Makenna Franks turns 5 this month. She'll probably start dance class soon. She noshes on Fritos twists, chases after her older brother and her boy cousins, and tries to climb trees the way they can in her suburban Houston neighborhood. Just a regular kid. But less than a year ago, she was on the operating table at Texas Children's Hospital for the third open-heart surgery of her four-year life.
Makenna was born with hypoplastic left heart syndrome, which affects about 1 in every 3,000 to 6,000 babies. In "hypoplasts," as doctors call these children, the left ventricle—the heart's main pumping chamber—and the aorta, which carries oxygenated blood to the body, stop developing before birth. Two of Makenna's heart valves also were defective.
Even as recently as the mid-'90s, most HLHS babies died. Gradually, however, a small group of surgeons has worked out a complex three-stage repair in which major vessels are rebuilt and rerouted to shift pumping chores to the right side of the heart. At Texas Children's, the death rate is now below 2 percent.
Makenna had the first stage as a 3-day-old infant and the second one at six months. Last August, Charles Fraser, chief of congenital heart surgery, performed the final step, which U.S. News chronicled in "Makenna's Heart" [September 3].
Recuperation wasn't fun. Her lymphatic system leaked fatty fluid into her lungs and around her heart, so Makenna was kept on a no-fat diet for six weeks after surgery. No Fritos. "Her big day was when she could finally eat those things," says pediatric cardiologist Michelle Grenier of Texas Children's, who has seen Makenna more than 10 times, most recently last month to run a battery of tests. "We want to watch these kids very, very closely," she says—measuring their blood oxygen, for example, to make sure their reworked hearts are pumping enough oxygenated blood.
The replumbed heart is working well, says Grenier. She pronounces her patient "just amazing—she knows she has a zipper on her chest, but she won't let it slow her down." Nor does Grenier think she should. "One of our concerns with our cardiovascular patients is that parents have a tendency to baby them, worry about them playing too hard, maybe feeding them a little too indulgently. Can she dance? Ride a bike? You bet."
Bobby and Brandi Franks aren't take-it-easy-sweetie parents. "We don't limit her," says Bobby. Nor do they assume that their daughter's life will now be perfectly normal. "Along the way I'm sure there will be bumps, and we'll do what needs to be done," says her father.
A recent study in the journal Circulation suggests that Makenna has a good shot at a smooth road. Researchers tracked every Children's Hospital Boston patient born prior to 1985 who had Makenna's type of surgery. Most, even those who had the procedure many years ago, were alive 20 and 25 years later.
"If Makenna came to me as a teenager and asked if she could run a marathon," says Grenier, "I would tell her she probably could—if she'd been doing as remarkably well as she has so far."