How 30 Years of Heart Surgeries Taught My Dad How to Live
[Editor's Note: This piece is the winner of our 2019 essay contest, which prompted readers to reflect on the question: "How has an advance in science or medicine changed your life?"]
My father did not expect to live past the age of 50. Neither of his parents had done so. And he also knew how he would die: by heart attack, just as his father did.
In July of 1976, he had his first heart attack, days before his 40th birthday.
My dad lived the first 40 years of his life with this knowledge buried in his bones. He started smoking at the age of 12, and was drinking before he was old enough to enlist in the Navy. He had a sarcastic, often cruel, sense of humor that could drive my mother, my sister and me into tears. He was not an easy man to live with, but that was okay by him - he didn't expect to live long.
In July of 1976, he had his first heart attack, days before his 40th birthday. I was 13, and my sister was 11. He needed quadruple bypass surgery. Our small town hospital was not equipped to do this type of surgery; he would have to be transported 40 miles away to a heart center. I understood this journey to mean that my father was seriously ill, and might die in the hospital, away from anyone he knew. And my father knew a lot of people - he was a popular high school English teacher, in a town with only three high schools. He knew generations of students and their parents. Our high school football team did a blood drive in his honor.
During a trip to Disney World in 1974, Dad was suffering from angina the entire time but refused to tell me (left) and my sister, Kris.
Quadruple bypass surgery in 1976 meant that my father's breastbone was cut open by a sternal saw. His ribcage was spread wide. After the bypass surgery, his bones would be pulled back together, and tied in place with wire. The wire would later be pulled out of his body when the bones knitted back together. It would take months before he was fully healed.
Dad was in the hospital for the rest of the summer and into the start of the new school year. Going to visit him was farther than I could ride my bicycle; it meant planning a trip in the car and going onto the interstate. The first time I was allowed to visit him in the ICU, he was lying in bed, and then pushed himself to sit up. The heart monitor he was attached to spiked up and down, and I fainted. I didn't know that heartbeats change when you move; television medical dramas never showed that - I honestly thought that I had driven my father into another heart attack.
Only a few short years after that, my father returned to the big hospital to have his heart checked with a new advance in heart treatment: a CT scan. This would allow doctors to check for clogged arteries and treat them before a fatal heart attack. The procedure identified a dangerous blockage, and my father was admitted immediately. This time, however, there was no need to break bones to get to the problem; my father was home within a month.
During the late 1970's, my father changed none of his habits. He was still smoking, and he continued to drink. But now, he was also taking pills - pills to manage the pain. He would pop a nitroglycerin tablet under his tongue whenever he was experiencing angina (I have a vivid memory of him doing this during my driving lessons), but he never mentioned that he was in pain. Instead, he would snap at one of us, or joke that we were killing him.
I think he finally determined that, if he was going to have these extra decades of life, he wanted to make them count.
Being the kind of guy he was, my father never wanted to talk about his health. Any admission of pain implied that he couldn't handle pain. He would try to "muscle through" his angina, as if his willpower would be stronger than his heart muscle. His efforts would inevitably fail, leaving him angry and ready to lash out at anyone or anything. He would blame one of us as a reason he "had" to take valium or pop a nitro tablet. Dinners often ended in shouts and tears, and my father stalking to the television room with a bottle of red wine.
In the 1980's while I was in college, my father had another heart attack. But now, less than 10 years after his first, medicine had changed: our hometown hospital had the technology to run dye through my father's blood stream, identify the blockages, and do preventative care that involved statins and blood thinners. In one case, the doctors would take blood vessels from my father's legs, and suture them to replace damaged arteries around his heart. New advances in cholesterol medication and treatments for angina could extend my father's life by many years.
My father decided it was time to quit smoking. It was the first significant health step I had ever seen him take. Until then, he treated his heart issues as if they were inevitable, and there was nothing that he could do to change what was happening to him. Quitting smoking was the first sign that my father was beginning to move out of his fatalistic mindset - and the accompanying fatal behaviors that all pointed to an early death.
In 1986, my father turned 50. He had now lived longer than either of his parents. The habits he had learned from them could be changed. He had stopped smoking - what else could he do?
It was a painful decade for all of us. My parents divorced. My sister quit college. I moved to the other side of the country and stopped speaking to my father for almost 10 years. My father remarried, and divorced a second time. I stopped counting the number of times he was in and out of the hospital with heart-related issues.
In the early 1990's, my father reached out to me. I think he finally determined that, if he was going to have these extra decades of life, he wanted to make them count. He traveled across the country to spend a week with me, to meet my friends, and to rebuild his relationship with me. He did the same with my sister. He stopped drinking. He was more forthcoming about his health, and admitted that he was taking an antidepressant. His humor became less cruel and sadistic. He took an active interest in the world. He became part of my life again.
The 1990's was also the decade of angioplasty. My father explained it to me like this: during his next surgery, the doctors would place balloons in his arteries, and inflate them. The balloons would then be removed (or dissolve), leaving the artery open again for blood. He had several of these surgeries over the next decade.
When my father was in his 60's, he danced at with me at my wedding. It was now 10 years past the time he had expected to live, and his life was transformed. He was living with a woman I had known since I was a child, and my wife and I would make regular visits to their home. My father retired from teaching, became an avid gardener, and always had a home project underway. He was a happy man.
Dancing with my father at my wedding in 1998.
Then, in the mid 2000's, my father faced another serious surgery. Years of arterial surgery, angioplasty, and damaged heart muscle were taking their toll. He opted to undergo a life-saving surgery at Cleveland Clinic. By this time, I was living in New York and my sister was living in Arizona. We both traveled to the Midwest to be with him. Dad was unconscious most of the time. We took turns holding his hand in the ICU, encouraging him to regain his will to live, and making outrageous threats if he didn't listen to us.
The nursing staff were wonderful. I remember telling them that my father had never expected to live this long. One of the nurses pointed out that most of the patients in their ward were in their 70's and 80's, and a few were in their 90's. She reminded me that just a decade earlier, most hospitals were unwilling to do the kind of surgery my father had received on patients his age. In the first decade of the 21st century, however, things were different: 90-year-olds could now undergo heart surgery and live another decade. My father was on the "young" side of their patients.
The Cleveland Clinic visit would be the last major heart surgery my father would have. Not that he didn't return to his local hospital a few times after that: he broke his neck -- not once, but twice! -- slipping on ice. And in the 2010's, he began to show signs of dementia, and needed more home care. His partner, who had her own health issues, was not able to provide the level of care my father needed. My sister invited him to move in with her, and in 2015, I traveled with him to Arizona to get him settled in.
After a few months, he accepted home hospice. We turned off his pacemaker when the hospice nurse explained to us that the job of a pacemaker is to literally jolt a patient's heart back into beating. The jolts were happening more and more frequently, causing my Dad additional, unwanted pain.
My father in 2015, a few months before his death.
My father died in February 2016. His body carried the scars and implants of 30 years of cardiac surgeries, from the ugly breastbone scar from the 1970's to scars on his arms and legs from borrowed blood vessels, to the tiny red circles of robotic incisions from the 21st century. The arteries and veins feeding his heart were a patchwork of transplanted leg veins and fragile arterial walls pressed thinner by balloons.
And my father died with no regrets or unfinished business. He died in my sister's home, with his long-time partner by his side. Medical advancements had given him the opportunity to live 30 years longer than he expected. But he was the one who decided how to live those extra years. He was the one who made the years matter.
60-Second Video: How Safe Are the COVID-19 Vaccines?
Comparing the Risks of COVID-19 vs. the Risks of the Vaccines:
Kira Peikoff was the editor-in-chief of Leaps.org from 2017 to 2021. As a journalist, her work has appeared in The New York Times, Newsweek, Nautilus, Popular Mechanics, The New York Academy of Sciences, and other outlets. She is also the author of four suspense novels that explore controversial issues arising from scientific innovation: Living Proof, No Time to Die, Die Again Tomorrow, and Mother Knows Best. Peikoff holds a B.A. in Journalism from New York University and an M.S. in Bioethics from Columbia University. She lives in New Jersey with her husband and two young sons. Follow her on Twitter @KiraPeikoff.
For Kids with Progeria, New Therapies May Offer Revolutionary Hope for a Longer Life
Sammy Basso has some profound ideas about fate. As long as he has been alive, he has known he has minimal control over his own. His parents, however, had to transition from a world of unlimited possibility to one in which their son might not live to his 20s.
"I remember very clearly that day because Sammy was three years old," his mother says of the day a genetic counselor diagnosed Sammy with progeria. "It was a devastating day for me."
But to Sammy, he has always been himself: a smart kid, interested in science, a little smaller than his classmates, with one notable kink in his DNA. In one copy of the gene that codes for the protein Lamin A, Sammy has a T where there should be a C. The incorrect code creates a toxic protein called progerin, which destabilizes Sammy's cells and makes him age much faster than a person who doesn't have the mutation. The older he gets, the more he is in danger of strokes, heart failure, or a heart attack. "I am okay with my situation," he says from his home in Tezze sul Brenta, Italy. "But I think, yes, fate has a great role in my life."
Just 400 or so people in the world live with progeria: The mutation that causes it usually arises de novo, or "of new," meaning that it is not inherited but happens spontaneously during gestation. The challenge, as with all rare diseases, is that few cases means few treatments.
"When we first started, there was absolutely nothing out there," says Leslie Gordon, a physician-researcher who co-founded the Progeria Research Foundation in 1999 after her own son, also named Sam, was diagnosed with the disease. "We knew we had to jumpstart the entire field, so we collected money through road races and special events and writing grants and all sorts of donors… I think the first year we raised $75,000, most of it from one donor."
"We have not only the possibility but the responsibility to make the world a better world, and also to make a body a better body."
By 2003, the foundation had collaborated with Francis Collins, a geneticist who is now director of the National Institutes of Health, to work out the genetic basis for progeria—that single mutation Sammy has. The discovery led to interest in lonafarnib, a drug that was already being used in cancer patients but could potentially operate downstream of the mutation, preventing the buildup of the defective progerin in the body. "We funded cellular studies to look at a lonafarnib in cells, mouse studies to look at lonafarnib in mouse models of progeria… and then we initiated the clinical trials," Gordon says.
Sammy Basso's family had gotten involved with the Progeria Research Foundation through their international patient registry, which maintains relationships with families in 49 countries. "We started to hear about lonafarnib in 2006 from Leslie Gordon," says Sammy's father, Amerigo Basso, with his son translating. "She told us about the lonafarnib. And we were very happy because for the first time we understood that there was something that could help our son and our lives." Amerigo used the Italian word speranza, which means hope.
Still, Sammy wasn't sure if lonafarnib was right for him. "Since when I was very young I thought that everything happens for a reason. So, in my mind, if God made me with progeria, there was a reason, and to try to heal from progeria was something wrong," he says. Gradually, his parents and doctors, and Leslie Gordon, convinced him otherwise. Sammy began to believe that God was also the force behind doctors, science, and research. "And so we have not only the possibility but the responsibility to make the world a better world, and also to make a body a better body," he says.
Sammy Basso and his parents.
Courtesy of Basso
Sammy began taking lonafarnib, with the Progeria Research Foundation intermittently flying him, and other international trial participants, to Boston for tests. He was immediately beset by some of the drug's more unpleasant side effects: Stomach problems, nausea, and vomiting. "The first period was absolutely the worst period of my life," he says.
At first, doctors prescribed other medicines for the side effects, but to Sammy it had as much effect as drinking water. He visited doctor after doctor, with some calling him weekly or even daily to ask how he was doing. Eventually the specialists decided that he should lower his dose, balancing his pain with the benefit of the drug. Sammy can't actually feel any positive effect of the lonafarnib, but his health measurements have improved relative to people with progeria who don't take it.
While they never completely disappeared, Sammy's side effects decreased to the point that he could live. Inspired by the research that led to lonafarnib, he went to university to study molecular biology. For his thesis work, he travelled to Spain to perform experiments on cells and on mice with progeria, learning how to use the gene-editing technique CRISPR-Cas9 to cut out the mutated bit of DNA. "I was so excited to participate in this study," Sammy says. He felt like his work could make a difference.
In 2018, the Progeria Research Foundation was hosting one of their biennial workshops when Francis Collins, the researcher who had located the mutation behind progeria 15 years earlier, got in touch with Leslie Gordon. "Francis called me and said, Hey, I just saw a talk by David Liu from the Broad [Institute]. And it was pretty amazing. He has been looking at progeria and has very early, but very exciting data… Do you have any spaces, any slots you could make in your program for late breaking news?"
Gordon found a spot, and David Liu came to talk about what was going on in his lab, which was an even more advanced treatment that led to mice with the progeria mutation living into their senior mouse years—substantially closer to a normal lifespan. Liu's lab had built on the idea of CRISPR-Cas9 to create a more elegant genetic process called base editing: Instead of chopping out mutated DNA, a scientist could chemically convert an incorrect DNA letter to the correct one, like the search and replace function in word processing software. Mice who had their Lamin-A mutations corrected this way lived more than twice as long as untreated animals.
Sammy was in the audience at Dr. Liu's talk. "When I heard about this base editing as a younger scientist, I thought that I was living in the future," he says. "When my parents had my diagnosis of progeria, the science knew very little information about DNA. And now we are talking about healing the DNA… It is incredible."
Lonafarnib (also called Zokinvy) was approved by the US Food and Drug Administration this past November. Sammy, now 25, still takes it, and still manages his side effects. With luck, the gift of a few extra years will act as a bridge until he can try Liu's revolutionary new gene treatment, which has not yet begun testing in humans. While Leslie Gordon warns that she's always wrong about things like this, she hopes to see the new base editing techniques in clinical trials in the next year or two. Sammy won't need to be convinced to try it this time; his thinking on fate has evolved since his first encounter with lonafarnib.
"I would be very happy to try it," he says. "I know that for a non-scientist it can be difficult to understand. Some people think that we are the DNA. We are not. The DNA is a part of us, and to correct it is to do what we are already doing—just better." In short, a gene therapy, while it may seem like science fiction, is no different from a pill. For Sammy, both are a new way to think about fate: No longer something that simply happens to him.