Finding out the hard way
UK employee Jason Conn is one of a growing number of adults who have survived congenital heart disease into adulthood. Although treated in childhood, the disease’s effects follow them through life, requiring a strong bond with a specialized care team.
Jason Conn was not about to miss the first meeting of a support group for adult congenital heart (ACH) patients at UK Chandler Hospital’s Gill Heart & Vascular Institute in May 2017. Conn is a mechanic for the University of Kentucky’s motor pool and a fan of anything with wheels, so he rode his bike the couple of blocks from home to the meeting.
Meghan Frost, MSW, CSW, a social worker in case management for UK HealthCare Ambulatory Services, was at the support group meeting, too. She works with congenital heart patients like Conn and had first met him a year earlier when he was so sick he could not stand up. Back then, on top of serious health problems, Conn was worried he might run out of sick leave and lose his job, so Frost had walked the worried patient through paperwork required for the Family and Medical Leave Act.
Now, he was fit and well, looking robust. Frost said, “I got chills, he looked so healthy. I kept saying, ‘Gosh Jason, you look so good!’”
Congenital heart disease requires lifelong specialized care
Jason Conn is among a growing population of cardiac patients with adult congenital heart disease, many of whom had surgery in childhood. By the time he was 13, Conn had had three surgeries in his home state of Ohio on a bicuspid aortic valve.
Four or five decades ago, children with heart problems were not expected to see adulthood; now because of improved surgical procedures and the care of pediatric cardiologists, these patients are surviving and, in many cases, living normal lifespans. That has resulted in adult congenital heart (ACH) programs, a new specialty area in cardiac care. Cardiologist Andrew Leventhal, MD, PhD, launched an ACH program at the Gill Heart & Vascular Institute when he joined UK HealthCare in early 2016.
“The care of these patients is extremely complex and requires coordination among many cardiac subspecialists,” said Leventhal. UK HealthCare has a multidisciplinary team of health care professionals – sonographers, nurse practitioners, obstetricians and electrophysiologists, to name a few – who specialize in adult congenital heart disease. Such a team is required to care for the complex issues faced by ACH patients.
First, convince patients they need you
One of the challenges of treating ACH patients is convincing them that they need specialized medical attention.
“Many of these patients aren’t being followed, so a big part of my job is identifying and convincing patients to come to our clinic,” said Leventhal. “Some were told as children that they were cured, and they were not. And, when they leave home at 18, the last thing they want to do is go see a doctor.”
And that, said Leventhal, is where trouble begins for patients like Conn. Over time, Conn’s follow-up appointments with a cardiac specialist in Ohio faded away. As an adult, he steered clear of doctors, seeing one only when he absolutely had to. By the time Conn came to Leventhal’s clinic, he was very sick.
Leesa Schwarz, APRN, NP-C, called to check on Conn when he missed his clinic appointment. By the time he arrived at the clinic a few days later, he was in a wheelchair and could barely stand. Her quick and accurate assessment of his condition is credited with saving Conn's life.
Getting reminders about an upcoming doctor’s appointment is not unusual. But few practices track down patients who fail to show up for their appointments as Leventhal’s does. “If a patient doesn’t show up in the clinic, we are going to find them and get them in,” said Leventhal.
Conn, in fact, missed his very first appointment at Leventhal’s clinic in spring 2016, prompting inpatient cardiology nurse practitioner Leesa Schwarz, APRN, NP-C, to call him.
“We need to see you.”
She reached Conn at home. He was sick, on antibiotics, unable to work, running a high fever. Schwarz said, “We need to see you.”
When Conn arrived at the Gill adult congenital heart clinic a few days later, “His friend was pushing him in a wheelchair; he couldn’t even walk,” said Schwarz. “He was so weak he couldn’t stand up.”
Conn told Schwarz he had not felt well since late 2015. He had lost weight and was running fevers. Schwarz had worked with ACH patients when she was at Emory University, and she immediately suspected endocarditis, an infection of the heart lining that often affects people who have damaged or artificial heart valves.
A sonographer trained in ACH issues did an echocardiogram that showed Conn indeed had endocarditis. It had caused an abscess to form on his aortic valve. The abscess extended to the tissue separating the heart’s bottom chambers and had caused a hole to open between them.
A series of important calls
Schwarz had also called Leventhal about Conn because she realized the magnitude of his illness. “Leesa probably saved his life,” said Leventhal. Now, it was Leventhal’s job to find the right cardiothoracic surgeon to handle the complex case.
Conn’s damaged valve would have to be replaced; infection would have to be cut away and holes in his heart tissue would have to be patched. “It was a massive undertaking,” said Leventhal, “and you need not just any adult cardiac surgeon but someone who understands congenital heart disease.”
Leventhal called Hassan Reda, MD, whose areas of clinical interest include aortic valve replacement and aortic root surgeries. “Being able to pick up the phone and call a guy like Reda is a huge luxury,” he said.
Calling on his experience despite the lack of symptoms, Reda ordered tests to see if infection had spread to Conn's brain. Infection can cause aneurysms in the brain, which left untreated before surgery, could cause fatal brain bleeds.
Before Reda could operate, Conn’s multiple infections had to be treated. He was admitted to the hospital. The UK Infectious Diseases team recommended the blood cultures needed to identify Conn’s infection so that a targeted course of antibiotics could be prescribed to fight it.
Reda first introduced himself from the doorway of Conn’s room; the doctor had a cold, and he did not want to further endanger Conn’s precarious health. “He told me he’d be back to see me soon,” said Conn.
When Reda returned, he spoke honestly about the surgery he would perform. Opening Conn’s sternum the fourth time was extremely dangerous, he said. “When someone reaches their 40s, what worries us most is the need to go into their chest again,” said Reda.
“Each time, you are losing material and you are working closer to the heart.” Conn remembered he was told.
Reda also said that there was a good chance that there was infection in the blood vessels around Conn’s brain. “It was a silent infection, he didn’t exhibit any symptoms but based on previous experience we look for it before we do surgery,” said Reda. The infection could cause aneurysms in the brain and if those were not treated before surgery, the blood thinners required for Conn’s heart surgery could cause him to have fatal brain bleeds.
Reducing surgical risk
Just as Leventhal was able to turn to Reda, Reda turned to another capable colleague, surgical neurointerventionalist Abdulnasser Alhajeri, MD, who specializes in endovascular surgical neuroradiology.
Unconvinced by the first test, neurointerventionalist Abdulnasser Alhajeri, MD, performed another to take a better look at blood vessels in Jason’s neck and brain. He found multiple infected brain aneurysms. If left untreated, surgery could have caused fatal brain bleeds.
Alhajeri would do a series of increasingly sophisticated tests to check Conn’s brain for aneurysms. Unconvinced that an MRA – an MRI with angiogram – was conclusive, Alhajeri performed a conventional cerebral angiogram, a minimally invasive test that accesses arteries from the groin and reaches blood vessels in the neck and brain via catheters. The catheters are then used to inject contrast media into arteries of the brain, guided by an X-ray camera. That test showed multiple infected brain aneurysms.
Using the catheters to access the blood vessels, Alhajeri cut off the blood supply to the aneurysms. The procedure posed a high risk of stroke because an artery that supplied viable areas of the brain was being blocked, but the painstaking procedure was a success and Conn awoke with no damage. Now, the risky surgery on his heart could go forward.
Conn works to regain strength in cardiac rehab
After surgery and time in the Cardiovascular Intensive Care Unit, Conn immediately began his physical rehabilitation three days a week at the Gill’s Cardiopulmonary Rehabilitation and Wellness Center at UK Good Samaritan Hospital. According to program supervisor Jacob Stone, MSEP, CSCS, the center designs a specialized exercise regimen for each patient.
In Conn’s case, because his job requires lifting tires and other heavy objects, rebuilding upper body strength was crucial. A team that included exercise physiologists and a nurse first did tests to see how well Conn was doing physically – for example, how far and fast he could walk. They also evaluated the effects his medications would have on his heart rate and blood pressure.
“He did everything we asked of him and sometimes he wanted to do more,” said Stone. “We were always trying to progress him safely.”
Conn was surprised by the social aspect of rehab.
Like a lot of the center’s patients, Conn was surprised by the social aspect of rehab. “I really enjoyed talking to the older gentlemen,” he said. Many are regulars at the center – after their rehab ends, they join the center’s wellness program so they can exercise in a safe place with medical professionals and a crash cart nearby and enjoy the company of other patients with whom they have become connected.
“Many of these people have made friends,” said Stone. “They go out to lunch after rehab or have other group activities. It is kind of a big support group really.”
Counseled to take better care of himself
A “champion” at cardiac rehabilitation, as Leventhal called him, Conn has also been attentive to Leventhal’s instructions on ways to live a healthier life as an ACH patient. “He is now my boss,” Conn said.
Leventhal has counseled Conn on everything from avoiding tattoos to the importance of regular dental cleanings and check ups. Most infections begin in the mouth.
Among Leventhal’s goals is to help his patients avoid acquired heart diseases, which would further compromise their health. He talks about proper diet and exercise and advises giving up harmful habits such as smoking. He helps them understand that little things like slight fevers are big deals for them. “These patients have to treat a fever seriously and always get a blood culture,” said Leventhal.
Conn knows he is never to take an antibiotic prescribed by another doctor without checking first with Leventhal. He has regular dental checkups and cleanings at the UK Dental Clinic. He has given up what he calls “gas station food,” and is eating healthier and drinking less alcohol. He tries to avoid cuts and scrapes that are common in his work and keeps his hands and feet “immaculate.”
After 20 years in auto repair, Conn knows well the benefits of preventive maintenance. Now, he realizes preventive maintenance is also critical to his health.
After 20 years in auto repair, Conn knows well the benefits of oil and filter changes, tire rotation and other preventive maintenance. Now, he realizes preventive maintenance is also critical to his health. “I’ve taken care of cars more than of myself. Before, I went to the doctor when something was wrong,” he said. Now, he says, “I’ll be doing preventive maintenance and taking better care of myself.”
“The choice I was facing was to go home and die or face a very risky surgery.” — Jason Conn, ACH patient
A return to work and a new support
Conn has been back at work since early July 2016, less than three months after his April 20 surgery. When he is not repairing cars in the UK motor pool, he might be working on a two vintage cars – a Fiat and a Thunderbird – that he owns. He also works on cars for friends, and as the summer of 2017 arrived, a VW Beetle that had not run in about 15 years awaited.
He also plans to attend future meetings of the adult congenital heart disease support group. Conn’s not a big talker, yet he connected with several other patients at the first meeting.
He thinks of the young woman who four months earlier had had a successful heart transplant. “That was not too long ago and she looks great,” he said. “It reinforces confidence in the clinic and what they do.”
Being at those meetings shows his support for patients who have driven hours to talk to others who share similar experiences, Conn said. “A lot of them have to drive from out of town, and I’m only four blocks away.” His presence also is a way to say thank you. “It is,” he said, “a way to support the clinic” that saved his life.Tell us your story More patient stories