Finding out your child has congenital heart disease (CHD) can send you on an emotional roller coaster. “You can’t help but think, is this my fault? What did I do wrong?” says Jessica Nigrelli, whose daughter Avery was diagnosed with CHD when she was 16 months old.
When Avery was a baby, she had an on-again, off-again heart murmur that was checked every three months. When the murmur persisted at 16 months, her primary care doctor recommended she see a cardiologist from Boston Children’s Hospital. At the Heart Center’s outpatient clinic in Waltham, Dr. Susan Saleeb discovered Avery’s atrial septal defect (ASD). An ASD is a hole in the wall that separates the heart’s upper two chambers, the left and right atria.
The diagnosis shocked Jessica and spurred a great deal of anxiety. “Avery appeared healthy. Looking at her, you would never know anything was wrong,” Jessica says. The word ‘defect’ just sets off a million bells and alarms in your head. Processing that took some time.”
Monday through Saturday, Kyle Cooper wakes up at 5:40 in the morning to get to his construction job by 7:00. On his only day off, he shoots trap at the local sportsman’s club with his grandfather. Things that would bother a typical teen—a long commute, arduous work, little time off for friends—barely faze Kyle. This 18-year-old has the quiet confidence and patience of someone twice his age.
Kyle’s demeanor may be due in part to having had to wait a lifetime for something he wanted so badly. He was born with hemifacial microsomia (HFM), a craniofacial anomaly that resulted in the left side of his face being underdeveloped. The cause of HFM is not well understood. Until this past February, what HFM meant for Kyle was that his face was noticeably uneven and barely any of his teeth touched. “I made it through and got used to it, but I couldn’t eat things like meat because it would take me three hours to chew.”
“People would say ‘I feel bad for her’…I’m the same person I ever was, but better, stronger,” says 14-year-old Shannon Curley, reflecting on the time she spent in isolation treatment at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. “I wouldn’t change anything. I’ve met so many amazing people and learned some important things.”
In December 2012, Shannon, a middle school basketball star and Billerica native, was diagnosed with acute bilineal leukemia. Due to the rarity and nature of her leukemia, doctors recommended an aggressive treatment plan. After three months of treatment, Shannon received a lifesaving bone marrow transplant and was put on a recovery plan that included a year in isolation to build up her immune system.
“After she got out of the hospital, Shannon had to go into a year of outpatient isolation treatment. Her immune system had been suppressed so her body would not reject the bone marrow, and that made her susceptible to infection,” explains Shannon’s mother, Carol Curley. “Although we were at Dana-Farber regularly, we’re lucky that she could go through isolation treatment at home and that she was able to make friends on the isolation floor during her visits to Dana-Farber.”
While in confinement Shannon kept a positive attitude and even offered support to other patients.
James Danna enters the Boston Children’s Hospital Cardiac Intensive Care Unit (CICU) with the tools he’ll use to treat Joy, a 9-month-old patient recovering from open-heart surgery. Instead of a stethoscope or scalpel, James carries only small percussion instruments and a guitar.
He gently opens the door to Joy’s room, taking a quick read of her heart rate—138. Joy is a tiny little thing in a great big bed, under bright lights and tethered to multiple machines. Over the course of her multiple procedures for a congenital heart defect, the noise of the monitors, air conditioning and loudspeakers have made for a very wary baby. Her medical chart describes Joy as “staff phobic,” as most adults who enter her room poke and prick her.
But Joy has met James many times before and knows him to be safe. She locks her eyes on him and waits for the music to begin. Keeping his distance, James quietly hums a tune while strumming a few chords on his guitar. “The music I play for Joy is soft like silk, a sensory blanket to swaddle and soothe her.”
Joy smiles, crosses her little legs and nods to the beat.