The young heart is in overdrive

Three-year-old Bethany Kerkstra was born without a head, missing most of the top of her skull. The condition is known as cutis aplasia. He also had two holes in his heart.

She spent a significant amount of time at the Gerber Family Neonatal Center at Corewell Health’s Helen DeVos Children’s Hospital and was also admitted to the PICU before going home.

Dr. Oliver Aregullin, a congenital interventional cardiologist at Corewell Health’s Helen DeVos Children’s Hospital, said her brain was mostly exposed at birth, which was her worst to begin with, and would require plastic surgery to close the gap before the holes could be closed. in the heart.

“He had a lot of complications,” she said. “He had skin grafts and several other procedures. After our plastic surgeon colleagues healed his skull, that’s when we started to fix his heart and lungs.”

Bethany’s parents, Eric and Sarah Kerkstra, said she spent 8 weeks in the NICU, which was difficult and traumatic for their family.

“He only came home from the NICU for 6 days and then was readmitted to the PICU for another month,” Sarah said.

He was also diagnosed with pulmonary hypertension and has a congenital heart condition that will follow him for the rest of his life.

“We were going back and forth to the hospital almost every day,” Sarah said. “We sent the older children to school and drove to the hospital, where I would stay until 7 in the evening. The days were very long.”

She said that it is extremely difficult to have a child with such a rare disease.

“Every time a new nurse came in, it was like learning a whole new lesson,” she said.

After a month in the PICU, Bethany returned home to her family.

He had several more brief hospital stays leading up to his “major surgery,” as his parents called it. He eventually had to have heart surgery to close his holes.

John Girotto, MD, a plastic surgeon at Helen DeVos Children’s Hospital, helped reconstruct Bethany’s head in collaboration with pediatric neurosurgeon Marian Berkou.

“These holes in the skull are usually the size of a nickel, but Bethany’s holes were almost the entire top of her head,” Dr. Girotto said. “It was a difficult procedure, but his family came to the right place because our team is skilled at fixing these situations.”

a donut-shaped device

Bethany was also diagnosed with pulmonary hypertension and suffered from fainting episodes and seizures. The fainting was caused by too much pressure in his lungs, which caused his heart to stop pumping blood.

He took three medications to treat it with no luck.

Doctors performed several cardiac catheterizations before surgery in April 2020. Dr. Neil Hillman repaired two holes in her heart, one an atrial septal defect (a hole in the wall that separates the upper chambers of the heart) and the other a ventricular septal defect (a hole in the wall that separates the two lower chambers of the heart).

When Bethany was 2 years old, Dr. Aregulin and Vettucattil performed a cardiac catheterization to place a research device in his heart. The probe device, the Atrial Flow Regulator (AFR), opens the upper chambers of the heart, and through the hole, doctors can reduce pressure on the heart.

This technique has been used in Europe but has not been approved by the FDA, so Bethany was the smallest and youngest patient to have this procedure in the United States.

Dr. Aregulin received special compassionate use approval from the FDA and placed the Atrial Flow Controller in Bethany’s heart in April 2022.

“The device is small, it’s inserted through a cardiac catheterization,” Dr. Aregullin said. “It looks like a little donut and helps divert blood between the two upper chambers of the heart.”

Bethany’s family has also applied for a second lung transplant, but for now, doctors want to wait and see if the medication and an additional shunt through the AFR device will help her body reach better oxygen levels and manage her pulmonary hypertension.

Thankfully, Bethany’s mom said she hasn’t passed out since the device was put in and is doing much better.

“When patients get too much pressure in their lungs, they have trouble breathing, they can go into cardiac arrest, and they can even die,” said Dr. Aregulin. “He never got that serious because the AFR device helped prevent that from happening.”

After the device was implanted, doctors tried various medications with good results.

And after a successful surgery and months of recovery, Dr. Girotto and his team were able to stabilize her head and proceed with plastic surgery.

“Living His Best Life”

Bethany’s mother Sarah said she lives a very full life, albeit a little limited. He can walk, run and play but is almost always out of breath.

“When he’s breathing, it’s pretty much his normal breathing rate,” Sarah said. “He’s a typical 3-year-old with a little attitude and sweetness all rolled into one. He’s learning the alphabet, numbers, letters and colors, but he’s not very vocal yet.”

Bethany is lucky to have two older siblings who happily speak for her: 10-year-old Olivia and 8-year-old Henry. Two Doberman siblings, Rocky and Ruby, complete their family.

They have been homeschooling their children since 2020, because every little illness can turn into a major setback for a child with a weakened immune system.

Sarah said Bethany has some developmental delays, motor skills issues and a speech delay, but is still quite active.

Bethany and her family live on 15 acres of woods where they enjoy outdoor activities such as four-wheeling and nature play.

“He also likes to splash and play in the dirt,” Sarah said. “Together with learning to read and looking at books.”

Bethany also loves Minnie Mouse and Hot Wheels.

Dr. Aregulin recently visited the family and said this is the first time he has been above the 50th percentile for growth and development.

“He’s doing great,” she said. “This is definitely a milestone for him.”

Bethany is currently on three powerful hypertension medications.

“He doesn’t have any significant heart failure concerns now,” Dr. Aregulin said. “He’s the smallest patient we’ve used this device on. And seeing him almost nine months later … from what I’m seeing and what I’m hearing, he’s doing a lot better.”

Her oxygen levels are also essentially normal, he said.

“Now she’s living life like a normal 3-year-old,” Sarah said. “His legs are a little weaker than other kids. But other than that, he’s just living his best life.”

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