October 17, 2002
Saying 'You're cured' is not enough; DFCI researchers study quality of life for pediatric brain tumor survivors
Christopher Turner, MD, with former patient Chris Johnson.
She's a model of hard work and diligence, yet for Arianne Del Rios, a petite, soft-spoken 7th grader at Trottier Middle School in Southborough, Mass., daily lessons are a struggle. She reads aloud beautifully, her mother says, but she may not grasp the main points. She excels in math and art, but has trouble with verbal matter. "If I'm studying for a test," Del Rios observes, "the information goes right through my brain and I don't remember it."
Her frustrating learning difficulties stem from damage to certain areas of her brain caused by eight weeks of radiation treatment for a tumor when she was 4 years old. Surgery and radiation destroyed the dangerous tumor, and for that she and her parents are forever grateful.
But now, Del Rios and her family are confronting the steep costs of medical success, as are many families of childhood brain tumor survivors. Children are being cured more often than in the past, at a success rate averaging 50 percent.Yet in the years that follow, patients may experience mild to severe memory loss, learning disabilities, lowered IQ, behavioral and personality problems, seizures, slow growth, weight gain, and sterility.
To meet these challenges, Dana-Farber has launched a new research program to assess the quality of life for pediatric brain tumor survivors and test different strategies for helping them cope.
"We're hearing from parents that they are very appreciative of what's been done for them here, but now they're trying to find a way for their children to have meaningful lives," says Tony Meyer, PsyD, the program's assistant director of quality-of-life research.
The side effects of brain tumor treatment have been apparent for some time, but in the past parents and doctors alike were mainly concerned with saving young lives. Now, survivors and their families want help overcoming obstacles to their life journeys. "It's a club you don't want to belong to," attests Maria Ternullo, whose son, Justin, is battling complications from brain tumor treatment nine years ago.
"Saying 'You're cured' is not enough," says Christopher Turner, MD, director of outcomes research for Pediatric Neuro-Oncology at Dana-Farber. "There are a host of issues and needs not being met among survivors of pediatric brain tumors. Dana-Farber is now focusing on these needs."
Brian Winston helps Del Rios with classwork.
A pediatric neuro-oncologist, Turner was recruited to DFCI by Mark Kieran, MD, PhD, to lead the quality-of-life program. Kieran heads the Stop & Shop Family Pediatric Brain Tumor Clinic, a partnership between the Institute and Children's Hospital Boston that bears the name of the supermarket chain that has long supported it.
Parents of these young patients have told DFCI researchers that their children need more help managing school, regulating their behavior, getting jobs, and generally becoming rehabilitated so they can be productive members of society.
The new effort is an extension of programs already in place at Dana-Farber for young brain tumor patients. These include a weekly multidisciplinary clinic, psychological consultation services, and the Bernon Family School Liaison Program, which helps teachers and school administrators better accommodate such patients.
In the works is a new clinic for brain tumor survivors who are several years past treatment. To be located in the Jimmy Fund Clinic after its upcoming renovation, the program is modeled after — and was developed with help from — the Institute's David B. Perini, Jr. Quality of Life Clinic, which serves survivors of all childhood cancers.
Staffing the new clinic will bean interdisciplinary team of neuro-oncology physicians, psychiatrists, psychologists, social workers, physical therapists, resource specialists, complementary medicine specialists, and others.
Refining the treatments
Brain tumors in children are among the most feared diseases, but, fortunately, they are relatively rare. About 2,200 are diagnosed each year in the United States, according to the American Brain Tumor Association — most in children under age 7. Pediatric brain tumors are the second-leading cause of cancer death in children after leukemia.
The Institute's Pediatric Brain Tumor Clinic sees about 150 new patients each year. They receive checkups, chemotherapy, and other outpatient services at the Jimmy Fund Clinic, radiation therapy at Brigham and Women's Hospital, and inpatient care at Children's.
There are many tumor types. Among the most common are low-grade astrocytomas, medulloblastomas, high-grade gliomas, and ependymomas. Some types are nearly always fatal, while others are usually curable. In general, however, the outlook for children with brain tumors has brightened in the past two decades, thanks to refined surgery and improved chemotherapy and radiation.
A tumor and its treatment unavoidably damage normal brain tissue. Some children are so mildly affected that there's little outward sign, while others can barely care for themselves. Researchers are working to find more selective treatments, but until they do, physicians walk a delicate line as they try to reduce radiation doses — or avoid giving it to the youngest patients altogether — without compromising the tumor-killing impact of the therapies.
As one example of progress in the field, brain tumor specialists at Children's have been at the forefront of efforts to give radiation in highly focused beams that zap just the tumor and its surroundings, sparing as much normal brain tissue as possible.
"We have also been using chemotherapy to help kids live longer and reach an age when radiation would be less toxic," explains Scott Pomeroy, MD, director of Neurological Neuro-Oncology at Children's and a DFCI consultant. "At first the goal was age 7, and now it's greater than age 10. Some people are wondering whether we can actually replace radiation with chemotherapy."
Studying the lingering effects
With its new clinic and research efforts in progress, the Dana-Farber team is gathering information on survivors' long-term mental and physical complications in a systematic way. Its goal is to help both patients and caregivers, now and into the future.
"This may allow us to change our current therapy and avoid these problems," Turner predicts. For example, a clinical trial at DFCI is now evaluating the use of chemotherapy injected directly into the spinal fluid as an alternative to irradiating the entire brain and spine of young brain tumor patients. Adds Kieran, "By working to understand these issues, we hope to improve the quality of life for patients here, as well as around the world."
In some cases, the consequences of treatment are predictable, giving parents a head start on preparing for the difficulties ahead. Robin Plumer, mother of Arianne Del Rios, says she took steps to adjust to her daughter's likely disabilities. "I would break down instructions into small sentences that she could comprehend," says Plumer. "I knew what to watch for."
The aftereffects depend in part on the tumor's location and how much of the brain was treated with radiation. Justin Ternullo, 20, of Assonet, Mass., had surgery when he was 10 to remove a life-threatening tumor buried in his brainstem. He had been diagnosed after complaining of headaches and blurred vision. The surgeons couldn't remove the entire tumor, and when it quickly began growing again, the boy underwent whole-brain radiation that is largely responsible for the problems he battles today.
Physically, Ternullo is short for his age, and thyroid damage has caused him to gain 80 pounds in the past two years, says his mother, Maria. His cognitive challenges have an irregular pattern; he has great difficulty with math, but his memory for verbal information is excellent. When it comes to inferring a big picture from assorted details, Ternullo may stumble if it's too abstract. And "if you joke with him, he doesn't realize it," his mother adds. He is also painfully aware that he's on a more isolated track socially. "His friends are growing and moving on," she attests, "and he's staying the same."
According to Meyer, the children who were older when treated are more likely to recognize their deficits. "They remember how they were able to play baseball and now can't, or formerly made the honor roll but now have trouble processing basic information."
Ternullo, who is attending Boston College this fall, has received brief psychological counseling at Dana-Farber, as well as educational help through the Bernon Family School Liaison Program.
For longer-term treatment, psychosocial staff at the Institute can help families find resources in their own communities. Parents, says Meyer, often need to talk about their frustration and (sometimes) guilt prompted by the changes in their child brought on by tumors or medical care. "They feel they have fought so hard to get the young patient through the treatment," he observes, "and now they're facing a new dilemma: dealing with the medical, psychosocial, and neuro-cognitive consequences."
Despite all its efforts, Dana-Farber wants to do even more for these patients, and the expanded program is a big first step. As it develops, says Meyer, "We hope our work can become a model to guide development of comprehensive, multidisciplinary services for young survivors at other institutions as well."
This story first appeared in the Fall/Winter 2002 issue of Paths of Progress magazine.
