A Trail Blazer for LMS Patients
After receiving her ULMS diagnosis in 2009, Estelle Bar Ness set out to learn all she could about LMS. By educating her doctors and advocating for herself, she gained access to treatments that had never been used in her country—and opened doors for all of the patients who would come after her.
Estelle’s Thriver story begins in 2005, when she was first diagnosed with uterine fibroids (also known as leiomyomas). Fibroids are common and not usually a cause for concern, but late in 2009, Estelle began having some unusual abdominal pains. Her gynecologist told her that one of the fibroids had become very large and wasn’t getting enough oxygen, which was causing it to shrink and begin to die. He recommended a hysterectomy. Another doctor said that Estelle didn’t need to bother with surgery: at 48, she was fairly close to menopause, and the fibroids would likely shrink on their own soon. A third doctor joined the first in recommending a hysterectomy, and Estelle decided to proceed with it.
Estelle, who is originally from South Africa, was living in Israel, and morcellation of fibroids was not uncommon there. When she checked into the hospital for her hysterectomy, a secretary told her that she needed to leave a check as a deposit in case the doctor decided morcellation was necessary and insurance refused to cover it. When the hospital gave her deposit back, Estelle felt that she had also been given back her life: the surgeon had recognized that her 6.5 centimeter “fibroid” was actually cancerous and removed it intact. Since the tumor was attached to her omentum, it was also removed.
At first, Estelle hoped that since her tumor had not actually been contained in her uterus, the diagnosis she’d received (uterine LMS) might be incorrect. She sent her pathology back for review, hoping that what she had had was a GIST (gastrointestinal stromal tumor)—a kind of tumor for which a medication was available. A GIST test came back negative. Ultimately, the tumor did prove to be LMS, and Estelle joined the ACOR (Association of Cancer Online Resources) email list, L-M-Sarcoma.
The list became a lifeline. List members included their signatures—quick summaries of their LMS histories—in their messages, making it easy for Estelle to find other patients whose experiences were similar to her own. After her surgery, Estelle’s doctors had begun recommending that she receive radiation. She was uncertain about this recommendation—although Israel is a world leader in technology and medicine, it is a small country, so none of her doctors had seen a high enough volume of LMS cases to truly be considered LMS experts. Estelle turned to the ACOR list for advice. The women she spoke with there said, “Only irradiate when there’s something to irradiate.” She had nothing—her tumor had been removed.
As an occupational therapist, Estelle had the expertise necessary to do her own medical research and reading, and as she sought to understand her options, she devoured every article about LMS that she could find. (It was very helpful that she spoke English, since most research is published in that language.) She read that survival rates for ULMS patients who had wide-field radiation were no better than for those who had not had it, and that this kind of radiation could have devastating side effects. Estelle had her radiation planning session and got her tattoos, but she kept looking for alternatives.
Estelle soon discovered a treatment she wanted to try. Some friends who visited her from California showed her LMS expert Dr. Martee Hensley’s article on a new chemotherapy treatment protocol using gemcitabine and docetaxel. When the first doctor she showed the article to pooh-poohed it, saying it was just a retrospective study and had a small sample size, Estelle sought a second opinion from Dr. Robert Benjamin in the sarcoma unit at MD Anderson. Estelle had her own medical data translated from Hebrew to English and forwarded it to Dr. Benjamin. He told her that since LMS is a systemic disease and since she no longer had a known tumor to target, chemo would be more appropriate than radiation. Estelle faxed the hospital to cancel her first radiation treatment and began searching for a doctor who would prescribe gem/tax.
Fortunately, Estelle was able to find such a doctor—but gem/tax was not covered for LMS in her healthcare system (though it was covered for other cancers). The gem/tax treatment was supposed to be started within 8 weeks of diagnosis, and Estelle knew time was running out. So she decided not to let the lack of coverage hold her back. Her friends in California approached the members of their synagogue about raising money to buy the medicine for Estelle, and they succeeded—the first dose was brought across by a volunteer who was visiting Israel. By the time she was ready for her second round, she had convinced her insurance to cover it, in her usual determined and direct way. She gathered letters from her doctors and brought them to the insurance office in person, then sat in front of the representative with her nose bleeding and her hair falling out and asked for what she needed. She got it. While Estelle’s own determination played a big role, she also credits Israeli culture; in her experience (30 years’ worth), Israelis are very compassionate and always willing to try to make things work to help someone who is in need.
Chemo was something of a nightmare for Estelle; she had many harsh side effects. So she was not happy when, as she approached the end of her four planned rounds, her doctor said, “Well, you’ve done four—you might as well go ahead and do six.” She said, “Show me the article that says six rounds gets better results than four.” There was no such article, so they dropped the subject.
Estelle continued to research LMS treatments. She was now very aware that while her doctors had to try to keep up with information about 50+ different kinds of sarcomas, she only needed to become an expert in one, and everything she learned had the potential to make a difference. At that time, testing tumors for estrogen and progesterone sensitivity was new and not widely practiced, but Estelle asked to have her tumor checked. She learned that it was 100% sensitive to estrogen and thought, “This is what is going to save me!” She proceeded to learn all she could about AI (aromatase inhibitor) therapy, which was also very new and had not yet been used in Israel. She gathered up some articles, showed them to her gynecological oncologist, and said, “What do you think? Could we do this after the chemo?” The doctor replied that while she had asked for gem/tax and received it, he was not operating a shop where she could just come in and pluck whatever she wanted off the shelf.
Undeterred, Estelle sought the opinion of a sarcoma expert at the Brigham and Women’s Cancer Center in Boston, who advised her—and her doctor in Israel—that letrozole was a very appropriate therapy in her case. Getting the letrozole, just like getting gem/tax, required some improvisation, but Estelle figured it out—and at one of her later 6-month scans, her doctor asked how she had managed to acquire it. He had begun recommending it to other patients, and it soon became widely used in Israel.
There were some complications with taking letrozole. Chemo had thrown Estelle straight into menopause overnight, and her doctor had said that her ovaries would never recover. But they did—and it’s not possible to take hormone therapy when your ovaries are functioning. For the next 3 or 4 years, Estelle had to receive regular injections to keep her estrogen levels down.
Estelle has been NED since her initial surgery and has followed the standard protocol for scans. Now, after 10 years, she will be moving to have them every 18 months. Her oncologist had been wanting her to stop letrozole for a while, and though she had few side effects from it and found it psychologically helpful (if nothing else!), she recently did. She says she will never know whether chemo was necessary, since she had no tumors at the time she underwent it and has not had any since, but she is feeling just fine now—she has had no serious lasting aftereffects from her treatments. This might not have been the case had she gone ahead and followed the advice of every single professor that she consulted prior to choosing her treatment plan. To this day, she remains thankful and relieved that she never did wide-field radiation treatment.
As time has gone on, Estelle has been able to put LMS behind her more and more. But she still gets occasional phone calls from other patients who are seeking advice. A friend of Estelle’s who had breast cancer wrote a book about cancer survivors that included Estelle’s story, and word about it spread. Israel has only a few specialists and survivors, so other patients are often eager to get her tips on navigating the system.
Estelle wants newly diagnosed LMS patients to know that it is important to follow up on anything odd in their scans. Estelle had a full-body PET/CT scan early on that showed a suspicious mass in her thyroid, but she might never have learned about it if she had not been getting two separate radiologists to review her scans. The mass turned out to be a second cancer (not LMS), and she had it removed in 2011. Estelle also recalls one scan report in which the radiologist mentioned being able to see that her uterus and ovaries had been removed—but her ovaries were still there. Doctors are human and can make mistakes, so patients need to speak up when they notice something that concerns them.
Estelle also wants to remind others that there is hope, and that they should not let discouraging statistics and advice stop them from pursuing treatment. In her job as an acute care worker, she sometimes has to argue with doctors who do not fight for patients because statistically they have little chance of surviving. Estelle’s own initial prognosis was bleak—at that time, statistics suggested that the 10-year survival rate in cases like hers was 15%. Some of her doctors and colleagues seemed to fully expect that she would die, but here she is 10+ years later, doing extremely well. At age 59, Estelle has learned that life is short and ending soon for all of us. People who have not had a life-threatening experience may believe that life goes on forever and make a huge fuss over nothing; she knows everything is temporary and appreciates her life all the more because of that.
If you are a leiomyosarcoma survivor of 7 years or more, please share your story with us! Pass the hope forward 2LMSDR@gmail.com