Dr. Nadya: With orphan cancers, choices are few

Cancer is a diagnosis no one wants to receive. Cancer brings with it an overload of test results, statistics, and medical advice that often leads to uncertainty, fear, and both physical and emotional anguish.  Among patients, confusion over treatment choices is common, especially as end-of-life fears blur even the most informed and educated minds.

There are not always clear answers in cancer. One tragic example of this is embodied in the story of Elizabeth Joice, a sarcoma patient  I knew both personally and professionally, who recently passed away shortly after the birth of her first child.

Sarcoma is a form of soft tissue cancer known as an ‘orphan cancer’ because it is so rare. Orphan cancers, like sarcoma, affect less than 200,000 people in the U.S. Because they are so rare, funding and research for orphan cancers is minimal compared to more common cancers like breast, lung, prostate and basal and squamous cell skin cancers.

In 2012, the National Cancer Institute allocated just $38.9 million to the research and treatment of sarcoma, compared to the $602.7 million it allocated for the study of breast cancer. Low levels of funding limit life-saving research and drug development in orphan cancers like sarcoma.

Consequently, the prognosis for survival when faced with a rare cancer is much less favorable and it can be difficult for physicians to set the stage for clear treatment choices, in terms of statistics of success.  Without sufficient research and data, or drugs that are consistently effective, there are no answers to questions about anticipated treatment times or what is recommended to optimize health and preserve life.

Elizabeth handled her diagnosis of sarcoma in exemplary fashion.  She started a blog in order to inspire others with cancer to be positive and hopeful.  Each day, she made an active decision to be outwardly happy.  Her goal was not only to cure her sarcoma, but everyone else’s.

During her chemotherapy treatments, Elizabeth regularly visited and comforted others with similar diagnoses. Laughter was an essential component of these visits. After chemotherapy, surgery and more chemotherapy, Elizabeth was declared cancer-free.

Three years later, Elizabeth became pregnant – something doctors thought would be impossible, due to the chemotherapy she had received to treat her disease. Knowing motherhood was always a dream for her, her friends and family considered this a miracle and a sign that everything in her life would be wonderful or as she liked to call it “normal.”

Unfortunately, just shy of her second trimester of pregnancy, Elizabeth noticed a bump in her back, at the site of the surgical scar from the removal of her original tumor.

She quickly sought counsel from the best doctors. But oncologists admitted they had never treated sarcoma during pregnancy, and therefore had no clear advice for Elizabeth. As a result, she proceeded with what doctors said seemed to be her best option: surgical removal of the tumor.  She made it clear she did not want to risk her life and questioned her oncologist as to whether sarcoma was a tumor that could be affected by pregnancy hormones. The answer was no.

Throughout Eizabeth’s pregnancy, I found comfort in the story of Kathy Giusti, a survivor and founder of the Multiple Myeloma Research Foundation. This March, Giusti was named one of “The World’s 50 Greatest Leaders” by Fortune Magazine, alongside Pope Francis, Warren Buffet, Bill Clinton and the Dalai Lama.

Shortly after Giusti was diagnosed with multiple myeloma, another orphan cancer,  she created her foundation to raise funds to accelerate pharmaceutical research into the next generation of multiple myeloma treatments.  Her efforts not only saved her life, but improved the five-year survival rate for multiple myeloma from 28 percent in 1998 to 43 percent today.

Giusti made the choice to become pregnant after her diagnosis; her children are now in their late teens. Giusti was always an inspiration to me personally and I prayed I was glimpsing Elizabeth’s future in hearing her story.

Sadly, though doctors removed the tumor discovered early in her pregnancy, Elizabeth experienced another recurrence of the disease towards the end of her pregnancy and she developed difficulty breathing.  Within days of discovering the cancer had spread, a C-Section was scheduled to deliver her baby six weeks early. After delivery, Elizabeth was to begin treatment of her cancer.  Elizabeth was able to give birth to a perfect gorgeous daughter; but six weeks later, she passed away, one week before her 37th birthday.

Elizabeth did not die in vain.  Her friends and family are doing all they can to support foundations such as the Sarcoma Foundation of America and Miles2Give.  Strangers who knew her have become each other’s dear friends, gathering together to support her cause and direct the publicity her story has attracted towards awareness and cure. These foundations are allowing innovations in cancer treatments such as gene specific targeted therapy and monoclonal antibodies, which have proven life-saving in many orphan cancers.

Elizabeth believed in the miracle of her baby and did not feel she had lost her battle with sarcoma; rather, by passing on her life torch to a small, resilient baby girl, she believed she had won. She also believed in staying happy, hopeful, and hard working, and wrote in her blog the following when describing her “smile campaign”:

Smile in the face of cancer because it doesn’t scare me
Smile while I get chemo because it is saving my life
Smile when I tell my story in hopes of changing the way people see cancer…
Together we can maybe change the way people see this disease because
Smiling More Illuminates Lives Everyday

-Elizabeth Ruotolo Joice

Click to visit Elizabeth's dedication page at CureSarcoma.org.