Jacob Bibb, 16, anxiously awaited the first game of his high school’s tiny 1A team in Mason. He’d be playing linebacker against a tough rival school and tried not to think about the swelling lymph nodes in his neck.
At 21, Barbara Garcia was living what she describes as the perfect life as a party-hearty fashion student at University of Incarnate Word. Even though she noticed a lump slowly growing underneath her left armpit and had a chronic cough, her doctor focused on treating what he thought was seasonal flu.
One mid-May morning, Krystal Madrigal, 21, worked the busy front desk at a San Angelo mental health facility, thankful for a brief break from her night classes at the local junior college and eager to get her summer started. She just needed to get a rash checked out.
“It wasn’t a rash,” said Madrigal from her hospital room on a recent September afternoon, a day after her twenty-second birthday, “It was petechiae, which is pretty much blood coming up through your skin.” The same day Madrigal visited her primary-care physician, she drove three hours to San Antonio’s Methodist Healthcare to begin treatment for Acute Myeloid Leukemia, a cancer normally occurring in patients 55 and older.
Across the courtyard, in Methodist Children’s Hospital, Bibb also underwent chemotherapy for what turned out to be Acute Lymphoblastic Leukemia. In a room with a Cinderella picture on its door, Bibb heard from friends the outcome of his September 9 football game. “They had my number on their helmets and they dedicated the game to me,” he said from his sister’s house in Boerne, where he now recuperates while receiving outpatient chemo treatment. “Then they won a really close game against a really good team. I was so proud of them.”
Garcia didn’t learn of her Hodgkin’s lymphoma diagnosis until after the surgical removal of her lump, a painful growth that had become visible through her clothes. “My surgery was on Friday, and on Monday they called and told me I had Hodgkin’s lymphoma,” she said, decked out in a long floral scarf just six days after completing her last radiation treatment. She underwent outpatient treatment at Cancer Care Center while finishing the spring semester.
Bibb, Garcia, and Madrigal, with their disparate ages and varied diseases, are nevertheless part of the same patient group, increasingly recognized as Adolescent/Young Adult, or AYA, oncology patients. Unfortunately, the group that accounts for six percent of all cancer diagnoses has seen the least amount of improvement in five-year survivorship in the past 25 years. Meanwhile, older and younger patients afflicted with the same cancers have seen their survival rates rise dramatically, leading the medical community to take a harder look at how they treat patients ages 15-39 years old.
Because AYA patients can contract cancers more common in young children, like Bibb’s Acute Lymphoblastic Leukemia, or older patients, like Madrigal’s Acute Myeloid Leukemia and Garcia’s Hodgkin’s Lymphoma, treatment may be handled either by medical (adult) oncologists or pediatric oncologists. The divided nature of medicine, wherein pediatric oncologists aren’t likely to pay much mind to adult oncology research and vice versa has put this population in an especially vulnerable, and ignored, position. In fact, the survivorship gap only became apparent in the late 1990s. Prior to that, the AYA population hardly existed in oncology research. The stressful nature of being young adults, with school work, new careers, new families, lower incomes, and lower levels of insurance enrollment, makes treating AYA patients more complicated than most physicians are accustomed to.
However, since the discovery of the survivorship gap, a movement to recognize the AYA population as distinct from children or older adults (the median patient age treated by a medical oncologist is 66), has been gaining momentum. Currently, Texas seems poised to take the lead, with major centers like Cook Children’s Medical Center in Fort Worth and MD Anderson Cancer Center in Houston dedicating resources specifically to the AYA population. This weekend, San Antonio’s Methodist Healthcare Center hosts both the First Annual Texas AYA Oncology Conference for health care providers and a tweetup social event for patients and survivors.
Stuck in the Middle
“It has always been an interest of mine to see this particular population of patients because they are not children anymore, and they are not adults,” said Dr. Jaime Estrada, a local pediatric oncologist and committee chair of this week’s AYA Oncology Conference. “They are sort of in between.” The 2006 AYA Progress Report Group paper, the first of its kind, defined 15-39 as the AYA age range because treatment at pediatric hospitals begins to drop off after age 14. On the older end, 39 is the average age where patients begin showing age-related hormonal changes, immune response decline, and chronic illnesses.
“These patients too frequently fall into a ‘no-man’s land’ between pediatric and adult oncology,” the report states.
Dr. Archie Bleyer, director of the world’s largest childhood cancer research organization, CureSearch’s Adolescent and Young Adult Cancer Research, and a scheduled conference speaker, was one of the first to notice the age group had strikingly poor survival progress. In 1996, while combing through the National Cancer Institute’s survival data from 1975-1996, the numbers leapt out at him. “I was looking for how we were doing as a nation in pediatric oncology,” said Bleyer from his offices in Bend, Oregon. “In looking at all of the variables, age popped out as an obvious problem. In fact, it was more obvious than any other issues, for example, race and ethnicity.” He focused on ages 15-19, but soon expanded his focus to determine when survivorship progress began improving. Bleyer found the tide only began to turn after age 40. “So there was this gap from 15-40 of no improvement in survival for a quarter of a century,” he said.
Meanwhile, another conference speaker, Dr. Karen Albritton, recently recruited by Cook Children’s Medical Center in Fort Worth from Dana Farber Cancer Institute’s Adolescent and Young Adult Oncology department, was noticing similar treatment gaps in her patients, a cross-section of her two specialties, mid-level pediatric patients (adolescents) and oncology patients. “I sort of had one of those ‘duh’ moments where I said, ‘What about these patients in between in my two fields? No one seems to really be comfortable with them.’ I got interested in their health service delivery issues and access issues and outcome issues. … I love the patient population and, my gosh, they’re totally underserved. It’s so easy to become passionate about it because they were so clearly the underdog.”
Both Albritton and Bleyer believe the first step forward in treating these patients is fostering communication between pediatric and adult oncologists, which is essentially Albritton’s mission in Fort Worth. It’s not always easy; specialists can get territorial about their patients and their treatment. “I have to convince the community, ‘Guys, I’m not trying to take your business,’” she said. “This is not a threatening situation, this is not a market situation. Could we all rise to the occasion and, pooling resources, improve the care? You may say, ‘I only have a few patients a year that fit that criteria,’ but so does the guy across the street. So does Cook’s. Once you combine all those, it’s not an insignificant number. It’s just that they’re spread out and therefore we all sort of feel under-resourced and under-knowledged.”
Currently, there is no oncology center in the U.S. dedicated specifically to AYA patients. “You should be treated by an expert in your disease. That’s number one,” said Dr. Michael Rytting, associate professor of pediatric leukemia and lymphoma at MD Anderson. “Hopefully that place has facilities for helping to take care of a young adult and unfortunately, that’s not what’s seen in most big academic centers.”
Albritton, who spent years as the director of Dana Farber’s AYA Oncology program, said, “I don’t think the answer is to treat all patients over the age of 15 at the adult hospital and I don’t think the answer is to treat all the up-to-24 patients at the children’s hospital. You’ve got to make both systems better for the population, which is truly harder to do.”
Under the current system, a medical oncologist treating a 20-year-old Acute Lymphoblastic Leukemia patient may not know their outcomes are much better following a pediatric protocol, which prescribes much more intense chemotherapy than the standard medical oncology dosage. AYA patients are also far, far less likely to enroll in a clinical trial that could prove lifesaving. According to the AYA committee of the Children’s Oncology Group, “while 55-65 percent of cancer patients younger than 15 years of age are entered onto clinical trials at CureSearch COG institutions, only 10 percent of 15- to 19-year-olds and two percent of 20- to 29-year-olds participates in cooperative group clinical trials.” While cancer in general is the leading disease-related cause of death in the AYA population, because the age group is susceptible to so many different kinds of cancer, the group’s presence in each specific disease is still rare. Too rare for even large cancer institutions to open clinical trials for them.
Then there is the struggle just to treat patients in a timely manner. Though AYA patients are six to eight times more likely to contract cancer than pediatric populations, a 2006 article in the Journal of Pediatric Hematology Oncology reported that among Canadian and American pediatric and adolescent cancer patients, the number of days from symptom onset to diagnosis increased with age, with older adolescents reporting more than double the elapsed days than pediatric patients under the age of 14.
“We need to really target primary care providers not to make young adult patients … feel so stupid, frankly,” said Albritton. “I can’t tell you how many young adult patients I’ve had come in whose doctors told them not to worry about it, that they’re too young to have cancer.” With symptoms as subtle as fatigue or aching legs, younger patients and doctors alike are prone to believe a less serious illness, usually the flu, strep throat or mono, is the cause. “You don’t think, ‘My legs hurt, I’ve got leukemia,’” said Madrigal. While Madrigal and Bibb had blood work done on their first physician visit, research suggests many AYA patients have primary care experiences closer to Garcia’s, who struggled with a hacking cough and growing lump for weeks before the doctor ordered blood work.
Invincible and Uninsured
Once diagnosed, the AYA population is among the most difficult to treat and faces the most complicated survivorship issues. This is poignantly evident at iConnect, a local support group founded by Methodist Healthcare oncology social worker and Hodgkin’s Lymphoma survivor Jenna Carrera and sponsored by the Leukemia Lymphoma Society. Attended by Garcia and nine other AYA survivors, coincidentally all female, between 21 and 45, September’s meeting celebrated Garcia’s recent remission and newly-wed Rebecca Boyd, a non-Hodgkins lymphoma survivor. Sitting in a La Madeleine meeting room, the women also talked about sexual-health issues (nearly all of them went through menopause as a result of chemotherapy), their illness’s toll on relationships, and what Acute Myeloid Leukemia survivor Kristen Nelson, 36, calls “that fucking cancer fear,” where any bruise, sore throat, or fatigue could mean relapse, or not.
“Not one person told me it’s going to be really tough and you’re going to go through depression,” said Hodgkin’s survivor Sarah Brannon. “They think that I’m in remission and the doors fly open, birds sing, and I drive away with the top down on the car.”
Because of their relatively young age, AYA cancer survivors deal with repercussions of toxic chemotherapy much longer than most adult cancer patients. Carrera has arthritis and suffered a heart attack at 37 due to damage caused by chemo. Nelson and friend Cassandra Farrias both contracted chronic graft-versus-host disease, an immune-system complication that affects the lungs, skin, liver, and gastrointestinal tract, thanks to a life-saving bone-marrow or stem-cell transplant. Garcia was shocked to learn that the radiation that eradicated her Hodgkin’s could cause breast cancer in as few as 10 years.
Many AYA patients also need extensive psychological and emotional support. Unlike children or older adults, patients in this age range are trying to get their lives going, so to speak, through education, career, and family. Most are either in an adolescent phase of seeking independence from their parents or have been independent for some time, yet find themselves again reliant on family to care for them. “A cancer diagnosis abruptly derails these important developmental processes,” noted the AYA Progress Review Group, “thrusting the individual back into uncertainty and sometimes an unwelcome or uncomfortable dependent state.”
Older teens treated in pediatric hospitals may feel especially awkward, with the bulk of care, social activity, and even interior design geared toward patients at least a decade younger than themselves. Bibb maintains a genial disposition about his treatment at Methodist Children’s, despite not seeing any patients close to his own age. His one suggestion for treating fellow adolescents in the future: “If they’re anything like me, 6’3” and in the `pediatric` hospital, maybe a bigger bed would be nice.”
Due to youth and inexperience with previous serious illness, the AYA population reports feeling invincible more often than other age groups. “They are grieving the loss of a healthy person,” said Nikki Yuill, who, like Carrera, works for Methodist’s oncology support team. Sometimes that grief manifests in the decision to cease treatment. “We have a lot of issues with young adult males who panic and almost want to leave the hospital situation,” said Yuill, “We have to come in … and talk them out of leaving, so that they’ll stay and get their first treatment.” Rytting, who has many patients in the 17-22 age range, said, “Kids aren’t able to say, ‘I’ve had enough, I don’t want any more chemo,’ but 22 year olds do it all the time. They simply say, ‘I think I’ve had enough, I’m going to take this chance that I’m cured and I’m not going to finish.’” Many patients are just eager to get on with their lives, to return to school or work, unhampered by chemo’s tiring effects and requirements to steer clear of alcohol and crowds.
AYA patients may also reject taking needed follow-up drugs like the commonly-used steroid prednisone, which in addition to causing migraines and insomnia, can create rapid weight gain, facial swelling, and acne. “It’s like the anti-self image drug,” said Bleyer. Garcia, the party animal, said her social calls started coming less and less frequently after diagnosis, and she was forced to spend her summer indoors. To add insult to considerable injury, she said some friends refused to see her unless she wore a wig or scarf to cover up her chemo-bald head.
While self-image or delusions of invincibility may account for some patients leaving treatment, another reason is simply work and insurance. Because they are newer to the workforce, many AYA patients worry they will lose their jobs if they take too much time off to deal with their illnesses. Madrigal worked at her front desk job for over 18 months, when she told her employer she needed six courses of four-to-six week treatment in San Antonio. She was let go. “It was called voluntary separation or something,” she said. With her job went her insurance; Madrigal recently applied for COBRA. “You’re sitting there, waiting, going, ‘I hope nothing goes wrong,’” said Madrigal. “Because you need it not to stop, you can’t just switch to another insurance company and say, ‘I have cancer, will you pay for all my stuff?’”
Other AYA patients may not have elected to pay into a health insurance system to begin with. In the U.S., citizens between 15 and 39 are by far the least insured, with more than 30 percent of people ages 18-24 and 25 percent of people ages 25-34 currently uninsured, a statistic that many physicians interviewed said they hope will change with healthcare reform. “If you don’t have insurance, it’s very hard to get into a big academic center and participate in a trial,” said Rytting. “You’re just not going to be accepted as a transfer patient with no insurance.”
A Building Storm
Over the past decade, several large non-profits like Planet Cancer, Livestrong Young Adult Alliance, and I’m Too Young for This! (see “Tweetup” below), have formed to help advocate for adolescents and young adults with cancer and provide comprehensive resources for questions outside their oncologist’s purview.
Matthew Zachary, a brash New Yorker diagnosed with brain cancer 15 years ago at age 21 and founder of I’m Too Young For This, capitalizes on social media to provide a directory of age-appropriate services, message boards, and organizations for the AYA oncology population.
“Fifteen years ago, no one got fair treatment in cancer,” said Zachary, “We weren’t even at a point where it was worth it to break out the young-adult population. We’ve reached a point now where patient care has improved, technology has improved, access has improved, where we can now finally single out what’s unique about getting cancer at this age versus this age.”
Just five years ago, a search for scholarly articles on AYA oncology returned a few results about survivors of pediatric cancer. Now, the National Cancer Institute boasts a growing webpage of resources specifically for the AYA population. Albritton, Bleyer, and Rytting point out that clinical trials designed for AYA patients are beginning to be opened, though participation is still poor, and Estrada noted that age limits for trial pediatric protocols have been extended to age 39 in most cases. In addition, a journal dedicated solely to AYA oncology will roll out next year.
Most healthcare professionals interviewed said the number one area of improvement is the development of best practices for any physician treating an AYA oncology patient. Albritton is helping to develop a computerized “pause” tool for physicians treating AYA oncology patients. “You put in a patient’s age, diagnosis and gender, it leads you through: Pause and think about fertility preservation, pause and think about what clinical trials might be available, pause and think about how the side effect might be different, pause and think about what their compliance issues might be, do they have transportation, school, or young kids. Whether you’re a pediatric or medical oncologist, you may need help thinking about those things because it’s not your daily routine,” she said.
The trick is still convincing the general public to support and advocate for AYA cancer awareness, much like community support boosted breast cancer and pediatric cancer awareness decades ago. “St. Jude’s is a machine and they’ve done an incredible job of marketing the cute bald kid, and you have to give money. It’s worked. It’s great. I’m not saying it’s not valid, but there’s eight times as many of those patients between 15-40 who don’t have any of that focus in terms of publicity, fundraising, or advocacy,” said Albritton, adding, “It’s building, the storm is building.” •
2010 Texas Adolescent and Young Adult Oncology Conference
123 Losoya St
Young People Get Cancer Too Tweetup
Methodist Children’s Hospital and LiveSTRONG host a “tweetup” social event for AYA cancer patients, survivors, and their friends and families. Keynote speaker: Matthew Zachary, founder and co-host of Stupid Cancer and I’m Too Young For This! Emcee: Karen Martinez, FOX News anchor and breast cancer survivor Music: The Tim and Bob Show. Plus: Local survivors’ panel, food, beverages, giveaways, and informational exhibits. 6-10pm Oct 1 at Tri-Point YMCA, 3233 N St Mary’s, facebook.com/youngpeoplegetcancertoo
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