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Childhood Cancer Survivors Face Lifelong Challenges

Her 7-year-old daughter Lexi returned from softball practice complaining of a pain in her chest along the breastbone, and though the most natural thing in the world would have been to assume this was some minor ballgame injury, Kayci Wilson sensed something much worse.

"My mother's intuition said something's really, really wrong here," she says. Instead of waiting to see how things developed, Wilson took Lexi to and urgent care clinic the next morning. The normally reserved Lexi piped up to say she was tired, and Wilson felt a twinge of maternal dread. After examining Lexi and drawing blood for tests, the doctor said she had a viral infection and sent Wilson home with the usual advice: rest and liquids.

But less than two weeks later, Lexi was hospitalized at Children's Mercy Hospital in Kansas City, Missouri, where doctors diagnosed her with anaplastic large-cell lymphoma, a rare form of non-Hodgkin lymphoma that affects white blood cells. The diseased cells then travel to other parts of the body, including the lymph nodes, spleen, bone marrow, blood or organs. It's often an aggressive cancer. With treatment, many stricken children survive. Still, in just 12 days, Lexi had gone from a rough-and-tumble softball player to a child so thin and weak her parents had to carry her out of the house and into the hospital.

The next few months were a whirlwind: chemotherapy, radiation treatment, a diagnosis of remission, a recurrence, a bone marrow transplant from Lexi's 4-year-old sister Audrey and then, finally, recovery.

The all-clear sign was far from the end, though. Lexi suffered an infection shortly after her bone marrow transplant, and she spent almost the entire next year in bed. "We were unprepared for how long it would take," says Wilson. Even now, Lexi still cannot run the way she used to-her hips are too weak. But with the help of tutors, she's managed not to fall behind at school. She even won an award for reading this year.

Though the specter of lymphoma is gone, the doctors gave Wilson some more bad news during a recent checkup: At the age of 9, Lexi received a diagnosis of primary ovarian failure. "She's basically not going to have children when she's older," Wilson says, her voice breaking. "I didn't realize it would happen that fast. I was heartbroken."

Despite their persistence in the face of disease, survivors of childhood cancer like Lexi are at high risk for what doctors refer to as "late effects." Chemotherapy drugs and radiation not only destroy cancer cells but also cause undetected damage to the DNA of normal cells nearby. The resulting late effects can include infertility, heart and other cardiovascular problems, neurocognitive effects, growth problems and even secondary cancers-not a metastasis but an entirely new tumor.

"By 30 years from diagnosis of the original cancer, 22 percent of [surviving] children will have developed a second neoplasm; that includes both malignant and benign tumors," says Dr. Gregory Armstrong, principal investigator of the Childhood Cancer Survivor Study, which contains data on more than 34,000 survivors nationwide. There are 400,000 childhood cancer survivors alive today; by 2020, that number will grow to half a million. Based on the current data, malignant tumors will strike about 11 percent of these survivors, most when they are still young adults. "These are people who are still in their 30s and 40s," says Armstrong, whose research shows that childhood cancer survivors are 15 times more likely to die from a second cancer than the general population dying from any cancer.

Roberto Martinez reflects on his experience battling germ cell (testicular) cancer at age 17, at his home in Los Angeles, May 09, 2008. In his 20s and cancer free, Martinez is heading into a career in health care, armed with deeper knowledge of cancer treatments. He worries about the late health effects on childhood cancer survivors.

To a large extent, this is a result of a necessary balancing act. Over recent years, the success rates for curing childhood cancers have inched up, and today survival rates are astounding. Dedicated doctors and nurses in pediatric oncology wards across the nation cure 80 percent of their patients. For many childhood cancers, the survival rate tops 90 percent. More important, "we have children who could survive not just six months, not just three years, five years, but for 60 years and 70 years," says Dr. Andrew L. Kung, professor of pediatrics at Columbia University and chief of the Department of Pediatrics' Division of Hematology, Oncology and Stem Cell Transplantation.

Unfortunately, that also means there are many children who, as adults, get secondary illnesses. Most of the late effects of cancer are due to exposure to radiation and some chemotherapy agents, including anthracyclines, which are "classic" and important drugs used to treat many different types of cancer, including breast and lung cancers and leukemia, says Armstrong. It is well-known that the higher the dose of radiation, the higher the risk of a second cancer-and much of the recent improvements in survival rates in children have been driven by oncologists using stronger doses to fight childhood cancer.

Research also shows that chest-directed radiation increases the risk for cardiac disease and cardiac death. Anthracyclines also affect heart function, says Kung, while another chemotherapy drug, etoposide, can cause genetic mutations leading to a second unrelated cancer.

Today, oncologists are working to limit the long-term health impacts of these cancer-killers by, for example, avoiding the use of radiation when treating some leukemias and lowering radiation doses used to treat Hodgkin lymphoma. Newer technologies, such as proton beam irradiation, will be able to treat the tumor while sparing nearby normal tissue from high doses of radiation. Future generations of survivors will not be exposed to quite so much radiation as generations past. And with the recent advent of precision medicine, the war chest of chemo drugs has increased, allowing doctors not only a greater choice of drugs but also the ability to use lower doses, which means fewer late effects for survivors.

"The good news is, 20, 30, 40 years down the road, those kids have lower mortality rates due to second cancers and heart disease," says Armstrong.

The other key to long-term health is ongoing "survivor care," designed specifically to address the fear, anxiety, nutritional challenges, physical disability and financial burdens that can come with the late effects of cancer survival. Ashley Dado was treated for brain cancer at the age of 10. At the age of 18, she transitioned to the Survive and Thrive Clinic, part of the University of Kansas Cancer Center for adult survivors of childhood cancers. Recently, Dado, now 22, graduated from MidAmerica Nazarene University with a degree in health and exercise science, yet she still makes yearly visits to monitor her late effects-hand-eye coordination, trouble concentrating and adrenal insufficiency, which requires her to take a growth hormone.

Gaps in survivor care still exist for some, says Carol Bush, an oncology nurse navigator with the Midwest Cancer Alliance and the University of Kansas Cancer Center. Addressing psychological problems can be particularly challenging. For example, though terminally ill patients often create strong networks and support groups among themselves, when one of them makes unusual progress-a long remission or becoming cured-they can develop survivor guilt, says Bush, and they feel isolated. There is no built-in support to address these types of difficulties.

In fact, between 13 and 17 percent of childhood cancer survivors experience mental health problems, pain or anxiety, with high levels of psychological distress, says Siobhan M. Phillips, assistant professor of preventive and behavioral medicine at the Northwestern University Feinberg School of Medicine in Chicago. These can lead to serious cognitive decline: Just over one-third of childhood cancer survivors between 20 and 49 have more trouble remembering things, solving problems and prioritizing tasks than their same-age peers, her most recent research suggests. "What's really striking is when you put this in context of how old these survivors are," Phillips says. "You wouldn't expect these conditions to develop until much older ages, yet everyone [in her recent study] was under the age of 50."

That's a major part of the problem, of course. While Bush favors addressing survivor problems immediately following a diagnosis, so that cancer patients and their families will know what to expect, she says that "all of those late effects don't really resonate because you're concerned about living."

The fallout of cancer treatment often becomes clear long after the fact. "I don't think Lexi's battle is necessarily over just because we don't have cancer," says Wilson. "Maybe the scary part is over, but we still have so many tough life situations ahead of us. There's probably many unshed tears."

By Susan Scutti, as published July 23, 2015 in Newsweek.