Hypoplastic left heart syndrome (HLHS) pediatric patient stories

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Avery Toole's Gift of Life

The day after Avery Toole was born, an echocardiogram revealed that she had a serious and rare heart defect called hypoplastic left heart syndrome. Her mother, Cheryl, is a nurse in the Neonatal Intensive Care Unit at Boston Children's Hospital knew her only chance for survival was immediate open-heart surgery.

Avery needed immediate open-heart surgery at Boston Children's, where her mother, Cheryl Toole, RN, MS, is a nurse manager. The newborn underwent the delicate procedure at just 5 days old, recovering well. Avery's parents were thrilled to bring their baby home, though they knew she had a long road ahead. Treating HLHS requires a series of surgeries. Avery had her second surgery at 6 months, followed by nine other operations, including leaky valve repairs.

By March, 2009, repairs could no longer save Avery's heart—she needed a new one. Doctors placed the 5-year-old on a Berlin Heart, a mechanical device that does the heart's work, while she waited for a donor. Avery spent 54 days on the Berlin Heart, struggling to gain strength for surgery.

Avery's life was saved by the heart that was donated by an amazingly generous family from Texas who had just lost their child in a car accident.

My baby’s congenital heart defect

By Casey BoltonParker patient HLHS

During a routine ultrasound I had in the 20th week of my pregnancy, I learned that my soon-to-be-born son, Parker, would be born with a complex congenital heart defect (CHD) called hypoplastic left heart syndrome (HLHS).

Parker couldn’t be born in our home state of New Hampshire because none of the local hospitals were equipped to handle his CHD, so we scheduled an induced birth in Boston, and almost immediately after birth Parker was brought to Children’s Hospital Boston’s Cardiac Intensive Care Unit (CICU).

Parker’s first 24 hours of life were so unstable that doctors weren’t sure he’d make it. His condition was so severe that even as his parents we weren’t allowed to see him in the cardiac ICU until they could stabilize him with an emergency catheterization.

Parker had to undergo his first heart intervention at just a few hours old. Then, at only seven days old, he underwent his first open heart surgery. His chest remained open after the surgery because his heart was so swollen; finally it was closed on Christmas Day. We spent that Christmas hoping for the best, but he was not adjusting well to the change.

Due to Parker’s condition, doctors said he required either three open heart surgeries or a full heart transplant. We choose the three surgeries because Children’s had a reputation of successfully performing surgery in that way.

Originally, Parker was supposed to come home between the first and second stage of his heart operations, but unfortunately he didn’t do well after the first surgery and needed to remain hospitalized with the support of the hospital equipment and medicine in order to survive to the second stage.

Parker had the second open heart surgery on March 3, 2008. This was one of the earliest stage II surgeries performed by cardiac surgeon Dr. Sitaram Emani.

Thanks to everyone at Children’s, after nearly 15 weeks in the hospital, Parker was finally discharged and able to come home for the first time in his life.

Parker is now home and he receives eight different medications, 16 times throughout the day. His next open heart surgery will be sometime between Thanksgiving and Christmas of this year. Eventually, Parker may need a heart transplant, but we are hopeful this isn’t for years to come.

Because of Parker’s condition we have learned to cherish each and every moment. Life is no longer about milestones, but about the moments. The difference between good days and bad days no longer seems so important; we are just thankful for the days. We are forever grateful for the first-class care our son has received at Boston Children’s; we truly felt he couldn’t have been in better hands.

The entire staff was amazing, and the dedication I felt from Dr. Sitaram Emani and Dr. David Brown goes beyond words. We were at Boston Children’s for 95 straight days and I felt like they were always available to us for assistance, guidance or to answer our questions. They never made me feel rushed or that my concerns weren’t valid.

Seeing their dedication to and passion for helping children made me want to make sure all future parents of children with heart conditions are aware of the amazing care their baby can receive from Boston Children's Hospital.

When I first received Parker’s diagnosis of HLHS, I had no idea what a congenital heart defect was, never mind what HLHS was. When I left the doctor’s office that morning in tears, the only thing I had was a tissue with hypoplastic left heart syndrome written on it.

Through our personal journey I’ve learned there just isn’t enough awareness or funding for research for CHDs. I’m amazed that the cardiologist we first saw in New Hampshire who diagnosed my baby couldn’t provide any information about his condition. I think most of my fears stemmed from the lack of education on the CHD and fear of the unknown.

As a result I have made it a life goal to raise awareness for CHDs so other expectant parents aren’t as lost as I was. I’ve contacted many people, medical professionals and organizations in attempts to open the lines of communication about the dangers CHD.

Thanks to work I’ve done with New Hampshire Governor John Lynch, this past February 14 was declared CHD Awareness Day in my home state. Currently, I’m working with New Hampshire State Representative Lynne Blankenbeker on having a pulse oximetry test (which could help detect signs of CHD in babies and save lives) added to medical screenings of all newborns born in New Hampshire.

While I’m glad to have the opportunity to be a part of the fight against CHD, I am most thankful for the work and dedication of Dr. Emani, Dr. David Brown and the entire staff at Boston Children's Hospital. They have given my son a chance at life, and I could never fully repay them—I am truly blessed to have Parker and my thanks go out to Boston Children’s for keeping him in my life.

About Hannah

Hannah Englert was born with hypoplastic left heart syndrome (HLHS), a rare and serious congenital heart defect. Her parents knew about her condition when Hannah’s mom, Denyse, was pregnant with Hannah, so they arranged to temporarily leave their Louisiana home so Denyse could have the baby at a hospital near Boston Children’s Hospital. That way, Hannah could be immediately transferred to Children's for treatment.

Hannah patient HLHS

At just two days old, Hannah had her first open heart surgery at Boston Children’s. Over the next few years, the Englerts flew back to Boston Children’s for Hannah’s follow-up surgeries, sometimes staying for weeks or months at a time. Last September, they arrived for her third—and hopefully final—heart surgery. Since the procedure was complex, Hannah has been slow to bounce back, but “her little personality is getting close to what it was at home,” says Denyse.

Despite the hardships caused by Hannah’s condition and treatment and spending so much time away from their Louisiana home, the Englerts have used Hannah’s health crisis to help others. They’ve partnered with the Louisiana Pediatric Cardiology Foundation to form a support network for parents of children with HLHS. “I want to help as much as I can,” Denyse says.

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