PATIENT STORY: Nancy Newsom Ridgway

Nancy Newsom Ridgway

This is the second post in our series of interviews with those affected by sarcoma. You can also read about leiomyosarcoma survivor Liselle Reppert.

If you're interested in participating in this series, please contact us to get the ball rolling.

Nancy Newsom Ridgway is a Texan and she does what it takes to get the job done.  She's a gentlewoman who never has an unkind word to say, but no one would ever call her timid. Nancy is strong and passionate about  family and God.  She is a wife, niece, parent, grandparent, great-grandparent - and she's a survivor. 

This is her story.

What is your sarcoma diagnosis?

Malignant Fibrous Histiocytoma (MFH), but they have changed the name of that type to Undifferentiated Phleomorphic Sarcoma.

In what year were you first diagnosed?

April 30, 2009….unforgettable!

Have you had surgery, chemotherapy, radiation treatments, something else?

I had surgery (resection and excisional biopsy) to remove a left quadriceps mass / medial margin left quad mass in the muscle.

They did the biopsy directly before the surgery and then proceeded with the surgery, and the surgeon got clear margins around the mass. They immediately took me for a chest x-ray after surgery to see if it had metastasized to the lungs (it had not).

I followed up surgery with 6 weeks of radiation every day. I had a wonderful doctor for my radiation, and I didn’t get much of a burn until the last week. I put pure aloe (no alcohol) on and it really helped. They gave me a lotion to use as well.

Had you heard of sarcoma before you were diagnosed?

When I was going in for the MRI with the lump on my thigh, I started researching and knew right away that sarcoma might be a possibility. Until then, I had never heard of it.

After having the first MRI and then getting the results to take to my oncologist, I looked up that photo on my MRI and compared it to one online of a sarcoma – they looked the same. I was sorry I looked it up!

What is your prognosis?

I am now 4.5 years out from my sarcoma, and so far all tests have been NED (no evidence of disease). They don’t really say remission now…it is NED.

I feel good about it, and at the same time I don’t take it for granted…and I know that it could come back. I have a really strong faith and if it does come back, God will be right there with me. I am thinking positively and if I make it past the five year mark, I understand that will be really good…not a guarantee, but really good.

I am very hopeful…and I pray a lot.

How old are you?

I am 63 years young!

Tell us a little about you.

My husband and I have lived in Texas most of our lives. We went to the same high school and have been married for 46 years. He has been my biggest support through cancer, although my family and friends have certainly all been there for me too.

We have two daughters, one is a nurse and the other teaches medical coding and works for a bunch of doctors. I have four grandchildren and two GREAT grandchildren. They are all very precious and special. My family and friends are my biggest blessings!

I also have two poodles who rule the roost here. I spend a lot of days letting dogs in and letting dogs out. I have a cat we adopted named “Bubba” (who has an identity crisis and thinks he‘s a poodle).

I was a florist in Fort Worth for many years at one of the largest florists in the USA at that time. Many holidays we would work into the night or all night long so Mothers and sweethearts all over the world could get their flowers on time. I chose to quit that job to take care of both of our Moms who needed help as they got older.

What is your biggest piece of advice for someone who is newly diagnosed with sarcoma?

Get a second opinion and go to a sarcoma specialist…maybe a third opinion if you aren‘t satisfied. I don’t regret getting all the opinions I could get because it helped me make a more informed decision. You have to be your own advocate.

Keep track of every test and everything in a notebook!

It's probably best not to believe everything you read on the internet about sarcomas! 

Always keep a sense of humor…you will feel better all around for it and you can always find something humorous.

"The surgeon herself even said it was probably a lipoma..."

Everyone has a diagnosis story. What is yours?

 I had this place in my thigh for months before it was diagnosed. It hurt. My rheumatologist looked at it before it was growing so large and he told me to put heat on it, and then my primary care physician thought it was probably a lipoma…I mean that was the obvious thing to think I suppose, and I don’t hold it against either of them for not knowing what it was!

The surgeon herself even said it was probably a lipoma and possibly a sarcoma. The sarcoma was most unlikely of the two…I get that. After several months of this, I had an MRI, then I saw the orthopedic oncologist and had surgery which showed it definitely was sarcoma.

Following the first MRI/surgery on my thigh, my oncologist thought I should have chemo and then radiation, and referred me to MD Anderson for a second opinion.

My surgeon, who was an orthopedic oncologist, said she would do radiation only, because of my already low white cells, which was (and still is) an ongoing problem. When I went to MD Anderson the first orthopedic oncologist I talked with told me he agreed with the radiation only, but when he talked with other doctors, they decided chemotherapy followed by radiation would be the better choice.

I asked them about the radiation-only option and they said that was my decision, but that without chemo the sarcoma would probably come back with a vengeance within 5 years. This was a very tough decision for me, and I didn’t want to do the wrong thing of course, or go against anyone’s best plan for me. I knew I was taking a chance, but I did have two oncologists and a radiologist all of whom thought 6 weeks of radiation would be enough for my treatment, so that is what I chose to have. 

My decision was right for me. I mean the final decision is ultimately up to us, and I had so many varying opinions, it was extremely confusing to me at that time. These are our bodies, and our choice…and heck yes, I wanted it not to come back….and my gut instinct was to go with the radiation only and a large part of the reason was because of my already low white count...and with chemo it would have plummeted.

My radiologist agreed with my choice, and said he thought I made a wise decision.

My husband and I had moved from the city to the country to retire. Six months after we got to the Texas countryside, I was diagnosed. I drove myself into town for radiation every day -an hour there and another hour home for those 6 long weeks.

How long did you wait to tell family/friends about your diagnosis?

I had part of my family in the waiting room when I had my surgery, so I didn’t have to tell them, the surgeon told my husband and some others, as I was waking up from anesthesia…and as in any family word spread pretty quickly I suppose.

How are you feeling today?

Today I am feeling great and I am 4.5 years out this month! I am very thankful to be alive and every morning when I wake up I am grateful.

My thigh still hurts a little bit to the touch. I still have tests regularly…having them this week and next as well. I hopefully will be able to tell MD Anderson things are still going well when I send back their questionnaire. They keep track of all of their Sarcoma patients.

I do have “survivors remorse” and I feel great remorse for friends who haven’t had it so good, it really grieves me that some of my fellow sarcoma patients keep having to fight it every single day. I really do care about all my sarcoma family and I keep them in my heart and my prayers.

　

"I have met so many wonderful, courageous, and caring people..."

Sarcoma diagnosis is a terrible thing, of course, but have there been any hidden blessings?

I have met so many wonderful, courageous, and caring people through having this cancer…and it is strange that blessings can come from being ill, but they clearly have.

I don’t know what I would do without my friends at Sarcoma Alliance.  They are funny and insightful and inspiring, not to mention courageous.

We all share this common bond…not the kind of bond we would have asked for, for sure, but there it is…and we all make the very best we can of it.

What keeps you inspired / motivated?

I love people and I like to do things for other people. Sometimes I go on overload…but I have always found I get back way more than I put in.

I totally love my volunteer job at Sarcoma Alliance and everyone involved is so sweet and kind. I have seen members go to the ends of the earth to get information when a friend needs it, and in helping someone new learn more about the things they need to know.

Music always inspires me as well…my family is musical and many of us play instruments. I love, love, love, to all get together to have music time. My brother used to play the keyboard professionally. My Aunt was a strings/orchestra teacher and she plays the violin beautifully, and even I dabble on the keyboard...and we all sing, some much better than others.

Nature truly inspires me too…always changing and always something new each day. I love animals and birds! I enjoy photography and all kinds of arts and crafts and painting. I love to travel… and have been to many foreign countries and I love bed and breakfasts because you can really get to know something about the people/cultures that way.

What is your favorite (clean) joke?

This teacher asked some children in a classroom to draw something really special to them…anything they wanted to. The teacher was going around the room and checking each one out and when she asked this little girl what she drew, the little girl said, “God”. The teacher said, “No one knows what God looks like“, and the little girl replied, “Well, they do now!”

If we came to your house for dinner, what would we have?

No telling…depends if I am in a hurry or I have a lot of time, if I am on my diet or if I am slacking!

I love to cook…and it would probably be Mexican food. My family loves my chicken enchiladas with sour cream sauce on them, or fajitas, beans, Mexican rice, and homemade guacamole with fresh cilantro in it. 

Nancy had her most recent MRI and CT scans after we'd completed the interview you just read. She told me the news was, "good and bad."  The lung CT showed some changes and her oncologist suggested another look in January. Nancy will be sending the results off to MD Anderson for another opinion.  Here's what she had to say about it, "...that's sarcoma for you...you have to stay on your toes with it and I am hopeful this is not sarcoma...but that being said, as a sarcoma patient, we have to be vigilant."

 Keep in mind that Nancy's story is just that - Nancy's story and experiences in her own words. The viewpoints expressed here do not necessarily represent those of Sarcoma Alliance and should not be taken as medical advice, though we fully endorse seeing sarcoma specialists and getting second opinions.

ANNOUNCEMENT: Sarcoma Alliance Store - Now Open

Sarcoma Alliance T-Shirt

Great big happy shopping news from us today:   Our Zazzle Store is open!

What the heck is Zazzle?  Basically, Zazzle allows us to easily offer a huge variety of  Sarcoma Alliance gear to our wonderful supporters. Like the t-shirt above.

And the snazzy luggage tag below....

Sarcoma awareness luggage tag from Sarcoma Alliance

And..oh my goodness...adorable clothing for your best friend....

How Cute is This? Doggy Shirt from Sarcoma Alliance

How about a little something for your morning commute...

Travel Mug - Sarcoma Alliance

You know what's really cool about Zazzle?  You can customize some of the items.

Want a stylish black shirt?  No sweat!

Sarcoma Alliance Black T-Shirt

And another great thing about Zazzle?  Returns. If you don't love it, they'll take it back. No questions asked.  We're pretty sure you're going to love everything there, though.  The Women's Shirt, for example, has more 500 reviews and received around 4.5 out of 5 stars.

As you use your swag from the Sarcoma Alliance Zazzle shop, you spread sarcoma awareness - and you'll be doing a great thing for others because a full 20% of everything you buy will come to Sarcoma Alliance.

So..what are you waiting for?  Go buy that keychain!

Researchers redefine sarcoma subtypes

By Suzie Siegel

Advances in science and collaboration across medical disciplines are redefining sarcoma subtypes.

Dr. Brian Van Tine

"Nowhere is that more evident than at CTOS meetings," said Brian Van Tine, MD, PhD, director of the sarcoma program at Barnes and Jewish Hospital at Washington University in St. Louis. "We are beginning to understand the complexity of sarcoma and how we will use this to design new therapies."

Dr. Van Tine will discuss new targets for metabolic therapies Thursday at the annual meeting of the Connective Tissue Oncology Society in New York.

"In the future," he said, "I believe we will not only use genomic information to subclassify sarcoma, but to also make treatment decisions based on the DNA sequence, the RNA expression, the activated proteins and the metabolites that are specific to an individual’s sarcoma. The work currently being done and presented at CTOS should soon have direct clinical application."

"Clearly the field of oncology is moving towards personalizing therapies and identifying new targets," said CTOS President Shreyaskumar R. Patel, MD, medical director of the sarcoma center at the M.D. Anderson Cancer Center in Houston."This trend is also true for musculoskeletal tumors, although slower due to the rarity and complexity of these malignancies."

The dictionary records the first use of the word "sarcoma" in 1804. In recent years, more than 50 subtypes have been identified, generally based on the normal cells they most resemble. For example, a leiomyoma (also called a fibroid) is a benign growth of smooth-muscle cells. A leiomyosarcoma is the malignant version.

Dr. Dei Tos

The World Health Organization classifies sarcomas. In 2002, for the first time, WHO used molecular genetics and immunohistochemical staining to refine its classifications, said Angelo Paolo Dei Tos, MD, who serves on the CTOS Board of Directors. He directs the department of oncology and anatomic pathology at the General Hospital of Treviso, Italy.

In January, WHO updated the 2002 categories. Dr. Dei Tos said he expects the next update in five or six years, as genetic aberrations are increasingly used to group tumors into categories.

This year, he said, WHO got rid of the subtypes “hemangiopericytoma” and “malignant fibrous histiocytoma,” commonly used in the 1980s and '90s. The former was divided into benign tumors; solitary fibrous tumors, which can be benign or malignant; synovial sarcoma; and malignant peripheral nerve sheath tumor.

Malignant fibrous histiocytomas were divided into leiomyosarcomas, rhabdomyosarcomas, dedifferentiated liposarcomas, undifferentiated pleomorphic sarcomas as well as lymphomas, melanomas and sarcomatoid variations of carcinomas.

In 2002, for the first time, WHO used clinical data to better determine the malignant potential of tumors, Dr. Dei Tos said. They were divided into benign, intermediate malignancies and malignant. The recent report substitutes two new categories for the term "intermediate malignancy":

• locally aggressive tumors that don't spread elsewhere, such as desmoid fibromatosis
• lesions that rarely spread (or metastasize), such as plexiform fibrohistiocytic tumor.

A few new subtypes have been added, such as pseudomyogenic hemangioendothelioma, he said.

International sarcoma meeting starts next week

By Suzie Siegel

At least 500 people from around the world are expected at the Connective Tissue Oncology Society's annual meeting Oct. 30 to Nov. 2 in New York City.

Dr. Patel

"CTOS represents the largest collection of sarcoma aficionados, including all disciplines – surgery, medicine, pediatrics, radiology, pathology and basic science. Also included are important components like nursing, patient advocacy groups and pharma partners," said President Shreyaskumar R. Patel, MD. Dr. Patel is the medical director of the sarcoma center and a distinguished professor of medicine at the University of Texas M.D. Anderson Cancer Center in Houston.

"Trends in this meeting include more detailed analysis of the genetic aspects of sarcomas, many of which have simpler defects like leukemias and lymphomas. Despite these similarities, it is not yet clear why leukemias and lymphomas are sensitive to chemotherapy while sarcomas are often resistant," said CTOS Program Chair Robert Maki, MD, PhD, a professor of medicine, pediatrics and orthopedics as well as medical director of the sarcoma program at Mount Sinai Medical Center in New York.

"There will be expert lectures on the development of new classes of drugs by Kevan Shokat, PhD, of the University of California-San Francisco, as well as a clinical lecture on sarcoma management by one of the recognized leaders in the field, Piero Picci, MD, of the Istituti Rizzoli in Bologna, Italy, one of the world's great bone sarcoma centers.

"In a similar light, management of complicated cases and new and emerging drugs to be considered for sarcoma treatment will be discussed at this year's meeting."

Our executive director will be there, as always. But we will also have five Alliance volunteers there, the most ever, at our own expense. We go to make connections and learn, in hopes we can help others.

Dr. Maki

"Sarcomas constitute fewer than 1 percent of cancers in adults, and about 15 percent of cancers in children," Dr. Maki said. "Perhaps, because of their rarity, they are a common diagnosis on television dramas like 'House.' But physicians don't see as many happy endings in real life.

"Sarcomas are cruel," he said. "Discoveries of new sarcoma treatments have been relatively few, compared with breast or prostate cancer. This is due, in part, to the funding set aside for these rare diseases.

"Our frustrations with the slow progress are balanced by the desire to help families, where we still too often see either children, young adults, or parents with young children dying of these cancers."

The Alliance recommends patients see experts. "But this is increasingly difficult," Dr. Maki said, "with the funneling of patients by insurance plans to general oncologists who don't see very many of these sarcomas as part of their practice."

The roots of CTOS go back 20 years, when a wealthy patient invited physicians and others interested in sarcoma to a meeting in Boca Raton, Fla. He believed better communication among experts would lead to better patient care. Herman Suit, MD, of Massachusetts General Hospital in Boston proposed that he and his colleagues keep meeting "to generate a more coordinated attack on sarcomas," Dr. Maki said.

CTOS, based in Alexandria, Va., formed in 1995. Its meetings alternate between North America and Europe. Next year's meeting will be in Berlin.

In 1999, a sarcoma survivor founded our nonprofit. The Sarcoma Alliance for Research Through Collaboration (SARC) was founded in 2003 at the University of Michigan in Ann Arbor. SARC brings together physicians from different institutions to collaborate on clinical trials. It will celebrate its 10th anniversary and discuss the latest on its clinical trials as part of the CTOS meeting.

PATIENT STORY: Liselle Reppert

Today, we begin a new feature - interviews with sarcoma patients, caretakers, family, and friends.

After talking the amazing Liselle Reppert into being our first interview, I closed my email with, "Let me know what you're thinking."

The first line of her two-part (!!) email back to me was, "What am I thinking? 'Oh Boy' and on so many levels."

Let's dive right in...

Liselle Reppert

What is your sarcoma diagnosis?

Undifferentiated Pleomorphic Leiomyosarcoma. There are characteristics of rhabdamyosarcoma that had them scratching their heads and may complicate my treatment later but for now we are going with leiomyo.

In what year were you first diagnosed?

My diagnosis started in May of 2013, but I found "Fred" in February of 2012. Yes. I sat on him for over a year and would most likely still be sitting on him if my friend Ashley hadn't taken control.  

Have you had surgery, chemotherapy, radiation treatments, something else? 

So far I have had two surgeries. One to remove "Fred," a 27cm incision to take out a 16x16x10cm section with clear margins. And another surgery to shoot blue dye into the lymph vessels when i stopped healing because edema kept re-opening my leg.   (Interesting stuff...made me pee like a Smurf.) 

And I found the Sarcoma Alliance while I was recovering with my wound-vac, which kept me in the hospital for 18 days.

I have only done one round of doxorubicin. 

Had you heard of sarcoma before you were diagnosed?

It was in college that I first heard of sarcoma and promptly forgot about it. Though I now recall every word of the brief lecture in my human anatomy classes.

"A lot can happen in 10 years
and I would like a chance to see it." 

What is your prognosis?

Since my last surgery I have had 3 CAT and 2 MRI scans. The plan was hunt, peck, and zap. Unfortunately, one lung met is inoperable and sitting in a nest that one-quarter of my blood volume passes through with every heartbeat. Now, we are looking to control its growth.

As I told my doctors, "A lot can happen in 10 years and I would like a chance to see it." Regardless, whether surgery advances or a cure is found, I want to help those who come after me. 

As things stand...I'm terminal.

That is NOT to say that sarcoma is going to kill me because it is NOT. From the moment we are born...we begin to die. That is what defines life. It is the definitive that separates life from existence. 

I am living.

How old are you?

44

"My inability to work dragged my family all
the way to the gutter. We live in a motel."

Where do you live?

Technically...I am homeless. My inability to work dragged my family all the way to the gutter.

We live in a motel. We could have moved out but God gave us the most incredible dog that can tell when i am going to fall. There are no apartments here that will accept a 95 pound dog without a service license and I have no way of getting him certified. We would rather live in a motel than risk losing my dog and the peace of mind he brings my family.

Tell us a little about you. Are you married, kids, pets? What do (or did) you do for a living?

I grew up in Long Beach, California; I live in Mount Vernon, Washington, with my husband, two grown sons, and my dog.

What is your biggest piece of advice for someone who is newly diagnosed with sarcoma?

My best piece of advice is be organized. NOTHING can prepare you for the chaos of hearing those three words from our nightmares. Getting organized gets your head back in the game.

Everyone has a diagnosis story. What is yours?

I'd lost some weight and the leftover skin flap shrank on one leg but not the other so I watched it. One day, I found a golf ball-sized sphere in the fat flap. 

"Oh great, a big cyst," I hoped. That was in February of 2012. I checked it periodically and by September there was no doubt I had a tumor that more than likely wasn't benign. Unfortunately, I had no insurance and having been disabled for the last eleven years and having landed in the gap between worker's compensation, social security, disability, and welfare, I had no hope of getting help. 

I went to the emergency room expecting to have to file bankruptcy as soon as they told me what was going to kill me and kicked me to the curb, but that's not what happened.  

Skagit Valley Hospital kept me and sent my labwork to California when the local lab couldn't give a clear diagnosis. They also got me the best oncology team they had and stayed on top of on welfare until they agreed to pay my ongoing bills.

Tell us why you name your tumors?

That is comedy relief. At first, my husband couldn’t even tolerate the word “tumor,” forget about lieomyosarcoma. Big scary “cancer” just ticked him and oldest son right off. My youngest understood that I think out loud when I’m getting a handle on a situation.

I thought of naming it “Henry” and the mets would have been “Winkler” and “Ford.”

My mother-in-love named the mets saying they were in my chest so they had to be girls.  My Sarcoma Alliance friends named a lymphnode that turned traitor and my named the latest met.

I have some more that need names. Any ideas?

How are you feeling today?

Actually, I feel surprisingly good, not at all what I expected.

"The only side effect so far is my
hair loss and that is no biggy."

What side effects of treatment are you experiencing?

Because of a pre-existing penchant for hyperemesis, we hit the antiemetics really hard.

The only side effect so far is my hair loss and that is no biggy.

What keeps you inspired / motivated?

How can I be so optimistic??? How can I not. From the get go, God has had His hand on this whole fiasco. 

Since you brought it up, how has your relationship with God been challenged throughout all of this?

My relationship with God is a little more blatant. He had always been obvious in my life, but I sometimes seemed to be sleeping on the job – letting Him appear to be taking a back burner in my active life.

When you were a kid, what did you want to be when you grew up?

As a child, I wanted to be a banker and after graduating from high school, I went into banking. Unfortunately, money weighs a ton and I injured my back forcing me to change careers. Several minor injuries later, two discs herniated leaving me partially paralyzed with chronic pain and facing full paralyzation. 

Sometimes God realizes you are stubborn and he pulls a pin to put on your brakes for you.

At one point, I studied to be a stenographer. I developed tendonitis. Rains...pours...I never understood what the lesson was. Still don't get it. :-P 

I just know He has a point. Somewhere.

One thing to keep in mind with this and all of our interviews - these are the words and experiences of the person being interviewed. The attorneys would like me to remind you that the viewpoints expressed here do not necessarily represent those of Sarcoma Alliance and should not be taken as medical advice. 

Join the Ocean of Hope Aug. 25 at the Classic

By Suzie Siegel

In 2011, when Robert Phelps was 20, his father was diagnosed with terminal brain cancer.

"I started paddling during this tough time to help myself." His father passed away this April. "Now I am paddling to help other people."

I'm going to tell you a little about Robert Phelps (above) and John Kucera, two new members of our Ocean of Hope (O2H) team. But first, I want to encourage anyone in the Los Angeles area on Aug. 25 to join us at the Catalina Classic Paddleboard Marathon.

Volunteers can come at 9 a.m. (If you want to volunteer, send an email to abeckert@sarcomaalliance.org.) The crowd arrives around 10:30. Paddlers will begin to come ashore around 11 if the winds and tides are good.

Meet other families at our bright-yellow tent, where we will have food, drinks, merchandise and raffle tickets. Once again, we will be raffling off a fast and sleek board by Joe Bark.

If you can't make it, but would still like to support O2H, click on paddleboarders and outrigger team members. O2H is the Alliance’s largest fundraiser and consists of paddleboard and outrigger-canoe races across the country. The paddlers also raise much-needed awareness about sarcoma, and everyone at the Alliance is grateful to them.

At the Catalina Classic, each paddleboard bears the name of someone we honor. People who donate $1,000 or more can have their name or the name of another on a board next year.

As of last year, O2H had raised $633,897.31. "We are getting close to $650K," said Arthur Beckert, the Alliance's executive director. 

The frontrunner in donations is Robert, who has raised more than $5,000. He will have the name of his father on his board. He competed in the Classic last year, but this is his first year on the O2H team.

"I was very impressed with the organization and wanted to be a part of it," said Robert, a lifeguard for the city of Coronado. He grew up sailing, fishing and surfing.

"My dad was a lifeguard in the late 70's and was very instrumental in getting me into junior lifeguards from age 9-16, when I got my first lifeguard job for California State Parks at Silver Strand. I've always loved being in, on or around the water."

John Kucera

Another new team member is John Kucera of Dana Point. On his board will be the name of his mother, his fiancée Christi, and Bob Chambliss, the only person with sarcoma who has attempted the race.

"When I lost my mother, Lucy, to a seven-month battle with pancreatic cancer in 1993, I became painfully aware of how devastating cancer can be. Then, just last year my fiancée, Christi, was diagnosed with leiomyosarcoma, and I again experienced firsthand the ups and downs and fears and hopes of cancer."

Christi has a good prognosis after a hysterectomy for leiomyosarcoma. Like others, she and John discussed how invasive a surgery she needed, before they knew whether it was cancer or not. They talked about complementary medicine and treatment after surgery.

"It was a very, very difficult year," he said. "We were planning to have kids."

Unlike most people, John had heard of sarcoma before. At the beach in 2006, he met Mark Schulein, former O2H captain, and he donated to O2H. "We shake our heads over this -- I'm not sure what to call it -- divine providence?"

John has long loved open-ocean swimming, and he was a lifeguard at Newport Beach in the 1980s. He entered the Classic once before -- 25 years ago. He lived out of state for years, but moved back in 2001-2002 and began to train and coach other paddleboarders.

"A lot of people pestered me, saying, 'You work so hard you're nuts if you don't do [the Classic again].' I love paddling, and I was looking for a significant date to do it."

He wrote on his Active.com page: "In my 1988 crossing, I placed 2nd in stock 12' class, and 10th overall with a time of 7 hours and 28 minutes. Today, the top 10 finishers will likely reach Manhattan Beach pier well under 6½ hours.

"Just as we have seen innovation and advancements in paddleboarding in the last 25 years through improved paddleboard design (mainly those by Joe Bark), better training techniques, and smarter sports nutrition so too have we witnessed innovation and advancements in the fight against sarcoma."

He reminds us to have hope.

High hopes for the experimental drug TH-302

By Suzie Siegel

We have high hopes for an experimental drug, TH-302, being studied around the world.

"I'm very confident of this drug in sarcoma," said William Tap, MD, section chief for sarcoma oncology at Memorial Sloan-Kettering Cancer Center in New York. He gave an update on TH-302 in June at the American Society of Clinical Oncology’s annual meeting. The first findings may be available next year.

Ninety-six cancer centers in the U.S., Canada, Europe and Israel have signed up for the clinical trial, according to the National Institutes of Health. “Australia is coming aboard soon,” said Dr. Tap, the trial’s principal investigator.

Having this many study sites is amazing, compared with sarcoma research a decade ago, he said. The study is being done by Threshold Pharmaceuticals in conjunction with SARC (Sarcoma Alliance for Research Through Collaboration). (SARC is sometimes confused with the Sarcoma Alliance, but it's a separate nonprofit for physicians and scientists.)

Dr. Tap said 338 patients have enrolled in the TH-302 trial so far, with a goal of 620. In one arm of the phase 3 trial, patients get doxorubicin (Adriamycin) and TH-302. In the other, they get only doxorubicin. Patients are selected at random to go into one arm or the other. He said the trial is enrolling patients who have intermediate- and high-grade soft-tissue sarcomas for which doxorubicin is an appropriate front-line regimen.

Some critics have suggested that clinical trials be limited to only the sarcoma subtypes that show the greatest initial response. But Dr. Tap said he wouldn’t want to focus on one or two subtypes while other patients who might have responded go untested.

"A phase 2 study showed broad activity across sarcoma subtypes."

Some recent drug studies have looked at progression-free survival, i.e., how long a patient lives without the sarcoma returning or advancing. The TH-302 trial looks at whether patients live longer, Dr. Tap said.

“In sarcoma, in more than 30 years, we haven't had a new drug that we believe affects overall survival," he said, except those used for gastrointestinal stromal tumors.

Some people think TH-302 may fail because palifosfamide did. Both are chemical cousins of ifosfamide, but less toxic. Ifosfamide, an older drug, is sometimes paired with doxorubicin for chemotherapy. The palifosfamide trial combined it with doxorubicin in one arm vs. doxorubicin alone – similar to the TH-302 trial.

"They're different drugs,” Dr. Tap said. “It's like comparing apples and oranges. The key is the delivery method."

Unlike palifosfamide, TH-302 activates in tissue with low oxygen, and those conditions often exist in the center of a sarcoma, he said. This differs from other drugs that attack the edges of tumors, which have more blood vessels and thus, more oxygen.

Tissue banks help with research

By Suzie Siegel

A national group of physicians and scientists who study sarcoma has opened a biospecimen bank to help research.

Denise Reinke

SARC created the bank three years ago, and its staff and volunteers have talked to researchers about it, said SARC President Denise Reinke, a nurse practitioner who also has an MS and MBA. Because the number of sarcoma researchers is relatively small, she said, SARC has never made a public statement about the bank before.

“We’ve never really advertised it or announced that we’re open for business,” she said, but she welcomes researchers to contact SARC if they have a specific study in mind.

Dr. Robert Maki

SARC’s scientific leadership decides who receives tissue and serum samples, said Reinke from her office in Ann Arbor, Mich. Those leaders are divided into three committees: developmental therapeutics, concept review and clinical research. Robert Maki, MD, PhD, medical director of the sarcoma program at Mount Sinai Medical Center in New York, is the director of translational research.

“He moves among the committees; he’s the glue that holds them together,” Reinke said.

SARC stands for the Sarcoma Alliance for Research Through Collaboration. It is sometimes confused with the Sarcoma Alliance, founded in 1999 to provide information and support to anyone affected by sarcoma. Both are nonprofits.

SARC brings together physicians from different institutions to collaborate on clinical trials. It will celebrate its 10-year anniversary in November at the annual meeting of the Connective Tissue Oncology Society in New York, Reinke said.

“We have been able to work together to make a difference for sarcoma patients.”

SARC has run 20 clinical trials across the country, she said, and has collected thousands of biospecimens. “We’ve even got samples from the first trial.”

Tissue is saved frozen, in paraffin blocks and in unstained slides. Serum samples from blood draws are also frozen. Samples may come from different locations in the patient’s body and at different times. For example, in a trial of the drug Gleevec and dermatofibrosarcoma protuberans, surgeons took flash-frozen tissue before the patient got chemotherapy and afterward to gauge the drug’s effect. 

“We have 20 paired samples, and we’re now looking for money to analyze them," Reinke said.

Samples are stored at the biorepository at Nationwide Children’s Hospital in Columbus, Ohio, where SARC has bought space. Cooperative groups for other cancers save samples there, too, including the Children's Oncology Group and the Gynecologic Oncology Group. COG runs sarcoma clinical trials, as does GOG to a lesser extent.  

SARC doesn’t store all of its samples, Reinke said. “In our IGF1R trial, for example, we had 25,000 serum samples, and it costs money to keep them in a repository.” SARC’s scientific leadership decided they needed to keep only a third of the samples.

“It costs to collect, save and analyze samples,” she pointed out. A SPORE (Specialized Programs of Research Excellence) grant helps them pay some expenses.

“The samples are being collected with annotated information along the way," Reinke said. "Having annotated information is a critical component.” 

The information includes the patient’s age, treatment, the response to treatment, survival, the location of the tumor, when the sample was taken, and whether the sample came from the original tumor or a metastasis. The data comes from medical records, not the patients themselves.

“Sometimes patients don’t know all the details of their cases,” Reinke said.

Dr. Dafydd Thomas

Comprehensive sarcoma centers also save biospecimens, often tied to patient details, said Dafydd G. Thomas, PhD, MD, co-director of the University of Michigan's Molecular Pathology Research Laboratory and its cancer center's Tissue and Molecular Pathology Core in Ann Arbor.

"University hospitals like Stanford, Moffitt and UM are sitting on a gold mine of tissue. I have over a thousand sarcoma specimens sitting in my freezers, linked to clinical data," he said. "I take the viewpoint that I have joint ownership with the patient of the tissue and that I will not refuse a reasonable request for tissue samples. Unfortunately, I do not have grant money to do much with this resource."

He's more focused on breast-cancer research these days because he has grant money for it.

Dr. William Tap

“We have a large tissue bank here at Memorial," said William Tap, MD, section chief for sarcoma oncology at Memorial Sloan-Kettering Cancer Center in New York.

MSKCC researchers collaborate with colleagues at other institutions, he said, but they don’t send tissue just because someone asks for it. Tissue is very valuable. "Once the tissue is sent out, it's gone.”

The National Cancer Institute (NCI) has given MSKCC a SPORE grant for sarcoma. MSKCC, SARC and NCI representatives have met to discuss ways to share information and material, Reinke said.

“There are clearly initiatives focused on working together to effectively use samples,” she said.

A 2004 NCI report called for “establishing a centralized sarcoma tumor and tissue repository.” Dr. Tap said: “It’s still critical to have a centralized bank, with the resources to properly manage it and establish quality control."  

Cost is one of the challenges, Reinke said, as is the high demand for biospecimens. “Sometimes institutions don’t want to send them out because they don’t want to compromise their own institution’s ability to do research.”

What's the difference between a centralized bank and SARC's? The SARC bank contains samples only from its clinical trials, while a centralized (and larger) bank would also include tissue and serum from patients who did not participate in clinical trials.

Like SARC, a centralized bank would be run by people who had no vested interest in one institution or another. This differs from 

a university hospital that manages its bank for the benefit of its own faculty, which may or may not choose to share the resources.     

Some nonprofits also are collecting tissue samples, Reinke said. “In the future, we will look at patient-driven initiatives.”

For example, Leiomyosarcoma Direct Research and the National LMS Foundation are urging people to send samples to LMSdr Executive Director Sharon Anderson in San Francisco, who calls patients or their survivors to get medical histories. Anderson, an LMS survivor, is the tissue coordinator for the Leiomyosarcoma Paraffin Tissue Block Repository at Stanford University Medical Center.

She turns over anonymous information and paraffin blocks to Matt van de Rijn, MD, PhD. Both nonprofits also raise money for his research.

This work is crucial, Dr. van de Rijn said. “I can’t emphasize that too much.”

In an ongoing study, he and his colleagues determined that people with more macrophages in their tissue samples tended to do worse. “We had 147 patients where we knew the outcome, and we could correlate macrophages and how patients did. These findings have been confirmed three times.

“The macrophages encourage angiogenesis, which leads to a better blood supply for the tumors. Macrophages have another major function – they eat up bad things in your body. We’ve found that if you inhibit a protein, CD47, on the surface of LMS cells, that will change the macrophages so that they will eat the LMS cells.” 

The cases were almost equally split between LMS that started in the uterus and LMS that began elsewhere. In some cases, the LMS spread, and the bank received tissue from the metastases.

Dr. Matt van de Rijn in his lab.

It’s important to find something, such as macro- phages, that can help the medical community make a prognosis in LMS cases, Dr. van de Rijn said. More important, however, is finding a better way to treat patients.   

“I’ve never felt so close,” he said, to developing a new therapy, whether a small-molecule inhibitor or the use of antibodies.

John Brooks, MD, started the LMS bank at the University of Pennsylvania Hospital in Philadelphia, with the hope that it would be a central repository. (See my 2007 article.) Due to lack of resources, the tissue samples were later transferred to Dr. van de Rijn. He describes himself as the curator of the tissue, not its owner.

"You can help researchers all over the world" by sending tissue samples, says the LMSdr website. "The advantage of having a tissue bank is that researchers can go to one place & access enough tissue to conduct research. ... Bottom line, without a tissue bank, it is impossible for researchers to get enough samples to conduct any valuable research on LMS. No samples, no research, no cure!" The NLMSF uses similar wording.

The consent form says researchers whose proposals meet the criteria of Stanford's Institutional Review Board can receive tissue samples.

Dr. van de Rijn said his primary focus is his research, not managing a tissue bank for other researchers. But he added: “I’m all for collaboration. We need to collaborate.”

Indeed, he has worked with Dr. Thomas and many others. In the past, he said, he has sent LMS tissue to other researchers in the U.S. and abroad, but he has had few requests.

“There are very few people working on leiomyosarcoma, and not all of them can use paraffin-block samples.”

For example, Sarcoma Alliance board member Matthew Anderson, MD, PhD, studies uterine LMS at Baylor College of Medicine in Houston. He has gotten frozen tissue from Dr. Thomas and M.D. Anderson Cancer Center, also in Houston.

The Affordable Care Act & sarcoma survivors

By Suzie Siegel

"Obamacare" will help many sarcoma survivors, but doesn't fix all the problems faced by people with rare cancers.

Under the Patient Protection and Affordable Care Act, called ACA or Obamacare, each state will set up a Health Insurance Marketplace to make affordable policies available from private companies. The federal government can create one if a state doesn't act. The process is expected to start Oct. 1.

The Marketplaces should help sarcoma survivors who couldn't afford insurance before or couldn't get a policy because of their medical history.

But many patients who are already covered find that their insurance company will not let them go out of network to see a sarcoma specialist for a second opinion, let alone continuing care. The ACA won't change this.

Some states and big cities have no sarcoma specialists. Of course, this differs from common cancers, in which patients are more likely to find specialists near home.

That's why we have a page to help people get insurance authorization for a referral. We also offer reimbursement up to $500 for people who need a second opinion from an expert.

People who don't have insurance through the government or their employer will be required to buy a policy from a private company, or be penalized. They can be exempt for religious reasons and financial hardship.

Major provisions of the ACA will take effect Jan. 1. People with existing policies may see little change. Insurance plans that were created before the law passed March 23, 2010, and have not been changed significantly will be "grandfathered in."

Under the ACA, insurance companies can no longer set lifetime dollar limits for "essential health benefits." The American Society of Clinical Oncology is working on a list of cancer-specific benefits, according to ASCO staff.

Brietta Clark, JD

"The law will provide more access to prevention services and treatment for many people, and it will protect them from financial catastrophe," said Brietta Clark, JD, a professor at Loyola Law School in Los Angeles.

"Under health-care reform, one way that insurance companies will try to control costs is through drug formularies," she said. A formulary is a database of medications that an insurance company will cover, as well as what percentage they will pay. The company may charge more for an expensive drug, and that has been a concern as more people get chemo in pill form.

The ACA will require insurance companies to cover routine costs associated with a patient's participation in a clinical trial. Now, some states require coverage, and others don't.

Matthew Anderson, MD, PhD

"I'm optimistic that this law will increase much needed access to care for many people," said Sarcoma Alliance board member Matthew Anderson, MD, PhD, director of clinical and translational cancer research in gynecologic oncology at Baylor College of Medicine in Houston. "I'm particularly excited because provisions of the ACA may help sarcoma and other patients access experimental treatments under circumstances where this previously may not have been possible." 

Because sarcoma is rare, it can be hard to get enough patients for the large clinical trials required for FDA approval. As a result, few drugs are approved for the disease; many patients get chemotherapy that was approved for a more common cancer.

It's still uncertain how insurance companies will pay for such off-label use under the ACA.

"One of the challenges that existed before health-care reform and will continue is who will pay for experimental treatment and how is efficacy being studied," said Clark, who specializes in the inequities of health-care law.

"Another challenge is when you talk of life-saving treatment, what are the trade-offs? The ideal is a system where patients and families can be really educated about all of the options and let them hear from people who have been there. They need to make decisions consistent with their values.

"The so-called 'Death Panel' was designed to help families do that. It would have paid for physicians' time to explain fully what the options were."

When some sarcoma survivors are eligible for Medicare, they choose Original Medicare, which gives patients more options on which doctor to see. Medicare Advantage and other plans may not pay for a sarcoma specialist out of network.

The law will not change Medicare Supplement Insurance, called Medigap, which picks up costs not covered by Original Medicare. In many states, companies that offer Medigap to seniors can refuse policies to people under 65. Or, they may be required to write only the most basic policy.  

There are only a few time periods when an insurer is required to write a Medigap policy despite pre-existing conditions. 

The Kaiser Family Foundation has noted that premiums for people under 65 are generally higher than for people 65 and older. Plan F premiums averaged 73 percent higher. (I pay almost $500 a month, for example.) Because of the younger age of sarcoma patients, this affects them more than many other cancer patients who are already 65 or older.  

The law allows doctors and hospitals to "bundle payments" from Medicare, instead of charging a fee for each service. When a patient has surgery, for example, a hospital might send one bill to Medicare, instead of having the surgeon, anesthesiologist, physical therapist, etc., bill separately.   

The goal is to save money and improve patient care by encouraging health-care providers to work together. Whether that will happen is unclear, according to the Rand Corporation.

Other provisions should help sarcoma survivors. The ACA:

1. Extends family insurance to members under 26 years old. This is especially important for sarcoma survivors because sarcoma represents 15 percent of childhood cancers. Even if a child or young adult no longer has cancer, they still need follow-up care.

2. Requires insurance companies to cover pre-existing conditions. They can't reject applicants or charge higher premiums because of medical history. On average, sarcoma strikes younger people than more common cancers, such as breast, colon and prostate cancer. If cancer survivors cannot get Medicare, some have found it hard to get insurance that will cover a recurrence.

3. Stops insurers from dropping someone because they get sick.

4. Requires companies with more than 50 full-time employees to  provide health insurance.

5. Expands the number of people eligible for Medicaid in states that have accepted the program. Click here to see if your state is participating. In some states, a person without children cannot get Medicaid, no matter how poor they are. 

6. Phases out the gap in drug coverage for Medicare Part D by 2020.

Because the law is so complicated, people should expect delays as it's fully implemented.

At the Alliance, we recognize that some people dislike the law because of their political principles, or feel they will be hurt as taxpayers and business owners. This post was designed to discuss only the health-care issues.

My thanks to the ASCO staff for their help.