In the fall of last year, a young woman named Gerdline walked into Hospital Saint-Nicholas in Saint-Marc, Haiti, carrying her baby son Rolensky. Only four months old, the boy was in a bad way: thin, breathing rapidly and lethargic, with a bluish tinge to his skin.
Little did Gerdline know as she crossed the hospital threshold that Rolensky’s heart was failing—because of a one-in-a-million blood vessel malformation in his brain. Nor did she know that the two of them would soon be on a plane to Boston, where doctors from across Boston Children’s Hospital would come together around her boy to save his heart by fixing his brain.
“When we first saw Rolensky, he was in respiratory distress, and had been for about a week,” says Christopher Carpenter, MD, a pediatrician and fellow with the Global Pediatrics Program (GPP) at Boston Children’s, who has worked in Haiti for the last two years. “He had congestive heart failure and pulmonary hypertension, but we couldn’t discern the cause.”
Over the next few months, Rolensky didn’t get worse, but didn’t get any better. Carpenter cared for him in the hospital’s makeshift ICU, keeping him on oxygen and giving him medications to keep his blood flow up. “He didn’t have enough energy to cry or to stand,” Capenter says. “His mother sat by his bedside for two months in a metal chair with no seat, comforting him and helping care for him.”
In January, when Carpenter’s tour at Saint-Marc ended, Rolensky’s care shifted to Sara Gonzalez, DO, another GPP fellow from Boston Children’s. “When I first met him, he was stable but clearly ill,” Gonzalez says. “He needed oxygen most of the time, and even though he was eight months old, he couldn’t yet sit up on his own. He was on the right medicines for his condition, but wasn’t getting any better. He also wasn’t gaining weight as well as we had hoped.”
She soon arranged for Rolensky to be seen by a pediatric cardiologist from upstate New York who was in Haiti with Partners in Health and was visiting a village about 50 miles away.
“The cardiologist did an echocardiogram but couldn’t find any structural reason for such a severe cardiac problem,” Gonzalez recalls. “Then he remembered a rare cause of pulmonary hypertension and put the echocardiogram probe on Rolensky’s scalp,” Gonzalez continues.
A rare condition
What the echocardiogram showed was a large blood vessel anomaly, called a vein of Galen malformation (VOGM), deep in Rolensky’s brain. In these extremely rare malformations, misshapen arteries grow and connect directly to a vein in the brain, shoving blood directly into it at high pressure and stretching it like a garden hose filling a balloon. It was the VOGM that was causing Rolensky’s heart to fail. Because so much of his blood was flowing through the malformation and right back to his heart and lungs, his heart was working overtime to get enough blood to the rest of his body.
A journey there…
Diagnosis in hand, Gonzalez got in touch with Boston Children’s multidisciplinary Vascular Anomalies Center (VAC) to see if there was anything to be done. “As I learned about VOGM, I realized that this was a problem that would require a high level of expertise if it could be treated,” Gonzalez says. “I reached out to the VAC team, and they agreed that he was a good candidate for surgery.”
“It’s a very rare condition,” says Darren Orbach, MD, PhD, a neurointerventional radiologist and VAC member who sees about a half dozen patients with a VOGM every year. “Usually children are diagnosed shortly after or even before birth, and it is unusual to see a child at his age first being diagnosed with it. But if you don’t catch it, the malformation can damage the heart severely and cause severe loss of brain tissue.”
Working with the local authorities, VAC staff and PIH, Gonzalez arranged for Rolensky and Gerdline to travel to Boston Children’s for a procedure, called embolization, to close his VOGM and relieve the stress on his heart. “I even took his passport photo with my phone and emailed it to our PIH team from the hospital parking lot.”
Rolensky’s plane landed April 2, and he was taken to the Boston Children’s Cardiac Intensive Care Unit to stabilize his heart. Two days later, Orbach—just one of the scores of people from across Boston Children’s involved in Rolensky’s care—conducted the first of two embolization procedures, threading a catheter through Rolensky’s arteries up into his brain and sealing as many of the malformed blood vessels as he could without threatening the blood supply to Rolensky’s brain.
From there, the team waited. “It’s a risky procedure, and our plan was to do it in the least dangerous way possible and monitor Rolensky along the way,” Orbach explains.
By all accounts, the change in Rolensky has been astounding. Within two weeks, he was in a regular hospital bed, and shortly after that it became apparent to his doctors that he didn’t need supplemental oxygen anymore. He was discharged from the hospital on April 30.
“His heart is much better, but we don’t know if it will ever get completely better,” says Mary Mullen, MD, PhD, a cardiologist and VAC member. “It’s so unusual to see a child his age with this condition, and there’s not much information in the literature. “But in front of our very eyes he’s grown,” Mullen continues. “He can pick up his arms, and he can eat on his own. He’s gotten so much better.”
“I saw a photo of him a few days ago,” Carpenter exclaims. “He looks like a completely different child.”
…and back again
On May 17, Rolensky celebrated his first birthday with his mother and PIH host family. And on June 7—a little more than two months after leaving Haiti—Rolensky went home.
But he’s not completely out of the woods yet. While much of his heart has healed, he still has pulmonary hypertension. If it doesn’t clear up with medication, Mullen says they may try to bring him back to Boston for further treatment.
In the meantime, Gonzalez, whose fellowship term in Haiti just ended, got to see Rolensky and help coordinate his ongoing care before returning to the United States. “He is doing remarkably well—standing with his hands held, playing and stealing my glasses. To see a child getting ready to take his first steps just a few months after he had been so weak feels incredible.
“In a case like Rolensky’s, I marvel at his good luck,” Gonzalez continues. “It seems the stars all aligned for him to have found his way from his village in Haiti to Boston Children’s Hospital and back again.”
Everyone whom Rolensky has met between Saint-Marc and Boston has been touched by his life and story. “This was such a great boost for everyone in the hospital that they were able to treat him here,” Carpenter explains. “The morale of entire hospital was lifted because of this child.”
Everyone recognized at the beginning that his was a very special case,” Mullen says. “We have all been thrilled to be part of his care.”