Traditionally, sisters share everything. “There isn’t anything we keep from one another,’’ Nicole Fearrington says of herself and her sisters. “We are best friends.’’ She pauses. “Of course, this was something we did not expect — or choose — to share,’’ she says.
By “this,” she means the heart condition that killed their father in 2003, and thus far afflicts four of six sisters, Nicole, 41, LaWanda, 39 , Candice, 34, and Kasi, 27.
In people with the disorder, familial dilated cardiomyopathy, the ventricles (the bottom chambers of the heart) enlarge and weaken, growing unable to pump blood effectively. Untreated, it can lead to heart failure and death.
The disease became critical enough for Nicole that she needed a heart transplant in 2014, and LaWanda had to get a new heart in April.
“This definitely has brought us closer together,’’ says Candice, who lives in Baltimore. (The other three affected sisters live in the Washington area.) “We’ve always been close, but now we are able to share the experiences we have with this disease, and coddle each other when one of us isn’t feeling well. We share laughter, and spiritual guidance. We keep each other going.”
The sisters’ father died of heart failure just before his 47th birthday. He was very private about his health problems and did not divulge medical information about himself, even to his family. But because the six sisters are the children of three different mothers — none with heart problems — their physicians at MedStar Washington Hospital Center’s Heart and Vascular Institute believe the father must have carried the genetic mutation responsible for the disorder and passed it to at least four of his daughters.
“They think our chances [of developing dilated cardiomyopathy] were higher because of what happened to him,’’ Kasi says.“It was hereditary. I think we were just destined to have it.’’
Familial dilated cardiomyopathy can be highly variable in its symptoms and progression.
Nicole and LaWanda took medication for years to try to keep the disease at bay, but the functioning of their hearts grew worse. Kasi and Candice, on the other hand, have been stable with medicine. In fact, Candice’s heart muscle has grown significantly stronger in recent years as a result of the drug treatment, according to Maria Rodrigo, the cardiologist who treats the four sisters.
The other two sisters — Ashley, 30, and Kamaryn, 16 — have shown no signs of the disorder, but they receive regular tests to assess their heart function.
Dilated cardiomyopathy is most commonly diagnosed between ages 20 and 60, and is more common in African Americans than whites, according to the National Heart, Lung, and Blood Institute. An estimated 750,000 people in the United States have dilated cardiomyopathy; about half of these cases are familial, according to the National Library of Medicine.
In addition to family history, risk factors for dilated cardiomyopathy include excessive alcohol use, complications during pregnancy, infections, certain drugs, and high blood pressure and diabetes, according to the American Heart Association.
Most inherited cases of the disease are the result of an autosomal dominant pattern, in which a genetic mutation from one parent is passed on to an offspring.
“This means that individuals with the disease have a 50-50 chance of passing the mutant gene and the disorder to their children,’’ Rodrigo says. “Many patients don’t even realize they have the inherited form of this disease until they start analyzing and looking back at their family history.’’
Someone can also have the genetic mutation and pass it to their children but not become ill themselves, although they do remain at risk of developing the disorder.
Other cases can result from a spontaneous genetic mutation in people with no affected family members who can then pass it to their offspring, according to the National Library of Medicine. This is one reason why some researchers believe that even cases with no apparent cause (known as idiopathic dilated cardiomyopathy) probably still involve a genetic component.
While researchers have found many gene mutations related to dilated cardiomyopathy, they believe more exist. “Our hypothesis is that most idiopathic dilated cardiomyopathy has a genetic basis, but we need” to discover the genes responsible, says Ray Hershberger, director of the Division of Human Genetics at Ohio State University.
One of two studies he is leading involves assembling a registry of patients with dilated cardiomyopathy, including those with idiopathic disease. The plan is to find new genes associated with the disease. Having this information may help identify cases early, when medicine might be able to keep the disease in check.
Family clusters like the Fearringtons’ can provide important data about the disease’s genetics and progression. MedStar Washington Hospital Center, one of the project study sites, plans to begin enrolling participants soon, and the Fearringtons expect to join. At that point, they would undergo genetic testing for the first time, which will help not only researchers but also their own children. “If they found the genes in us, the children would be tested, too,” Nicole says.
In 1997, about six months after giving birth to twins, LaWanda, a case manager for a home health agency, felt extremely tired and short of breath, with her legs swollen from fluid retention. All were symptoms of an enlarged heart unable to properly pump blood. She was the first of the sisters diagnosed with dilated cardiomyopathy, later recognized as familial when her sisters also developed it.
Three years after LaWanda’s diagnosis, Nicole got hers. Candice followed in 2011, and Kasi in 2012.
Because pregnancy can stress an ailing heart, the four sisters have decided against having more children. LaWanda’s twins are 20, Kasi has a 3-year-old, and Candice has a 13-year-old and a 4-year-old. (Nicole has no children.) The children are healthy, but their mothers recognize they too are at risk and plan to monitor them as they age.
Nicole, who is currently job hunting, was grossly overweight when she found out she had cardiomyopathy and was suffering from Type 2 diabetes and hypertension. Her symptoms were similar to LaWanda’s. “I couldn’t lay flat to go to sleep,’’ she says. “Sometimes I had to sit up to sleep; I felt like I was drowning.’’
As her heart deteriorated, doctors told her she needed to lose weight — she reached 325 pounds at one point — before they could consider a heart transplant, she recalls. She had gastric bypass surgery in 2011, and now weighs 145 pounds.
It took a while to recover from her transplant on April 19, 2014, she says, but now “I feel great.’’
In LaWanda’s case, her heart had weakened enough by 2006 that she needed to use a cardiac defibrillator that would shock her heart when its rate started to get unsteady. Last August, with her heart function continuing to deteriorate, doctors also implanted a left ventricular assist device (LVAD) to her heart — a battery-powered mechanical pump to keep it pumping until a donor organ could be found.
LVADs are not easy to live with. The external battery pack and pump controller must be worn around the clock, so LaWanda couldn’t take a bath (or swim) because water can damage the pump. LaWanda named her device Horatio, after a character on the TV show “CSI Miami.”
“Horatio goes where I go,’’ she said about a month before her transplant. “I drive with it. I work with it. I shower with it [using a special bag that keeps it dry]. I sleep with it. Believe me, I’m more than ready to end this relationship. It’s been nice, Horatio, but — no hard feelings — you’ve got to go.’’
Twice, LaWanda had seemed on the verge of a transplant, only to be disappointed. But in April, she got a middle-of-the-night call that her time had come.
At 4:45 a.m. on April 7, to be exact. She awoke to the phone ringing: The transplant coordinator said a heart was available and LaWanda needed to get to the hospital immediately.
Ezequiel Molina, the cardiac surgeon who also performed Nicole’s transplant, spoke with LaWanda as she was being prepped for surgery. “This is a good heart,’’ he told her. “I’m very confident about this. I’m going to take good care of you.’’
Nicole arrived in the afternoon, minutes after LaWanda had been wheeled into the operating room. The nurses brought her back out so Nicole could hug and kiss her. Nicole kept the other sisters apprised of what was going on by phone.
LaWanda was in the OR for 13 hours, Nicole recalls, as surgeons removed the LVAD, replaced LaWanda’s damaged heart with a new one and then stabilized her.
But Molina was right: The donor heart was a good match.
Heart transplants can cost $1 million or more, plus the cost of post-surgical medications, according to the National Foundation for Transplants. Fortunately, LaWanda has good medical insurance through her employer.
In recent years, better technology and drugs are enabling transplant recipients to live longer: About 88 percent survive the first year, and 75 percent survive for five years, according to the National Heart, Lung, and Blood Institute. (One of MedStar’s earliest transplant patients. Thomas Cook, recently celebrated his 27th year with a new heart.)
LaWanda knew that the first few months after surgery would be difficult. Her medication schedule is complicated, and she was briefly rehospitalized because of side effects from the anti-rejection drugs she will need to take for life. Butit helps that she saw Nicole go through it first — and that Nicole today is vibrant and healthy.
“It is a daunting process,’’ LaWanda says. “But I am feeling seriously grateful, and really looking forward to the life ahead of me.’’
Candice and Kasi, meanwhile, hope that researchers and physicians will learn more about their faulty family genes and are optimistic that they, their other sisters and their offspring will have an easier time than Nicole and Lawanda. “I hope with proper medication and a healthier lifestyle I won’t have to undergo a transplant,” says Kasi, an office manager.
Candice adds: “I want to see my children go off to college. I want to see my grandchildren. I don’t think my time is done, and I don’t believe the time is done for my sisters, either.”