My Child Just Fainted: No Big Deal or Sudden-Death Warning?

Until March 1999, I was a happy mother of two boys, working as a firefighter/EMT-I at an industrial plant in North Dakota. On March 25, I received a phone call at work from the high school, saying that my oldest son, Shannon, was not in school that morning. On checking, my father found Shannon dead in his bed.

In November 1999, EMS Magazine published an article by Alan R. Cowen, MA, EMT-P, about the sudden death in 1989 of a seemingly healthy young lady named Cory Prince. Long QT Syndrome (LQTS), once thought to be a rare disorder, was the culprit. I was referred to the article in 2000, and realized that, coincidentally, at the time the article was written, I was searching for answers to the tragic death of my 17-year-old son.

After Shannon’s death, rumors started to fly in our small town. Suicide and drug overdose were the popular explanations for his death. It was quite obvious at the scene that it was not suicide, and I hoped no one had given him something that could kill him. The North Dakota state forensic medical examiner and I had many discussions during the wait for toxicology reports. He told me he was puzzled and couldn’t see any reason for cardiac arrest. We waited three months for autopsy and toxicology reports to be finalized. The reports said there was no evidence of street drugs in my son and no apparent reason for cardiac arrest. I was told, “You may never find out the cause of death.” In short, there were no answers.

In all my years in EMS, I had been taught, and believed, that you would know if a patient was sick enough to die. At age 17, 6´1" and 190 lbs., Shannon seemed to be perfectly healthy. We had annual physicals; we ate well-balanced meals. What had happened?

In May 1999, when my younger son Dustin, age 13, needed a sports physical to attend summer football camp, I took him to our local doctor, and everything seemed normal. When I suggested that “maybe he should have a baseline ECG,” the doctor told me to bring my son back when he was older. When it was time for football camp, I could not find the copy of the sports physical. As I searched my house, I found a copy of Shannon’s sports physical conducted 13 months prior to his death. The information on it brought me to my knees.

Shannon had checked yes to the following questions:

• Do you experience dizziness during and after exercise?
• Do you experience chest pain during and after exercise?
• Has anyone in your family died of heart problems or sudden death before age 50?
• Do you experience trouble breathing during and after activity?

I called the doctor and, the following morning, took Dustin for an ECG, which showed abnormal T waves that warranted seeing a specialist. We traveled to the Mayo Clinic in Minnesota to see a pediatric cardiologist. My son had an ECG, echocardiogram, chest x-ray and a physician consultation. All tests came back normal. I had brought along a copy of Shannon’s autopsy report and ECG results from my mother, father, sister and myself. Although the doctor said my ECG would be labeled normal by anyone who did not know specifically what to look for, it wasn’t a completely normal ECG. On it were borderline prolonged QT intervals, and the doctor told us they needed to do further testing and draw blood for genetic testing to detect the presence of defective ion channels in the heart.

Michael Ackerman, MD, PhD, conducted the testing, which included an exercise ECG, epinephrine QT stress test, Holter monitoring for 24 hours and a research test called a “dobutamine challenge.” We were among the first LQTS patients at Mayo Clinic to have some of these tests performed and to be clinically diagnosed with LQTS. I was diagnosed based on how my heart behaved during the epinephrine and dobutamine challenges. Dustin was diagnosed and placed on a beta-blocker because of his abnormal epinephrine QT stress test and documentation of a brief episode of nonsustained ventricular tachycardia captured during his 24-hour Holter. I was placed on a beta-blocker by phone after we returned home. We were told “no competitive sports, never swim alone, and always try to have someone with you who knows CPR.” At the time, it was believed that beta-blocker therapy should take care of any LQTS problems.