by Linda Janower
(Contact AKBTC)

This candid and heart-wrenching story, shared by a grandmother whose grandchild has JPA, reminds us all that JPA can show up in any child, at any age. Not enough research is in progress, diminishing our hopes for kinder, gentler treatments, not to speak of a cure. Samantha wanted to be a ballerina. Suddenly, she started stumbling on her left foot and couldn’t lift her left arm. She had JPA growing in her brain.

I am dreaming of my granddaughter, Samantha, 4 years old, laughing and healthy. Suddenly a huge black cloud covers her, and she cries as she limps towards me. I wake up in a sweat. I am having a nightmare about Samantha and her so-called benign brain tumor.

I fly home to visit the hospital where Samantha is lying in bed with a high fever, connected to tubes for intravenous antibiotics and hydration. She is nauseous and listless. Her bare scalp peeks through thinning hair. Her usual cheerful greeting has been replaced by a feeble smile. Her emaciated body is down to 32 pounds. The drug cocktail she receives through a port implanted in her chest has destroyed her bone marrow. Robbed of her body’s crucial red blood cells, she lies in bed, dozing. I remember when she kept after me while I was reading the morning newspaper: C’mon grandma, let’s run!

Twenty years ago, Samantha’s diagnosis would probably have been fatal. Now, 70% of children with Samantha’s tumor, JPA or pilocytic astrocytoma, survive for at least l0 years. However, the drugs, which are used to control it, for which I am so grateful, have compromised her immunity. Her white blood count is perilously low. A coughing child poses a threat to her survival. If she gets sick, we could lose her to a fatal pneumonia. I anxiously eye the sign on a nearby hospital room: Air Bourne Virus; Do Not Enter. Samantha giggles as Allie, her 14 month old sister, dashes into the forbidden room when the nurse opens the door. Samantha startles me with her four-year-old perspective: It’s Allie’s turn to get a brain tumor, right Grandma? I leave the room. I do not want my children and grandchildren to see me cry.

Samantha’s condition has had dramatic effects on our family. An estimated 2,000 children have brain tumors each year, and 40% of them (1,100 children under age l8) are diagnosed with pilocytic astrocytomas. That’s thousands of shattered families, relatives, friends, neighbors, coworkers, schoolmates, every year that know and love one child. Samantha’s mother, had to leave her job after surgery, in order to manage Samantha’s multiple appointments – weekly chemotherapy infusions, physical therapy (for the weakness in her right side caused by her tumor), occupational therapy, family therapy, CAT Scans, MRIs, blood tests, etc. Mom or dad rushed her to the ER when she spiked a fever. And they took turns sleeping at her side when she was hospitalized. When she was home, she was up frequently at night; both parents were sleep deprived. How could my son concentrate at work? I wondered. How can they stay grounded?

Samantha’s sister, Michela, age 7, missed Samantha when she was hospitalized. However, sometimes she was resentful: Why can Samantha eat all the ice cream she wants, but I can’t? If Samantha is your miracle child, what am I…just your plain old ordinary one?

“Siblings are affected, that’s for sure. My older daughter asked, ‘If Samantha is your miracle child, what am I? Just your plain ordinary one?’ ”
– Samantha’s Dad
Samantha’s dad turned his grief into action last May. Believing that research was our best hope for cure, he raised $450,000 for brain tumor research; the funds were used for promising approaches for adult malignant tumors, following government research priorities, which though understandable, do not directly affect JPA, Samantha’s disease. Brain tumors do not attract a lot of research money anyway because the number of people affected is small compared to other life threatening illnesses. But children’s brain tumors get even less funding. Federal funding for non-malignant brain tumors for children is non-existent and private donations from friends and family cannot possibly support the research needed to find a cause and a cure for JPA.Funding for children’s brain tumors is affected by many factors ranging from number of cases, mortality, and survival statistics, to the level of national awareness, celebrity involvement, lobbying, and politics. But children can’t vote and parents of children with brain tumors can barely get through the day, much less write their congressmen for research funds.So-called benign tumors like Samantha’s have been almost totally overshadowed in research funds because they are technically not malignant, although certainly life-threatening and potentially devastating; they are far from benign. (The Federal Government did not even keep benign brain tumor statistics for adults or children until 2004). I cannot find any research projects focused on JPA (a grade 1 or stage l, low-grade glioma which has its own biology structure, behavior and treatment, and is a quite different disease than even Stage 2, 3 or 4 gliomas in children).

I am not a scientist, but I searched the Internet and asked varied experts at pediatric cancer centers, children’s hospitals, and children’s and pediatric foundations across the country, and could find no currently funded JPA research in any of the diverse promising areas for adult or even children’s brain tumors (e.g., genes, DNA, molecular, proteonomics, etc.). I could not find a single clinical trial or even pre-clinical trial involving JPA patients either, and yet I know that the parents hang onto the belief that all this is happening and search the Internet constantly. While there are many dedicated and expert scientists, there is nothing in the pipeline working specifically on or for children with JPA, because of its low statistics (1,100 U.S. cases a year), low mortality rate (30%) and the current view that it is manageable. Tell that to the thousands who have lost a child or to the current families witnessing the daily heartbreaking changes they see in their child’s quality of life. Benign is not the operative word for JPA for this grandmother.

Now what about Samantha? After a skilled team at the Dana Farber removed 75% of the tumor embedded in Samantha’s brain stem (leaving 25% which was too close to her vital functions), and 60 subsequent weeks of chemotherapy, Samantha’s tumor is only slightly smaller. At this stage, 60-70% of JPA tumors grow within 6 years; the average period is 3 years. I am grateful for the four more possible chemotherapy protocols available, but pediatric oncologists tell us that they can wear down children’s systems and have real and potentially life-altering side effects.

Radiation, the next alternative, is risky and pediatric neuro radiologists do not like to radiate a child so young, since radiation has potential for killing growing brain cells, potentially compromising vital functions like sight, speech, movement, intelligence, even the ability to have children (if the child survives to adulthood); even with the best of current equipment available, and assuming they can get to the tumor with radiation, her normal childhood and quality of life can be seriously compromised.

Samantha is happier now, but she has a significant shadow over her life, because the tumor is still in her brain stem near all her vital signs and has a 70% chance of growing within an average of 3 years

Now Samantha is thriving and well. She is a friendly, kind, bubbly, adorable, four year old girl who loves to laugh. She has a sense of wonder about everything. “Now I can come and sleep over,” she announced to me after her chemo was over. “But actually there is one thing you need to know, and one thing you should know,” she added emphatically. “You need to know how to give me my medicine,” and she detailed all the instructions. “And you should know that I like ice cream, and I prefer strawberry!” she added with a wink. While I am overjoyed with her return to a normal schedule and routine (but still including physical therapy, MRIs every 3 months, etc.) and try to live moment to moment, I wonder about her future. How can I be hopeful if there is no research in the pipeline to identify a cause, and no 100% cure? The truth is I am a 65-year-old grandmother, who is petrified.

And so I continue to ask: What can I do? How can I hope to keep my granddaughter and the thousands of other children? How can I inspire the many thousands of others, volunteer and professional, worldwide (when I don’t even know who they are) to work with any time and resources they have on or for JPA research? What can I do to use any skills I might still have in cause-related health marketing to become a JPA catalyst for action? And finally will we get the research funded in time? Will Samantha and so many others benefit from new breakthroughs in treatment and thus have normal childhoods?

Prayers are not enough for me. I have renewed my faith in God believing in the compassion of others who are blessed with powerful, intact brains to create blessings for those young ones who are not so fortunate.

I must believe something good can come of this journey, so when Samantha asks me the inevitable question, “Will I die?”, I can definitively say, “No, not if grandma can help it.”

I am happy to provide advice, counsel and support to any volunteer fundraising project (yours or mine) to benefit CBTF, my charity of choice, or your own if you prefer, as long as you and they share the commitment to use all of the funds to accelerate JPA research and education. To e-mail Linda, e-mail [email protected]