Our daughter, Vivian Rose Weaver, is a smart, articulate, funny, sweet, thoughtful, imaginative, precocious, 3.5 year old who lives in Husum, Washington. She is full of love for life and people. She loves princesses and superheroes. She loves art, she loves her friends, she loves her large extended family and she really loves ballet. She is excited for her first recital in June. Like most children at this age life is full of discovery and she’s full of joy and happiness.
On the evening of Monday January 29, 2018 Vivian was riding her electric 4-wheeler and complained of a headache. Her Dad, an ER Physician, sat Vivian on the lawn and briefly examined her. She had previously been diagnosed with strabismus, the most common eye deviation problem in children, and we hadn’t been too worried about it. But when her father noticed a subtle facial nerve palsy he was immediately concerned. The likelihood of two cranial nerve palsies without significant pathology was unlikely. An MRI performed 2 days later confirmed our worst fear.
February 1, 2018 Vivian was diagnosed with a large brainstem tumor. Due to the obstructive effects resulting from the tumor significant pressure within the brain had developed. She was immediately transferred to Oregon Health and Sciences University where a VP Shunt was placed later in the day. This drain allowed pressure in the brain to normalize and the facial weakness and eye deviation gradually improved. That day we heard the term DIPG for the first time. As we discussed Vivian’s case with the pediatric neuro-oncologist the significance of the diagnosis became clear: No surgical options. No known effective chemotherapy options. A uniformly fatal disease. Average life expectancy from time of diagnosis, 10-12 months. It’s difficult to describe the crushing nature of this news.
After extensive conversation with the pediatric neuro-oncologist and pediatric neurosurgeon we made the decision to biopsy the tumor. Her clinical course was atypical, it was unclear over what time frame these symptoms developed, and our hope was that the biopsy would prove this tumor to be something less aggressive. Reaching the brainstem is no easy task, but the skilled physicians at OHSU accomplished this task with no complications. The pathology report, however, confirmed the tumor to be DIPG, Diffuse Intrinsic Pontine Glioma.
Over six week spanning February and March of 2018, Vivian bravely completed 6 weeks of daily radiation therapy at Oregon Health & Science University. Concurrently, she participated in a trial of a chemotherapy drug, AZD 1775. This was a challenging six weeks for Vivian and our family. We moved into a friend’s condo in Portland, returning home each weekend. Each day we were at the hospital, and given Vivian’s young age and presumed inability to remain motionless for the duration of each radiation treatment she was sedated for each treatment. Each day Vivian insisted on walking into the radiation suite and climbing onto the radiation table herself. She would hold out her arm so they could access her PICC line, and she would insist on holding something scented near her nose so she wouldn’t taste the medicine as it was infused. She never cried. She never complained or struggled, but she didn’t like it at all. Each day as the medicine was infused and as we snuggled closed to one another she would fall asleep in our arms. We would lay her limp body onto the table and watch them carefully position her. We would step out of the room and wait for them to call us back. Then we would sit with her as she gradually awoke.
Vivian couldn’t eat for 6 hours before the sedation. Immediately after awakening from sedation she would take her chemo pills, then wait 2 more hours to eat. This was probably the most difficult and challenging aspect of these 6 weeks. Vivian would miss breakfast every day. She was typically cleared to eat by noon, but her appetite was gone and she would often fall asleep by then. She would often go 16hrs at a time without food. She lost 12% of her body weight, blood counts went down, and she received one blood transfusion during the 4th week of treatment. Despite all of this, and the many appointments and long days in the hospital, Vivian rarely complained.
Well before Vivian’s diagnosis we had scheduled a Spring Break trip to Hawaii. Vivian didn’t miss a single day of therapy, so we were able to make the trip as scheduled 2 days after she finished radiation treatments. We were able to spend time together as a family recuperating, resting, sleeping better and trying to expand Vivian’s diet. Vivian loves to swim, and after having a PICC line for 6 weeks which prevented normal bathing she swam like a fish the entire week. She was able to regain some of her spunk, stamina, and energy. Today, she is almost symptom free. We believe we picked up on her symptoms earlier than normal, allowing for an earlier diagnosis and initiation of treatment earlier in the disease process. We are thankful she is doing so well.
At the end of April we went to Disney World through the Make-a-Wish Foundation. Each time I realize we have a “Wish” kid it brings tears to my eyes and puts a knot in my stomach. Vivian is running around, dancing, laughing and behaving like any normal 3 year old, and we struggle to rationalize what we see and what we know.
There are no treatment recommendations following radiation. We, along with our doctor, spent several weeks coming up with an experimental treatment plan for her in hopes of prolonging her life long enough to find a cure or possibly actually being a cure.
After six weeks of paperwork and reviews by the FDA and internal review board, she received thru compassionate care a “halo” device called the Voyager. She is the very first DIPG patient to use this. The company, Nativis, has been conducting clinical trials on the Voyager for adult patients with glioblastoma for several years. We believe that God led us to this unique treatment by way of my brother happening to be at an investment opportunity meeting for the company one week after Vivian was diagnosed. In theory the device inhibits cell division by emitting low frequency radio energy around her head. This will, in theory, mimic chemotherapeutic actions without all of the side effects. It is specifically good for brain tumors since chemotherapy agents have such a hard time crossing the blood brain barrier.
Concurrently, we spent two months getting insurance approval for Vivian to use a chemotherapy drug called Panobinostat. After two appeals, Simon spent several days preparing and appeared before the board of Providence Health Plan to argue the case for using the drug. We had letters from our Pediatric Neuro-oncologist as well as the leading researcher, Dr. Michelle Monje at Stanford, to argue her case. This drug has shown in clinical trials to prolong life in DIPG patients. We hope and pray that the combination of both of these treatments will be a miracle for Vivian and a cure for all kids suffering from this terrible disease.
The chemotherapy drug has some somewhat severe side effects. Constant fatigue, nausea, diarrhea, vomiting. She takes it 2 weeks on and 2 weeks off. It is so hard to see her not feeling well. We hope the side effects will dissipate as she gets used to the drug and that we can alleviate some of them with supporting medications.
Simon and I are immensely appreciative for the help and care Vivian has received from her providers. Her physicians have been amazing, and the nurses and supporting staff have all been kind and caring. As we’ve investigated this disease we are increasingly thankful for the many physicians, researchers and teams working behind the scene to identify a cure for this terrible disease, and we have created this foundation with the intention of doing our part to raise awareness of DIPG and to raise crucial funds that have spurred much of the recent and promising research that is slowly whittling away at DIPG. A cure will come, and private research funding will have, and has already had, a pivotal role in slowly unraveling the complexities of this disease.
Finally, we find solace knowing that our God in heaven loves Vivian more deeply than we can comprehend. We do not know what God has in store for Vivian, and even as we struggle to understand His plan we find peace and joy in God’s many blessings. Vivian was an answer to prayer and we continue to thank God daily for blessing us with her life, for allowing us to love and parent her each day.
Brief updates…January 31, 2019, one day shy of one year since diagnosis, Vivian’s MRI showed her tumor was growing again. We began a second round of 12 radiation treatments. This second round of radiation was something very new in the world of DIPG that had been happening only a few months prior to her needing it. The first round meets the criteria of lifetime maximum allowable dose. The risk of secondary issues because of the extra radiation continues to go up, but researchers realized that these kids are all going to die anyway, so why not try it. This has bought Vivian, and many other children more time with their families.
On September 25, 2019 Vivian’s bimonthly MRI once again confirmed that her tumor was growing again. We discussed an unprecedented third round of radiation but decided we felt her body had already had enough. She was already only walking with a walker and having a lot of difficulty speaking.
Today is November 18…she can no longer walk with her walker, she is having difficulty swallowing, we can understand a few words, and she rarely tries to talk. Yet, we keep her busy doing art projects, helping make cookies, we got our Christmas tree a week ago so she would be here to help us decorate it and the house which she loves. She is happy and smiling, loves her Mom, Dad, and sister, Lucie, immensely. She is actually at school for over half of the day today. She loves her class and her teacher but can only do so much. They have been amazingly accommodating. We love this sweet girl so very much and it is entirely heartbreaking to watch her go thru this.
Below are a few texts we sent to family when Vivian was first diagnosed: