Hugo Chavez and rhabdomyosarcoma

By Joan Darling and Suzie Siegel

Venezuelan President Hugo Chavez may have been the most famous and most powerful person to ever have rhabdomyosarcoma.

If the disease was indeed rhabdomyosarcoma, it was very unfortunate that he chose not to make this fact public. Regardless of what one might think of his politics, Chavez was a prominent and influential figure, and his openness could have resulted in publicizing the plight of thousands of current and future rhabdomyosarcoma sufferers, especially older adults for whom there is practically no effective treatment.

Publicity brings awareness, and awareness brings demands for more and better treatments. By announcing the type of cancer he had, the family of Chavez, so proud of bringing political change to their own country, could have a positive impact on the lives of people throughout the world.

Before his death March 5, Chavez had undergone treatment for cancer in his pelvis for two years. He never specified what kind of cancer he had, but there was widespread speculation that he had soft-tissue sarcoma, specifically rhabdomyosarcoma, as journalist Dan Rather reported last year. After his death, Reuters and other news agencies said the sarcoma began in his psoas muscle.

Chavez and his vice president suggested his enemies might have injected him with cancer. But a healthy person's immune system would fight off such an injection.

Rhabdomyosarcoma is a rare cancer of the voluntary, or striated, muscles. About 350 children, adolescents and young adults are diagnosed with it each year in the United States, along with a small number of older adults.

Children and teenagers are treated extremely aggressively to beat what is an extremely aggressive cancer. They typically receive numerous rounds of multi-agent chemotherapy and radiation, as well as surgery, if feasible. Side effects of treatment can be debilitating and are often life-threatening.

If the cancer hasn't spread by the time they are diagnosed, young people have about a 70 percent chance of surviving. In contrast, older adults have less than a 5 percent survival rate.

Adults often have a different subtype of rhabdomyosarcoma that appears less responsive to treatment. Also, the oncologists who treat adults are not as familiar with this disease or the latest treatments as are the pediatric oncologists who treat children. Finally, just by being older, adults may not be able to withstand the rigors of the extended and intense treatment, which can take more than a year to complete.

Given political expediency, it is not surprising that a diagnosis of rhabdomyosarcoma would be hushed up, particularly as Chavez was up for re-election in 2012. It would have been very discouraging for his supportive constituency, and likely devastating to his political allies, to find out that he was facing an extremely bleak future.

Up with patient services, down with doping

By Suzie Siegel

Some people affected by sarcoma have heard of the Livestrong Foundation but not the Sarcoma Alliance, even though our missions are similar.

Meanwhile, many survivors have followed the Lance Armstrong coverage, not realizing they took legal forms of EPO.

Armstrong has admitted blood doping, including the use of erythropoietin, commonly called EPO. The hormone regulates the production of red-blood cells. Erythropoiesis-stimulating agents, or ESAs, increase red-blood cells, which deliver more oxygen to muscles, increasing endurance. These drugs are sold legally under the trade names of Epogen, Aranesp and Procrit.

“We used to give it all the time," said sarcoma specialist Gina D’Amato, M.D., a board member of the Sarcoma Alliance. Like me, many patients got shots when our red-blood count fell too low for us to continue on chemo. "Its use expanded until studies found that continuing to give it once blood levels were normal can cause serious complications.”

ESAs remain useful for various conditions, such as anemia in patients with kidney disease, she said.

The FDA black-boxed the drugs in November 2007. The information warns that they have “not been shown to improve quality of life, fatigue, or patient well-being.” Instead, the FDA said, they increase the risk of death, stroke, blood clots, heart attacks and the progression or recurrence of tumors. The drugs “shortened overall survival and/or increased the risk of tumor progression or recurrence in clinical studies of patients with breast, non-small cell lung, head and neck, lymphoid, and cervical cancers,” the FDA said.

The same is presumed to be true in sarcoma patients, said D’Amato, who sees patients at Georgia Cancer Specialists in Atlanta. Because of the risk, oncologists are warned to give the drugs only to patients with metastatic disease who cannot be cured by chemotherapy, not to those getting chemo in hopes of being cured.

“There’s a theory that sarcomas have erythropoietin growth-factor receptors,” and thus, it’s possible that an ESA could stimulate slow-growing tumors, she said. A triathlete, she said she would never dope, of course. “It’s a shame that there’s so much pressure on athletes in professional competitions.”

In endurance sports, "it's bad to push your hemoglobin higher for the tiny advantage it will give you," said Ellis J. Neufeld, M.D., Ph.D., associate chief of the division of Hematology/Oncology at Boston Children's Hospital, a teaching hospital of Harvard Medical School. "It is clearly dangerous."

Armstrong stepped down from the board of Livestrong last year, and the Lance Armstrong Foundation changed its official name to the Livestrong Foundation. 

People who think Livestrong raises money for research are mistaken. Like the Alliance, Livestrong advocates for research, but doesn't fund it. Instead, both nonprofits focus on patient services and awareness.

In the fight against cancer, the vast majority of money goes toward basic science research, said Doug Ulman, (right) president and CEO of Livestrong in Austin. Ulman, who survived chondrosarcoma and melanoma, responded to email questions last August.

“While research is absolutely important, the needs of patients and families today are also critical,” he said. "Even with better treatments being developed, individuals and families will still endure the diagnosis, treatment (hopefully successful) and post-treatment survivorship. There will always be a need for survivorship services.

“I think that historically patient services have not been viewed on the same level as other medical services. They have been seen as add-ons or nice-to-haves rather than critical to a patient's survival, well-being and quality of life. That is changing, and we must continue to prove the impact of these services, and the value that patients and survivors place on them.”

Armstrong founded Livestrong in 1997, around the time that Wendy Sommers started giving information and support to other sarcoma patients. She answered emails and telephone calls from around the world, said Alliance board member Kevin Plottner of Alameda, Calif., who was her husband and designed the Alliance’s first website.

She became friends with another sarcoma patient, Suzanne Leider, who had the same doctor. After Wendy died in 1999, Suzanne founded the Alliance in her home in Mill Valley, Calif.

“You are not alone” is our motto, and our mission is guidance, education and support. Like Livestrong, we help people navigate the health-care system. Livestrong refers people to a nonprofit that provides peer-to-peer support. We run a similar program. We  help people get insurance reimbursement, access good treatment, and find clinical trials. We have various ways to connect people with one another so that they can give emotional support, with resources for children and young adults. We also give grants to help reimburse people who get second opinions.

The enormous Livestrong offers help to all cancer patients, while the Alliance is much smaller, reflecting the rarity of sarcoma. To illustrate our difference in size: Ulman's salary is higher than our annual budget.

But that doesn't mean we're insignificant. The National Cancer Institute has a list of "National Organizations that Offer Cancer-Related Support Services." Search it for "sarcoma" and the Alliance will pop up. The same goes for the American Cancer Society and Cancer.net, the patient-focused site for the American Society of Clinical Oncology.

Livestrong.org doesn't link to us. A search for "sarcoma" will yield stories of survivors. (We also offer stories of survivors, as well as those who have died but whose lives still can inspire and teach us.)

Livestrong.org partners with Livestrong.com, a for-profit website on diet, exercise and other lifestyle issues. If you search the site for "sarcoma," you will get links that people have paid to appear first. Next comes an article that talks about a sarcoma diet and "sarcoma-friendly foods."

No link has been established between diet and sarcoma, as we explain on our nutrition page. (As part of lifestyle issues, we also have pages on exercise and sexuality.)