Claudia Irizarry was just five months pregnant when a routine ultrasound revealed that her unborn daughter, Angela, had a life-threatening heart defect that left Angela’s heart with only one functioning chamber to pump blood.
Babies born with this serious condition usually live just a short time unless their walnut-sized hearts undergo immediate reconstruction. For Angela, that meant open heart surgery to implant a synthetic blood vessel to help her heart deliver blood more efficiently to her lungs. While the surgery was successful, it wouldn’t be Angela’s last. Because her synthetic graft wouldn’t grow with her, each year, Angela would need open heart surgery to replace the graft. With the surgeries also came the risk for infection – and rejection, a problem that could occur if Angela’s body reacted to the plastic in the synthetic graft.
Claudia says that while it was difficult knowing that her daughter would need so many risky procedures, as Angela grew into an active toddler who loved to run and play, it was obvious that her life depended on them.
“She would get tired easily. If she was crying or being active, her lips would turn purple or blue from not having enough oxygen,” recalls Claudia.
“So it would be another year, another surgery.”
At age three, when Angela was getting ready to have her third graft surgery, clinician-researchers Christopher Breuer, MD and Toshiharu Shinoka, MD offered the Irizarrys another option – an experimental procedure to use a bioengineered blood vessel, made from Angela’s own cells, instead of the plastic graft.
“We went online and read about the research they had been doing, and saw that this could help Angela avoid future surgeries and potentially reduce her risk for infection,” said Claudia. “This looked like her best chance.”
At age three and a half, Angela became the first person in the United States to receive a tissue-engineered blood vessel, marking a historic moment for the field of regenerative medicine. The graft was created by infusing a biodegradable scaffold, shaped like a blood vessel, with Angela’s own cells. After a few weeks inside her body, the scaffold melted away, leaving behind a perfectly functioning vessel that can grow with Angela.
Angela also became the first child to enroll in a five-year study to follow her progress, opening the door for several other children with organ defects who could benefit from tissue-engineered vessels.
“I always told Angela that she is special because she has these problems in her heart, but now she's more special because she's helping another kid,” says Claudia. “She may not have a normal heart, but she has a big heart.”
Today, six-year old Angela’s ‘big heart’ is pumping strong and telling her to be a firefighter. And a doctor. When she isn’t busy pedaling around on her bike with her big brother Alexander, she’s dancing, drawing and writing. She recently took up gymnastics, and loves school. Doctors expect her – and her grafted vessel – to continue to grow and thrive.
Even though Angela’s family is based in Pennsylvania, they make regular check-up trips to Nationwide Children’s to see Dr. Breuer and Dr. Shinoka and their team. For the Irizarrys, the trip is a labor of love – not just for Angela – but for the physicians and researchers they feel changed their daughter’s life.
“We love the hospital. From the doctors, to the nurses, to the people in the cafeteria. They are angels and they are friends,” says Claudia. “They helped our child and they want to help everyone’s child – not just in Ohio, but kids all over the world. I feel so lucky to have them in our life.”
Led by Dr. Breuer and Dr. Shinoka, Nationwide Children’s Tissue Engineering Program, is developing some of the nation’s most innovative technologies in regenerative medicine today. The expertise that created a functioning blood vessel for Angela’s heart is bringing that same advanced knowledge to children with other organ defects that can be treated or potentially cured using tissue-engineered implants.