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Claire Elizabeth Kowal

A story about heart repair

claireAfter having two healthy boys, Jennifer and Pete Kowal thought it was strange that the ultrasound technologist and obstetrician were having trouble seeing their baby's heart. They had brought the boys to the ultrasound thinking it would be a routine appointment. Their boys, Simon, not even 2 ½, and Oliver, then 1, had reached their limit of patience after 20 minutes of scanning, so Pete took them out for a break. That's when the doctor turned to Jennifer, pointed to the baby's heart on the screen and told her that they weren't having trouble seeing the heart, there was something wrong with the heart.

Jennifer was stunned. She left the exam room to find her husband and told him the news with tears stinging her eyes. The couple was in a daze until their appointment at the Children's Heart Center at Golisano Children's Hospital at the University of Rochester Medical Center. It didn't feel real until Carol Buzzard, M.D., a pediatric cardiologist, laid out the series of defects their baby had: Hypoplastic right heart syndrome (HRHS), tricuspid regurgitation and stenosis and severe pulmonary stenosis, which all means that the right half of the heart is too small, some blood flows back into the heart instead of out and a blockage in the pulmonary valve prevents blood from flowing into the lungs for oxygenation. Then she laid out the series of three open-heart surgeries the baby would likely need after birth.

That diagnosis was not a good one and the prospect of open-heart surgery was scary, but Jennifer somehow felt a little better. She now had a name for what was happening to her baby.

“We walked out of there as new people,” Jennifer said. “It’s amazing what you can learn in an hour and how your life can change.”

She headed straight to her computer to find out more. She joined online support groups and became an advocate for her baby.

At the beginning of the pregnancy, the couple had decided not to find out the gender of their baby, but everything was so different now and with so much uncertainty looming, they decided to find out—Simon and Oliver would have a little sister. A little sister with an underdeveloped heart. A little sister who would need a series of open heart surgeries to survive.

Prepping for the arrival of Claire Elizabeth

At Jennifer’s next obstetrical appointment at 25 weeks, Marit Sheffield, M.D., Jennifer’s obstetrician at Park West Women’s Health, assured her that she’d be a part of the team at their baby girl’s delivery. She would perform Jennifer’s C-section, just as she had for the boys.

“I could not imagine not having her there,” Jennifer said. “So it was comforting to hear that she will be there with us on this journey.”

Unfortunately, even though the Kowals got the good news from their amniocentesis that their baby girl didn’t have a genetic anomaly, their next echocardiogram showed that her heart was getting worse. Her right ventricle hadn’t grown since the last scan, the pulmonary valve had closed off and it looked like her right ventricle might also end up blood-starved. A subsequent scan showed the same thing, which meant it didn’t get any better, but it didn’t get any worse either. The Kowals decided to focus on the positive, and the technologist, Marianne Rees, helped them.

“Marianne treated us to a few minutes of face time with baby girl – we watched her playing with her face and moving around,” Jennifer said. “Usually the appointments are all-business. It was a wonderful break from all the worry.”

Then it was time to meet their surgeon, George Alfieris, M.D., chief of pediatric heart surgery at Golisano Children’s Hospital. He and Buzzard went through how they predicted the baby’s care would evolve from birth through three separate surgeries. In the initial surgery, within days of her birth, Alfieris would put in a shunt between the baby's aorta and her pulmonary artery to allow her blood to pick up more oxygen from the lungs before heading back out into her body. He would also open up with pulmonary valve with a patch. The second surgery would take place at about 6-months-old and is called the bi-directional Glenn procedure. In that surgery, the blood from her head and upper limbs is rerouted directly to the lungs, bypassing the right ventricle. The last surgery would take place several years later and is called the Fontan procedure. This surgery reroutes the blood from the lower body directly to the lungs, once again bypassing the right side of the heart.

“We were comforted by the fact that Dr. Alfieris was extremely familiar with our type of defect and knew what he intended to do once she was born,” Jennifer said. “After meeting with him and Dr. Buzzard, Pete and I felt much more comfortable and knew our baby was in great hands.”

The First of Three Surgeries

Claire Elizabeth arrived at about 11 a.m. Monday, July 27, 6 lbs. 4 oz. and 18 ½ inches long. A peanut compared to her brothers, but pink and surprisingly healthy looking. An echocardiogram in the neonatal intensive care unit (NICU) shortly after her birth and a cardiac catheterization the next morning confirmed what doctors saw in utero. She would need surgery, and soon.

The day after his little sister was born, Simon scrubbed his hands and arms and went into the NICU to meet her. He wore a face mask and stood on a stool to see her. He asked about her boo boo on her heart and then he reassured his mother that the doctors would make her better.

“Although he was aware he had a new baby sister from pictures and such, seeing her was very important. He talked about her all the way back to my room. Pete told me when he got home he made the neighbor girls sit down on the porch and look at the picture of his baby sister,” Jennifer said.

On Claire Elizabeth’s third day in the world, Jennifer and Pete waited in the operating room preparation area, holding their daughter. After Alfieris explained what he was going to do in this first surgery again, they kissed Claire Elizabeth goodbye at about 9 a.m. and went back to Jennifer's room to wait. Gina Cable, the nurse practitioner for the pediatric cardiac surgery program, came to their room to give them updates. At about 2 p.m., Alfieris came to the room and told them that Claire Elizabeth had done very well. Her chest was left open and covered with gauze in case of swelling.

“When we walked into the PCICU (pediatric cardiac intensive care unit ) to see our little girl ... a sense of calmness came over us. I don't think we can explain the relief to have her out of surgery and now on the mend,” Jennifer said. “I'm not sure what we expected to see, but Pete and I were thrilled to see how good she looked.”

“It is evident that God has blessed Dr. Alfieris with an unsurpassed talent.”

Taking Baby Steps

Over the next few days, Claire Elizabeth did very well keeping her blood-oxygen levels up and her swelling went down. She was weaning off several medications and was stabilized. Jennifer even felt comfortable enough to leave the hospital for a few hours to see her boys and was surprised by her favorite dinner brought over by a friend. After her chest was closed, Claire Elizabeth practiced taking some breaths with her ventilator output lowered. Once she got the hang of it, the ventilator was removed and Jennifer and Pete could hold her again and Claire Elizabeth could breastfeed. A major hurdle for babies who've been through surgeries like this is gaining weight and strength.

Over the next week, Claire Elizabeth took baby steps toward normalcy, as tube after tube was removed and she ate enough to please her doctors. At a little more than 2 weeks old, Claire Elizabeth got to go home for the first time and the family started adjusting to having three small children, one of whom needed extra care.

Simon and Oliver dote on their little sister, and she adores them back. She's growing and learning to love her special place in the Kowal family. Claire Elizabeth still has two major surgeries to go, but she's doing very well.

“She laughs, hysterically, at her brothers and smiles quite a bit,” Jennifer said. “Looking at her reminds me that the small things don't really matter. Family and friends are what make us strong and give us the courage to wake up the next day.”