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Familial Cardio-myopathy Initiative

One in three patients affected with unexplained cardiomyopathy has an inherited form of the disease. Hopkins’ Familial Cardiomyopathy Initiative conducts research, screens patients and maintains a registry of people with the condition. Info: Nicole Johnson, genetic counselor, 410-502-2578; hopkinsmedicine.org/
cardiology/heart/
familial_cardio-myopathy_center.htm

 

A Heart Full of Thanks

A grateful patient preaches the gospel of lifestyle changes to fellow parishioners


Like families everywhere, the Williams clan, above, will celebrate Thanksgiving this month. But for James Williams, center, every day since last spring has been a day for giving thanks.

James Williams is a convert with a mission. And when he shares his story at church, people pay attention.

“Different ones walk up to me and say, ‘I never knew that,’” says Williams, a machine operator for a computer hardware company in Hunt Valley.

But Williams doesn’t preach religion. Ever since he was given the news that he has a disease of the heart muscle, or cardiomyopathy, he has become a model patient. And he is consumed with spreading the word about the importance of lifestyle changes for people like him with congestive heart failure.

Last winter, Williams had a cough, and he was prescribed an inhaler to help him breath easier. When he didn’t get better, his doctor wondered if the chemicals Williams was exposed to on his job might be to blame. On a weekend in late March, he became so short of breath and lightheaded that he was admitted to Good Samaritan Hospital. He was stunned to learn that what he assumed was a bad case of the flu was something much more serious.

“They explained to me that only 22 percent of the heart was working and that I might need a heart transplant,” says Williams, 41, the father of five. “Then they sent me to Johns Hopkins.”

Although Williams and his family were reeling from the news, for Hopkins cardiologist Daniel Judge, Williams was a straightforward case. Judge followed the standard approach for new onset of unexplained cardiomyopathy—he cleared out the extra fluid causing congestion and put Williams on standard medications. Then he delved into Williams’ medical history.

Williams’ father had, in fact, died at a young age. That put Williams at risk for having inherited his condition, called dilated cardiomyopathy, in which the heart becomes enlarged and is unable to pump blood efficiently around the body. Judge recommended that Williams’ children and siblings be screened for the condition, which causes 10,000 deaths each year.

It took just two days for Judge to repeat some tests and bring Williams’ symptoms under control. Still, the family was bracing for bad news. “I remember precisely that [Dr. Judge] said, ‘We’re gonna be letting you go,’” recalls Williams. “I thought he meant that was it for me, they did all they could do.” Instead, Judge announced he was releasing Williams back to his referring physician and that he wouldn’t need a heart transplant unless his symptoms worsened.

Although there is no treatment for cardiomyopathies, that doesn’t mean patients can’t learn to live with the disease. Before Williams left the hospital, he and his wife had an hour-long consultation with Amanda Ryan, clinical dietician specialist with the Department of Nutrition. Ryan taught them the importance of a low-salt diet. Using actual food labels from the grocery store, she compared “low sodium” with “reduced sodium,” labels, she says, “that can trick you.”

Williams has taken every recommendation seriously. He is sticking to 2,000 mg of sodium a day and exercising by walking regularly around his Rosedale neighborhood. All of the Williams children were screened and came out disease-free.

Now Williams feels he wants to give something back. He has taken on the role of educator to “the older gentlemen with heart conditions” in his congregation. “They were slack,” he says. “They’d miss their medications, not eat properly, still mess with salt. I tell them, Even if you feel good, you must take your medicines properly for them to work as they should.”

He wants to write a book to share his story, his wife’s recipes, and the literature he’s discovered to help him. “Even if something like this comes upon you, if you do as modern medicine dictates, you can survive this. There’s life after this!”

- MEM


 

 

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