Wednesday, 6 July 2011

SIDS: Long Q-T Syndrome

DAVID WAHLBERG : June 23, 2011

 CRAIG SCHREINER – State Journal
Doug Bartow, 56, center rear, is pictured with his children and grandchildren at his house in Montfort, west of Madison. All of them, except for Quinn Hoeper, have Long QT Syndrome — a genetic heart condition that can cause sudden death, though medication and avoiding strenuous activities reduces the risk. From left: Sherri Seitz, 30, and her children Mayleigh Seitz, 1, and Rhylinn Seitz, 3, Jonah Hoeper, 5, Quinn Hoeper, 3, and their mother, Amy Hoeper, 32.
When Doug Bartow learned he had a genetic heart condition that can cause sudden death, he had mixed emotions: fear, blame and relief.
He worried about his two daughters and four grandchildren, then felt guilty after discovering he passed the condition to all but one of them.
But knowing the family has Long QT Syndrome — a cause of sudden death in children and young adults, especially athletes — also brought comfort.
Bartow now almost certainly knows what killed his sister at age 14 and brother at age 5. His daughters and their children, ages 1 to 5, can take steps to try to prevent the same fate.
“They have all the information up front so they can make their choice,” said Bartow, 56. “We didn’t have that before.”
Some in the Bartow family are taking medications and avoiding strenuous activities to ward off dangerously irregular heartbeats, and all are being treated at UW Health’s Inherited Arrhythmias Clinic.
The clinic, which started in 2004, treats more than 100 families with hereditary heart diseases. Doctors and genetic counselors help the families navigate the expanding world of genetic medicine, where knowledge of diseases can bring fear and hope, often at the same time.
A serious syndrome
Long QT Syndrome, in which part of the heartbeat lasts too long, can cause dizziness, fainting, seizures and sudden death, especially during exertion. The condition, found in up to 1 in every 2,000 people, explains some of the rare but visible sudden deaths of young athletes, though two other heart problems are more frequently the cause.
Half of people with Long QT Syndrome have potentially dangerous episodes, including 10 percent who die suddenly, said Dr. Craig January, a cardiologist and co-director of the clinic.
The other half don’t have problems. The longer people live without an episode, the less likely they are to have one, said Dr. Kathleen Maginot, a pediatric cardiologist and the other co-director.
The condition is diagnosed through family history, electrocardiograms and genetic testing. In an example of a typical situation, Maginot said, a child faints while running and then the family realizes other relatives fainted or died suddenly.
Some miscarriages and cases of Sudden Infant Death Syndrome can be attributed to Long QT Syndrome, Maginot said.
Most patients take beta blockers, drugs that lower the risk of problems by preventing the heart from beating too fast. Some receive implantable cardioverter defibrillators, or ICDs, devices that deliver electric currents if they detect abnormal heart rhythms.
Patients are told to avoid competitive sports, especially swimming, football and basketball. That can be a big challenge for children, Maginot said. Some parents heed the advice, but others don’t want to give up such an important part of childhood, she said.
“There’s a lot of family dynamics,” Maginot said. “But we’re pretty cautious with children. We’re trying to protect them and give them as close to a normal life as possible.”
Reacting to the news
The Bartow family has had a variety of responses to Long QT Syndrome.
Doug, from Montfort, about 60 miles west of Madison, learned
he had the condition last year during testing for another ailment, for which he had a liver transplant.
After he tested positive for Long QT Syndrome, his daughters — Amy Hoeper, 32, of Livingston, and Sherri Seitz, 30, of Fennimore — learned they have the condition. Each had a 50-50 chance of having it.
Then their kids were tested. Amy’s son, Jonah, 5, has it. Her 3-year-old daughter, Quinn, doesn’t. Both of Sherri’s kids have it: Rhylinn, 3, and Mayleigh, 1.
Amy, who said she was already “that crazy, protective mom,” was worried about Jonah, wondering if the kindergartner should take swimming lessons and participate in physical education class.
She decided those things are OK as long as she is present or lets teachers know about his risk. But she won’t let Jonah play competitive sports and hasn’t let him sleep over at friends’ houses.
“It’s hard to let go of that control,” she said.
Amy and Jonah are on beta blockers. She and her husband Abe decided against having more children because she doesn’t want to risk passing on the condition again.
‘Kids want to be kids’
Sherri, whose children are on beta blockers, isn’t taking the drugs because they can make her asthma worse. She and her husband Jamie plan to have more kids.
She’s not going to restrict her children from any activities, including sports.
“Kids want to be kids,” Sherri said. “I’ve lived with it my whole life.”
Doug, a volunteer for 26 years with the Montfort Fire Department, continues to go on fire calls despite doctors’ advice against it. “They’ll never take that away from me,” he said.
He takes beta blockers and has an ICD. He’s glad to finally know what likely killed his sister while swimming years ago and his brother as he ran up the basement stairs.
Five of Doug’s nine remaining siblings have been tested, and four have the condition. Some of their children and grandchildren have it too.
As Doug sat at his dining table with his family nearby, he touched on the magnitude of complex feelings Long QT Syndrome can bring.
If he learned he had it decades ago, he said, “I know I wouldn’t have had children.”
Then he looked around. “But I’d never give them up now.”

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