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The Heartbreak Kid
Mike Mader was born in Las Vegas to 1 in a million odds—the unlucky kind. He was diagnosed at age 4 with familial hypercholesterolemia, a rare genetic disorder that affects both the liver and the heart.
The condition, which occurs in just 1 in 1 million births, is caused by a genetic mutation, of which Mader inherited two copies—both from his mother. His liver lacked a certain gene needed to process cholesterol; as a result, cholesterol built up in the arteries of his heart and, over time, led to coronary disease.
Mader’s mother required a seven-bypass surgery to reduce her cholesterol buildup. Mader’s sister, Katie, is able to manage her elevated cholesterol through statin medications.
But Mader’s body did not respond to the cholesterol medications, and he and his family knew since his diagnosis at age 4 that a heart attack was inevitable.
At times, Mader was on about 25 different prescriptions at once, with limited success. He also ate a selective diet and exercised to remain healthy and stave off cholesterol buildup.
But Mader still had a heart attack—earlier than his doctors expected, and then many more heart attacks; more than they had anticipated.
His first came at age 22, while he was attending Southern Utah University and working full-time for a chemical company as a quality-assurance manager.
Mader was at work when the heart attack arrived. He recalls that he felt sick, and then, he says, “It’s almost like a switch flips in your head. Your body just tells you that something is catastrophically wrong. I just got an overwhelming feeling of doom.”
He went home, told his mom, and was then rushed to the emergency room, where it was confirmed the occurrence was a heart attack.
A month later, he had a second heart attack.
After the second heart attack, Mader skipped two semesters at SUU, but continued to work through five more heart attacks. After his eighth, he quit his job.
“Not being able to work was very difficult,” he says. “There were times when I tried to go back to work and I was just too sick to do it. The cost of living and not being able to work can be pretty stressful at times.”
With each heart attack Mader experienced, doctors placed more stents—ducts made of metal and fabric mesh—in the valves of his heart to allow blood to flow. At age 30, he had 36 stents throughout his arteries.
And as the years wore on, Mader experienced more and more heart attacks, with varying intensities.
Mader had more than 18 heart attacks total—surprisingly, that’s not a record. In 2005, Leslie Hackwell of England had around 32 heart attacks within two minutes and survived, which led many doctors to the conclusion that it is not the number, but rather the severity of a heart attack that matters.
Mader became somewhat accustomed to the heart attacks but no less aware of their seriousness.
“The amount didn’t faze me, as it didn’t matter how many I had survived—it would only take one to kill me,” he says.
Mader moved to Salt Lake City in November 2007 to have lipid apheresis treatments to remove the cholesterol in his bloodstream. The sessions, which occurred as often as once a week, took four to eight hours and involved inserting a large needle in each arm to run his blood through a cleansing machine.
But the treatments made Mader anemic and did not stop the heart attacks.
And with each heart attack, Mader wound up back in the emergency room. “Time is everything if you’re having a heart attack,” he says. “So the faster you can get in, the more likely you are to survive it.”
In summer 2009, while vacationing with friends at Lake Powell, Mader had a severe heart attack. And within two weeks, he had another. He was officially in heart failure—leaving his body without adequate blood flow and necessitating a dual transplant of a heart and a liver.
As a bridge until a suitable donor was found, doctors implanted a defibrillator into Mader’s chest that would administer a resuscitating shock if his heart went into a fatal rhythm.
Mader was shocked three times by his pacemaker during the 2 1/2 years he had it. The third time was during a jog; he says the sensation was so jolting that he thought he had been hit by lightning.
But the pacemaker could not save him from a deadly heart attack—only a transplant could.
“It was horrible to have to wonder if every holiday or birthday would be his last,” says his sister, Katie. “He had been so lucky to survive as many heart attacks as he had.”
The family could not help but feel, Katie says, “that we were pushing our luck.”
Mader was caught in a catch-22. Because of his innate cholesterol buildup, if doctors replaced his liver, he would still have a diseased heart; if doctors replaced his heart, he would still have a malfunctioning liver.
“If someone’s heart failure has a genetic cause, there isn’t any additional treatment that we can do; there’s no genetic therapy,” Budge says. But with Mader, she says, getting a liver transplant could act “sort of like a gene transplant. By giving him a new liver, we were giving him the genes that were then needed to handle the cholesterol.”
It seemed the only option for Mader was a combined transplant of both a heart and a liver—a laborious procedure with a lengthy waiting time. Mader was placed on the transplant list in late 2010 to await a fitting heart and liver.
According to the U.S. Department of Health & Human Services, the median national wait time for a liver is 361 days; it is 113 days for a heart. And Mader needed both at the same time. He also needed a larger heart to support his system, limiting the pool further.
In addition to matching size and blood type to the organ donor, a patient must be adequately healthy to receive a transplant. So to prepare himself to manage two new organs, Mader pushed himself physically. He was not allowed to lift weights, so he focused on cardio. As time went on, Mader eventually found it even difficult to walk on flat ground. Still, he lost 30 pounds before the operation.
It was a silver lining to his otherwise cloudy long wait—718 days.
“Waiting on the list, it’s hard not to count the days that have gone by,” Mader says. “A lot of the waiting period during those three years, I wasn’t really sure if I’d make it. The transplant was the only thing that could have saved me. I had to hold on long enough to get that done. I think, mostly, I just learned how to survive heart attacks.”
His family, meanwhile, had to learn how to survive waiting.
“I felt as though the transplant was never going to happen,” Katie says. “Or that by the time he received the transplant, he was going to be too critically ill and his body wouldn’t be healthy enough to survive the dual transplant.”
She was in constant worry, although Mader showed no obvious physical manifestations of illness.
“Just looking at me, you’d never guess I was having health problems since I wasn’t wearing a full-body cast and everything was internal,” he says. “People just socially didn’t recognize that I was sick.”
But suddenly, on the night of April 22, 2013, Mader had less than a day to wait. A phone call came from the hospital informing Mader they had received a transplant liver and heart from the same donor. Now he was just 17 hours away from entering the surgery room and leaving it with a new life.
“Once I got wheeled into the operating room, things got serious real quick,” Mader says. “At that point, it really hit me. That’s when I got nervous.”
Playing Operation
There are no second chances in a heart transplant. It is literally do or die in the operating room.
“A heart transplant is an exciting thing. It’s also a stressful time until it is actually complete,” Budge says. “There’s always some risk when it’s taken out of the donor that something could go wrong in that process. From the moment we get that donor call, there’s some anxiety until that heart is in and it’s working.”
Mader’s procedure was the first combined adult heart and liver transplant in the Intermountain Region; fewer than 10 are done each year in the entire country.
The operation took more than 12 hours and required the coordination of more than 100 people, Budge says. Calls had to be made. Staff had to meet to determine which operating room and intensive-care unit to use, and to make the decision of which organ—the liver or the heart—would be transplanted first. The actual organs had to be acquired from Intermountain Donors Services, and everything had to happen quickly—the heart can be kept outside of the body, on ice, for just four hours.
On the day of an operation, Budge’s job as a cardiologist is to observe how her patients are responding to the donor heart—which might be more tense than wielding the scalpel. Thanks to medical advances, it’s now relatively uncommon for patients to reject the new heart immediately, but it can still happen.
And despite the initial joy, waking up from surgery is when more risks become real.
When Budge sees the donor heart warmed up and pumping in a patient’s body, she experiences a feeling that she refers to as “a measured amount of cautious relief.” Even after an initially successful heart transplant, the patient still faces the possibility that his or her body will reject the new organ and treat it like a disease.
“The biggest uncertainty would be how long a person will live after transplant,” Budge says.
