A New Normal: Lauren’s Story

One day last October, 15-year-old Lauren Riley was climbing the stairs to class at her West Hartford high school and felt it again: out of breath, dizzy and like she might faint. This was the third time in recent weeks she’d nearly fainted from a flight of stairs. Soon after, she started having belly pain, and then vomiting.

Everyone thought she was probably just under the weather. But her family decided not to take any chances.

She came to Connecticut Children’s. When her care team evaluated her with laboratory studies, a chest X-ray and an echocardiogram, they saw something alarming.

Then and there, Lauren was admitted to the Intensive Care Unit with a probable diagnosis of severe pulmonary arterial hypertension, a type of high blood pressure that affects the lungs and the right side of the heart. She was in right heart failure. She needed immediate treatment. She was very, very sick.

She and her family were stunned.

There was one piece of good news. Although there are a lot of kids with pulmonary hypertension, not a lot of pediatric health systems specialize in treating it. Connecticut Children’s is one of the few.

> Related: Emergency Room, Urgent Care or Video Visit? When Your Child Needs Care, Here’s Where to Go

“A lot of times that doesn’t happen, even for adults”

Most people have never heard of pulmonary hypertension, especially in young people. Although it’s a life-threatening disease, it often goes undiagnosed. That’s partly because the early signs of it – like trouble breathing during exercise – can be easy to ignore. Even when symptoms become more dramatic, they’re often mistaken for asthma or just being a little out of shape. But the truth is, pulmonary hypertension is a challenging medical problem that involves the whole body, and can occur in infants, children and adults. There are a lot of different reasons someone may have it, from an undetected heart problem to complications from another disease. Diagnosing it and figuring out the right treatment is like solving a puzzle. Treating it correctly, and early, can save a patient’s life.

Lauren’s care was led by an expert in pulmonary hypertension in children, Joseph Kuruvilla, DO, FAAP, from Connecticut Children’s Division of Cardiology. In a sense, he became the quarterback of her team, which also included physicians from the divisions of Rheumatology, Pulmonary Medicine and Intensive Care.

 Joseph Kuruvilla, DO, FAAP, a cardiologist with an expertise in treating pulmonary hypertension in children

“A pulmonary hypertension program requires a lot of coordination and collaboration with multiple specialties,” says Dr. Kuruvilla. “You have to look at the body in its entirety, and consider other systemic causes that may initially sound unrelated to the heart and lungs. That open exchange of ideas among other disciplines elevates care across the entire health system.”

While Lauren was in the hospital, doctors from all the specialties would gather every morning to discuss Lauren’s health, and Lauren and her family were always included. Lauren’s mother, Denise, marveled at it. “Sometimes you need to have a whole group together from other specialties to make things work,” she says. “A lot of times that doesn’t happen, even for adults. But that’s what happened.”

Lauren is a thoughtful teenager, who weighs what she says very carefully. It meant a lot to her that Dr. Kuruvilla took a thoughtful approach too. When he explained the seriousness of pulmonary hypertension, and how it would impact her life, he sat in a chair beside her hospital bed so he could speak to her directly.

“The information that he gave me was easy to understand, and right to the point,” Lauren says. “He’s really helped me understand what’s going on.”

“From the start, Dr. Kuruvilla knew what to do and we trusted in him,” says Denise. “It was hard to hear about the gravity of Lauren’s situation, but we appreciated hearing the real deal. He helped us understand that our life was going to change. He said we were going to have a new normal.”

> Related: When newborn Delilah needed surgery for a rare heart defect, Connecticut Children’s was there

“A diagnosis doesn’t set in until you hear what it means”

Dr. Kuruvilla explained that there is no cure for pulmonary hypertension, and living with it requires a partnership with open communication, trust and aggressive medical treatment. This means that, unless another treatment is developed, Lauren will have a continuous delivery of medication, either by IV or a catheter placed under the skin, for the rest of her life.

“A diagnosis doesn’t really set in until you hear what it means,” says Lauren. “And mine really hit me when he said ‘for the rest of your life.’ It made me imagine what I’d look like with this catheter in my arm in 10 years, in 20 years, in 30 years. It wasn’t a pretty thought. But I keep thinking: It’s not as bad as it could be.”

Meanwhile, research is changing what doctors know about the disease every day. Dr. Kuruvilla has used these findings to keep Lauren’s life as normal as possible. For example, her medication is delivered into her arm using a small pump about the size of an iPhone. She and her family can care for the subcutaneous line at home, and it’s small enough that Lauren can slip it into her jeans pocket. If you didn’t know to look for it, you would never know it was there.

Lauren was in the hospital for a month before she was healthy enough to go home. The first thing she did was close the door to her room and flop on her bed. She’d been imagining exactly that moment for weeks. It meant she was finally healthy enough to return to her life.

> Related: With Video Visits, you can connect with our pediatric experts right from home

“Connecticut Children’s was here at the right time”

After several months of treatment, Lauren is doing great. She can now walk nearly 10 times as far as when she was first diagnosed. She can enjoy her favorite foods again. Thanks to the convenient way her medicine is delivered, she can move around like usual, and easily curl up with a good book – one of her passions. In many ways, life is the same as it was before she got sick.

In other ways, it is – as Dr. Kuruvilla promised – a “new normal.” She has frequent doctor’s appointments, and at home, she and her mom must be diligent about making sure her medication line and pump are operating the way they’re supposed to. If there are any issues, she needs to see a doctor immediately.

For families in other parts of the country, whose nearest pulmonary hypertension specialist is several hours away, it can be a stressful situation. For Lauren and Denise, it’s a comfort to know that Dr. Kuruvilla and Connecticut Children’s are just a few miles up the road.

“Had Dr. Kuruvilla not been here, we would’ve had to go to Boston or Manhattan,” says Denise. “The treatment has been outstanding. Connecticut Children’s was here at the right time for us. This is what we needed.”

Learn more about Connecticut Children’s cardiology services >>

Want more articles like this from pediatric experts you trust?

Sign up for our weekly email.
Subscribe

Related

 

 

Share This Post

Newsletter Sign-up
Want our latest Blog posts sent directly to your inbox once a month? Sign-up below.
* indicates required
Back to Top
Searching Animation
Searching