Where do kids with rare diseases, like Isabella, find hope when the odds are stacked against them?
Isabella weighed just one pound, four ounces when she was born three months early at a nearby Boston hospital. She struggled to breathe and feed, but fought to survive.
She first visited Boston Children’s at 39 days old, for an innovative procedure to patch a hole in her heart with a mesh device. But the cardiac specialist discovered another hole in her heart.
Tests revealed that Isabella had a rare, life-threatening condition, Pulmonary Vein Stenosis (PVS). Cells in her veins blocked blood and oxygen flow from her lungs to the heart. The worst part: when surgeons clear the obstructions, the cells quickly grow back—sometimes in a matter of weeks.
A revolutionary solution
Ten years ago, a PVS diagnosis was terminal, but thanks to trailblazing research at Boston Children’s, Isabella had a chance.
A team of heart, lung and cancer experts at Boston Children’s worked together to identify two chemotherapy medications that are safe for young children. The drugs slow cell regrowth and give kids and their veins time to grow so blockages are less likely to recur. This approach—only used at Boston Children’s—boosted the survival rate from 0% to 80% for children at five years old.
Before 5-month-old Isabella could start treatment, she spent three weeks in a medically induced coma to gain strength for open-heart surgery.
“It was so hard seeing her like that," says Kimberly. "But the nurses and the PVS care team never let us lose hope.”
Two days after surgery, when Isabella opened her eyes, everyone was overjoyed.
“The nurses and the PVS care team never let us lose hope.”
Kimberly, Isabella’s mom
Cherishing every milestone
Over the next months, as the medication took effect, Isabella got a tracheotomy tube to aid breathing and a feeding tube for nutrition. By the time she was eight months old, she had become a chubby, happy baby that smiled and giggled when music therapists visited.
Before leaving the hospital, Isabella’s mom, dad and grandmother were trained in the simulator lab to confidently meet her complex needs. Finally, at 10 months old, Isabella went home with her family.
Now 2, Isabella no longer needs medication. She just started walking and celebrated her birthday at the zoo. Her family cherishes every milestone she reaches and continues to work closely with the PVS team. They are forever grateful for what they found at Boston Children’s: lifesaving answers and hope.
