The Guilt and Grief That Comes With Waiting for a Heart For Your Child
“For the longest time I did have it stuck in my head, that I was praying for a heart to come for my child; does that mean I'm praying for somebody's child to die?"
On October 5, 2008, two-and-a-half-year-old Madi Rippy woke up from a nap, unfocused and groggy. Her baby blue polka dot pants and matching shirt were wrinkled from sleep.
Her mom, Linsey, was making waffles in the kitchen. When she called out her daughter's name, Madi— who usually turned her head to look back—didn't respond. Instead, she stared forward as if in a trance, her left pupil wandering off to the side, "like she wasn't with the world," Linsey says.
Linsey and her husband, Noel, buckled Madi into their Jeep to bring her to the local hospital in Blaine, Minnesota. Then, they noticed that Madi had lost all movement on the left side of her body. Linsey called an ambulance and they waited five minutes—an impossible, surreal five minutes—Madi sitting in her carseat, and Linsey pacing in circles.
“I don't think I was feeling anything,” Linsey tells me. "Basically in survival mode. We never thought it might be something so unbelievably serious.”
When they arrived at Mercy Hospital Coon Rapids, they were told the hospital didn’t handle serious pediatric cases. They took another ambulance to Children’s Minnesota Minneapolis Hospital about twenty minutes away. There, an MRI confirmed that Madi had had several small strokes. “They were like, ‘Why is this two-and-a-half-year-old child, who had been previously healthy, having strokes?’” Linsey remembers.
The answer came by way of chest x-ray. Madi’s heart was the size of a softball—two times the size it was supposed to be at her age. It was so big it almost filled her whole body—chest wall to chest wall—and had collapsed her left lung.
Madi was diagnosed with dilated cardiomyopathy, a condition in which either the left or right ventricle of the heart is enlarged and the heart can’t pump blood normally. Up to 50 percent of people with dilated cardiomyopathy have an immediate family member with this condition. When it shows up in young children, though, the cause is murkier—and the outcomes are often more serious.
Dilated cardiomyopathy can occasionally be caused by myocarditis, a viral infection that impairs heart-muscle function. Other times it's caused by random new mutations in the genome. Some kids get better and stabilize with only medication, Linsey and Noel were told. But some get worse. Dilated cardiomyopathy is the most frequent cause for heart transplants in pediatric patients. There are more than 50 gene mutations linked to dilated cardiomyopathy, but Madi wasn’t positive for any of them. She had recently had a respiratory virus, and her doctors thought maybe that triggered her cardiomyopathy.
After two weeks in the hospital, she was discharged with an arsenal of heart drugs: Lovenox shots two times a day in her legs to thin her blood; Enalapril, Lasix, Aldactone and Coreg. And later, Digoxin was added to help her heart squeeze better. “When we left, we thought that life was going ridiculously different than we had thought it would be, but at least the genetic testing showed everything was fine. It'll be fine," Linsey says. "Well, we were wrong.”
Not even a year later, in July 2009, one of Madi's medications, Digoxin, became toxic in her blood. Her heart rate dropped so low she had to be readmitted to a hospital for an open chest surgery to put in a pacemaker. After the operation, she had been off the ventilator for two hours when suddenly, her body was no longer keeping up. Her blood pressure and other vital signs were going in the wrong direction.
“She just wasn't sustaining herself,” Linsey says. “So they sent her to the Mayo Clinic and they put her on the transplant list.”
Aristotle viewed the heart as the most important organ, “the seat of intelligence, motion, and sensation." English physician William Harvey, while describing the heart’s function in 17th century, called it the “king” or “sun” of the body.
When the ancient Egyptians mummified a body for a person’s spirit to return to, they discarded the other internal organs, including the brain (by liquefying it and draining it through the nose)—but preserved the heart. After Chopin died, his body was buried in Paris, but he requested that his heart be sent back to his homeland, Poland.
We live in an age that’s ruled by the brain, which is credited with holding the secrets of our personalities and consciousness. And yet the heart still holds a privileged position.
“If I give talks and I say to people, ‘Could you point to the location of your mind?’ they point to their head. When I say ‘Where is your soul?’ they point to their chest,” Fay Bound Alberti, an honorary senior research fellow at Queen Mary University of London who wrote a book about culture and the body, told The Atlantic in a 2016 interview. “There’s metaphors about the heart that are enduring in the language that we discussed, about feeling that something is heartfelt, you're being true to your heart and so on. So the organ carries on that meaning above and beyond any scientific explanation.”
Madi was joining the small population of children that needed their hearts replaced. Bound Alberti says that among transplant recipients and doctors alike, a new heart comes with extra emotional baggage. Some patients worry about their personality being changed after receiving a new heart, or refuse hearts from donors of a different sex. In a recent episode of Grey's Anatomy, a teenage boy doesn't want a donor heart for fear that he won't love his boyfriend anymore, as if his love is embodied in the organ itself. Another heart cannot actually change who "you" are—but the stories and myths we tell surrounding the heart belie its persisting symbolic importance.
Congenital heart disease is the number one birth defect, affecting about 40,000 children each year. That’s about 1 percent of all babies that are born, and of those, only a handful will need a heart transplant. All in all, it’s not a huge population. There are between 300 to 500 pediatric heart transplants done each year, says Elizabeth Preze, a cardiac nurse practitioner, who is launching the pediatric heart transplant program here at Children’s Minnesota Minneapolis Hospital. In 2017, there were 431 pediatric heart transplants. They account for roughly 12 percent of the total heart transplants. Data provided to Tonic from UNOS says that as of April 11, there are currently 372 children waiting for donor hearts in the United States. 58 are under a year old, and 133 are between one and five.
But the fact that there are so few cases adds to the hefty emotional weight of this kind of surgery. There are large patient groups and resources for childhood cancers or cystic fibrosis—still terrible illnesses, Preze says. But heart transplant families can often be the only ones in their towns or counties. They have to leave home, embarking to urban hospitals that can perform the transplants and wait for hearts—the average waiting time is around six months. A study from 2009 found that 533 children died waiting for hearts between 1999 and 2006.
She says that families are often hit hard psychologically, developing post traumatic stress, anxiety, depression. "The numbers aren't huge and, unfortunately, the resources for mental health are already stressed," she says. "There's just not enough of them and so these families really struggle to find people that are like them."
Where do transplant hearts for children come from? Joseph Dearani, the chair of the department of cardiac surgery at the Mayo Clinic, lets out a big sigh when I ask him this. It's not a happy topic, he says. The most common source is trauma: Car accidents, shaken baby syndrome, or any instance when a child's head gets injured and they experience brain death.
“Another category is child abuse, just to be candid,” he says. “It's not something that we like to talk about, but that’s one of them.” There’s also crib death, when babies die in their sleep, drownings, and in rare cases asthma, due to low oxygen levels in the brain.
“It's always alarming on the donor end, and it's always upsetting, and it's always tragic,” Dearani tells me. “It's also quite dramatic on the receiving end. As dramatic, disappointing, and upsetting it is on the end of the donor, there's exhilaration on the recipient end. Many of these children are waiting for quite a while—many, many months, even longer sometimes—for an organ to become available.”
This is one of the most difficult psychological struggles, Preze says. The dire state your own child is in, and the guilt that comes with what they need to get better. “They hope that their child gets a heart, but they know that another child has to die to receive that. You can try to explain it away by saying you didn't cause it to happen, but the fact is that there's another parent out there going through their worst nightmare when you're getting what you were wishing for... That really is a very difficult thing to hope for, especially when you think that there’s another set of parents and family that are grieving the thing that you just avoided.”
Not only does a family have to wait for the impossibly tragic to happen, they also have to wait for a heart of around the same size. Dearani tells me that when you list a child for a heart transplant, you do so by weight. In general, they have a 20 percent rule. You can go up 20 percent, or down 20 percent in weight—any bigger and smaller and the heart won’t fit, or won’t do the job. There are some exceptions to this: In kids who have very dilated hearts, they could accept a heart 40 percent larger than expected, because they have the room for it.
I can’t imagine the intricacies of operating on a normal size heart—around the size of two fists— let alone one that's half that size, or even less. But Dearani assures me that most heart surgeons are pretty technically skilled. The size isn’t the issue, it’s that some children have abnormal “plumbing,” meaning they have blood vessels entering or leaving the heart in different locations. The donor hearts come from healthy children, so Dearani says it can be a challenge to reconnect all the blood vessels so that everything lines up.
Normally, when a heart is taken out of the body, it continues to beat for a little while. That’s its natural drive: to pump blood. When Madi’s heart was removed, it immediately stopped moving. Dearani, who performed the operation, told Linsey later that Madi's heart was on the brink of total failure. He had also found a huge blood clot, waiting insidiously in the wings.
There's a rating called ejection fraction, which essentially measures how strong your heart is squeezing and pumping blood. You want the ejection fraction to be at least 55 percent; a healthy heart's ejection fraction is around 70 percent. Madi’s was less than 10 percent. Her doctors said that her heart wasn’t so much beating as it was quivering.
She was also extraordinarily lucky. Eighteen hours after she was put on the transplant list, she got a heart. “Even at Mayo, they said, 'We've never seen this,'" Linsey says.
When Madi first went to the hospital, Linsey was about 12 weeks pregnant with another daughter, Sydney. When Sydney was born, her doctors preformed an echocardiogram on her heart, a non-invasive way to see visually how the heart is functioning, and it was normal. A year later, it remained that way. But at two-and-a-half, Sydney was diagnosed with dilated cardiomyopathy too. Her heart was getting too big.
“We kind of fell apart,” Linsey says. “We were like, 'Oh my God, we thought this wasn't genetic,' and they said, 'Well, I guess it must be genetic.'”
When she turned three, Sydney got a stomach bug. Noel took her to the emergency room, and as he walked in the door Sydney began to seize. She coded, and needed 28 minutes of CPR. The virus had depleted her sodium levels, causing the seizure. When they intubated her, she went into cardiac arrest. She coded again, and was put on extracorporeal membrane oxygenation (ECMO), or a bedside bypass machine that circulates blood through the body when the heart can't do it itself. She was flown down to the Mayo Clinic for a heart transplant, and received a heart two days later.
"I was kind of numb the second time around," Linsey says. "How was this happening to me again?”
Madi and Sydney’s hearts had failed on them at almost the exact same age, and so the Mayo Clinic devoted a research team to the Rippy family. “They were stunned by how quickly they went downhill, by how aggressive the disease was, and how it started at a freakishly similar time for both girls,” Linsey says.
The team at Mayo took blood and skin samples, and biopsies; Linsey and Noel each had echocardiograms and gave detailed interviews about family history. Five years later, a new genetic mutation was found. It’s a recessive mutation in a gene called TAF1A that Linsey and Noel both carry. One copy of the gene has no apparent impact on the heart, but having both copies leads to rapidly progressive heart failure, says Timothy Olson, one of the authors on a paper about their genetic findings.
“Basically, had we married anybody else, this probably wouldn't have happened,” Linsey says.
Cells in the heart have limited capacity to divide and regenerate, Olson tells me, but the proteins in the heart are made and replenished every five to ten days. They think that when there are defects in the TAF1A gene, it interrupts a key step in this protein renewal, which is critical for maintaining normal heart muscle function. When the researchers knocked out the analogous TAF1A gene in zebrafish, it caused heart failure.
"TAF1A can now be incorporated into clinical genetic testing panels, informing family planning decisions and possibly predicting a rapidly progressive form of dilated cardiomyopathy that warrants close clinical monitoring,” Olson says. “Defining precise molecular genetic causes for dilated cardiomyopathy enables further research to develop targeted treatments that prevent heart failure.”
“It's a little surreal,” Linsey says. “And on top of that, my husband and I had originally wanted one more child. When Sydney turned up sick we said, Nope, we're not doing this again. So, no more kids. There was a certain grieving to that. We didn't make the decision to stop having children necessarily. Life did, you know?”
But the genetic findings also mean that Linsey gained some peace of mind about why both her girls got sick. “There was absolutely nothing that we did or didn't do that caused it,” she says. “As a mom, you automatically think, 'Oh my God, what did I do? Or what did I not do?' Through my whole pregnancy with Madi, I exercised, I didn't drink caffeine. So I felt like, 'What did I do? what did I do? It was kind of a relief to know that we had no control over it at all.”
I asked Linsey if she knows where her daughters' hearts came from. She says that Madi’s came from South Carolina, and Sydney’s came from Texas. Madi’s heart was from a ten-month-old who had been in a car accident. Sydney’s was from a child closer to her age, around three years old. Other than that, she doesn’t know anything.
“For the longest time I did have it stuck in my head, that I was praying for a heart to come for my child; does that mean I'm praying for somebody's child to die?" she says.
One day, a cardiologist at Children’s Minnesota Minneapolis Hospital pulled her aside, and told her not to think that way. He said that child would have died even if Madi or Sydney didn’t need hearts. “He said, 'You have to try to look at not as if you're wishing for a child to die, but that you're wishing for the parents to be brave enough to save other lives.'”
Linsey now serves on the board of a charity called Ireland’s Hope, which was started in Minnesota by Patty O'Connor in honor of her niece, Ireland, who waited ten months for a heart. Linsey says Ireland’s Hope was started not only for pediatric organ donor awareness but to help financially support families whose children are in the hospital waiting for a heart near them. “If your kid has to have a heart transplant, you can't just call your work and be like, hey, I'm gonna be back sometime,” Linsey says. “It could be two months, it could be two years. I don't know. It just doesn't work like that and your mortgage company doesn't care either.”
Madi is now more than eight years post-heart transplant, and Sydney is almost six years post-transplant, but both still have a cavalry of doctors they see regularly. Dearani says that for transplant families, the story never really comes to an end, even though right after the transplant it might feel that way.
“The children are on the edge of death before surgery," he says. "And then after the transplant, usually within weeks, they can be up, and they can be running around, and they can be walking in the hallways. And their color... Sometimes they go from blue to pink. The extremes are so dramatic in this particular area of pediatric cardiac, that it tends to be very rewarding, and really great for the patients and their families because they see that too.” But there is no future without worry, there is no such thing as “medicine-free" or "cured."
“Whereas most other things in health care, you have a problem, you get fixed, it's intense for a while, you get better. This isn’t like that.”
The anti-rejection medication can be hard on the kidneys, and many heart transplant kids need kidney transplants eventually too. And a new heart isn’t a guarantee. The average adult survival rate following heart transplantation currently is ten years, and data presented at a 2014 Annual Meeting of The Society of Thoracic Surgeons found that 54.3 percent of pediatric heart transplant cases survived at least 15 years. Linsey says it's always in the back of her mind, that ten or fifteen-year anniversary that no one wants to face.
But when her girls go in for an echocardiogram and the image of the heart comes up on the screen, Linsey says she’s overcome by a sense of extreme gratitude and also amazement. Inside both her daughters are hearts that used to belong to someone else, now beating in their chests, getting bigger with them, as they grow up to ages they wouldn't have reached on their own.
“I have total awe," she says, "at what has happened for these girls to be able to live.”
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