In many ways, Isabelle Wagner is an ordinary first-grader: a creative, contented girl who loves drawing, making mud pies and climbing trees.
But one thing sets her apart: She has survived cancer. On Feb. 10, she passed the five-year anniversary of a lifesaving surgery at Lucile Packard Children?s Hospital to remove a malignant, golf-ball-sized tumor from her brain. It?s a significant milestone in a journey that included 14 months of chemotherapy and radiation, and still requires regular brain scans to ensure the tumor has not returned.
?It?s fantastic,? said Isabelle?s dad, Derek. ?And yet there?s a bit of holding back, the feeling that this isn?t over.?
?I?m really excited. But that scares me, to be excited? said Isabelle?s mom, Heather, who still worries about letting her guard down about her daughter?s health.
A generation ago, few patients with Isabelle?s diagnosis of medulloblastoma made it to the five-year mark. The cancer is the most common malignant pediatric brain tumor, and today, more than half of the 350 U.S. patients diagnosed each year survive, though reaching the five-year landmark does not mean their journey is finished. Isabelle still faces the risk of late-appearing side effects from the treatment she received ? a legacy of the delicate balance her doctors faced between killing the cancer and minimizing harm to her developing brain. And her parents, Derek and Heather, are still working to maintain equilibrium between helping their daughter enjoy her childhood and survive a life-threatening illness.
Excising the tumor
Isabelle?s medulloblastoma, a tumor at the base of her brain, was discovered in an MRI a few months before her second birthday. Isabelle?s pediatrician requested the scan after the family had experienced several months of seeing her go gradually downhill. The little girl was vomiting frequently and regressing developmentally, forgetting words she had already learned.
When Isabelle?s tumor was identified, she was immediately scheduled for surgery with Michael Edwards, MD, who is chief of pediatric neurosurgery at Packard Children?s. Even in the two days between the MRI and her surgery, Derek and Heather saw Isabelle decline further, suffering severe pain.
?We started living life in segments of time,? Derek said. ?Things changed by the minute in those first couple of days, and we just lived for the next few minutes.?
Derek and Heather sat down with Edwards so he could explain the planned operation. After he described the procedure, he asked if they had any questions. ?We didn?t know what to say,? Derek recalled. ?Dr. Edwards said, ?Well, you guys are in shock right now, so let me ask the questions that you should ask, and I?ll give you the answers.? He walked us through everything and set expectations for us. It was amazing.?
The next segment of time that stands out in the Wagners? memories is the eight-hour wait during Isabelle?s surgery.
?The surgery went very well,? Edwards said, noting that the team removed all of the visible tumor. ?She came out without significant neurologic deficits.?
After surgery, Isabelle began improving right away.
?Instantly she was in better shape,? Derek said. ?You could tell that she wasn?t in the same kind of pain.?
?In the hospital, she cracked a joke for the first time ever,? Heather said, recalling her amazement at the transformation Isabelle went through once the tumor was gone. ?Every day she would have at least five new words.?
One month after surgery, Isabelle began a course of chemotherapy and radiation designed to prevent the cancer from returning. She was part of the first group of young medulloblastoma patients in the world to receive radiation only at the site of the tumor ? a departure from the previous standard of irradiating the whole brain and spinal column.
?We wanted to tailor her treatment such that we could cure her but also not devastate her,? said Isabelle?s oncologist, Paul Fisher, MD, chief of neurology at Packard Children?s. The hospital?s active role in clinical trials often allows his team to offer patients groundbreaking therapies, Fisher said. In many cases, this means the Packard team is working to achieve the best balance between saving the child?s life and minimizing long-term effects from heavy-duty radiation and toxic anti-cancer drugs.
In Isabelle?s case, the new therapy spared her brain function. Young children who receive radiation to the entire brain and spinal cord inevitably experience significant developmental delays.
?Isabelle is in the vanguard of lesser treatment to create better long-term outcomes,? Fisher said, noting that the treatment she received is now widely used.
Isabelle?s family and physicians remain on the lookout for long-term complications, including problems with her cognitive function, school performance and psychological health. There are also physical risks that stem from her treatment. Childhood cancer survivors can have strokes in their 20s, 30s and 40s, and some experience impaired fertility as adults. Isabelle also runs a small risk of developing a secondary cancer from her radiation exposure.
But in spite of the risks, Isabelle is doing extremely well. And she has the support system she needs to make sure that complications can be caught early and treated aggressively. ?Isabelle is very, very lucky in that things all synched for her,? Edwards said. ?She has received really good care and follow-up, and her family is making sure she?s getting maximal management and intervention for any problems that are identified.?
?She is ? gosh, she?s thriving,? Fisher added.
Heather and Derek certainly agree. Though they will always be extra-vigilant about her health, they are thankful to be enjoying every minute of Isabelle?s ordinary adventures ? tree climbing, mud pies and all.
Foto’s Courtesy of the Wagner family.