Allyson Fowler, 7, returned home from Hopkins Children’s last Monday a few pounds lighter and with a new heart. She brought with her some saline syringes, the better to squirt the unsuspecting, as she had lab technicians and doctors at Hopkins.
“It was my nurses’ idea,” she says innocently of her small (and needle-free) arsenal of improvised-squirt guns. “They gave them to me to take home.”
Delight in a little payback is understandable in the young Kent Island, Md., resident who, since her first complaints of feeling unwell in mid June, had been hospitalized and diagnosed with an untreatable heart disease, gone into heart failure, been placed on an artificial heart on July 19, and then, on July 24, undergone a heart transplant.
After a breakfast of pancakes, sausage, eggs and cereal, on August 2, she was ready for the traditional ride in a wheelchair out of the hospital for the trip back home, where her brother Tyler, 9, and dog Sebastian, also known as “SeaBass,” awaited her.
“I’m happy to be going home,” she said, as Bev Fowler brushed out the braids her daughter had worn during her hospital stay, “but a little scared.”
Who wouldn’t have some trepidation after such a sudden catapult into a critical illness? Allyson’s odyssey from the everyday certainties of a healthy, active childhood to a second chance at life itself began in mid-June when she told her parents that her stomach hurt. The pain would come and go, but appeared to be worse at night. Worried that she might have appendicitis, they took her to the local emergency room. After physicians there found no appendicitis but a heart murmur, they drove her across the Chesapeake Bay Bridge to Baltimore and the emergency room at Hopkins Children’s. There, pediatric cardiologist Janet Scheel and a team of specialists began investigating the cause of Allyson’s symptoms.
“Her heart is enlarged, all four valves were leaking, fluid is backing onto her lungs and liver and there is minimal heart function at the moment,” Fowler wrote in her blog on the Caring Bridge site the next day. “They were thinking this was a virus that led to an infection but all of the blood tests that they have run have come back completely normal. Not typical for myocarditis cases.”
At Hopkins, she continued, they were “receiving the best possible care and attention.”
A heart biopsy at Hopkins Children’s Center showed that Allyson had significant fibrosis or ‘scarring’ of her heart muscle, a result perhaps of a previous viral infection or a genetic mutation. As is the case in 75 percent of children, the cause will likely never be determined.
“The presence of the ‘scarring’ meant that there was little hope that Ally would recover enough heart function to return to the activities of a normal life, let alone return to the active lifestyle of crabbing with her father and playing with her dog, which she so enjoyed before her illness,” says Scheel, director of the Pediatric Cardiac Transplant Service at Hopkins Children’s.
Allyson was diagnosed with severe dilated cardiomyopathy, a rare disease of the heart muscle and the leading cause of sudden deaths and heart transplants in children. It advances quickly and relentlessly, usually without any warning. With this disease, the heart muscle stops functioning normally and the heart dilates in an attempt to compensate, until it can no longer supply sufficient blood flow to all the vital organs. “This is what happened when Ally presented to the emergency room,” Scheel adds.
At Hopkins Children’s, the child was listed for a heart transplant and placed on a continuous intravenous infusion of medicine to help maintain an adequate flow of her blood until a new heart could be found. Weeks later, she went into complete heart failure, despite the medication, and was placed on a Berlin heart, a mechanical device that would pump her blood through her body, keeping her alive while she waited for a donor heart.
On Saturday, July 24, a perfect match was found. Luca Vricella, director of Pediatric Cardiac Surgery and Heart Transplantation at Johns Hopkins, led the child’s transplant surgery.
While there is no known cure or treatment to prevent or eliminate cardiomyopathy, which was formally recognized as a disease in the 1950’s, Johns Hopkins is leading research to understand the genes involved and why the disease progresses so quickly in children. In its Helen B. Taussig Congenital Heart Center this includes studies to understand the fundamental basis of cellular flux in heart muscle cells and how it relates to the development of heart abnormalities, as well a hunt for biomarkers that could lead to the development of a screening test for the disease. Learn more about related research.
In the pediatric intensive care unit after surgery, Allyson began to recover.She squirted the unsuspecting with water, played hospital bingo and welcomed visits from the hospital clowns. Then, she was moved out of critical care to a general unit. When her best friend, Carmen, stopped by, they sat together on Allyson’s hospital bed and worked on a puzzle. With physical therapy, she was soon back up and walking. Then, it was time to go home.
Throughout their daughter’s 45 days in the hospital, Fowler and her husband commuted from Kent Island to be with their son, too. “It is remarkable that when you have a sick child, life goes on, the world doesn’t stop, in particularly when you’ve another child at home,” she says. “You adjust your schedules, put work on hold and hold tight onto what you have.”
The outpouring of support not only at Hopkins but from the community at large has been tremendous, Fowler continues. “We can’t thank everyone enough for their incredible support. It means more than you’ll ever know,” she writes to well-wishers, friends and family on her blog.
Touched by her daughter’s story, “at least 20 people have told me they now want to change their status to become organ donors, now that they see what it has meant for Ally,” she adds. “That’s a wonderful thing.”
Follow Ally’s journey and care at Hopkins Children’s, and her progress at home on Bev Fowler's blog.