Treating pulmonary hypertension

“I had never even heard the words 'pulmonary hypertension,’ ” Mitchell said.

The family was referred to Children’s Healthcare of Atlanta’s Sibley Heart Center.

“Before we could make an appointment, we got a call from out of the blue from Patricia Lawrence [a pediatric nurse practitioner], who told us that they could see Tucker within a couple of days,” Mitchell said. “I didn’t know her or anything about the pulmonary hypertension program at CHOA, but going there was the best thing we could have done. When I think about that call now, I thank God. We couldn’t have asked for a better team of doctors and nurses.”

Children’s Healthcare of Atlanta established its hypertension program in early 2011. The only such center in the Southeast and one of just a few in the nation, the facility serves 108 patients and their families.

Tough disease to treat

“We call pulmonary hypertension an orphan disease because it is rare, relatively young and there has not been as much research into it as other diseases. We’ve only had treatments for the last 15 years or so,” said Dr. Usama Kanaan, pediatric cardiologist and director of the pulmonary hypertension program at CHOA.

Kanaan  learned about the disease during his training at the University of California, San Francisco, because doctors there were conducting research about it. He came to   Sibley Heart Center, knowing it was one of the busiest and best facilities in the country.

“I didn’t come with a mission, but seeing patients referred to other centers for follow-up treatment, I saw needs that weren’t being met,” Kanaan said.

Along with others, he advocated for a program to fill the gap.

“With a program, we’re able to devote the attention that a complex, serious disease like pulmonary hypertension deserves,” Kanaan said. “We now have the facilities and resources to provide high-quality, consistent and expert care for these patients. It’s become a significant part of my work. It’s both challenging and very rewarding.”

Building and managing the team fell to Lawrence, RN, MSN, CPNP-AC/PC. A cardiac specialist, the pediatric nurse practitioner was recruited from Boston, where she had created a pediatric fetal care center for families expecting infants with birth defects for three Harvard hospitals.

“The idea of building this program from the ground up excited me,” said Lawrence, program manager. “When I got here, all I had was a desk, some pens and a vest that said CHOA, but there were already 10 to 15 patients waiting to be enrolled.”

She had worked in cardiology long enough to know the challenges and dismal outcomes of the disease.

“This isn’t like system hypertension where you slap on a blood pressure cuff and get the results,” Lawrence said. “It’s a difficult disease to diagnose, because its symptoms are vague and mimic other common childhood problems, like asthma.”

The definitive test calls for a heart catheterization to measure pressure in the lungs.

“It’s not uncommon for there to be delays in diagnosis for up to two years, and all that time the disease is getting progressively worse,” Lawrence said. “Once diagnosed, the disease is just as difficult to manage. There is no prescribed treatment that works for every patient.”

Identifying and eliminating some of the associative causes can reduce blood pressure in some patients, she said. But often there are no known causes and doctors must find individual treatments for each patient.

With no cure, patients with idiopathic pulmonary tension often have shortened lives.

Treatments aim to improve symptoms and slow down the disease.

“You have to be an investigator in this work and you can never get too comfortable or bored. Every patient is different,” she said. “The beauty of our program is that it’s a one-stop shop for families.”

The power of teamwork

Patients can have tests performed  at the clinic before meeting with a team consisting of a cardiologist, a pulmonologist and a nurse who discuss test results and create a holistic treatment plan. Regardless of the issue, families know they can call for help — and they often do.

“Patricia Lawrence gave me her cellphone number and has called us when she was on vacation. Doctors will call us to discuss results and answer questions. There isn’t much research about this disease and what you read is grim,” Mitchell said.

Since Tucker has been enrolled in CHOA’s program, the team has  eliminated many of the causes of his pulmonary hypertension. They have closed up his heart defect, performed a sleep study and treated his severe obstructive sleep apnea with night-time oxygen and eliminated his aspiration swallowing problems by thickening his food.

“They performed another catheterization last June and we got great results. The pressure was only a little above normal and he doesn’t even need medication,” Mitchell said. “In September, we were able to take him off the oxygen and he’s started a special-needs preschool. He’s a real flirt and rarely meets a stranger.”

She credits the CHOA team’s persistence and expertise  for her son’s progress.

“Tucker is one of our success stories and we want to see more of them,” Lawrence said. “But even with treatment, this disease can be relentless. It keeps us all on our toes.”

To better understand the disease and what treatments are appropriate for children, the pulmonary hypertension program has several research projects and drug trials under way.

The program is also trying to raise awareness among physicians and the general public so  children can be diagnosed earlier. “Most experts agree that earlier treatment prolongs life,” Kanaan said.

With increased awareness and more research nationally, he is optimistic that a cure or significant strides in treatment will come  in the future.

“Right now, we know that we’re improving the quality of care and lives of our patients and we’re supporting their families as they struggle to cope with this chronic disease,” he said.