Pulmonary Hypertension: Olivia’s Story

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Olivia has been treated for pulmonary hypertension at Children’s Hospital of Philadelphia (CHOP) since she was a baby. Now 9, she’s a happy, caring child who loves books and music.

Olivia dressed up standing outside smiling Olivia was born prematurely, at 33 weeks, in Somerset, NJ. She seemed to be doing well at first, but doctors discovered a ventricular septal defect (VSD), which is a hole between the two lower chambers of the heart, during an echocardiogram. Cardiac specialists believed that the VSD was a minor issue and after 10 weeks, Olivia was discharged.

Finding expert care

Still concerned, Olivia’s parents, Christina and Manuel, took Olivia to see several different specialists over the next few weeks for follow-up. A pediatric cardiologist at St. Peter’s University Hospital in New Brunswick, NJ, which is part of CHOP’s Care Network, recommended that they take Olivia to CHOP’s Main Campus in Philadelphia for a cardiac catheterization, a procedure used to diagnose and treat heart conditions.

Christina made an appointment with Jonathan Rome, MD, a cardiologist at CHOP and Director of the Cardiac Catheterization Laboratory. After the procedure, Dr. Rome had difficult news for Olivia’s parents: Olivia’s VSD had led to pulmonary hypertension, a condition in which the pressure in the vessels leading from the heart to the lungs is too high, causing extra strain on the heart. If left untreated, pulmonary hypertension can lead to heart failure.

Dr. Rome referred Olivia to CHOP’s Pulmonary Hypertension Program for specialized care. Olivia was started on oral medications right away. Within a few days, her condition had improved enough to go home.

A challenging time

At first, the oral medication did a reasonably good job of controlling Olivia’s pulmonary hypertension, but the effect diminished over time. When she was 2, a cardiac catheterization measured pressure within her heart at dangerous levels. During the procedure, a central line was placed in Olivia’s chest so that the team could deliver a stronger medication directly to her heart.

Olivia stayed at CHOP for four weeks after the procedure so the team could monitor her condition while the medication dosage was adjusted. Christina and Manuel were coached on how to care for her central line and how to store, mix and administer her medication.

“Those years were really tough,” remembers Christina. “We had to mix the medicine every two days. Olivia’s skin was sensitive to the adhesive in the standard dressing, so we had to use gauze and a different type of tape, which didn’t provide as much protection. If she got a fever, we had to immediately go to CHOP, and she would be put on antibiotics until the culture results came back.”

Olivia did get a couple of infections, and she had to have the line replaced several times over the next three years, which is common for children with central lines.

Manuel recalls a particularly trying night when they brought Olivia home after a long day at CHOP, where doctors had put in a new line. “We were so tired. As I was picking her up; I accidentally tore the line. So we had to go back to CHOP and get it repaired.”

“It was a time of high anxiety,” says Christina. “Things that could go wrong went wrong.”

A breakthrough option

During an appointment when Olivia was 5, Christina and Manuel discussed the challenges of giving Olivia medication through the central line. After considering different options, it was suggested that they try a new way of administering Olivia’s medications. In this method called subcutaneous delivery, the medication is injected under a patient’s skin, rather than into a vein. The method had never been tried with this medication in such a young child, but it was clear that the central line was no longer a good option for Olivia.

The change was successful. Olivia continued to receive the medication she needed, but now without the risk of a central line infection. The new delivery caused some discomfort each time the injection site was changed, but the team has worked with Olivia’s family to minimize her discomfort.

The change made a big difference for Olivia and her family. They no longer had to rush to the Hospital each time Olivia had a fever. With a central line, a fever could indicate an infection in the blood stream, which is very serious and needs to be treated immediately.

There were other benefits, too. Without the central line, Olivia began sleeping better. The pump for the central line used to occasionally disturb her at night, and the adhesive used to hold the line in place had been causing discomfort from time to time.

Manuel and Christina felt their anxiety levels drop, too.

Now when we get a call from school, we don't worry.

Subcutaneous delivery continues to be the method used to administer Olivia’s medications.

Olivia today

Olivia sitting outside smiling Today, at 9, Olivia is a happy little girl, full of smiles and generous with hugs for her parents and her two younger brothers. She likes music and dancing, and loves to have her parents read to her. “She’s very loving. Very kind,” says Christina. “She goes out of her way to help other kids.” Before her parents read to her, she always checks with her brothers to see if they want to listen, too.

Thinking back over the years of Olivia’s treatment, Manuel remembers the family’s first meeting with Brian Hanna, MDCM, a pediatric cardiologist and the Director of the Pulmonary Hypertension Program, when Olivia was first diagnosed.

“He was very reassuring. He told us, ‘We’re going to take care of her. I’m going to be in your life for at least the next three years.’ We felt confident in him. I still have full confidence in him. He’s open to listening, and to new approaches,” says Manuel.

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