From The Orange County Register
By Nathaniel Percy | npercy@scng.com | Orange County Register
TRABUCO CANYON – There are few things that can put 12-year old Sofia Rodriguez in a foul mood.
Telling her she’s not allowed to travel for six weeks? Yeah, that’s one of them.
When she got a reminder about that from her mother, Sonia Rodriguez, in their Trabuco Canyon home, Sofia folded her arms and gave a little huff.
After a recent operation to switch out her pacemaker for a new wireless, leadless model, Sofia was told by doctors she’ll need to stay grounded for six weeks.
Luckily, that time will be up in time for the family’s winter vacation, said her father, Edgar Rodriguez.
Doctors at Children’s Hospital of Orange County who performed the procedure in late August say they believe Sofia is the youngest person to receive the new technology — the battery-operated Micra Transcatheter Pacing System created by Medtronic.
Sofia has needed a pacemaker all her life. Her first was installed when she was just six months old.
She was born with Tetralogy of Fallot, a congenital condition that causes four defects, including a hole between the lower chambers of the heart and an obstruction from the heart to the lungs. In addition, the aorta lies over the hole in the lower chambers and the muscle surrounding the lower right chamber becomes overly thickened.
With the defect, not enough blood is able to reach the lungs to get oxygen and oxygen-poor blood flows to the body. According to the National Heart, Lung and Blood Institute, the birth defect affects five out of every 10,000 babies.
“We were broken, crying in the chapel of the hospital,” Edgar Rodriguez said about her first operation when she was 10 days old. “We didn’t think she was going to make it (at first).”
When she was 6 months old, she had a second procedure to replace a pulmonary valve.
“(Doctors) decided it was a good idea to have a pacemaker in there just in case,” Rodriguez said. “Maybe at that point, she didn’t need it, but at some point, she might.”
Despite the procedures, it is still possible Sofia could develop heart arrhythmia.
The new pacemaker has metal hooks that connect it with the tissue on the inner part of the heart. There is no need for wires or to perform additional operations to extend those wires when they break due to Sofia’s childhood development.
Doctors told Edgar Rodriguez that the new pacemaker, about the size of a large pill, could keep Sofia out of the hospital for up to 14 years.
Sofia, however, has never let the defect or the fact that she has a pacemaker change the way she lives her life. She enjoys playing soccer and swimming and loves animals on top of her love for travel.
At Serra Catholic, if she feels too fatigued as a result of the procedure, she can alert her teacher and head home early for the day. Her mother said she has yet to receive a call from the school to come in early to pick up her daughter.
“I just go for it,” Sofia said.
She’s been doing that all her life, according to her parents, who watched as doctors tried to find what was wrong with her just days after birth. She’s the youngest of Sonia and Edgar Rodriguez’s three children.
“She’s always been an active kid,” Sonia Rodriguez said. “When she was a baby, she would go to the playground. We never put any limitations on her.”
Five days after Sofia was born, she was diagnosed with Tetralogy of Fallot, and on the 10th day she endured an 11-hour open-chest procedure. Doctors had to keep her chest open for the following 24 hours because of the swelling that ensued.
“It was a very tough, uphill battle,” her father said. “She had to recover from a really complicated surgery. We had a glimmer of hope, but it was still a long procedure.”
About six months later, Sofia needed a second procedure to replace a pulmonary valve to allow for better blood flow from the heart to the arteries.
She had a relatively normal childhood until the pacemaker needed to be replaced when she was 7, her father said. That procedure was to correct wires broken due to her growth.
“She didn’t remember any of this, except for the last procedure she had; it’s so unknown to her,” he said. “She grew up feeling like normal, except every now and then she would mention the little lump she had.”
In August, the family jumped on the opportunity for an alternative — the micra pacemaker that could keep Sofia’s heart beating at normal rhythm and could keep her out of the hospital for up to the next 14 years.
The micra pacemaker not only is wireless, the procedure to install it is much less invasive. Surgeons send the small device through the femoral artery, where it finds its way to the heart and adheres with the four small hooks.
Once it’s stable, the device senses heartbeats. If the heart doesn’t beat at a certain point, the device can send energy to the heart to make it beat, said Dr. Anthony McCanta, a surgeon at CHOC who performed the procedure on Sofia.
“It can sense her movement and activity and pace her heart faster if it needs to,” McCanta said. “This technology…will really give children and young adults with congenital heart disease a chance to lead more normal lives.”
McCanta was impressed by Sofia’s courage and resolve throughout the hospital visit.
“She’s somebody who has had to deal with major surgeries her entire life, so she has this inner strength about it,” he said. “(About a week) after the procedure she had so much joy, she was relieved.”
The biggest difference, McCanta said, was that the smaller pacemaker leaves no visible trace.
“It will not remind her every day that she needs a pacemaker,” he said. “The scar on the chest and the battery under the skin, those are daily reminders that you have major heart problems, so the fact that there’s no real evidence on her body is going to be a great thing for her.”
The new system requires no medications and the timeline is a relief for Sofia’s parents, who no longer worry about the pacemaker malfunctioning because of broken or worn wires.
Sofia is interested in entering the medical field, but wants to work with animals, following in the footsteps of her father, who is a veterinarian at the Portola Plaza Veterinary Hospital.
As for travel, the family hasn’t yet made plans about where they will go for the holidays, but Sofia is counting down the days until she can take off again.
“That’s all she’s worried about right now,” Sonia Rodriguez said.
