Von Hippel-Lindau syndrome (VHL): Bryce’s story

Bryce Munson recalls the very first time he had a panic attack that he thought was a panic attack. Mother Erryn Westerhold too. “I was in seventh grade math class when I started having trouble breathing, my chest hurt, I couldn’t concentrate, and my vision was blurry,” Bryce said. Erryn took Bryce to the family’s pediatrician, who prescribed anti-anxiety drugs.

The panic attacks stopped for a while, but over the next three years they became more frequent. “At first it seemed like I had a really, really bad case of anxiety,” Bryce said. But he didn’t. Erryn and Bryce’s father, Jerald Munson, was beginning to wonder if something else could be causing their son’s anxiety attacks and uncontrollable spikes in his blood pressure.

Jerald’s mother had an inherited condition 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 can be cancerous or noncancerous and often first appear during young adulthood.

Although Jerald’s mother had died of VHL, he had never shown any signs or symptoms of the condition. “We knew in the back of our minds that it was possible that Jerald had VHL and that the guys inherited it, but it had never been a problem,” Erryn said. Until Bryce’s anxiety attacks started.

Diagnosis of VHL

Erryn began researching VHL and found that people with the condition often develop a type of tumor called a pheochromocytoma. These tumors occur in the adrenal glands, which are located on top of each kidney. While pheochromocytomas aren’t usually cancerous, they can cause headaches, panic attacks, excessive 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 investigation.

Jerald and Erryn remember the day they got the call that Bryce had a pheochromocytoma in his right adrenal gland. “It was actually terrifying,” Erryn said. “I was at work when the phone rang and they said to take Bryce to Children’s Mercy immediately. I told them, ‘I’m leaving work at 5:30 p.m. and then I’ll bring him up,’ but they said we had to bring him 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 he was in the hospital, Bryce said specialists were queuing 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 looking after him at Children’s Mercy. That’s the kind of care this condition requires.”

After a few days in the hospital, he returned to Warrensburg to recover. “I had a lot of drugs to regulate,” Bryce said. “And I had to measure my blood pressure 100 million times a day.” Mom said it wasn’t that bad, but she kept a diary of his blood pressure readings which she still has. “I would send the measurements 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 through a cryoablation procedure. He has been tumor free ever since. No treatment other than medication was required.

On the SPOT!

When Bryce tested positive for VHL, it sparked a whole new level of concern that anyone in the Munson family, except Erryn, could have the condition. First, Jerald tested positive for VHL. He found that he also has several non-cancerous tumors throughout his body. One has been removed from his kidney and his doctors are watching him closely.

While the odds were 50/50 that Jerald had passed VHL to Clayton, 14, and Michael, 11, they also tested positive for VHL. So far, neither has shown any symptoms of the condition, but to make sure the brothers stay healthy, they are now being monitored at the Children’s Mercy Surveillance for Predisposition to Tumors, or SPoT Clinic. The clinic has been developed to follow patients with different 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 ensures that the patient is aware of all surveillance tests. “We target children with complex cancer risks, meaning their risk of tumor development requires more complex screening schedules,” explains 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, especially when multiple children are involved, like the Munsons.”

The SPoT Clinic team includes Caitlin, Kevin Ginn, MD, a pediatric neuro-oncologist, and Erin Guest, MD, director of the Cancer Genomics Program and the Cancer Center Biorepository. They run the outpatient clinic eight times a year, following about 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 of missing out and improving the quality of life for these families.”

We are behind you

Bryce, Clayton and Michael take comfort in the knowledge that the SPoT clinic team is closely monitoring their health so they can focus on what matters to three active boys. “I’m in high school now,” Bryce said. “I’m a college graduate and thinking about getting a business degree.”

Clayton is the daredevil of the family. He likes to do tricks on his bike and play football. He has broken several bones, but so far has not developed any tumors associated with VHL. And Michael, known to his brothers as Mikey, enjoys playing soccer and taking care of his bunny, but also has no symptoms of VHL.

All three are followed annually in the SPoT Clinic. “To be honest, we dread that time of year,” admitted Jerald. “We know there’s a risk that we’re going to have to deal with something, but so far we’ve had some good news.” However, care is always in the back of their minds. “If one of the guys has a headache for more than a few days in a row, or if they’re not feeling well, we’re worried it’s not just a headache or a virus, but it’s more serious,” Erryn said. Jerald agreed. “We worry that the little things can turn into something big.”

Counting their blessings

While Erryn and Jerald have been through a lot since Bryce’s diagnosis, they’re thankful they discovered it when they did, and that Children’s Mercy was near them. “Children’s Mercy is a great hospital,” Jerald said. Erryn is grateful that their home in Warrensburg is only an hour from the hospital’s Adele Hall Campus and the SPoT clinic. “I feel very blessed that we live so close to Children’s Mercy, and a team of specialists who know what VHL is and how to deal with it.”

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