Jimmy Kimmel’s heartfelt monologue Monday on the “terrifying thing” that his family had gone through had everyone talking about the proposed replacement for the Affordable Care Act and pre-existing conditions.
“Before 2014 if you were born with congenital heart disease like my son was, there was a good chance that you would never be able to get health insurance because you had a pre-exiting condition, you were born with a pre-existing condition…” Kimmel said on his show “Jimmy Kimmel Live!” “No parent should ever have to decide if they can afford to save their child’s life.”
People also wanted to know more about this heart condition — Tetralogy of Fallot — that Kimmel’s son, Billy was born with.
About 8 in 1,000 babies are born with a congenital heart defect, says Dr. Camille Hancock Friesen, the medical director of pediatric cardiovascular surgery at Dell Children’s Medical Center of Central Texas. About 15 to 20 percent have Tetralogy of Fallot. Hancock Friesen estimates she does surgery about two to three times a month on babies with this condition.
Tetralogy of Fallot happens when the muscle that is the housing for the pulmonary valve never grew properly.
This causes four things to happen, Hancock Friesen says:
1. The blood flow that goes from the right ventricle of the heart to the pulmonary artery is obstructed.
2. The right ventricle has to work very hard to compensate.
3. There is a hole where the muscle that holds the pulmonary valve should be.
4. The aortic valve and pulmonary valve are supposed to work together, but the aortic value is pulled forward.
What doctors do to fix this problem is to open up the right ventricle to save prevent pressure from building up in that ventricle, which causes the muscle to thicken, and they repair the hole left by the muscle not being there. They do not automatically replace the pulmonary valve, though.
“People can survive without a pulmonary valve quite well,” Hancock Friesen says.
While not having a pulmonary valve can be tolerated for years, eventually these babies will need a valve, but usually not until they are teenagers.
Like Jimmy Kimmel, Austinite Erin Campbell knows what it means to have a baby born with this condition. Her son, Colton, was born May 26 last year with Tetralogy of Fallot. Unlike the Kimmels, her family knew Colton had it before he was born. About 70 percent of babies are now diagnosed in the womb.
At Colton’s 20-week-gestation ultrasound the technician couldn’t quite see all the images of the heart that needed to be seen. Campbell had repeated ultrasounds every three to four weeks until at 32 weeks doctors found the problem.
They could plan for his delivery, and she and her husband, David could get to know Colton’s medical team before it was an emergency.
“It was critical for me that I had very good prenatal care,” says Erin Campbell, who is an assistant principal at Anderson High School. “My husband and I asked a lot of questions with our doctors to really understand what was going on, to understand the situation.”
She thinks about the Kimmels not getting that time to plan and take in the diagnosis. “It would have made that situation more frightening exponentially. We’re very fortunate our doctors caught it ahead of time.”
When Colton was born by scheduled Cesarean section, the neonatal intensive care unit team was in the delivery room. They evaluated him to see if he was a Tetralogy of Fallot baby that would need immediate surgery, recognizable for blue-tinted skin because the blood flow to the lungs is so restricted, or a Tetralogy of Fallot baby that would need surgery at four to six months of age. These babies have pink-tinted skin.
Colton was pink and could wait for surgery to repair his heart.
When he was 4 months old, Colton and his parents met with Dr. Hancock Friesen, who looked at his dusky skin color and scheduled the surgery for the next week. Erin Campbell says, she hadn’t noticed the change in his coloring because she was seeing him every day, but as soon as the surgery was finished, she says, “His coloring completely changed. He become so much more pink. Now I look at the photos right before the surgery and he definitely had some bluish-grayish tint.”
“For me the hardest thing about that experience is you have that strong maternal instinct from the moment your kid is born to keep your baby close to you and protect him,” she says. “To not be able to protect him and be close to him during such a major surgery feels helpless.”
Colton will be able to have a regular childhood, except will be monitored by doctors every couple of years. When he’s a teenager or possibly in his early 20s, he’ll need a pulmonary valve transplant.
He will know it’s time when he’s not able to do things like running as quickly as he once did.
Technology has come a long way. It used to be that doctors would wait to do a repair of the heart and would just put in a shunt to treat the symptoms. Now, as heart-lung machines have gotten better and techniques have become more sophisticated, doctors often can repair the hole and save the right ventricle from becoming overworked in a single-stage operation.
In cases in which the intervention isn’t done early, sometimes it becomes too late. Hancock Friesen says when she travels to countries where people don’t have access to medical care, sometimes the right ventricle is so thickened that it cannot be repaired.
When babies like Billy Kimmel and Colton Campbell do grow up and have a valve replacement, the exciting thing is they might not have to go into an operating room. Hancock Friesen says they now can replace a pulmonary valve by going in under the skin in Dell’s catheterization lab rather than do an open-heart surgery in an operating room.