A new procedure is available to help infants with a heart defect that affects hundreds of children yearly. But because the Food and Drug Administration doesn't recognize the procedure, few families know about its usefulness and health benefits.
After watching his daughter suffer through heart failure, Chris Miles and his wife, Ashley, are hoping to get the word out so that more families can be spared the grief of finding a procedure to help their child with a certain heart defect.
While living in France, pursuing a basketball career after four years spent playing in college, Chris and Ashley Miles learned they were pregnant with their second daughter. About six months into the pregnancy, an ultrasound was conducted to evaluate the health of their soon-to-be-born daughter.
The doctors told the Mileses that there would undoubtedly be something "wrong" with the pregnancy, but "didn't know exactly what was wrong."
"They said, 'Your daughter is going to have some health issues when she's born, if she does survive,' " Chris said.
The doctors in France recommended Ashley that have an abortion, but the Mileses did not entertain the thought of aborting their second daughter. Instead, they put the health of their unborn child as a top priority and moved back to Utah to get further testing. Doctors there said their daughter would likely be born with Down syndrome and that there was a 50 percent chance she would have a heart defect.
"I found out that she had Down syndrome, and that kind of rocked my world," Ashley said. "I never thought it would happen to me; I'm young, I'm healthy. That was hard on its own, and I thought it was the hardest thing I've ever had to handle and accept in my life."
After their daughter, Zuzu, was born a few weeks premature and with Down syndrome, doctors found three holes in her heart from congenital heart disease. As a result, Zuzu was going into heart failure and required surgery to live.
"She was four days old and I was standing there by myself looking at this daunting task ahead of me, and the doctor comes up and says, ‘She's in heart failure,' " Ashley recalls. "And I didn't know what that meant, so I'm just immediately sobbing, freaking out. I thought that meant she's going to die."
Though Zuzu needed surgery to repair her heart, doctors said she was too weak and that immediate surgery would be too dangerous. The Mileses were instructed to watch her condition, looking for symptoms such as her skin turning gray or her not being able to breathe.
"For five months of her life I'm hardly sleeping, watching for my daughter as she slowly goes through heart failure, waiting for that exact moment where it's not too late, but that it's just the right time, which is really hard," Ashley said, holding back emotions.
The months took their toll on Zuzu. "You just see her and she looked like a swollen marshmallow," Ashley recalled. "It was terrible."
After a sleepless and emotional five months of watching and waiting, Zuzu was finally healthy enough for her first open heart surgery. The surgery would be the first of two surgeries to correct the heart defects at birth.
Zuzu was in surgery for more than nine hours as her parents waited in the lobby, cautiously optimistic and hopeful that their daughter would be OK. A nurse would send the Mileses periodic updates via a pager that received text messages, but the unknown left both parents on an emotional roller coaster.
"You kind of have to say goodbye and you don't know if your baby is going to make it through the surgery. There's a high likelihood that they will, but there's no guarantee," Chris said. "It is a very scary situation because it's so unpredictable.
"Intensive care units and babies don't make for a peaceful, happy emotion for parents," he added.
Despite an extensive first surgery, Zuzu was immediately in heart failure again and would need to undergo another procedure to correct her mitral valve, which was damaged in surgery. The mitral valve is an extremely sensitive valve that leaves surgeons few options when attempting to keep an infant alive.
Zuzu's cardiologist recommended a mechanical valve as an option, but the quality of life would be severely restricted and would likely present other complications, such as strokes or blood clots, in the future. The cardiologist, however, said he would be looking for other options to help Zuzu.
The cardiologist would go on to find a breakthrough procedure being performed at Boston Children's Hospital: implantation of a Melody valve, which was generally used in a different position for the heart and not in the mitral position, to replace failing pulmonary valves.
The Melody valve is made from a bovine jugular and can be used on infants and toddlers to correct mitral valve disease. The valve can be as small as 9mm but can be enlarged non-surgically, which keeps young children from needing multiple open heart surgeries.
Additionally, a Melody valve does not require the blood thinner drug Coumadin, which is often adminstered as a shot with a syringe and a needle several times after a mechanical valve surgery. Often, parents of children who have mechanical valves have to keep a close watch following the surgery in case of strokes, blood clots or other medical problems. A Melody valve only requires a half dose of baby aspirin.
"Going to a baby aspirin and not having to worry about strokes, blood clots that could be fatal or be paralyzing, the half a baby aspirin is a pretty big deal," Chris said.
In its three years of use, the Melody valve had only been used on 15 infants in the United States at Boston Children's Hospital. It was a fairly unheard of procedure and not backed by the FDA - an essential step to alert surgeons of the new procedure and give them the confidence to recommend the procedure as a good alternative for children.
Nevertheless, the cardiologist told the Mileses the history of the procedure was "extremely good" and was a better alternative to a mechanical valve, but that it would have to be their decision.
"We thought about it, we prayed about it, we talked to the cardiologist, we called the surgeon in Boston with our cardiologist and explained things to him," Chris said. "We felt that it was going to be the best option for our daughter."
The Mileses would spend two weeks in Boston as Zuzu went through another open heart surgery using the new procedure. The surgery was a success and Zuzu eventually recovered from heart failure.
Zuzu will eventually have to undergo another surgery when she's older to expand the valve, but the surgeon will be able to use a catheter with a balloon on the end, which will go up through the leg, instead of having to perform another open heart surgery.
Looking to the future
As a result of Zuzu's Melody valve surgery, the technology and know-how was brought back to cardiologists at Primary Children's Hospital.
"It was really exciting for us because it was nice to have something good come out of something bad," Chris said.
Zuzu, now 1 year old, is healthy and free of the effects of her heart defects.
"She's starting to stand on her own two feet at just over 1 year old, which is huge progress for a baby that had heart surgery because if they have one or two heart surgeries, they spend a lot of time in the hospital rather than moving, crawling, playing with toys and eating," Chris said. "She's almost crawling and her health benefits and the fact that she's starting to kind of catch up, we attribute a lot to the Melody valve."
Now, the Mileses are doing all they can to get the Melody valve approved by the FDA so other children don't have to settle for a mechanical valve.
"When our infant cardiologist proposed the idea of Zuzu receiving a Melody valve, the last thing on our mind was the FDA. We just wanted to know what the history of the experience was with the valve and how successful it was," Chris said. "We wanted to make sure it was the best option.
"It got us really excited to do anything we really could to make it available for other babies because it really has been the biggest blessing to our daughter's life," he added.
"We just felt a huge responsibility because it's changed our life; it was the best thing that's ever happened to Zuzu," Ashley said.
The Mileses, in addition to about six other families, have created a website to provide information for families going through similar struggles with their children and to get 100,000 signatures to petition the FDA for approval. They feel that increased knowledge of the procedure will help expedite the process to get an FDA approval of the Melody valve.
"We want to let people know that there's options when you feel like you're in a hopeless situation," Ashley said. "Mechanical valves are not a good thing; it's kind of the last resort, it's kind of a lose-lose. The quality of life isn't very good; it just keeps them alive.
"It's the last thing (parents) should have to worry about when it's (the Melody valve procedure) such a successful surgery and can really improve the quality of the baby's life, and the families," she added.
As a result of the Mileses efforts and that of the other families to make the procedure known, another family was able to benefit from the surgery, saving their child. In total, more than 30 infants have had the surgery performed and "more kids are being benefitted from the surgery," Chris said.
To read about other families and their experiences or to learn more about the Melody valve, visit melodyvalve.org.