Accreditation puts PH team in rare company

By Emma Mann, for Riley Children’s Health

As an aspiring baker/chef, Rebecca Cooley says that brownies are her favorite treat to make for a very specific reason.

“I made these brownies as a surprise for my mom and dad, and I had to substitute something out, because we didn’t have it, but they still turned out really good!”

Similar to baking, 17-year-old Rebecca’s life has been full of substitutions and changing ingredients.

When Rebecca was 8 years old, she fainted during recess at summer school. She was given an albuterol inhaler because she had a history of asthma, and she recovered quickly.

To be safe, Rebecca's parents, Gary and Julianne Cooley, took their daughter to her primary care doctor in Fort Wayne. He believed there was a possibility that Rebecca had an upper respiratory infection.

To confirm his suspicion, he ordered a chest X-ray. Results identified an unsuspected cardiac concern. Rebecca was referred to a pediatric cardiologist in Fort Wayne, who performed an echocardiogram, which showed that her heart was enlarged.

Within two weeks of her initial fainting episode, Rebecca was referred to Riley Children’s Health, where doctors diagnosed her with pulmonary arterial hypertension, which occurs when the pressure in the blood vessels leading from the heart to the lungs is too high, damaging the lungs. The condition can be managed to a point, but there is no cure.

“It was definitely a scary diagnosis,” Julianne Cooley said. “There’s not a cure; there are just treatments. So, I mean, it was very hard.”

For Rebecca, who also was diagnosed with an atrial septal defect in her heart, this meant a new recipe for life. Her journey would involve adding, subtracting and changing medications and treatments for stability and the best results possible.

“I think a lot of adults who would have this diagnosis would probably be taking it a lot worse than what she is,” Gary Cooley said during a visit to Riley last month. “She is very resilient. She’s taken the punches as they come. We have hurdles, roadblocks and speed bumps that she just fights through, and she adapts to whatever changes come along.”

Since 2014, Rebecca has been under the care of the pulmonary hypertension team at Riley, which includes cardiologist Dr. Michael Johansen, pulmonologist Dr. Gregory Montgomery, program coordinator/nurse Tisha Kivett, two nurse practitioners, a PH nurse, social worker and dietitian, as well as genetics and pharmacy.

The team was recently recognized as a Pulmonary Hypertension Association-accredited PH care center, just the ninth accredited pediatric program in the country.

It’s a rigorous process to become accredited, requiring highly experienced staff, advanced treatments and comprehensive care.

Dr. Montgomery understands the significant effects of this “unforgiving” disease on his patients and stresses the importance of collaboration between patients and physicians. He works closely with Rebecca and her family to navigate her care to provide the best possible outcome and find solutions when problems arise.

“For long-established patients like Rebecca, the formal accreditation of our pulmonary hypertension program by the PHA serves as validation to these kids and their families that they have indeed received the absolute best possible care in our hands,” he said.

When Rebecca was first diagnosed, she was put on remodulin and was on a continuous drip of the drug until 2021, when she had heart surgery in St. Louis. At that time, a Potts shunt was put in, and her numbers started to improve. However, over time, her numbers started to trend negatively again, so the doctors decided to put Rebecca back on remodulin.

The medications and treatments at Riley have allowed her to continue to be mobile and have improved her breathing.

She recently underwent pulmonary rehab to increase her lung function and improve her breathing. Under medical supervision, she exercised an hour a day three times a week for 12 weeks. In addition, she has heart caths every three to six months.

Rebecca relies on an oxygen tank and has a BiPap machine (a type of ventilator) for nights. She calls her oxygen tank Clank because “he always kind of clanks around.”

Clank is her constant companion. Her friends and family understand that Rebecca and Clank are a package deal.

“My friends are wonderful,” she said. “They help me. They change my oxygen. They are excited to change it for me.”

Even Julianne and Gary like to have a good time with Clank.

For dress-up day at school, not only did Rebecca and her parents find the perfect outfit for Rebecca, but they chose the perfect bow tie for Clank.

"That bow tie is from when we went to prom,” Gary explained. He and Julianne were high school sweethearts, and he let Clank wear the bow tie that he wore to their senior prom.

Rebecca hasn’t had an easy time, but she says her medical team helps keep her spirits high because they can match her sassy attitude and energy.

“They’re really a fun group of doctors,” her dad said. “They joke around and have a good time. She gives them a hard time, and they give her a hard time, so they’re a really great staff of people.”

While hopeful for a cure, the family continues to trust in the treatments that Riley offers and in the team that supports her.

“The team is phenomenal,” Julianne said. “I’m able to text or email and they’ll get back to me right away.”

That confidence in the team should only grow, with accreditation status bolstering the program’s reputation, allowing families to continue to put their trust in the Riley PH program with the knowledge that “Riley Hospital is a care destination for PH,” Dr. Montgomery said.

For now, Rebecca will continue baking brownies and other treats with her sous-chef Clank, ready to add, subtract or change any ingredients that put her on the path to success in her medical journey and in her future baking career.

Photos by Mike Dickbernd, IU Health visual journalist, mdickbernd@iuhealth.org