Regina’s journey with HLHS: Four years later

Regina was just 25 days old the first time she made the trip from her home in Mexico to Boston. Regina was born with a serious congenital heart defect called hypoplastic left heart syndrome (HLHS), resulting in the underdevelopment of the left side of her heart. Her doctors in Mexico told her parents it was unlikely she would survive, but they refused to accept that answer. Instead, they anxiously reached out to Dr. Gerald Marx, a cardiologist at Boston Children’s Hospital.

Dr. Marx said that he could help Regina if she arrived in Boston immediately. Over the following 19 months, Regina underwent three successful operations enabling her functioning right ventricle to do the work normally done by both ventricles.

Her parents know Regina is one of the lucky ones. There aren’t many opportunities to care for children with complex congenital heart disease in Mexico. “They are good cardiologists, but they don’t have the experience with HLHS like they have in Boston,” says Regina’s mom, Elizabeth. She knows of only two other children living with HLHS in Mexico.

The first time Boston Children’s shared Regina’s story, she was an energetic 4-year-old. Now 8, Regina is becoming a young lady who continues to defy expectations.

Educating others about HLHS

Growing up, Regina always knew that she had a heart condition. But, as she has gotten older, she is becoming more curious about understanding HLHS.

“Little by little, she began to ask us more questions, and so little by little we began to tell her more information,” says Elizabeth. “She slowly became more aware of her condition and aware that she had to take care of her body.”

Elizabeth also credits Dr. Marx with deepening Regina’s curiosity about her heart. “In Mexico, the doctors only check her heart. But Dr. Marx will answer all of Regina’s questions. He’ll tell her how his stethoscope works.”

Beyond learning more about HLHS herself, Regina has taken an interest in educating her friends and peers about the condition. This year, when her class had to do presentations with a family member, Regina chose to talk about her heart. She and her father, Eduardo, taught her classmates about HLHS and her experiences at Boston Children’s.

‘You are here and you are okay’

Although Regina is now an expert on HLHS, it isn’t always easy growing up with a serious condition. Last year, Regina had an operation to change the battery of her pacemaker. “Before the operation she was scared and kept asking me, ‘Mama, why do I have to do this, why was I born like this?” Elizabeth says.

“I told her some people might be born with blue eyes, some people might be born with a problem in their tummy, and some people might be born with a problem with their hand. It’s you who was born like this, but we are thankful that you are here and you are okay. That’s what is important.”

Elizabeth believes that the health challenges Regina has faced have helped her become more compassionate and understanding. Whenever Regina sees a person who seems sad, she wants to know what happened and how she can help them.

Determination in the face of HLHS

Regina loves swimming so much Elizabeth calls her “sirena,” or mermaid, in Spanish. When she’s not in the water, Regina is playing tennis, riding horses, or spending time with her friends.

Although Regina is not allowed to play contact sports and knows she needs to rest when she gets tired, she doesn’t let her heart condition limit her. Elizabeth says nothing holds her back, especially when playing sports at school. “I can think of two times when she said she was tired. She sat, rested for a moment, and then got up and continued.”

Recently, at the school’s Aquathlon, Regina brought her mother to tears with her determination. All the students participating in the Aquathlon had to run four laps around the schoolyard, get in the pool and swim four laps, and finish by running another four laps.

Regina did so well, she was in first place for more than half the event. She ran her final four laps to the loud claps and cheers of her classmates, “Regina!” Regina!” Regina!”  

It was a very emotional moment. “She put her all into it, and she did it,” Elizabeth says through tears. “She was tired, but she did it. Other mothers might say ‘Ah, how sad. She swam very well, but she couldn’t run.’ But for me, who has seen what it takes for my child to be able to do these things — to run, to jump, to go to school — it was thrilling. These are all milestones I wasn’t sure she’d ever be able to achieve.”

Elizabeth reminds Regina of that moment when she is struggling or feels frustrated. “When she says she can’t do something, I say ‘Of course you can. If you could run, swim, and run again, then you can achieve anything you set out to do.”

Prioritizing Regina’s care, from Boston to Cancun

Although Regina and her family live in Cancun, far from her team at Boston Children’s, she still visits regularly. Next year, she will return for a fitness test and catheterization.

However, even living in a different country, Elizabeth never feels as though she is far from Regina’s care. She is regularly in contact with nurse practitioner Anne Johnson, whom she calls her “security.” They met Anne eight years ago, when they first arrived in Boston. “If I need anything, if I have any question, I send her a message and she answers me,” Elizabeth says. Sadly, Anne will be retiring to working just two days a week and Regina’s family will be working with a new nurse practitioner. However, Elizabeth knows she’ll still be able to email her whenever she needs.

She feels similar affection for Dr. Marx. “I’m not going to allow Dr. Marx to retire. Maybe once Regina is 30 years old, but before that, definitely not,” Elizabeth jokes.

Learn more about the Single Ventricle Program.