In 2008, when our twenty-seven month old daughter Alexis was diagnosed with something called DIPG, or diffuse intrinsic pontine glioma, I knew nothing about the world of childhood cancer or even the terror that those letters cause. Alexis was by all accounts a normally developing child. She enjoyed music, TV shows, blowing bubbles, art projects and so much more. She ran, she jumped, she played and she was going to her first nursery school program. There was an issue for several months when she was vomiting at night, as well as a very slight and intermittent concern with her right eye turning inward. What we anticipated to be nothing more than lazy eye turned out to be a pediatric brain tumor.
What we soon learned was that this was no ordinary brain tumor; this was DIPG. Within the first three minutes of her diagnosis we were told that even with current treatment, Alexis had nine to twelve months to live. I will never forget the first evening of Alexis’ initial 2 day admission to the hospital when she was diagnosed. Late at night on the very first night in the hospital, I found myself standing behind the neurosurgeon who was attending at the hospital. This was the doctor who essentially diagnosed Alexis by looking at her original CT scan. He wrote down the words “Pontine Glioma” on the back of his business card and then said several words that will haunt me forever: “Do me a favor, don’t Google this.” Of course we did just that and immediately discovered the universally dismal prognosis of children with DIPG.
My immediate thought was to contact the best neurosurgeons in the world and get the tumor out of Alexis’s head. With naïve ambition, we emailed several well-known neurosurgeons in what was of course a futile effort. Over the course of the next thirty-three months, we researched, we enrolled Alexis in five clinical trials, and we raised money to fund additional research. But unfortunately, not much changed, and Alexis passed away in January 2011.
In some respects, Alexis’ story is one of the past, yet it also continues to be very much one of the present as well. Since her diagnosis and passing, more focus and financial resources have been concentrated upon treating and finding a cure for DIPG. In 2011, the very first DIPG Symposium was held in Cincinnati, Ohio. About 60 researchers with some focus on DIPG sat in one room and discussed nothing but DIPG and potential future collaborations. In another room, approximately fifteen family foundations discussed how to collaborate to create change for children with DIPG. Something unique occurred during that meeting. Both rooms came to the same conclusion: collaboration was necessary to break the cycle of loss and lack of treatments for children with DIPG.
Since 2011, the DIPG Symposium has convened on two other occasions. The most recent meeting was held in Chicago on April 24 and 25th, 2015. This time there were more researchers with primary career focuses on DIPG who filled the seats . More money is now being provided to DIPG research than ever before as a result of the efforts of the DIPG Collaborative and other family run foundations. This year, when the meeting came to a close, the researchers gathered were told that $2.5 million is available on June 1, 2015 for the next grant cycle of the DIPG Collaborative. This is what promise and hope look like for a community that for so long has had little of either.
The unfortunate reality is that there have been over two hundred clinical trials initiated for children with DIPG without any noted efficacy. Trial after and trial has universally failed for most of these children. The only standard treatment ever to show efficacy is radiation, and that is simply palliative in nature. Most children diagnosed with DIPG are between five to eight years old and are generally provided with the prognosis of nine to twelve months to live following diagnosis. This is the story that continues regardless of the efforts to date. I have often wondered how it was possible that man walked on the moon in 1969 and yet there has not been a cure found for a brain tumor resting just inside a child’s skull. The irony of this fact is that the man who took those first steps on the surface of the moon, Neil Armstrong, lost one of his own children to DIPG in 1962.
So, where does one find hope in the story of DIPG? The hope is based upon a community that has rallied together to insist that the story changes. It is borne in the work that so many are doing now in a more concerted and desperate effort to break the cycle. This hope takes shape in the following ways:
Mark Souwedane, MD, from Weill Cornell Medical Center in New York City is pioneering his research on a targeted delivery system called convection enhanced delivery, which is a method of inserting catheters directly into the tumor to bypass the blood brain barrier and ensure the drug of choice is delivered directly into the tumor. A drug that has undergone three years of preclinical testing, and has shown good levels of penetration into the brain will soon be moved into a new clinical trial. Although the drug is not a cure, the hope is that it provides an overall improvement to survival. New attempts at utilizing vaccine therapies to treat children with DIPG were discussed by Oren Becher, MD, from Duke University. The DIPG Registry (www.dipgregistry.org) that was formed as a result of the first symposium in 2011 now has hundreds of tissue samples, patient records and close to one thousand individual MRI scans for analysis. The overall sense of compassion demonstrated by the researchers and clinicians alike was beyond palpable. And finally there is Lauren Hill, the courageous college basketball player who simply wanted her legacy to be one of helping other children diagnosed with DIPG. She did just that by increasing the profile of the disease and raising a lot of money in the process. Lauren personified hope in every facet of the word.
I wish that I were writing about a cure being found. Of course I am not. With that said, I remain hopeful that this is simply a matter of time. Over the years I have gotten to know many special children who have been diagnosed with cancer. Unfortunately it just so happens that I know more of those children who have not survived than those who have. Recently I’ve gotten to know one special little girl in my area who was diagnosed with DIPG in December 2014. It is both beautiful and painful at the same time to grow to love this little girl. I do have hope for her, I truly do, but it is always mixed with caution. It is a long road to hope, but it is a road that I truly believe leads to a finish line with a cure on the other side.
Jonathan Eric Agin is a childhood cancer advocate, speaker and an attorney (www.jonathanagin.com) in Washington, DC. He is married to Neely Agin and is the father of Alexis, Gabriel and Trevor Agin. Alexis was diagnosed in April 2008 with a tumor in her brainstem and battled for 33 months before passing away on January 14, 2011. Mr. Agin is the Director of External Affairs for the Max Cure Foundation and the General Counsel for the Children’s Cancer Therapy Development Institute. Mr. Agin, along with his wife, run a Washington, DC chapter of a pediatric brain cancer foundation. In his spare time, Jonathan participates in endurance events, such as triathlons and running races to raise increased awareness for childhood cancer.