Finding community to fight pediatric renovascular hypertension
The rare condition brought one Oregon family to Michigan, where a new international collaborative is improving outcomes and inviting families affected by the disease to join the network.
Today, Heidi Van Natta is a vibrant 8-year-old. But in 2014, she was a critically ill baby with pediatric renovascular hypertension, also commonly known as pRVH—a rare disease that necessitated a six-week stay at University of Michigan Health C.S. Mott Children’s Hospital.
Mott has long attracted families from across the country coping with a diagnosis of pRVH. Now, pediatric vascular surgeon Dawn Coleman, M.D., and others have launched a new global collaborative to help critical stakeholders in this disease around the world advance research and clinical care—and to help patients and their families feel less alone.
The pediatric Renovascular Hypertension Patient Centered Outcomes Research Collaborative, also known as PCOR, provides support and resources for patients with the condition through their Virtual Research Network.
Families can sign up for research updates, to participate in PCOR training, to partner in research opportunities and to access outreach, family support, clinical and educational resources, and more. The voice and experience of these unique patients and their families is critical to patient centered outcomes research. With their partnership, the Collaborative can work toward outcomes that are important to children and their families affected by pediatric renovascular hypertension.
“Families make long trips to come here. They sacrifice a lot and often put trust in a medical team they haven't met in person,” Coleman said. “The collaborative will really help bring families together.”
The condition is so rare its national incidence isn’t known. Mott is one of the country’s most high-volume centers for the condition, seeing just a few hundred patients over the past couple of decades.
Coleman says it is the multidisciplinary team approach—anchored in the groundbreaking work of vascular surgeon and active emeritus James Stanley, M.D.—that makes Mott uniquely suited to surgically treat this condition.
For Coleman, all patients are special. But Heidi holds a special place in her heart.
When Heidi came to Michigan at 8 months old, she was incredibly sick; she was immediately admitted to the ICU and needed a long, complex surgery.
“Her case is particularly memorable to me because it was so challenging and she did so well,” Coleman said.
Patients like Heidi motivate Coleman and her collaborators to push care for pRVH forward.
Heidi’s mom, Lisa Van Natta, had a normal pregnancy and birth, and Heidi was doing well at all her regular checkups. Then, when Heidi was about 7 months old, Lisa noticed an alarming, sudden change.
“From the belly button up, her skin was nice and peachy. But from her belly button down, her skin was blotchy and purple looking,” Lisa said.
She quickly called the pediatrician and a battery of tests led to a diagnosis of a previously undetected heart murmur.
This set off a chain of events that initially led to more questions than answers for the Beaverton, Oregon, family. Heart murmur diagnosis in hand, the Van Nattas were referred to a cardiologist at Doernbecher Children's Hospital in Portland. There, her blood pressure was found to be dangerously high.
One thing led to another and Heidi was admitted to the intensive care unit that same day. Heidi then received a CT scan of her abdomen.
“We thought we'd get some answers, but when the doctor came out, he leaned against a wall and said it was worse than he thought. She had a coarctation in her abdominal aorta, narrowing in both renal arteries. Her right kidney had not grown. It was the size of a newborn’s and the narrowed artery supplying blood to that kidney was shooting her blood pressure high.”
Heidi stayed in the ICU in Portland for two weeks, and her blood pressure was never successfully stabilized. Heidi’s nephrologist in Oregon who she still sees today, Randall D. Jenkins, M.D., says Heidi’s condition was so rare he’d only seen it once before in a teenager—never in a baby as small as Heidi.
From early on in their stay, Jenkins said he thought the family needed to go to Mott because of the hospital’s reputation for taking on high-risk or difficult cases. He conferenced with the renovascular hypertension team at Mott to discuss Heidi’s case and try to find some answers.
“It was terrifying and shocking,” Lisa said of the time. “I personally kept blaming myself thinking, ‘What did I do wrong? What could be different?’ It’s not comforting when you're in the ICU and your child is suffering and the doctors say, ‘We don’t ever see this.’”
“We asked Dr. Jenkins if this were his child, what would he do, and he said, ‘I would go to Michigan,’ so that is where we went.”
From Oregon to Michigan
Once Heidi was stable enough to travel, Heidi and Lisa took a life flight from Portland to Michigan while her husband John Van Natta flew the couple’s then two-year-old son, Tommy, to stay with Lisa’s parents in California.
Initially, the care team at Michigan, including pediatric specialists from critical care, cardiology, nephrology, wanted to avoid operating because Heidi was only 8 months old.
“Mott tried to stabilize her and couldn't and within a week she was on a ventilator,” Lisa said. “She ended up being intubated three times.”
This indicated it was time for surgery.
On April 28, 2014, Coleman and her partner, vascular surgeon Jonathan Eliason, M.D., with their team performed a right nephrectomy (kidney removal), bypassed the blocked portion of her abdominal aorta, and fixed the blood flow to the remaining kidney with a reimplantation. Essentially, they took her left renal artery and moved it down on her aorta, and all told, it was an 8- or 9-hour surgery.
Heidi pulled through, and her recovery was remarkable.
“They said she would be in the hospital another two months, and she went home two weeks later,” Lisa said.
A small community for a rare illness
Before coming to Michigan, the Van Nattas hadn’t heard of any other families fighting the same condition.
“It was really hard to find any information, and it felt like there was nobody out there to turn to,” Lisa said. “It was isolating, and it felt like nobody could understand. Even when we went to Michigan, they said we do see this but it's very rare.”
Coincidentally, while they were at Mott for Heidi’s surgery, Coleman had another patient in the hospital at the same time. The two families were able to meet and found it very comforting to be able to talk to each other.
Coleman said, “This is a really tricky thing for families to navigate. What I've learned over the course of caring for these kids is in most cases we can’t tell parents why their children are affected by this problem. Parents feel guilty and think they did something to cause this, but this is not the case.”
“It’s terrifying and very isolating, and it’s important for parents to know that other parents have walked this pathway and come through it successfully. That really matters so people don’t feel alone.”
Within a year of Heidi’s surgery, Coleman connected the Van Nattas with another family.
"That year on our follow up, we went back to Michigan and got to meet each other because the other two families were there at the same time,” Lisa said. “It was nice to see what the kids are going through, and how much they're growing and thriving.”
The new collaborative is meant to help, in part, with this isolation and advance patient-centered research and clinical care.
“With the voices and active engagement of patients and families, we have an incredibly powerful international team prioritizing this disease and these rare patients,” Coleman said. “I’m excited about the potential to leverage all of these stakeholders to move forward high-impact research, and to bring patients and families together to enhance clinical care and peer support.”
"I feel like sharing her story is one way Heidi can give back to help somebody else,” Lisa said. “I'm excited about this project because it can feel isolating and lonely, so I'm hoping that this gives some insight to people who are newly diagnosed or currently going through [treatment].
‘Spunky, silly, a little spitfire’
Seeing Heidi today, her parents say you would never know she was so sick. She loves to dance, play soccer and swim. She is obsessed with horses, participates in Cub Scouts, and loves to read and write stories.
“She's spunky, silly, a little spitfire, and very caring,” Lisa said. “When her brother gets hurt, she's the first one to make sure he's feeling OK. She adores her brother. She’s very tender and caring about people and animals.”
The family hasn’t been back to Michigan since a visit one year after Heidi’s surgery. They see her nephrologist and cardiologist at home for regular follow-ups. But her bypass will have to be replaced, likely within the next couple of years, and they plan to come back to Mott for that operation.
“We know them, we trust them, and they know her,” Lisa said. “I send Dr. Coleman updates on her, and we've stayed in touch.”
Coleman said, “I’m grateful we have an enduring relationship. It’s really a blessing to have them now as partners in this important work together [through the collaborative]. It’s the most meaningful set of aims and goals one could have as a surgeon.”
Visit the pediatric Renovascular Hypertension Patient Centered Outcomes Research Collaborative’s website for more information and to join the pRVH Virtual Research Network.