Baby Asher sits in his high chair at a park with a homemade “smash cake” in front of him. He looks around with his big blue eyes while his loved ones sing Happy Birthday. He gingerly touches the cake and starts taste testing. Before long, he dives in with both hands and devours his very special cake in this very special moment, covering his precious face with white icing. It’s his first birthday, after all, and this little heart warrior deserves it.
“I was thinking how blessed we are that he is here,” says Rhenda, Asher’s mom. “A year ago, we weren’t sure if he would be, and it was a rollercoaster day of holding back tears.”
Rhenda and her husband, Jeremy, are from Port Charlotte and were eager to welcome their newest addition to the world. Rhenda was considered a high-risk patient because of her age and was referred to Karen Raimer, M.D., a maternal-fetal medicine specialist in the Johns Hopkins All Children’s Maternal, Fetal & Neonatal Institute, but she wasn’t expecting the news that came during her sonogram anatomy scan at 20 weeks. Due to the COVID-19 pandemic, Jeremy joined the appointment via a video call.
“The sonographer, Denise Aviles, kept going back and forth over the heart, and I didn’t think anything of it, but I could tell by Jeremy’s face that something was bothering him. Then the doctor came in,” Rhenda explains, pausing as she gets choked up reliving the moment — she apologizes, “I’m going to cry. I’m sorry.”
It was an overwhelming and emotional day. There was something wrong with Asher’s heart — likely a significant congenital heart defect. Taking on the role of more than sonographer, Denise stood by her side.
“She held my hand during it and everything,” reflects Rhenda. “I was scared. I cried.”
After the initial shock, she and Jeremy prepared.
“I can’t even tell you how much research we did to prepare ourselves because we figured the more we knew, the better off we would be. It was important for us to have as much information as possible to better understand our journey ahead with our little hero. We prepared for the best, and we also prepared ourselves for the worst-case scenario,” Rhenda says.
Part of that preparation was getting to know the cardiovascular intensive care unit team at Johns Hopkins All Children’s and learning the possible scenarios for after Asher was born.
“We had telemedicine conferences where I met with different staff members at Johns Hopkins All Children’s, in case the baby had to receive care from different departments/specialties,” Rhenda says. “We met with the cardiac unit and then about a month before Asher was going to be born, I had a telemedicine with his surgeon, Dr. Q.”
James Quintessenza, M.D., is co-director of the Heart Institute and chief of cardiovascular surgery at Johns Hopkins All Children’s Hospital. He and Awais Ashfaq, MBBS, would work together to perform the three heart surgeries needed to give Asher a chance at life.
At 37 weeks, Rhenda’s blood pressure spiked, so Asher was delivered via cesarean section.
“I thought I could lose my son and wife. Sitting behind Rhenda while they were delivering Asher has been the hardest thing I have faced, but facing it with her made it a lot easier,” Jeremy says. “The moment they showed us Asher all I wanted to do was hold him and kiss him.”
Rhenda recalls, “In the operating room, they had the cardiology team there. There were so many people in there waiting to evaluate Asher immediately after birth to make sure that he was OK. He was stable enough for me to be able to hold him, thank God.”
Asher was born with tricuspid atresia with d-transportation of the great arteries (d-TGA) and small ventricular septal defects. This meant he had only one ventricle instead of two, and a smaller than usual aortic valve.
“He was born with half of a normal functioning heart with the major artery emerging from the underdeveloped half,” Quintessenza explains. “This is a rare form of congenital heart disease. He required a major surgery in the first week of life.”
At just 6 days old, Asher was whisked away to the operating room for his first of three surgeries.
The Road Ahead: Asher’s Series of Surgeries
Norwood procedure, 6 days old: This is major surgery. Previously, 15 percent of babies discharged after this surgery died at home nationally. Now, with a new follow up protocol and medical advancements, the risk is down to 5 percent. During surgery, the blood vessels from the heart are rearranged to allow for good blood flow to the body. A shunt is also placed to help get blood to the lungs.
Glenn procedure, 5 months old: Reroutes blood flow from the upper body. The plastic shunt, which provides blood to the lung circulation, is removed and replaced with a major vein from the upper half of the body that is connected to the lung circulation.
Fontan procedure, around 2 years old: Palliative surgery, which makes the heart as close to “normal” as possible. A major vein from the lower half of the body will be connected to the lung circulation.
Between Asher’s first and second surgeries, he was seen every two weeks in follow-up appointments by Benton Ng, M.D., and the cardiology team. It was hard on Rhenda and Jeremy, who have two other children, but they remained positive.
“Asher had an echocardiogram at each appointment to check on his heart,” Ng says. “The family was very committed to Asher’s care. Knowing how far they traveled for each visit speaks to that. They were excellent about reaching out to us with questions. They were clearly on top of every aspect of their care.”
Rhenda and Jeremy had access to a special phone line to ask questions or express any concerns from medicines to his gastrostomy tube, or G-tube, (which help him receive the nutrients his body needs).
Asher is now also seen by Joelle Miller, M.D., and the cardiology team at Johns Hopkins All Children’s Outpatient Care, North Port. He still needs the G-tube to continue gaining weight and growing, and also does feeding therapy, with physical therapy on the horizon to help with developmental milestones. But he has come very far, and his recent birthday celebration commemorated that.
“For everything he's been through, he's one of the happiest babies. I cannot thank the staff at Johns Hopkins All Children’s enough — they are simply angels on Earth. Dr. Q, he’s an angel on Earth walking. I will forever be grateful.”
“I love all of the folks that have touched our lives, caring for our little hero,” Jeremy says. “My hopes and dreams now for Asher are that he can reach his hopes and dreams no matter how big or small. I hope he can do anything he sets his mind to and his condition never limits his ambitions and dreams.”
Visit HopkinsAllChildrens.org/Heart for more information about the Heart Institute teams.