by Amy Wood

Raymond A. Wood Foundation Tackles Survivable Brain Tumors That Pose a High Price for Survival

Amy Wood, Executive Director, Raymond A Wood Foundation

United States

It was a regular Thursday morning in May 2015 . My then four-year-old son Alex was up and ready for library day, his favorite day of the week in pre-kindergarten . While I was having my coffee and getting awake, something about Alex’s left eye caught my attention . It seemed to be off-center, almost crossed inward . We have a history of lazy eye in the family, so I thought maybe I hadn’t noticed it before . Nothing else seemed wrong, so off he went to school, and I made an eye doctor appointment . But as the day progressed, I started getting messages from his teacher . “Have you seen Alex’s eye?” she said . “He seems to be having trouble with his balance . ”

A shocking diagnosis We went to see Alex’s pediatrician that afternoon, and he immediately instructed me to take Alex to the nearest children’s hospital, which was three hours away . He simply said it could be a brain tumor or brain cancer . That was so incredibly shocking to hear, and I will never forget that moment . It was the most harrowing next 24 hours — the drive to the hospital, the first MRI and then the news that Alex had a mass in the middle of his brain causing hydrocephalus . They moved him to the pediatric intensive care unit at the hospital that night, and early the next morning, they took him into surgery .

The neurosurgeon told my husband, Shawn, and me that it was a benign tumor believed to be a craniopharyngioma and that it was fully removed . Hearing the tumor was benign seemed like a relief, but as the hours and days unfolded after surgery, we soon learned that we had a long road ahead . As a result of the tumor surgery, Alex developed diabetes insipidus (DI), the absence of the antidiuretic hormone vasopressin due to pituitary damage, which made it challenging to manage his body fluid balance and keep his blood sodium level in a safe range . This kept him in the hospital for six weeks . When we asked how we measure his sodium at home, we were quickly made aware that this is a rare condition and that at-home testing of blood sodium is not available — unlike typical diabetes where you can test glucose .

Amy Wood, Executive Director and Co-Founder of the Raymond A Wood Foundation based in Ocean City, Maryland, USA

Alex was also adipsic in that he lost any sense of thirst from hypothalamic damage, so we could not rely on thirst as an indicator of his hydration level . Additionally, he developed panhypopituitarism which is loss of endocrine function, adrenal insufficiency, and hypothalamic obesity (HO) which is excessive weight gain combined with an insatiable hunger .

Challenges for optimal care After discharge, Alex was getting lab work done up to four times a week where we waited hours for results . His veins were blowing out from constant poking . We were measuring how much he drank and how much he urinated to calculate fluid intake . He had three seizures due to low blood sodium that put us on medical flights to the children’s hospital, where it took him days to recover . I felt strongly that this situation was not sustainable, and we needed a solution . We discovered a hospital-grade handheld blood analyzer that could do full basic metabolic panels at home, with a finger stick producing accurate results in less than five minutes . There were significant roadblocks to getting the device — the price tag is around US $12,000 with test cartridges and the fact that it would be used off-label where there was really no prior experience with using it for this purpose . But we sensed it would be a game-changer . With the help of Alex’s pediatrician and support from our local community, we raised money and purchased one .

This device was life-changing . We could test Alex’s sodium at home and make quick medication and fluid adjustments to keep his sodium in range . Since receiving the device, he has had no sodium-related hospitalizations . It also peeled away one of the most complicated layers of management of his challenging post-tumor treatment . Shawn and I knew there were others like us that may benefit from using this device, so we created the Raymond A . Wood Foundation (RAWF) in 2017 to raise money to purchase and provide these devices to pediatric brain tumor patients with adipsic diabetes insipidus . We also realized early in this brain tumor journey that there was no U .S . based organization primarily focused on craniopharyngioma . Because of the excellent survival rate in craniopharyngioma, the high cost of survival is often not satisfactorily addressed as a priority . Many survivors face complete loss of endocrine function, neurocognitive impairment, blindness, uncontrollable obesity (hypothalamic obesity), temperature dysregulation and memory issues . Craniopharyngioma in particular affects both children and adults, and is also highly recurrent and characterized as “malignant in place . ”

The Raymond A Wood Foundation We began to position the foundation to be the premier patient advocacy organization for survivors of hypothalamic-pituitary brain tumors . In 2020, I left my full-time job as a creative director of a web development agency to become the executive director of the Raymond A . Wood Foundation . We added key opinion leaders to our board and redeveloped our mission to serve adult survivors in addition to children, because we discovered a significant unmet need in ongoing treatment of the comorbidities of these tumors in adults . ➤

Right: The Raymond A. Wood Foundation recently attended the Chan Zuckerberg Initiative, “Rare As One Convening” where 50 rare disease organizations joined to learn and share around advancing treatments. The Raymond A Wood Foundation delegation included (from left to right), Eugenie Hsu, Ph.D, Board Chair; Amy Wood, Executive Director; Nathalie Kayadjanian, Ph.D, Scientific Advisor; and Cassie Kline, MD, Neurooncologist, Children's Hospital of Philadelphia Raymond A. Wood Foundation founders, Shawn and Amy Wood with son Alex, days after craniopharyngioma surgery

Currently, RAWF’s focus is on supporting efforts to develop better treatment outcomes particularly for craniopharyngioma, while finding solutions for the myriad long-term challenges like diabetes insipidus (DI) and hypothalamic obesity (HO), which is characterized by uncontrollable obesity recalcitrant to diet and exercise and can also present with hyperphagia, or an insatiable hunger . RAWF recently hosted a patient listening session with the US Food and Drug Administration (FDA) on HO, and we are working closely with pharmaceutical companies and key HO researchers to find an effective treatment . We also host a yearly family conference in collaboration with Children’s Hospital of Philadelphia (CHOP) where we offer the opportunity for families to engage with physicians and researchers while also connecting with each other on these topics .

Making a real difference for craniopharyngioma patients In 2020, we initiated the development of a sodium meter, akin to a glucometer, that could be FDA-approved, insurancecovered and more user-friendly by using a small blood volume . This project is in phase one development in collaboration with Drs . Shana McCormack and Khushbu Patel of CHOP and Giner Labs, whose initial development work has been funded by an NIH SBIR[MOU2] grant . Recently, we were named a grantee of the Chan Zuckerberg Initiative’s (CZI) “Rare As One Network” . Under that three-year grant, we are developing a research roadmap and building a patient-led collaborative research network with a focus on reaching underserved craniopharyngioma patient populations . Fortunately, my son Alex never had a recurrence, but this tumor takes up a big part of our day-to-day lives, impacting everything from his ability to learn and make social connections, to his physical capabilities, sleep and wake cycles, and even his speech articulation . I have made it my life’s work to increase awareness around the need for more patient-centered, future-focused treatments to mitigate these lifelong effects . Survival is a true blessing, but the price is steep . I believe we can make a difference in that cost for future craniopharyngioma survivors . n

Dr. Craig Alter (left), neuroendocrinologist at Children’s Hospital of Philadelphia and Amy Wood (right), co-presented on diabetes insipidus management at the inaugural Pituitary Brain Tumor Family Conference

For more information about the work of the Raymond A. Wood Foundation, visit www.rawoodfoundation.org

The IBTA maintains a list of neuro-oncology and relevant cancer conferences on its website at www.theibta.org

The Brain Tumour Patients’ Charter of Rights has worldwide relevance and sets out an aspirational framework for improving healthcare systems and communications. Read the Charter at https://theibta.org/charter/