IN THIS ISSUE
Home | Contact Us | About Us | 
Subscribe
From the Spring 2005 Issue
Patients Talk
Mother and Daughter Receive Defibrillators for Heart Condition
For years Karla Aaland thought that people in her family simply didn't have a long lifespan. Her mother died when Karla was 8 years old, and four other close relatives also died fairly young.
However, in 2000 Karla found out that she and others in her family have a genetic condition called long QT syndrome. It was a relief to find out, but upsetting to know that she might have passed it on to her two daughters. Laura, age 16, does not have long QT. But Jenna, 19, does.
Long QT syndrome is an inherited electrical disorder of the heart (Figure 1). It can cause rapid heartbeats (arrhythmias), which may make the person faint. If dangerous arrhythmias occur, sudden cardiac death could result. Without immediate treatment, a person can die within minutes.
Sometimes patients take medications called beta blockers to help prevent arrhythmias. But if arrhythmias occur anyway, the medications can't restore the heart's normal rhythm. Other patients receive an implantable cardioverter defibrillator (ICD), which can stop arrhythmias and restore a normal heartbeat. Some patients may be treated with both beta blockers and an ICD.
Karla, 42, lives in Fargo, North Dakota, with her husband, David, and Laura. Jenna, a freshman at the University of Minnesota, lives in Minneapolis. Karla and Jenna shared with LifeBeat Online some of their experiences.
How did you learn that you had long QT syndrome?
KARLA: Five years ago my Aunt Sharon died at age 50. Her doctor started asking questions and found out that most of her siblings also died quite young. He got in touch with a long QT specialist in Rochester, Minnesota, who said that we should all see a cardiologist and be tested for long QT.
JENNA: I was tested after my mom found out she had long QT. Both the DNA blood test and the ECG [electrocardiogram] pointed to long QT.
Have either of you ever had a blackout?
KARLA: Yes, twice I've passed out in the water—once when swimming and once when snorkeling. Luckily people saw me go down both times and rescued me. I've since learned that many times people with long QT pass out in water. So my snorkeling days are over.
JENNA: Unlike my mom, I haven't passed out.
How many family members do you think might have had long QT?
KARLA: Four aunts and uncles died while still in their 20s to mid-50s, and my mother died at age 35. She actually died at my Aunt Sharon's wedding. At the time the doctors, at a loss to explain her death, said it was from heat exhaustion. But clearly it was long QT. People with this disorder usually die at young ages—from the teen years to middle age. And the arrhythmias are often triggered by excitement, a medication, or a loud noise like an alarm or a door banging.
What were your treatment options?
KARLA: You can take beta blockers or get an ICD [implantable cardioverter defibrillator]. I tried the beta blockers first but they made me feel drowsy and I couldn't even hold thoughts together. At the time I was president of a manufacturing company, I was getting an advanced business degree, and I was raising a family. I really had to be able to think clearly! [Editor's note: Some patients may be treated with both beta blockers and an ICD.]
JENNA: Knowing what my mom had gone through, I didn't want to try the medications. Plus I was only 14 at the time, and I didn't want to take pills for the rest of my life.
How did you both decide on an ICD?
KARLA: I was never hesitant about getting a device. It was a bigger concern for Jenna, since she had proms and summers in swimsuits ahead of her.
JENNA: I had second thoughts about it. Do I want this bump in my chest? What's it going to be like at dances? Is everyone going to stare at my shoulder? At that age I was self-conscious. But looking at the long term, I thought 'Drugs like beta blockers can't prevent sudden cardiac death, but the device can.'
KARLA: Our doctor at Mayo Clinic showed us the ICD and said it was cutting-edge technology from Guidant. The device was very small. The procedure would take about an hour and we'd be home the next day. So we were implanted on the same day, one right after the other.
What was your recovery like?
KARLA: There was a little soreness where the incision was for maybe 5 days. Then we were back to normal. Getting an ICD has meant zero limitations for me.
JENNA: I was in pain for about a week. And I couldn't lift anything over 5 pounds for a while. But that meant I didn't have to do grocery shopping—that was nice! I also found that I didn't need to worry about dances and that sort of thing. People supported me rather than making fun of me. And they told me they couldn't even really see the device. It is pretty hard to see unless I pull my shoulders way back. Then you can see its shape.
What has this experience meant for you?
KARLA: Having long QT has changed my life by helping me find balance again. In 2003 I quit my high-pressure job and took nearly a year off to be with the girls before they left home. Now I am executive director of the Fargo-Moorhead Area Foundation. We give grants to nonprofits and scholarships to students. So I've tried to put my business skills to work for our community.
JENNA: With the long QT I can't participate in contact sports. I had to stop playing basketball in high school, and I was upset about that for a while. But I found other ways to be involved. I managed the girls' basketball team and was an athletic trainer. Plus I continued to play golf.
All of this changed my focus on life. I realized that I can be happy with what I'm able to do instead of thinking about what I can't do. And I'm thankful for every day that I wake up in the morning.
I've also decided to major in biomedical engineering. I want to be able to make an impact on others, like the people who helped me.
KARLA: As for the device, it gives me peace of mind. People ask if I'm afraid the ICD will go off [deliver therapy]. That's the last thing on my mind. If it goes off I'm glad, because I probably needed it. Now I don't even think about the fact that I have long QT. The entire experience has helped me see that every day can be a gift.
Note: Individual symptoms, situations, and circumstances may vary. Please consult your physician or qualified health provider regarding your condition and appropriate medical treatment or before beginning an exercise program. The information provided is not intended to be used for medical diagnosis or treatment or as a substitute for professional medical advice.