Hypoplastic Left Heart Syndrome
Jordan and Jarrett Franklin were excited about becoming parents, and confident they would introduce their son Jase to his new nursery a few short days after birth. They never imagined it would take nearly a month, and a team of exceptional health professionals, for him to become well enough to go home. Jase was born at a local hospital where, initially, he appeared healthy. Then, his caregivers noticed his oxygen saturation levels were low, so he was transferred to the Cardiovascular Intensive Care Unit (CVICU) at Children’s of Alabama. “It was a real roller coaster of emotions,” his parents say. “We were getting ready to be discharged from the hospital to go home. Then the next thing we knew, he was being transported to Children's.” Jase was quickly diagnosed with a severe congenital heart defect; hypoplastic left heart syndrome (HLHS), which means he was born with only one pumping ventricle. The devastating news was compounded by the realization that he would not survive without undergoing a high-risk surgery called a Norwood operation. They were relieved, however, to learn that the cardiovascular (CV) team at Bruno Pediatric Heart Center has helped many infants survive this condition and go home with their families. Three days later, Jase’s parents kissed him goodbye as he was taken to the CV operating room. After eight long hours, the procedure was complete, and Jase returned to the CVICU in very critical condition. The family was told he was not doing well. His oxygen saturation levels continued to be dangerously low, despite aggressive resuscitation from the medical team. “It was clear that Jase wasn’t recovering as expected, and he needed more surgery,” says cardiothoracic surgeon Robert Dabal, M.D. So Jase was rushed to the hybrid cardiac catheterization lab - a cutting-edge suite where fluoroscopic imaging and surgery can be performed simultaneously to manage the most complex cardiac cases. He remained in the hybrid suite overnight, surrounded by a team of cardiothoracic surgeons, interventional cardiologists, cardiac intensivists, anesthesiologists, nurses, technicians, CRNAs, and perfusionists. Despite fatigue, the team worked diligently to restore the blood flow in the newborn’s tiny heart. “Working through the night, the interventional cardiology team worked with me and my surgical team to diagnose and successfully fix his problem.” Dr. Dabal says. The following morning, Jase returned to the CVICU. He remained critically ill, and required ECMO, a type of heart/lung bypass machine, to support his fragile heart and lungs. However, his heart was now fixed and began to grow stronger. He was weaned from ECMO the following day, and continued to improve. After a week, he no longer required life support. “Jase’s miraculous recovery, from his heroic interventions and through his tenuous days in the CVICU, would not have been possible without the hard work, dedication and teamwork of too many people to mention - most important our cardiac nurses, who were crucial in the transformation of Jase from the sickest child in Alabama to the baby boy his parents were praying for,” says Jeffrey Alten, M.D., Medical Director of the CVICU. Jase went home with his parents twenty-four days after they first learned about his grave heart condition. “The whole time we were in the hospital, our goal was to get him home,” his parents say. “When we finally were able to take our son home, we were elated.” They were understandably nervous, too. “The nurses really prepared us for taking care of Jase when we went home,” says his parents, “but taking care of a child with such a serious condition can be daunting, especially for first time parents who are learning how to take care of any baby.” Because of the high-risk nature of his heart condition, Jase was eligible for the “Hearts at Home” program, which provides home monitoring and support for babies with complex congenital heart disease. “Most of our parents feel overwhelmed by taking their baby home following surgery,” says Sarah Torsch, CV nurse practitioner and coordinator of the “Hearts at Home” program. “With close monitoring, we are able to help them identify problems early. We want them to know they are not alone in caring for their baby.” Jase is now thriving. He returned to Children’s briefly, in December, for the second of three major cardiac surgeries he will need during his childhood. And his CV family will never forget the miracle baby, Jase Franklin, who is happily back home again with his parents. "We are grateful to all the doctors, nurses, and other caregivers at Children's for giving us the opportunity to do what we wanted to do in the first place - get our son home and watch him grow,” the Franklins say.
