Dorien “DJ” Garnett, a 17-year-old junior from Brockton High School in Massachusetts, had been a heathy sports star all his life, but while playing basketball one day in 2009, he suffered sudden cardiac arrest.
“He clutched his chest and just dropped,” said DJ’s mother, Carmina Taylor of Penllyn, Pennsylvania. She got the heartbreaking news from her ex-husband and her son, who had witnessed DJ’s death on the court.
As schools open across the country, an estimated 2,000 Americans under the age of 25 can be expected to die of sudden cardiac arrest in the coming year, according to the Centers for Disease Control and Prevention. It is the leading cause of death on school property.
DJ’s autopsy revealed that he had hypertrophic cardiomyopathy (HCM), an enlargement of the heart muscle that is the leading cause of sudden death among athletes. And his family is helping lead a charge to get other at-risk kids tested — before it's too late.
Tests showed DJ's father carried a gene for the disease. And although his mother was herself a heart disease educator, she had never heard of cardiomyopathy until her son died.
“The irony was DJ was a very loving person who died of an enlarged heart — he had a humongous heart,” Taylor, 42, told NBC News.
Taylor is speaking out on behalf of the Children’s Cardiomyopathy Foundation during September to educate other parents about the pediatric form of this lethal disease, which, if detected early, can be successfully treated.
Cardiomyopathy has five forms and affects about 30,000 children in the United States, according to the Children’s Cardiomyopathy Foundation. Eventually the heart loses its ability to pump blood and heart failure or dangerously irregular arrhythmias may occur.
CCF is the only organization that advocates exclusively for pediatric cardiomyopathy. It was founded by New Jersey mother Lisa Yue, who lost two boys to the disease: 11-month-old Bryan died in her arms in 1999 and 9-month-old Kevin succumbed waiting for a heart transplant in 2001.
“We don’t want to alarm families — it’s still rare,” Yue told NBC News. “Sudden cardiac death doesn’t happen every day, but it is a possibility and 25 percent of the cases occur during sports.”
“Our goal is to raise awareness about the signs and symptoms and risk factors so we can help identify more of these undiagnosed children and get them the proper medicine and attend to their needs so they don’t become victims,” she said.
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Although not routinely offered, an EKG or echocardiogram can identify cardiomyopathy. Symptoms include:
Shortness of breath
Cardiomyopathy can also strike in infancy. Recent studies funded by CCF show that children are 10 times more likely to develop the disease during the first year of life than ages 2 to 18 combined. Infants may experience poor weight gain, difficulty breathing, excessive sweating or lethargy.
Lucy Blythe Farmer of Austin, Texas, died of dilated cardiomyopathy just three months after her first birthday on Mother’s Day in 2013 after her parents noticed her fast breathing. She died of ventricular tachycardia while waiting for a heart transplant.
Anna Grace Kaelin, a 5-year-old from Sellersburg, Indiana, was diagnosed with the disease last year and has received a Berlin Heart as she awaits a transplant. The artificial heart cannot be unplugged for more than 25 minutes at a time.
Yue said that it is also important for families to review their cardiac health history, identifying anyone in the family who had heart disease and died before the age of 50.
“Asking those questions can be little red flags to investigate further,” she said. “Many times a child is diagnosed first, and then you find out other family members have had the disease, but they didn’t say it was cardiomyopathy.”
Older children who participate in competitive sports are also at higher risk for sudden death if they have underlying disease. Yue’s foundation is developing standardized questions that might be incorporated into pediatric and school medical exams.
“It’s not going to catch all cases, but it’s a start,” she said. “If we can save one life, we have done our job.”
CCF has also introduced legislation to protect children against sudden cardiac death, including such measures as requiring automatic external defibrillators (AEDs) in the schools and requiring staff to know where these life-saving devices are located and how to use them.
Cardiomyopathy is an insidious disease, according to Dr. Steven E. Lipshultz, pediatrician-in-chief at the Children’s Hospital of Michigan and chair of pediatrics at Wayne State School of Medicine.
“Children can be asymptomatic and then have an event where they pass out or need to be resuscitated from a dangerous heart rhythm,” he told NBC News. “For many the first event is the last event — it’s fatal.”
“Sometimes in the family when you put a pedigree together and see four people over two or three generations who had sudden death under 50, you can start to understand there is a genetic predisposition and how potentially dangerous it is.”
Treatments may include medications like statins, beta blockers and calcium channel blockers; surgery to burn away the thick part of the heart muscle; and implanted defibrillator devices.
Those with HCM, the second most common form of the disease after dilated cardiomyopathy, should not compete in sports, he said.
“So much more needs to be done to raise awareness about the disease. It’s a silent killer."
Children at risk should have an echocardiogram to see if the heart muscle is enlarged. That must be repeated every five years until adulthood and is not always conclusive. Many doctors do not recommend genetic testing because of its complexity — there are more than 1,000 genes associated with cardiomyopathy.
Although pediatric screening for the disease is not required in the United States, Lipshultz says some parents may want to consider testing, “if you have a fair amount of anxiety or a history of sudden death arrhythmias.”
“If you don’t look, you won’t know,” he said. “It’s not an insurance policy, but it does give you some assurance."
As for Carmina Taylor, she said she wished she had known DJ was at risk for sudden death. But she now looks at her son as a “hero” for helping to alert other parents about pediatric cardiomyopathy.
Luckily, she said, DJ’s 20-year-old brother Taylor has been tested and does not have the disease, but he had to deal with “the loss of his best friend.”
“So much more needs to be done to raise awareness about the disease,” she said. “It’s a silent killer."