It sounded crazy to Sophia Smith’s parents that doctors would use an adhesive similar to Krazy Glue to plug the tiny holes in her brain, but in essence that’s just what they did.
“I was like, ‘Krazy Glue?’” Sophia’s mother Rebecca Raezer said just a few days before Sophia’s life-saving surgery. “I thought they were just calling it glue for me, but it is a permanent fix. I found it hard to believe.”
Sophia, who is 3 months old, has vein of Galen malformation, or arteriovenous malformation (AVM). The large, deep vein at the base of her brain lacks capillaries, so her blood flows much too quickly from the arteries to the vein, which becomes overwhelmed by the intense bloodflow.
On Dec. 4, Dr. Alejandro Berenstein, director of the Hyman-Newman Institute for Neurology and Neurosurgery at St. Luke’s-Roosevelt Hospital in New York City, performed an embolization: He inserted a hollow tube containing the medical glue, or N-butyl-cyanoacrylate Trufill, through Sophia’s groin and fired bursts of it into the holes of the arteries.
This was Sophia’s first embolization, which is the best treatment for infants with the rare condition of vein of Galen — the most extreme of AVMs — but it definitely won’t be her last.
She came home from the hospital on Dec. 7. Raezer called it “the best day of my life.”
‘She Really Was a Miracle’
Raezer, 41, and her husband, Rich Smith, of Long Branch, N.J., who have been together for 13 years and married for three, had just about given up on the idea of having a biological child.
“I had breast cancer at 32 and a double mastectomy,” Raezer told FOXNews.com. “Doctors didn’t think I’d be able to get pregnant naturally. She really was a miracle.”
Because one of her two tumors was estrogen-receptor positive (the tumor's growth was fueled by estrogen), doctors advised Raezer not to try fertility treatments, since they would require her to take extra hormones and might prompt a recurrence of cancer.
But then a miracle happened: Raezer found out she was pregnant.
The pregnancy wasn’t exactly smooth sailing. Because of her age, Raezer was considered high-risk, and she developed gestational diabetes. Then the bomb dropped: At 32 weeks, during a routine ultrasound, her doctor told her the baby had vein of Galen malformation.
“They drew me a picture,” Raezer said. “They showed me a thick artery going into a vein and explained the capillaries weren’t there.”
Raezer and Smith sought out Berenstein because of his experience with vein of Galen malformations. Just last month he saved the life of Ella-Grace Honeyman, a 17-month-old from England with the same condition.
Each year, there are about 300 to 400 new cases of vein of Galen malformations worldwide, Berenstein said.
The United States sees only about 25 or 30 of those cases.
“Three days prior to her being born, they gave me a large dose of medicine so her heart wouldn’t be working too hard,” Raezer said. “They took her (via Caesarean section) and I didn’t get to see her right away ... but, she went into heart failure, and that was scary.”
Besides congestive heart failure, vein of Galen babies can suffer from hydrocephalus, seizures or become developmentally delayed if the condition is not treated in a timely manner.
How the Glue Works
Sophia recovered from the heart failure, but she did have a hole in her heart, which doctors said is the result of the vein of Galen malformation. Because of the holes in her brain, her heart was working overtime.
So by plugging the holes in Sophia's brain with the glue, Berenstein decreased the demand on her heart, which has already begun to repair itself.
The glue figures into Sophia's prognosis in two ways: First, her brain is receiving a better blood supply, and second, the venous drainage is much less congested, Berenstein said.
"In three months when we repeat this surgery, the vein of Galen will be a lot smaller than it was," Berenstein said.
The concept of using medical glue is constantly evolving. Different versions of N-butyl-cyanoacrylate have been used for dental and eye surgeries, and Band-Aid and Nexcare have liquid bandages that are a form of cyanoacrylate.
“The first time we used Krazy Glue, the patient had a ruptured vein and we literally went to a hardware store,” Berenstein told FOXNews.com before Sophia’s surgery. “And, going back in history, in the Vietnam War, we used the same kind of glue to put livers back together. Now we inject it into a microcatheter to seal holes back together.”
Although the glue has been used for several decades in emergency situations, the Food and Drug Administration did not approve its use for embolizations until 2000.
A Bright Future
Although vein of Galen malformation success stories are fairly recent, Sophia’s prognosis is good, according to Berenstein.
But she will require at least one more surgery in a few months, and each surgery has a 5 to 6 percent chance of stroke or death.
“Alternatively, if she did not have the surgery, she would not live to the age of 5,” Berenstein said of Sophia. “It is a lethal disease.”
Rebecca Raezer still keeps a smile on her face and is convinced Sophia's battle makes her family stronger. The thought of a bright future with her daughter excites her.
“I want to be a soccer mom, I never thought I’d say that,” Raezer said, smiling. “I want to go her wedding and her graduation. I want her to talk about this, and what she’s gone through, and maybe have people learn more about AVM.”