REGAL EAGLE — These American Bald Eagles, the standard of our nation, were captured on the camera just north of town. A bird of prey, the Eagle was also the standard of the Ancient Romans and many other countries as well. They are known for their strength, size, gracefulness, keen vision and power of flight. Where these were photographed, there was a third Eagle off to the right, but too high on a pine tree to get a good photo. Seems like there are a lot of these birds in our area this year. I-O Photo by Buck Traxler
By Deanna Wakkinen, I-O Reporter
A silent threat waiting patiently inside what is now believed to be one in every 5,000 to 7,000 people in the United States has its genetic make-up in the family of Kathy Hager, longtime Conrad resident.
Long QT syndrome (LQTS) is a rare heart rhythm disorder, often present from birth, which can potentially cause fast, chaotic heartbeats.
These rapid heartbeats may trigger a sudden fainting spell or seizure. In some cases, the heart may beat erratically for so long that is can cause sudden death.
This was the unfortunate case for Marena Steele, Hager’s oldest daughter, who died suddenly in the night less than two years ago at age 39.
Steele leaves behind three children, her two daughters, ages 7 and 10, have since been diagnosed with LQTS as well and have had defibrillators implanted next to their hearts.
Steele was unaware of her later to be found diagnosis but was on the road to discovery slightly before her death; a doctor’s appointment at the Mayo Clinic in Minnesota was just a few weeks away.
Her aunt, and Hager’s sister, Diana Krumm of Billings shared with the Yellowstone Valley Woman that “Marena had been sleeping. She must have known something was wrong. She was trying to get up to get to the door to get help and she died in front of the door.”
Krumm, who said there have been times in her life when she was afraid to fall asleep because her heart was beating so fast, was one of 10 family members who had their heart checked after the loss of Marena. All 10 women received the same diagnosis and had to have defibrillators surgically implanted.
A person can either have acquired LQTS or inherited LQTS. Acquired is genetic and inherited is caused from medication. Those genetically dispositioned have a 50 percent chance of passing the gene on to their children. Once a person is clear, they cannot pass on the gene.
For most people with LQTS, it is likely that the genetic abnormality has been passed on from previous generations of the family. It may affect several family members. Often the syndrome has been undiagnosed, so the frequency with which it appears is unknown. This means that all relatives need to be evaluated carefully for LQTS when the inherited form is diagnosed.
To date, researchers have identified 12 types of genetic defects involved in long QT syndrome. Within those 12 types, researchers have found hundreds of mutations. However, 65 to 75 percent of all inherited LQTS can be explained by defects in the three most common types of long QT syndrome genes, identified as LQT1, LQT2 and LQT3.
Additionally, scientists have been investigating a possible link between sudden infant death syndrome (SIDS) and long QT syndrome. Some researchers believe that approximately 10 to 15 percent of babies with SIDS had a genetic defect or mutation for LQTS.
LQT1 often strikes early in life and comes on through excerise. LQT2 comes through excitement, such as when a person becomes surprised or scared. LQT3, which is the type prevalent in Hager’s family, comes with rest or sleep.
All forms of LQTS involve an abnormal depolarization of the heart which is why Hager’s family now uses defibrillators to deliver electrical shocks, sense the cardiac rhythm and sometimes pace the heart, as needed.
Treatment for LQTS varies depending on the type present but can include medication, medical devices, surgery and life style changes.
While medications won’t cure the condition, they do provide some protection against potentially fatal disruptions of heart rhythm.
Mayo Clinic suggests not overexerting yourself, knowing your symptoms, informing people around you and controlling your environment as precautions.
As Hager reflects back on her life, she re-examines her family history. “My Dad died 24 years ago of leukemia but he had uncles and cousin that all died by the time they were 40 of ‘heart attacks’.” Her twin daughters also come to mind as one died at only six months of age and the other collapsed and died at age 15.
There is an organization whose mission is to save the lives and support the families of children and young adults who are genetically predisposed to sudden death due to heart rhythm abnormalities. The SADS Foundation is dedicated to achieving public awareness, providing patient and family support services and providing advocacy and research to LQTS. You can see more at www.sads.org.
Editors note: Information for this article was found at www.mayoclinic.com.