases,” says David G. Pfister, M.D., chief of head and neck oncology at Memorial Sloan-Kettering Cancer Center in New York City. “The more you see something, the more experienced you get at dealing with it. We know if one drug works better than another and are able to understand the course of the disease.” With orphan cancers, most physicians and patients have no blueprint. “In terms of treatment, there aren’t many patients going through what I am,” Linn says. “And with such a small pool, there isn’t much incentive for drug companies to invest in research.”

Getting a cancer diagnosis is an isolating experience under the best of circumstances. But minimal medical options and few support groups mean people with orphan cancers are apt to feel especially singled out. “It’s lonely and frustrating,” admits Linn, who has gone through a series of different chemotherapies at Memorial Sloan-Kettering. Recently, her oncologist told her she is free of measurable disease. Remission is normally cause for celebration, but Linn has been given good news three times before, only to see the cancer return each time. When she asked what came next, the answer was less than reassuring. “I don’t know,” the doctor told her. “There’s a 50-50 chance that it’s coming back. We’ll keep scanning you every 10 weeks, and if something turns up, we’ll deal with it.”

It’s the uncertainty patients with orphan cancers find most agonizing — “Watching and waiting is the hardest part,” Linn says — especially coming on the heels of baffling symptoms and catch-as-catch-can treatments. Yet despite the challenges, people with orphan cancers are working urgently — swapping advice online, pushing for more clinical trials and raising awareness through efforts such as Cycle for Survival, which is now run by Memorial Sloan-Kettering. “I needed an outlet, but I also created the organization selfishly,” Linn says. “If I leave it up to someone else to develop new therapies, it might never happen.”

Awareness brings money, which in turn fuels research, and last year’s federal stimulus package spurred new grants for the study of orphan cancers. “This is the era of orphan diseases, in large part because of the patients,” says Alexandria T. Phan, M.D., associate professor in the department of gastrointestinal medical oncology at the University of Texas M.D. Anderson Cancer Center in Houston. “Their voices are even more important than doctors’, because it’s the patients who get the rest of the world excited.” In the process, they are changing the destiny of almost anyone with cancer, uncommon or not, creating new treatments and forging a path to survival where none existed before.