Adolescent and young adult cancer survivors must be their own advocates to live long and well.
Despite the fact that 23 years have passed, Diana DeVoe still cries when she talks about the day she learned that she had cancer. Then an 18-year-old college freshman at The Ohio State University, she waited until she went home for Christmas to have her family doctor look at the knot that had popped up between the knuckles of the first and second fingers of her left hand. He referred her to a specialist for a biopsy. She woke to find herself alone in the hospital while her father had gone to get her mother.
“Before my parents even got back, because I was 18 and considered an adult, they told me alone that I had cancer,” DeVoe says. “And it was bad.”
A cancer of the connective tissue, called alveolar rhabdomyosarcoma, had wrapped itself around her fingers and the connecting muscle, requiring that two fingers be removed along with most of the muscle. The surgery was followed by radiation and more than two years of chemotherapy. During the week of surgery, DeVoe celebrated her 19th birthday.
The treatment kept her cancer-free, but at a price.
When DeVoe received her diagnosis in 1988, she fell in the, as yet, unnamed category of an adolescent and young adult (AYA) cancer patient. Now defined by the National Cancer Institute as people ages 15 to 39, AYA patients have seen growing attention because of their unique position at the crossroads of pediatric and adult oncology.
Researchers have identified the primary concerns for AYA survivors as detection and management of late effects, assessment and support of psychosocial functioning, provision of health-related education, and assistance with identifying and meeting financial challenges.
Kevin Oeffinger, MD, director of the Memorial Sloan-Kettering Cancer Center Adult Long-Term Follow-Up Program, says by bringing AYAs into the system for surveillance, other issues can be identified and referred, but the biggest challenge remains convincing them to follow up on any recommendations once treatment has ended.
Research by Oeffinger and others has shown that survivors of pediatric and young adult cancer face a substantial risk of late effects and early mortality. By 30 years from the initial diagnosis, 73 percent of pediatric cancer survivors will develop at least one chronic physical health condition; in 42 percent the condition will be severe, life-threatening, disabling or result in death.
If there were a poster child for both the needs and the challenges of this age group, it would be DeVoe.
After her fingers were amputated, DeVoe went home with her parents, crawled into her childhood bed and took pain killers one after the other so she wouldn’t wake up. She lost 55 pounds during two years of combination chemotherapy using cisplatin, vincristine, doxorubicin and cyclophosphamide, during which she remained home, enrolled in and dropped out of a community college to maintain her status as a full-time student so she could stay on her parents’ insurance plan. When she returned to campus, where she focused on getting out of college and keeping people at arm’s length, she told new friends that she lost her fingers in a car crash. After graduation, she took a position with a national hotel chain, where she had interned, moving to Dallas to manage hotels.
Then, in 2006, on her 36th birthday, she woke up and couldn’t feel her toes. As the numbness turned to pain, a visit to her internist began a physician odyssey that took her to an orthopedic surgeon, a sports doctor, a back doctor, a neurologist, a pain specialist and, finally, a neurosurgeon who performed a nerve biopsy. Nothing helped. She went back to work, struggling with the pain, which had now moved up her right leg to her hip and her left leg to her calf. By early 2011, DeVoe was receiving disability benefits, unable to drive more than 10 minutes at a time and struggling emotionally. Then she was referred to Angela Orlino, MD, director of the Young Adult Program at After the Cancer Experience (ACE), a survivorship program of Dallas Children’s Medical Center and the University of Texas Southwestern Medical Center, one of the oldest AYA programs in the nation.
Orlino identified the neuropathy and pain as probable late effects of DeVoe’s cancer treatment and helped her obtain insurance coverage for the surgery to implant a spinal cord stimulator to help with the pain.
On a positive note, after more than 20 years, DeVoe began to make peace with her cancer diagnosis when she met other AYA survivors at local and national meetings.
“I ran away for 22 years,” she says. “I am finally getting it that God didn’t give me cancer; it’s just something that happened. I was in anger and denial for so long and now I am working through it.”
While trying to launch their lives and find their emotional footing, AYAs are also faced with complicated psychosocial issues ranging from isolation and family dependence to insecurities resulting from a disease that brings with it treatments that may alter their appearance or life. Their ability to continue their education or earn a living may change in a multitude of ways, and with that their ability to afford health insurance when they age out of their parents’ healthcare policies.
Nicholas Romanelli, who received a diagnosis of diffuse large B-cell lymphoma in October 2006 at age 21, is among the new generation of AYA survivors who recognize that an altered lifestyle and life-long monitoring come with the territory.
Romanelli, who was attending college in upstate New York at the time of his diagnosis, stayed with his parents on Long Island during initial treatment and then managed the year-long chemotherapy on his own, thankful that his parents respected his independence. Two months after finishing treatment, scans showed his disease had returned and he would need a bone marrow transplant.
“I had total body radiation, hardcore chemo and a transplant in March 2008. I remained inpatient for 19 days,” he says.
I would like to see a more formal way to educate the primary care physicians about late effects.
“I would like to see a more formal way to educate the primary care physicians about late effects,” says Orlino, DeVoe’s physician. “But right now the patients are the best repository of their information and they have to own it.”
Orlino follows the young adults in the ACE clinic, which is a subclinic of her general internal medicine practice where she also follows other AYA patients who have found her through research or referrals. These are often patients such as DeVoe who were treated in other cities earlier in their lives and who now need a primary care physician who understands their cancer history.
“The Leukemia and Lymphoma Society will refer adult patients to me who want to blend primary care and late effects,” she says. Her job, she says, is educating patients about their increased risk for late effects and helping them make changes that will reduce those risks.
“The challenge is finding a way to tell this healthy population in a way that will motivate them to make those changes now.”
As Romanelli recovers, he continues receiving intravenous immunoglobulin due to his depleted B cells (antibody-producing white cells). In 2011 he met Oeffinger, who set up his survivorship care plan for monitoring, exercise and nutrition—a relationship that Romanelli sees as lifelong. Now working in financial services in Manhattan, he also says he is grateful to be in a city with an AYA follow-up clinic.
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Melissa Hudson, MD, director of the Cancer Survivorship Division at St. Jude Children’s Research Hospital in Memphis, Tenn., and chairwoman of the ASCO Cancer Survivorship Committee, has focused on late effects of AYA and childhood cancer treatment in her clinical and research work. She now follows some 5,000 survivors from across the country, the oldest of whom is in his late 60s.
“We not only think about the medical outcome but also the psychosocial and emotional impact of the cancer diagnosis, especially its impact on academic and vocational progress,” Hudson says. “Those AYAs treated in pediatric centers are more likely to have access to a multidisciplinary psychosocial support staff that proactively addresses these issues.”
Hudson says follow-up at St. Jude’s takes into consideration a patient’s age at diagnosis and developmental issues, she says. As patients are followed, she and her team evaluate if survivors are achieving the milestones expected for their developmental stage, she says. “Young adults want a normal life. They have had this horrific experience and they want to move on from that, and they don’t want to be reminded of the health risks,” she says. “But if they are proactive about medical follow-up and healthy lifestyle, they can modify those risks.”
Patients have a survivorship care plan that has information about the drugs they’ve taken and their potential late effects, as well as a monitoring and surveillance plan. Hudson says they encourage patients to become their own advocates with their local primary care physicians.
This becomes increasingly important as statistics from a recent survey of doctors showed a lack of knowledge of the late effects of common chemotherapy drugs used to treat breast and colorectal cancers. More than 2,000 oncologists and primary care physicians were asked to identify late effects of four chemotherapy drugs used for breast and colon cancers: doxorubicin, oxaliplatin, cyclophosphamide and paclitaxel.
Of the primary care doctors, only 55 percent knew that cardiac problems could be a late effect of doxorubicin. About 27 percent and 22 percent, respectively, knew that paclitaxel and oxaliplatin could cause peripheral neuropathy. Oncologists performed better on the survey, with 62 to 97 percent being able to connect the drug to a late effect.
In February 2012, the National Comprehensive Cancer Network responded to many of these concerns with new practice guidelines for the AYA patient population that address the “critical issues that AYA patients with cancer and their caregivers encounter at diagnosis, during treatment and after therapy.” The guidelines also call for AYAs to be assessed across domains of individual function, relationships, socioeconomic issues and supportive care services/interventions. In each of these categories the NCCN offers recommendations.
The “AYA gap” has also garnered researchers’ attention because of the lack of improvement in survival—unlike the age groups on either side—prompting some to call for more clinical trials for the AYA population.
Researchers in this area point to the need for improvement in the survival rate for this age group of around 70,000 new patients a year—nearly eight times the number of new diagnoses of those under 15. Researchers also ask that because of the unique emotional and physical aspects of AYA cancer, it become a separately recognized specialty within oncology.
In its guidelines, the NCCN lists the most common cancers for the AYA population, which include not only those more identified with younger AYA patients, such as Hodgkin lymphoma, but also those associated with older adults, such as cancers of the breast, thyroid and colon.
The guidelines call for monitoring when “certain medications associated with irreversible organ damage may be essential” for treatment. For these drugs, the toxicities can be associated with cardiac, renal or hearing impairment.
Oeffinger served on the panel that created the NCCN guidelines and has a particular interest in the follow-up and monitoring that will help AYA patients go on to live long, productive and meaningful lives.
“Whether we are talking about someone with testicular or ovarian or breast cancer at a young age, many of these patients have an increased risk for heart disease,” he says. “Sometimes it’s directly related to therapy and often it’s indirectly related.”
Oeffinger says he and his staff follow around 1,000 patients who range in age from 17 to 85 who received a cancer diagnosis prior to their 40th birthday. In this group, he has seen numerous second cancers, as well as other late effects. A particular interest for Oeffinger is women who had radiation to the chest area before age 30 and are now at risk for breast cancer. According to a study released at this year’s annual meeting of the American Society of Clinical Oncology (ASCO), their risk is comparable to women who carry the BRCA 1 and 2 mutations.
“At the end of the day, each person has to make his or her own individual decision about whether or not to be followed and monitored,” he says. “For example, I recently reviewed records of a young adult Hodgkin lymphoma survivor who was not followed in our program. She was not getting any screening, felt a mass in her breast and then waited six months before seeing a physician. Unfortunately by then, her options were limited. Her death was highly avoidable.”