Monae and Phillip Johnson dreamed of having children. Eleven years ago, they welcomed their beautiful daughter, Simone. During the pregnancy, Monae developed lupus, an autoimmune condition that also affected the child’s heart, leading Monae to wonder what her chances were of developing the condition again in future pregnancies.
Monae and Phillip wanted to give Simone a sibling, and after multiple attempts to have another baby – and enduring a miscarriage along the way – the Johnsons learned they were expecting again.
“We were surprised, happy, and nervous at the same time,” said Monae. “When I was nine weeks along, I worked with Dr. Michael Barsoom, the same high-risk OB-GYN who delivered Simone. Because of my prior experience, and since I had the tendency of developing lupus during pregnancy, our baby, Eva, and I were monitored closely with weekly ultrasounds.”
At 20 weeks, Eva’s heart went from normal to second-degree heart block to complete heart block, prompting Children’s Cardiology team to recommend fetal heart ultrasounds every week. Fetal cardiologist Dr. Jennifer Winters was assigned to Eva’s case, reading the ultrasounds, and Dr. Jeffrey Robinson became her primary cardiologist.
Welcoming Baby Eva into the World
On Sept. 23, 2020, the Johnsons welcomed Eva into the world. She weighed 4 pounds, 12 ounces and was delivered via C-section at 36 weeks.
Later that evening, Eva — at less than one day old — had her first heart surgery. Her cardiac surgeon installed temporary pacemaker wires since Eva wasn’t big enough for a permanent pacemaker.
Setbacks along the way required additional surgeries. Finally, when Eva was 2 months old, she reached her goal weight of 6.6 pounds and underwent another surgery in November to receive her permanent pacemaker.
“Everything was going well,” said Monae. “She wasn’t on a ventilator, and she wore clothes for the first time, which was an exciting ‘normal baby’ moment for us. Her doctors even told us that she was on her pathway home, which was very reassuring.
“Then, as I was changing my daughter’s diaper, I noticed drainage from her sternal incision. Her pacemaker got infected, and she was heading back to surgery. This was the ultimate high to low for our family. We broke down many times. We put our hope in God and in the hands of her medical team.”
More Surgeries – and a different approach
On Jan. 4, 2021, Dr. Ali Ibrahimiye, a pediatric cardiothoracic surgeon, and Dr. Oluwaseun Adetayo, Children’s Division Chief of Plastic Surgery, were involved in Eva’s fifth surgery, in which they cleaned up the incision and began investigative surgery to identify the cause of the recurring infections. Following the procedure, it was decided that the permanent pacemaker would need to be removed. It was left in place and gradually turned down over the next couple of days; however, Eva started to show signs that she couldn’t tolerate the low heart rate, so her pacemaker was turned on again.
Eva was then taken back for her sixth surgery. The permanent pacemaker and leads connected to the heart were removed, and new temporary pacemaker wires were placed and turned on again. This allowed the infected “pocket” to heal so the Cardiothoracic Surgery team could safely place a permanent pacemaker later without ongoing infection risk.
Though Eva was back to square one – wearing a temporary pacemaker again until she was ready for a permanent one – Dr. Ibrahimiye and Dr. Adetayo learned what was causing the multiple infections. They noticed the wires from the permanent pacemaker to the heart had worked their way up toward the skin. Eva’s body did not respond well to the sutures, which also contributed to the recurring infection. With the mystery solved, Dr. Ibrahimiye and Dr. Adetayo worked together to plan and perform Eva’s permanent pacemaker surgery.
“We took a different approach to this surgery,” said Dr. Ibrahimiye. “We first made a small incision in the abdomen to insert the pacemaker leads and connect it to the outside surface of the heart. The device was placed on the right side of Eva’s abdomen because there was too much scar tissue on her left side from her previous surgery. Together with the Plastic Surgery team, we elevated a safe, deep place of muscle flap, so we were able to create a new pocket of muscle to secure the pacemaker.”
“When I opened Eva’s sternal wound, I noticed several of her muscles were no longer properly attached to the sternum or her upper abdomen,” said Dr. Adetayo. “Due to the infection, the muscles had retracted, and the scar had shortened, similar to when anyone’s body tries to heal with a scar. In addition, her tissues were very stiff with poor blood flow because it had been infected so many times.”
The goal of surgery was two-fold: fix the recurrent problems Eva had experienced over several months and prevent subsequent reoccurrence. The scarred and stiff muscles were repositioned and reconstructed in the chest and abdomen to restore both function and appearance in their normal anatomic position. For a female patient, the team must consider breast development later in life, so the approach for closing the chest with muscle flaps is done with meticulous attention to detail. The Plastic Surgery team took several layers of tissue and closed each incision securely to reconstruct Eva’s chest wall and close her abdomen.
“I appreciated the strong collaboration of the Plastic Surgery and Cardiothoracic Surgery teams to give Eva the best outcome,” added Dr. Adetayo. “Multidisciplinary specialty collaborations are crucial in complex patients to help improve their outcomes, and we were able to accomplish this for Eva by working together as a team.”
Bringing Eva Home
After a month of considerable progress post-surgery, 7-month-old Eva was ready to join her sister, Simone, at home. Monae says she is grateful to Eva’s entire care team, spanning Pulmonology, Gastroenterology, Plastics, Cardiology, Physical and Occupational Therapy, Endocrinology and Wound Care, for taking great care of her daughter and providing a support system for their family.
“The care team at Children’s was a saving grace for me,” said Monae. “The love they had for Eva melted our hearts. Dr. Adetayo was a ray of sunshine, and she did an amazing job reconstructing Eva’s abdominal muscles so they can function properly. As for Dr. Ibrahimiye, he was a gentle spirit and so calm and collected and gave me the confidence to know Eva was in good hands with the medical and surgical teams. Dr. Barsoom and the Wound Care team were great to work with, too. Besides the outpouring of support from her care team, our faith and hope in God helped us stay strong for our daughter.”
Plastic & Reconstructive Surgery
At Children’s Nebraska, we understand that birth defects or traumas can be difficult and overwhelming — especially when they are visible or affect a child’s development. Our specialists are here to help children ages 0 to 21 through all of their plastic surgery needs.
Cardiology & Heart Surgery
From minor murmurs to major defects, disorders, and diseases, Children’s Nebraska offers comprehensive cardiac care programs for both children and adults with congenital heart issues.