Von Hippel-Lindau Syndrome (VHL): Bryce's Story
Bryce Munson remembers the very first time he had what he thought was a panic attack. Mom Erryn Westerhold does, too. “I was in my 7th grade math class when I started having trouble breathing, my chest hurt, I couldn’t focus and my vision was blurry,” Bryce said. Erryn took Bryce to the family’s pediatrician, who prescribed anti-anxiety medication.
For a while, the panic attacks stopped, but over the next three years, they became more and more frequent. “On the surface, it looked like I had a really, really bad case of anxiety,” Bryce said. But he didn’t. Erryn and Bryce’s father, Jerald Munson, started to question whether something else might be causing their son’s anxiety attacks and uncontrollable spikes in his blood pressure.
Jerald’s mother had had an inherited disorder called von Hippel-Lindau syndrome, or VHL. VHL is characterized by the formation of tumors and cysts in different parts of the body. These tumors may be cancerous or noncancerous, and often first appear during young adulthood.
Though Jerald’s mother had passed away from VHL, he had never shown any signs or symptoms of the disorder. “We knew in the backs of our minds that it was possible Jerald could have VHL and the boys could have inherited it, but it had never been an issue,” Erryn said. Until Bryce’s anxiety attacks began.
Erryn started researching VHL and found people with the condition commonly develop a type of tumor called a pheochromocytoma. These tumors occur in the adrenal glands, which are located on top of each kidney. Though pheochromocytomas are usually not cancerous, they can cause headaches, panic attacks, excess sweating and dangerously high blood pressure—all symptoms Bryce had. After discussing Jerald’s family history with their pediatrician, he referred Bryce to an endocrinologist at Children’s Mercy for further testing.
Jerald and Erryn recall the day they got the call that Bryce had a pheochromocytoma on his right adrenal gland. “It was terrifying, actually,” Erryn said. “I was at work when the phone rang and they said we needed to get Bryce to Children’s Mercy immediately. I told them, ‘I get off work at 5:30 p.m., and I’ll bring him up then,’ but they said we needed to get him there NOW.” Erryn and Jerald dropped what they were doing, and rushed Bryce to the hospital. There, doctors removed the pheochromocytoma and his right adrenal gland.
While hospitalized, Bryce said specialists lined up outside his door. “I felt like I had 30 doctors!” he said. “He was pretty popular,” Jerald said. “Endocrinologists, neurologists and oncologists— Bryce had a team of specialists taking care of him at Children’s Mercy. That’s the kind of care this condition requires.”
After a few days in the hospital, he returned home to Warrensburg to recover. “I had lots of medications to regulate,” Bryce said. “And I had to take my blood pressure like 100 million times a day.” Mom said it wasn’t quite that bad, but she kept a journal of his blood pressure readings that she still has today. “I would send the readings to his endocrinologist every day, and she would adjust his medications accordingly,” Erryn said.
A few months later, Bryce returned to Children’s Mercy to have a smaller pheochromocytoma removed from his left adrenal gland via a cryoablation procedure. Since then, he’s been tumorfree. No treatment other than medication has been necessary.
On the SPoT!
When Bryce tested positive for VHL, it generated a whole new level of concern that everyone in the Munson family, except Erryn, could have the disorder. First, Jerald tested positive for VHL. He discovered he also has several non-cancerous tumors throughout his body. He’s had one removed from his kidney, and his doctors are watching him closely.
Though the odds were 50/50 that Jerald had passed VHL on to Clayton, 14, and Michael, 11, they also tested positive for VHL. So far, neither has shown any symptoms of the disorder, but to be certain the brothers stay healthy, they’re now followed in the Children’s Mercy Surveillance for Predisposition to Tumors, or SPoT Clinic. The clinic was developed to follow patients with a variety of hereditary cancer syndromes that predispose them to different types of tumors in all parts of the body.
At each clinic visit, the team reviews national screening guidelines specific to the patient’s diagnosis, and makes sure the patient is current on all surveillance testing. “We focus on kids who have complex cancer risks, meaning their risk for tumor development requires more complex screening schedules,” explained Caitlin Schwager, MS, CGC, Genetic Counselor for oncology and Manager of the SPoT Clinic. “That can be a lot for families and primary care providers to manage, especially if there are multiple children involved, like the Munsons.”
The SPoT Clinic team includes Caitlin, Kevin Ginn, MD, pediatric neuro-oncologist, and Erin Guest, MD, Director of the Cancer Genomics Program and the Cancer Center Biorepository. They conduct the clinic eight times a year, following approximately 30 to 40 patients. “The SPoT Clinic is like a safety net for these patients and their families,” Caitlin said. “We can improve adherence by following established protocols, reducing the risk that something will be missed, and improving the quality of life for these families.”
We've got your back
Bryce, Clayton and Michael take comfort in knowing the SPoT clinic team is keeping a close eye on their health, allowing them to focus on what’s important to three active guys. “I’m a senior in high school now,” Bryce said. “I’m taking college courses and I’m thinking about getting a business degree.”
Clayton is the family daredevil. He loves doing tricks on his bike and playing soccer. He’s broken several bones, but so far hasn’t developed any tumors related to VHL. And Michael, known to his brothers as Mikey, loves playing football and taking care of his pet rabbit, but has no symptoms of VHL either.
All three are followed in the SPoT Clinic annually. “To be honest, we dread that time of year,” Jerald admitted. “We know there’s a risk there could be something we might have to deal with, but so far, we’ve gotten good news.” Still, the concern is always in the backs of their minds. “If one of the boys has a headache more than a couple of days in a row, or they don’t feel good, we’re concerned it might not just be a headache, or a virus, it could be more serious,” Erryn said. Jerald agreed. “We worry that the minor stuff could turn out to be something major.”
Counting their blessings
Though Erryn and Jerald have had a lot to deal with since Bryce’s diagnosis, they’re thankful they discovered it when they did, and that Children’s Mercy was in their corner. “Children’s Mercy is a great hospital,” Jerald said. Erryn is grateful their Warrensburg home is only an hour away from the hospital’s Adele Hall Campus, and the SPoT Clinic. “I feel very blessed we live this close to Children’s Mercy, and a team of specialists who know what VHL is and how to treat it.”
Inflammatory myofibroblastic tumor: Clara's story
Clara was born with a tumor the size of a golf ball on the back of her tongue. Genetic testing of the tumor showed that Clara had a rare form of cancer. By obtaining approval for a compassionate use trial—essentially a clinical trial just for her—Clara's doctors were able to successfully treat her cancer.
- Cancer Center
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- Experimental Therapeutics
- Genomic Medicine in Cancer Treatment
- Histiocytosis Program
- Leukemia and Lymphoma Program
- Liver Cancer Treatment
- Proton Therapy
- Soft Tissue and Bone Sarcomas
- Spanish-Speaking Cancer Clinic
- Surveillance for Predisposition to Tumors (SPoT) Clinic
- Survive & Thrive
- Cancer Center Family Care Team
- Hematology, Oncology, and Blood and Marrow Transplant
- Meet the Team
Neuro-Oncologist; Director, Brain Tumor Program; Interim Director, Experimental Therapeutics Program; Associate Professor of Pediatrics, University of Missouri-Kansas City School of Medicine; Clinical Assistant Professor of Pediatrics, University of Kansas School of Medicine
Associate Director, Pediatric Hematology/Oncology Fellowship Program; Hematology/Oncology; Director, Cancer Genomics Program; Director, Cancer Center Biorepository; Associate Professor of Pediatrics, University of Missouri-Kansas City School of Medicine; Research Assistant Professor of Pediatrics, University of Kansas School of Medicine