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Megan’s miracle: A happy ending after a four-year medical mystery

Cardiologist Gonzalo Wallis and heart surgeon Thomas S. Maxey with Megan Edney after her heart transplant. [CONTRIBUTED]

By Bill Moss, Published: August 17, 2018

For four years, no one could pinpoint what was causing Megan’s mysterious stomach pain. Then her condition cascaded into a crisis that plunged her to the edge of death and led to a successful heart transplant. This is her story.

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Megan Edney remembers when she could run and play, free as the wind.

But then the stomach-aches started. She had persistent pain in her belly from about age 10.
“Just my stomach hurting every day,” she said. “When I was younger I used to be able to run. And then when I got older I kept getting out of breath more where I was finally to the point where I couldn’t even go up the stairs.”
Her father, Michael, added: “She’d have to stop at the landing.”
Megan, who turned 14 on May 7, was back home in Hendersonville, settled on a couch, talking about a miraculous medical journey that brought her from the brink of death to a new life with a new heart — and no belly-aches.
Already, she can climb stairs without resting at the landing. She can play outside. Although visitors can’t get near her without wearing a mask, her beloved dogs, Lady and Chloe, can cuddle close.
Michael, an attorney who is chairman of the Henderson County Board of Commissioners, and Lisa, personnel director for a daycare center, have spent the past three months doing little besides accompanying Megan on her medical odyssey, an experience Michael described in the beginning as a “rollercoaster ride from hell.”
Megan, shown with Chloe, a Yorkshire teacup, missed her dogs when she was hospitalized.Before this past Memorial Day weekend, Michael and Lisa had been struggling with Megan’s medical ordeal for years.
“We were told it was acid reflux so that’s what we were treating,” Lisa said. “We were treating acid reflux but she still had all the symptoms.”
When Megan told her mom that the pain had moved from her stomach to her side, Lisa, on a hunch, made an appointment on May 30 with her ob-gyn at Pardee/UNC Health to check for an ovarian cyst. An ultrasound detected a large volume of fluid in her abdomen and a CT scan showed potential heart failure.
“That’s what saved her life,” Lisa said of the diagnosis at Pardee. “At that point she was already down to 14 percent of her heart working. That’s when it became a rush.”
Pardee doctors sent the Edneys to Mission Health and its cardiac specialists. Even the highly regarded heart doctors there recognized that her condition was more serious than they could treat. Doctors sent her to Levine Children’s Hospital in Charlotte.
“They wanted to fly us there but the helicopters were grounded,” Lisa said.
An ambulance took Megan through the torrential downpour that night to Levine’s highly respected Sanger Congenital Heart Center. She arrived at 1:30 a.m. on May 31, a Thursday.
Cardiologist Gonzalo Wallis and surgeon Thomas S. Maxey would be Megan’s main doctors. They confronted a patient who was critically ill, suffering heart failure and kidney failure, the cause of which was a mystery.
“Every 14-year-old girl that has stomach pain is clearly not having a heart attack,” Maxey said in an interview. “The parents obviously feel bad that — quote unquote — they’ve missed the diagnosis. It’s a difficult thing to kind of talk the parents off the ledge. The mom feels responsible. It’s a difficult diagnosis. If we end up doing a $50,000 workup on every teenage girl with abdominal pain we’re going to break the hospital system by lunchtime.
“In retrospect, her symptoms were related to poor cardiac function. All of those are signs of Danon disease, which typically doesn’t come to the forefront of every pediatrician’s practice. I don’t blame them.”
Even the most conscientious pediatrician would check a lot of boxes before he or she ever reached a rare cause like Danon disease.
“The chances of a 14-year-old girl having numerous heart attacks are much further down your working diagnosis list than other things that cause abdominal pain,” Maxey said.
No parents could have done more than the Edneys, who had taken Megan to specialists locally, at a hospital in Greenville, S.C., to Duke and UNC.
“Actually they went above and beyond,” Dr. Wallis said. “They were going to three different hospitals to try to find answers, so they did exactly what they needed to do. It just takes a highly coordinated medical care team to come up with a diagnosis like this.”

‘Missing the forest for the trees’

Dr. Wallis and Maxey had seen Danon disease once before — coincidentally the patient was also a teen-age girl from the N.C. mountains — but nothing in Megan’s medical record pointed specifically to that cause.
Family members and the Levine medical team, from left, are social worker Katie Boulware, Michael Edney, Lisa Edney, Dr. Gonzalo Wallis, Megan, Dr. Tom Maxey, heart transplant coordinator Kati Robinson, advanced care practitioner Celin Wittman and dietician Christine Smith.“In her case there was a bunch of red herrings that didn’t make a whole lot of sense,” Wallis said. “She had an eye problem, she had some concerns with her learning, and to top it off, during her evaluation, we found that she had an anatomic abnormality. She had a coronary artery that was not arising from the right place. At that time, people thought that her heart disease was related to the coronary problem. It didn’t look like that to me.”
Danon disease results in hypertrophic cardiomyopathy, an abnormal thickening of the heart muscle, a chronic condition for which there is no cure.
“The majority of those are genetic in nature,” Wallis said.
So the team brought in its geneticist to try to nail down what was causing the life-threatening breakdown in her heart function. The DNA test confirmed Danon disease. It was Saturday, June 2, day 2 of Megan’s stay at Levine.
“It was a good diagnosis that
captured everything that we were seeing,” Wallis said. “The heart was
thick. (Danon disease sufferers) have some learning issues. They can have vision problems. The interesting thing with Megan is that Megan had been seen for years and years and years at other hospitals but each one was seeing a different problem. They were missing the forest for the trees. They were trying to find the cause but
they couldn’t until they could take a step back and see the whole constellation of problems and say, ‘This makes sense.’”
As the doctors told them more about Danon disease, the Edneys looked back on four years of symptoms, medical visits and diagnoses and saw them as connected dots. Now they knew. Megan wasn’t an exaggerator or a hypochondriac or a stressed-out teenager. She had been suffering from repeated heart attacks. Her heart was gradually killing her.
“Three or four years ago, we went to Chapel Hill and Duke to have her eyes checked because a local doctor thought she might have retinitis pigmentation,” Michael said. “They did all these tests. They couldn’t figure it out. Now that we know what her Danon’s is, part of Danon’s is a discoloration of the eye.”

Berlin heart

It’s a hard message to hear but one that Maxey uses to explain to parents the ordeal his pediatric patients face.
“To get a heart transplant I’m going to hurt you before I help you,” he said. “You need to have enough reserve to be able to tolerate that. And someone that is so debilitated that their kidneys aren’t working (is weak), they’re malnourished because they can’t eat, they’re on a breathing machine, you’re in a bed, you’re losing muscle by the day.”
Lisa and Michael Edney hold a board displaying Megan’s beads of courage.Knowing now that Megan was suffering from an incurable heart condition and that her heart and kidneys would soon fail completely, Drs. Maxey and Wallis also knew they had just one option to bridge her to a transplant. Named for the city in Germany where it was developed and is still manufactured, a Berlin heart is a miracle machine for children awaiting a transplant.
“It’s better off to put in a VAD — a ventricular assist device — to allow them to recover,” Maxey said. “They can eat, they can do physical therapy, they can walk the halls. We do not know when hearts come. A heart can be available in 10 days or 10 months. … We bridge patients with these VADs and that allows them to recover.”
By then, Michael and Lisa had learned how close to death their daughter was.
“This is an overwhelming concept,” Maxey said, recalling the conversations with the Edneys, not unlike other initial meetings when parents are still in shock. “Your child was normal, healthy, now you’re in the intensive care unit. ‘What’s going on?’ ‘Oh my goodness, your kidney’s bad.’ ‘Now we’re talking about a heart transplant.’ That floors families within about five minutes.”
“The smarter you are, usually the harder it is to maneuver through these things,” he added. “Every family, I tell them — and it takes a couple of days to believe me — ‘I have every answer for you, you need to have faith in me. I can sit down and discuss for two hours every problem in the world. I have nothing to hide from you.’ … Families lose control. They lose control of their child, which nobody can handle. And you’ve got to put the control in somebody else’s hands who you haven’t met and that is an impossible task for every single family. Your mind spins 24/7 and you can’t get out of that tornado.”

 

‘Why is Dad crying?’

Bad as the weather had been on the way down the mountain, the downpour of new revelations about Megan’s condition seemed to get worse by the minute. Yet, within hours, the team had worked to stabilize Megan, inserting the breathing tube that she came to loathe almost immediately, surrounding her with machines and alarms and constant beeping and above all surrounding her with doctors and nurses delivering one-on-one coverage around the clock.
On June 5, six days after she was admitted, the Berlin heart arrived in Charlotte. The surgery to connect her to the machines — one on either side of her heart — lasted several hours. The first news in post-surgery alarmed her parents.
“Bleeding more than expected,” Michael wrote on Facebook. “Having to go back in and see why.” Hours later, he posted an update: “Doctor just came in and said this was a bump in the road not a mountain. Megan is stable and will be fine.”
“That operation went very well,” Maxey said. “She was on the breathing machine for two days and was actually starting to get up and exercise. Those are all good things. And if we had to wait six months for the heart, it would have been fine.”
Amazingly, Megan did not have time to start a regular exercise regimen, which the Edneys had been told could have included walking the hospital corridors and even going outside to soak up the sun. She was luckier than that.
Stabilized by the team at Levine, Megan was expected to survive on the Berlin heart and wait — for weeks, maybe months. The transplant team told the Edneys to be prepared to wait six months, nine months, maybe longer.
Then, one night, just 12 days after she left Mission’s emergency room in an ambulance, Megan saw her father sobbing in her hospital room. “Why is Dad crying?” she asked Lisa.
“She said he’s crying because they called about your heart,” Megan said in the interview at her home. “I didn’t really know what to think about it at that time.”
“Miracles do happen,” Michael exulted the next morning on Facebook. “At about 11 p.m. yesterday we received a call saying a donor heart had been found.”
“They never had another heart patient to our knowledge that got a heart that quick,” Lisa said. “We were expecting the long haul.”

‘Shopping out of both markets’

Drs. Maxey and Wallis say they have no information they can share about the heart donor.
Governed by the nonprofit United Network for Organ Sharing, organ matching and allocation is beyond the control of individual doctors and hospitals. Once Megan’s specs were put in the system, all anyone could do was wait.
“If you’re No. 1 on the list and the heart is no good for you then you’re not getting a heart,” Maxey said. “If you’re No. 8 on the list and the heart is perfect for you, you are getting a heart.”
Megan had a strike against her — a rare blood type. Her age was a factor in her favor.
“I will only put a heart younger than 39 years of age in a pediatric patient,” Maxey said. “Clearly it’s an adult size heart for an adult-size recipient. She could actually shop out of both markets, which was a huge advantage. The bad news is she’s an O. The good news is she’s an adult size pediatric.”
On Tuesday, June 12, the team wheeled Megan into the operating room for the heart transplant.
“They called us every hour during the procedure,” Lisa said. “It was awesome.”
Later that afternoon, Michael reported the successful outcome.
“Transplant is complete and the doctors say everything went as planned,” he wrote on Facebook. “Surgeon said her heart was as big as his head when it should have been the size of his fist. Praise God! Today was huge but she has a long journey ahead, please continue to pray for her and our family. Please Lord, somehow let the donor family know our eternal gratitude and be with them now and forever. We will always feel a very special connection.”
Heart transplant protocol allows the Edneys the opportunity to get in touch with the donor’s family.
“We can reach out to them in six months,” Michael said. “They can decide if they want to have any communication.”

‘She looks like a rock star’

Nine weeks since she got her new heart, Megan is passing every medical test with flying colors. She makes weekly visits to Levine for bloodwork and other tests.
One of the unusual things about Maxey’s treatment of Megan is that he had no chance to build a doctor-patient rapport before he put her on the Berlin machine and then sewed in her new heart. Friendly and often humorous, Maxey would prefer to have more time with his young patients before he plunges them into such major medical journeys.
“I did all that without even talking to her, which was a little awkward,” he said. “Normally, I have the ability to talk to kids in that situation, so all this was through her parents. I really didn’t speak to Megan until after her heart transplant. That was a little unique with Megan. I haven’t experienced that a lot.”
When she woke from surgery, she expressed feelings that the transplant surgeon often sees.
“She’s an emotional teenage girl, got a raw deal,” Maxey said. “I think she had confidence in me and our team and realized we are going to get through this. She’s got a metal zipper down her chest and has someone else’s heart in her. I think the reality of that takes a while for you and I to absorb — you can imagine a 14-year-old trying to absorb that.”
A month later, on July 11, Megan impressed her heart surgeon with her progress.
“Saw her today and she looks fantastic,” he said that day. “She looks like a rock star. She looks like a normal teenage kid walking around but she’s still emotionally trying to deal with all this.”
Wallis also was pleased at how well his patient was doing.
“She looked amazing,” he said. “That girl is resilient and those parents are resilient. She basically went through three surgeries back to back to back and she recovered amazing. She was intubated and sedated and then she went for a Berlin heart and then she went for a heart transplant. Those are things that each take its toll. … It’s impressive that she recovered like she did.”

Hundreds of get-well cards

Although she takes a medicine chest worth of pills every day, Megan otherwise enjoys a life that gets closer all the time to a normal teenager’s.
For now, she is home-schooled through a special program at Mountain Community School; she expects to go back to school after Christmas. She attends classes through Google Hangout and teachers come to her home two days a week.
The Edney family — Lisa, Michael, Megan and older brother Mitch — are filled with gratitude for the medical team at Levine.
“They colored with her, did her hair, they treated her like she was their daughter,” Lisa said.
Cards, letters and gifts from Hendersonville and across the U.S. filled a large box.
“They said she got the most mail,” Lisa said. “They’ve never seen a child get so much mail. We got cards from lots of churches. Tennessee, New York, Florida. All kinds of people that just read her story.”
Every time she had a medical procedure at Levine — some major milestones, others more routine — the staff added a bead to her collection. There are dozens, displayed on a board in front of a message: “I am enrolled in beads of courage!”
On Sunday, Aug. 5, Michael posted pictures from a lakeside home at Lake Norman, loaned to the family by WHKP radio station owner Art Cooley.
“We finally had a mini-vacation at Lake Norman (thank you Art),” Michael said. “Megan was able to go swimming, we had a great time! Family means everything, thank you God for ours.”
With medication, regular checkups and periodic biopsies, Megan should enjoy a healthy life.
“I expect no restrictions for her,” Maxey said.
Asked whether he would hand Megan off to a cardiologist closer to home, Wallis said, “Oh, no no. I told them at the beginning, this is a marriage. I’m not going anywhere.”
“The most important thing is none of this would be possible if people would not donate hearts,” Wallis added. “If you can emphasize something, people need to donate, so that other kids can have a chance of life. That’s a key message here. We need organs to do good work.”
A transplanted heart doesn’t last forever. But with medical advances, they can endure.
“It’s not a cure. That’s the difficult part of this,” Wallis said. “Transplantation is not a cure. We do it so you can have better quality of life.” A transplanted heart can last 14 to 20 years “but that number keeps getting better and better all the time. I think with appropriate medical care she will have great quality of life until we have a better solution. These kids grow normal. They go to school, get married, have kids.”
Can Megan run in the grass, swim in the ocean, dance at her senior prom?
“Yes, absolutely,” Wallis said. “That’s why we do this thing.”

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A “Hit for Megan’s Heart” golf tournament fundraiser is scheduled for Sept. 24 at Kenmure Country Club. The fundraiser is in coordination with the Children’s Organ Transplant Association, a nonprofit organization that provides fundraising assistance and support to families of children who need a life-saving transplant. To register visit cota.donorpages.com/MiracleMakers/TeamMeganEEvents. For more information about the golf tournament or about sponsorship, fundraising and volunteer opportunities, call (828) 692-4130 or office@edneylaw.com.