We loved the Sarcoma Exchange!

By Suzie Siegel

The Sarcoma Exchange proved so successful that even before it ended, survivors were asking about the next one. 

“We were so encouraged by the outpouring of participation by the sarcoma patient, caregiver and survivor community. We had 147 attendees, but many more expressed that they wanted to attend,” said Alyssa O’Driscoll, executive director of the Sarcoma Alliance. “We’re planning to make this a regular part of our programming should funding continue.” 

The Alliance, founded in 1999, is an international nonprofit dedicated to the education, guidance and support of people affected by sarcoma. Although survivors and families meet each other at Alliance events, this the first time it has held a patient-education conference. 

The conference was July 20-22 in Atlanta. Patients and caregivers came from 29 states and represented 21 subtypes. The patient who had survived the longest was Susie Wright of Cairo, GA, who was diagnosed with synovial sarcoma 48 years ago. The subtype with the most survivors in attendance was leiomyosarcoma (LMS).  

LMS survivor Sharee Whitmer (left) and Lisa Kessler, whose husband survived LMS, but their son did not.    

"The information gleaned from the sarcoma oncologists and researchers empowered me in a way that really let me feel I have some options that I have not exhausted," said Sharee Whitmer of Madisonville, VA, an LMS survivor. She came with her husband and their therapy dog, Stella. "It reassured us that the sarcoma oncologist and team that I am with now are staying well-informed and that I am indeed in good hands. And when and if the time comes to move on and find another team, I have many other choices.

"For us to have some idea of how up-do-date our care and our options, including trials, really are, it is imperative to attend conferences aimed at educating and empowering patients. Not everyone is seen at a large sarcoma center.

"The BIG take away from the conference for us was to really be able to connect in person with other sarcoma patients and their caregivers that we have met through online support groups. And during the conference to be able to meet so many more that we would not have met if it were not for attending the conference. Knowledge is incredibly valuable for patients like us, and having the opportunity to exchange our experiences while enduring this diagnosis and the treatments that follow are invaluable."  

Mary Prince, Lea Custer and me

Mary Prince of Portland, OR, came from the farthest away. Her sister, another LMS survivor, lives in Murrayville, GA. 

“Attending this wonderful event with my longterm-thriver/ BFF / sister, Lea Custer, was the highlight of the year for me. Meeting other LMS survivors and meeting all the wonderful medical professionals and hearing what's coming up on the horizon made it an incredible conference. Love was everywhere; you could feel it!" Mary said. "A special shout out to Dr. Gina D'Amato, Lea's primary sarcoma physician, who made it all happen.”  

Dr. D’Amato, a Sarcoma Alliance board member, practices at Northside Hospital’s Cancer Institute in Atlanta. She’s grateful that sarcoma specialists from across the South volunteered to speak, along with her colleagues from Northside.

The medical oncologists who spoke were Dr. Robert Benjamin and Dr. Shreyaskumar Patel from MD Anderson Cancer Center in Houston, Dr. Jonathan Trent from the Sylvester Cancer Center in Miami, and Dr. Richard Riedel from Duke Cancer Institute in Durham, N.C. Dr. Scott Davidson, a surgical oncologist, and Dr. Hamilton Williams, a radiation oncologist, attended from Northside.

Breakout sessions covered patients in treatment, survivors and caregivers.

Amanda Smith with the late Roger Henderson

"Never quit the fight. That was my mindset," said Amanda Smith of Havelock, NC, who facilitated the caregiver section. She lost her boyfriend, Roger Henderson, to chondrosarcoma. An LPN, she's in school to become an RN. 

Sarcoma Alliance board member Marites Tullius, a nurse practitioner, spoke on palliative care. From Northside, registered dietitian Rebecca Perez spoke on nutrition; social worker Myra Bazell, stress management; RN Debbie Bickes, patient navigation; and occupational therapist Melora Rennie, exercise and fatigue.

Osteosarcoma survivor Woody Roseland, whose "S#!%Cancer Patients Say” brought knowing laughs, entertained us one afternoon. That night, hypnotist Ricky Kalmon evoked a great deal of silliness from his targets.  

Sponsors were drug companies Lilly, Eisai, Ignyta, Novartis, Immune Design and Epizyme as well as  Northside Hospital’s Sarcoma Program. Thanks to them, we had free rooms at the Marriott Perimeter Center Hotel and delicious meals. 

  

Presentations from the conference are posted here: https://sarcomaalliance.org/event/sarcoma-exchange-2018/ Here are a few tidbits:

Dr. D'Amato with Dr. Benjamin

"Stage 4 has a bad connotation. I don't even use it in clinic,” Dr. D’Amato said. The problem with "stage 4" or "terminal" is that these labels include people whose sarcoma has spread far and wide as well as those with oligometastatic disease, which means they have only a few small tumors in one or two distant locations. Some people think "stage 4" or "terminal" means they are going to die soon. They may decline treatment that could help them live longer.

Dr. D’Amato noted that "remission" applies to people who no longer have any evidence of sarcoma after their initial treatment. The term for people who have no evidence of disease after they have been treated for metastatic sarcoma is NED. For example, my original leiomyosarcoma was 2C. Soon after, one small lung metastasis was found; 18 months later another appeared. Thanks to radiation, chemo and surgery, I’m NED from oligometastatic disease.

Dr. Riedel

“When you’re educated, you’re empowered,” said Dr. Riedel, who spoke on immunotherapy. “The future is bright.” 

Don’t let community doctors take you off of a drug just because your tumors aren’t shrinking, he said. His mantra is: “Stable disease is a good thing.”

“Patients want their sarcomas to shrink and disappear, but doctors are happy with stability,” Dr. D’Amato stressed. Sarcoma specialists may try new treatments only if tumors start to grow. 

Some times the tumor stays the same, or even looks larger, but is actually dead after treatment. Some tumors shrink, but not enough to meet the definition of shrinkage in clinical trials. 

Researchers refer to “progression-free survival” (PFS) when tumors stay stable. Some people don’t think the FDA should approve drugs based on stability. They want clinical trials to prove that the people who got the new drug lived longer, which is called “overall survival” (OS). 

Dr. Benjamin thinks PFS can be a good sign that a drug is working. The problem with OS is that it measures  things that weren’t part of the clinical trial, he said. For example, the clinical trial on doxorubicin (adriamycin) + olaratumab (Lartruvo) compared it with dox by itself. But people who got olara may have gone on to get better treatment, improving their survival.  That’s why MD Anderson is doing a clinical trial to compare dox + olara to the older combination of dox + ifosfamide.

Dr. Patel

Dox + ifos is a common treatment for sarcomas, and some community doctors may try to treat all sarcomas with it, Dr. Patel said. They shouldn’t, he added. They need to look at each subtype individually because some, such as alveolar soft part sarcoma (ASPS), don’t respond to this chemo combo. 

        

Because immunotherapy is in the news, he spends a lot of time educating patients on why chemo may be better than immunotherapy for their particular sarcomas. He also discusses the advantages of a clinical trial vs. standard treatment.

“Patients must have information to make a decision,” Dr. Patel said.

A variation on doxorubicin, called aldoxorubicin, “clearly has less cardiac toxicity. It’s a very good drug,” Dr. Benjamin said. We await the results of clinical trials and a decision by the FDA. 

Dr. Trent

Dr. Trent joked about the crazy names that pathologists give to sarcoma subtypes, such as dermatofibrosarcoma protuberans (DFSP). Nevertheless, medical oncologists like Dr. Trent greatly respect sarcoma pathologists. You can’t get the best treatment without an accurate diagnosis. 

For example, most sarcoma cells look like spindles under the microscope, Dr. Benjamin said. If pathologists cannot identify the tumor’s subtype, they may use the catch-all term of “spindle-cell sarcoma.” 

Instead of taking a tissue sample, some doctors are looking at tumors’ DNA circulating in the bloodstream (called ctDNA). They want to see which treatments are working and which might work for the patient in the future. Although these “liquid biopsies” are still experimental, Dr. Trent said: 

“This is what the future is.”

Boston Strong athlete honors her brother

By Suzie Siegel

Shawna Kleftis rode her bike across America in 2014, ran the Boston Marathon in April and paddled across the rivers and lakes of the Adirondacks in September. She has raised as much as $25,000 for cancer patients.

“I’ve had the immeasurable support of family, friends and total strangers. It was never just me,” the 24-year-old said. “I feel like my job is easy. I just have to talk and write about what inspires me – my brother – and people respond. It never ceases to amaze me.”

On October 11, 2009, my older brother, Cullen, passed away from osteosarcoma at the age of 19,” she wrote on her fundraising page for the Adirondack Canoe Classic.

“Every time I sign up for another so-called crazy physical challenge – be it a bike ride across the country, a marathon, or this [canoe] race – I think about my brother and the level of mental and physical strength he exuded throughout his four years of being sick. I think about cancer’s inability to take away his drive, even as it ate away at his body. I carry his spirit with me whenever I think I cannot bike another mile, run another step, or paddle another stroke. It is with him in my mind and heart that I seek to raise funds for the Sarcoma Alliance.”

Shawna, who works in law enforcement in Boston, participated in the Adirondack race as part of Ocean of Hope, the Alliance’s largest fundraiser. She raised $2,877 and is still accepting donations at: https://www.crowdrise.com/donate/project/oceanofhope/shawnakleftis She entered the race before she knew that Cullen had received a grant from the Alliance's Sarcoma Hand in Hand: The Suzanne Renée Leider Memorial Assistance Fund to help with his trip to Houston to get a critically important second opinion.

The Adirondack race, known as the 90-Miler, was a Kleftis family affair. With her mother and younger brother as pit crew, Shawna and her father shared the canoe for their first race together. Not that they were new to water.

“I had grown up sailing and doing all variants of water sports on Lake Ontario,” she said, and she was on the sailing team of her college her freshman year. She and her father started slow but made good time the second day. She’s proud they paddled 69 miles and survived some hardships despite the last day of the 3-day event being cancelled due to high winds.

“Much to my Dad’s dismay, I insisted on camping out each night between the legs of the race, like Native Americans and early settlers would have done on their travels through the same series of lakes and rivers.”

During one portage – when they had to carry the canoe between bodies of water – she disturbed a hornet’s nest while looking for a place to empty her bladder. She was stung about 20 times.

Shawna is an all-around athlete. “In high school, I played varsity soccer and volleyball and ran cross-country. I knew how to play hockey from growing up with my brothers and watching their games, and playing pickup hockey in our driveway. So I decided when I got to Wellesley College that I wanted to play hockey instead, and I sported a #11 jersey in memory of Cullen, whose jersey/lucky number was always #11.”

On her 18th birthday, she went skydiving with her father and Ridge Thorbahn, who had osteosarcoma and two other cancers. He had known Cullen. “When my family learned he wanted to skydive, we paid for his dive. Cullen got to jump before he died; we made sure Ridge also got that chance.”

In 2014, Shawna earned her bachelor’s degree in International Relations-Political Science and Middle Eastern Studies. She had spent a year abroad studying Arabic, and we talked about how cancer is not discussed as openly in many countries as it is in America. “It was quite interesting to learn and witness firsthand how cancer was perceived while living in Jordan,” she said. “I would say in general, it's kept hush-hush. There's almost a sense of shame surrounding disease, as well as a sense of resignation (things happen at God's/Allah's will).

“Beyond that, I think cancer is in some ways ‘new’ to the region because it's still very much developing, and cancer rates tend to increase as countries develop. The prevalence of certain behavioral and environmental factors unique to the culture and region also dictate which types of cancers people are being diagnosed with. As you can imagine, the popularity of smoking manifests as lung cancer, as being in the desert and unyielding sun manifests as skin cancers.”

Only hours after college graduation, Shawna flew to Baltimore to ride 4,000 miles to San Diego in the Ulman Cancer Fund for Young Adult’s 4K for Cancer. (The founder survived chondrosarcoma.)

“It was very appropriate,” she said. “Cullen received treatment in Baltimore at Johns Hopkins, and it was also his birthday weekend. He would have been 24 the day before I started biking.” Cyclists stopped along the way to talk to young people about cancer and visit them in hospitals. Shawna also helped with logistics and publicity. She had hoped to raise $11,111.11, but she surpassed those lucky 11s to raise $14,560.

In April, she ran the Boston Marathon. “I had been dreaming of running it since my first year at Wellesley, which serves as the famous halfway point of the race. It was always such a celebratory day on campus, as it is for Boston and the surrounding areas. And I have always loved running – running has been my outlet all these years. After the bombings in 2013, my desire to run in the marathon increased tenfold. I finally got that chance this spring, and it was wonderful!”

 She and her family have organized other fundraising events and done various volunteer work. “I'm now trying to determine how I can get involved in volunteering on weekends at any of the local Boston hospitals,” she said.

“I know my Dad and I will be doing the 90-Miler again, and I'm also looking at some other paddle-kayaking events. The hardest part is being in Boston – the weather and seasons are not conducive to year-round training – which is unfortunate because I'm always training for my next adventure/physical challenge, regardless of weather!”