Inherited Arrhythmias Clinic: The Bartows
For families such as the Bartows, above, UW Health's Inherited Arrhythmias Clinic provides them with hope not only through the clinical care they receive, but also through the research being conducted on their condition.
A Heart Condition that Crosses Generations
When Doug Bartow of Montfort, Wisconsin, became unexpectedly ill last year, he had enough to worry about as he awaited a life-saving liver transplant.
Little did he know this illness would lead to another diagnosis, one that would affect not only him, but his daughters, and grandchildren, too.
During a pre-transplant screening, doctors discovered that Doug has Long QT Syndrome (LQTS), a serious heart condition that can result in sudden cardiac death in an otherwise-healthy person. Individuals with LQTS can experience fainting spells or seizures, often at a young age. In 10 percent of the cases, the first symptom is sudden cardiac death itself. LQTS can be inherited and several members in multiple generations of a family can be affected.
Doug was fortunate as a child, but upon reviewing his family history, the unexplained deaths of his 5-year-old brother and 14-year-old sister began to make sense. Doug’s first concern was not only his health, but more importantly the health and well-being of his two adult daughters Amy and Sherri, and their children - Doug’s young grandchildren. Had they inherited this risk?
When Doug's condition was identified, his transplant surgeon's first concern was to make sure his heart was stable enough to endure the transplant surgery. The illness itself had put his heart under great strain and caused the LQTS to manifest in Doug now, at a later age.
Dr. Lee Eckhardt, Doug's cardiologist, worked closely with the transplant anesthesiologist and surgeon to develop a plan to manage his heart condition during surgery. This plan included medication, electrolyte replacement and an implantable cardioverter defibrillator. Without these, Doug might not have survived the transplant surgery.
Once Doug was diagnosed with LQTS, the entire family was referred to the UW Health Inherited Arrhythmia Clinic, where Dr. Eckhardt and fellow adult cardiologist Dr. Craig January, pediatric cardiologist Dr. Kathleen Maginot, pediatric nurse practitioner Ann Dodge and genetic counselor Kate Orland form a unique team to care for patients of all ages.
According to Dr. Eckhardt, it is one of the only clinics in the country designed to diagnose, treat, and provide comprehensive follow-up care for entire families with inherited arrhythmia syndromes.
"By definition, an inherited heart condition such as LQTS is a family concern," she explained. "Through our clinic, we are able to screen all family members, and ultimately can identify and treat patients sooner."
The family was grateful for the ability to be treated at the same clinic. Orland noted that this ability is essential in identifying an inherited condition such as theirs.
"When a family is as motivated and cooperative as the Bartows, we are able to make the connections between family members and generations, which allow us to get a clear picture of the entire family," Orland said.
Amy and Sherri appreciate that. They, too, are treated by Dr. Eckhardt and their children by Dr. Maginot. Both Amy and her 5-year-old son Jonah share her father's gene for the class one LQTS mutation, which presents serious risks. Sherri and her daughters have the class two mutation of the gene, about which researchers still have much to learn.
Amy and Jonah are being treated with beta blockers to manage their risks, and Amy has been proactive in assuring Jonah's safety in their community. The local EMTs have taken a class on LQTS and Amy has met with officials at Jonah's school to discuss automatic external defibrillator education.
At this point, Sherri and her daughters, 1-year-old Mayleigh and 3-year-old Rhylinn, follow a list of drugs they must avoid, and the daughters are also treated with beta blockers.
"When working with children in the clinic, our goal is to determine their risks, treat them effectively, and most importantly, help them to have a safe and normal childhood," Dr. Maginot said.
Both mothers have faced their family's diagnoses with strength and grace.
"At first we didn’t want to know, but knew we had to find out for our kids' sake. We are thankful we did," Sherri said.
Amy agrees: "It was scary at first, but now we feel in control because we have the resources, support and treatment to protect our children," she said.
Doug and his wife Chris couldn’t agree more.
"It’s been wonderful knowing Dr. Eckhardt is there for Doug and my daughters, and that Dr. Maginot is there for our grandchildren," Chris said. "Knowing that their conditions can be managed and treated has given me a sigh of relief."
Doug added, "The worst part of this disease is that so many people don’t know about it. We want to help people understand and protect their families the way I want to protect my mine. I am so grateful for the resources and reassurance we have found at UW Hospital's Inherited Arrhythmias Clinic."
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