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.
How a Deadly Fire Gave Birth to Modern Medicine
On the evening of November 28, 1942, more than 1,000 revelers from the Boston College-Holy Cross football game jammed into the Cocoanut Grove, Boston's oldest nightclub. When a spark from faulty wiring accidently ignited an artificial palm tree, the packed nightspot, which was only designed to accommodate about 500 people, was quickly engulfed in flames. In the ensuing panic, hundreds of people were trapped inside, with most exit doors locked. Bodies piled up by the only open entrance, jamming the exits, and 490 people ultimately died in the worst fire in the country in forty years.
"People couldn't get out," says Dr. Kenneth Marshall, a retired plastic surgeon in Boston and president of the Cocoanut Grove Memorial Committee. "It was a tragedy of mammoth proportions."
Within a half an hour of the start of the blaze, the Red Cross mobilized more than five hundred volunteers in what one newspaper called a "Rehearsal for Possible Blitz." The mayor of Boston imposed martial law. More than 300 victims—many of whom subsequently died--were taken to Boston City Hospital in one hour, averaging one victim every eleven seconds, while Massachusetts General Hospital admitted 114 victims in two hours. In the hospitals, 220 victims clung precariously to life, in agonizing pain from massive burns, their bodies ravaged by infection.
The scene of the fire.
Boston Public Library
Tragic Losses Prompted Revolutionary Leaps
But there is a silver lining: this horrific disaster prompted dramatic changes in safety regulations to prevent another catastrophe of this magnitude and led to the development of medical techniques that eventually saved millions of lives. It transformed burn care treatment and the use of plasma on burn victims, but most importantly, it introduced to the public a new wonder drug that revolutionized medicine, midwifed the birth of the modern pharmaceutical industry, and nearly doubled life expectancy, from 48 years at the turn of the 20th century to 78 years in the post-World War II years.
The devastating grief of the survivors also led to the first published study of post-traumatic stress disorder by pioneering psychiatrist Alexandra Adler, daughter of famed Viennese psychoanalyst Alfred Adler, who was a student of Freud. Dr. Adler studied the anxiety and depression that followed this catastrophe, according to the New York Times, and "later applied her findings to the treatment World War II veterans."
Dr. Ken Marshall is intimately familiar with the lingering psychological trauma of enduring such a disaster. His mother, an Irish immigrant and a nurse in the surgical wards at Boston City Hospital, was on duty that cold Thanksgiving weekend night, and didn't come home for four days. "For years afterward, she'd wake up screaming in the middle of the night," recalls Dr. Marshall, who was four years old at the time. "Seeing all those bodies lined up in neat rows across the City Hospital's parking lot, still in their evening clothes. It was always on her mind and memories of the horrors plagued her for the rest of her life."
The sheer magnitude of casualties prompted overwhelmed physicians to try experimental new procedures that were later successfully used to treat thousands of battlefield casualties. Instead of cutting off blisters and using dyes and tannic acid to treat burned tissues, which can harden the skin, they applied gauze coated with petroleum jelly. Doctors also refined the formula for using plasma--the fluid portion of blood and a medical technology that was just four years old--to replenish bodily liquids that evaporated because of the loss of the protective covering of skin.
"Every war has given us a new medical advance. And penicillin was the great scientific advance of World War II."
"The initial insult with burns is a loss of fluids and patients can die of shock," says Dr. Ken Marshall. "The scientific progress that was made by the two institutions revolutionized fluid management and topical management of burn care forever."
Still, they could not halt the staph infections that kill most burn victims—which prompted the first civilian use of a miracle elixir that was being secretly developed in government-sponsored labs and that ultimately ushered in a new age in therapeutics. Military officials quickly realized this disaster could provide an excellent natural laboratory to test the effectiveness of this drug and see if it could be used to treat the acute traumas of combat in this unfortunate civilian approximation of battlefield conditions. At the time, the very existence of this wondrous medicine—penicillin—was a closely guarded military secret.
From Forgotten Lab Experiment to Wonder Drug
In 1928, Alexander Fleming discovered the curative powers of penicillin, which promised to eradicate infectious pathogens that killed millions every year. But the road to mass producing enough of the highly unstable mold was littered with seemingly unsurmountable obstacles and it remained a forgotten laboratory curiosity for over a decade. But Fleming never gave up and penicillin's eventual rescue from obscurity was a landmark in scientific history.
In 1940, a group at Oxford University, funded in part by the Rockefeller Foundation, isolated enough penicillin to test it on twenty-five mice, which had been infected with lethal doses of streptococci. Its therapeutic effects were miraculous—the untreated mice died within hours, while the treated ones played merrily in their cages, undisturbed. Subsequent tests on a handful of patients, who were brought back from the brink of death, confirmed that penicillin was indeed a wonder drug. But Britain was then being ravaged by the German Luftwaffe during the Blitz, and there were simply no resources to devote to penicillin during the Nazi onslaught.
In June of 1941, two of the Oxford researchers, Howard Florey and Ernst Chain, embarked on a clandestine mission to enlist American aid. Samples of the temperamental mold were stored in their coats. By October, the Roosevelt Administration had recruited four companies—Merck, Squibb, Pfizer and Lederle—to team up in a massive, top-secret development program. Merck, which had more experience with fermentation procedures, swiftly pulled away from the pack and every milligram they produced was zealously hoarded.
After the nightclub fire, the government ordered Merck to dispatch to Boston whatever supplies of penicillin that they could spare and to refine any crude penicillin broth brewing in Merck's fermentation vats. After working in round-the-clock relays over the course of three days, on the evening of December 1st, 1942, a refrigerated truck containing thirty-two liters of injectable penicillin left Merck's Rahway, New Jersey plant. It was accompanied by a convoy of police escorts through four states before arriving in the pre-dawn hours at Massachusetts General Hospital. Dozens of people were rescued from near-certain death in the first public demonstration of the powers of the antibiotic, and the existence of penicillin could no longer be kept secret from inquisitive reporters and an exultant public. The next day, the Boston Globe called it "priceless" and Time magazine dubbed it a "wonder drug."
Within fourteen months, penicillin production escalated exponentially, churning out enough to save the lives of thousands of soldiers, including many from the Normandy invasion. And in October 1945, just weeks after the Japanese surrender ended World War II, Alexander Fleming, Howard Florey and Ernst Chain were awarded the Nobel Prize in medicine. But penicillin didn't just save lives—it helped build some of the most innovative medical and scientific companies in history, including Merck, Pfizer, Glaxo and Sandoz.
"Every war has given us a new medical advance," concludes Marshall. "And penicillin was the great scientific advance of World War II."
This Boy Struggled to Walk Before Gene Therapy. Now, Such Treatments Are Poised to Explode.
Conner Curran was diagnosed with Duchenne's muscular dystrophy in 2015 when he was four years old. It's the most severe form of the genetic disease, with a nearly inevitable progression toward total paralysis. Many Duchenne's patients die in their teens; the average lifespan is 26.
But Conner, who is now 10, has experienced some astonishing improvements in recent years. He can now walk for more than two miles at a time – an impossible journey when he was younger.
In 2018, Conner became the very first patient to receive gene therapy specific to treating Duchenne's. In the initial clinical trial of nine children, nearly 80 percent reacted positively to the treatment). A larger-scale stage 3 clinical trial is currently underway, with initial results expected next year.
Gene therapy involves altering the genes in an individual's cells to stop or treat a disease. Such a procedure may be performed by adding new gene material to existing cells, or editing the defective genes to improve their functionality.
That the medical world is on the cusp of a successful treatment for a crippling and deadly disease is the culmination of more than 35 years of work by Dr. Jude Samulski, a professor of pharmacology at the University of North Carolina School of Medicine in Chapel Hill. More recently, he's become a leading gene therapy entrepreneur.
But Samulski likens this breakthrough to the frustrations of solving a Rubik's cube. "Just because one side is now all the color yellow does not mean that it is completely aligned," he says.
Although Conner's life and future have dramatically improved, he's not cured. The gene therapy tamed but did not extinguish his disorder: Conner is now suffering from the equivalent of Becker's muscular dystrophy, a milder form of the disease with symptoms that appear later in life and progress more slowly. Moreover, the loss of muscle cells Conner suffered prior to the treatment is permanent.
"It will take more time and more innovations," Samulski says of finding an even more effective gene therapy for muscular dystrophy.
Conner's family is still overjoyed with the results. "Jude's grit and determination gave Conner a chance at a new life, one that was not in his cards before gene therapy," says his mother Jessica Curran. She adds that "Conner is more confident than before and enjoys life, even though he has limitations, if compared to his brothers or peers."
Conner Curran holding a football post gene therapy treatment.
Courtesy of the Curran family
For now, the use of gene therapy as a treatment for diseases and disorders remains relatively isolated. On paper at least, progress appears glacially slow. In 2018, there were four FDA-approved gene therapies (excluding those reliant on bone marrow/stem cell transplants or implants). Today, there are 10. One therapy is solely for the cosmetic purpose of reducing facial lines and folds.
Nevertheless, experts in the space believe gene therapy is poised to expand dramatically.
"Certainly in the next three to five years you will see dozens of gene therapies and cell therapies be approved," says Dr. Pavan Cheruvu, who is CEO of Sio Gene Therapies in New York. The company is developing treatments for Parkinson's disease and Tay-Sachs, among other diseases.
Cheruvu's conclusion is supported by NEWDIGS, a think tank at the Massachusetts Institute of Technology that keeps tabs on gene therapy developments. NEWDIGS predicts there will be at least 60 gene therapies approved for use in the U.S. by the end of the decade. That number could be closer to 100 if Chinese researchers and biotech ventures decide the American market is a good fit for the therapies they develop.
"We are watching something of a conditional evolution, like a dot-com, or cellphones that were sizes of shoeboxes that have now matured to the size of wafers. Our space will follow along very similarly."
Dr. Carsten Brunn, a chemist by training and CEO of Selecta Biosciences outside of Boston, is developing ways to reduce the immune responses in patients who receive gene therapy. He observes that there are more than 300 therapies in development and thousands of clinical trials underway. "It's definitely an exciting time in the field," he says.
That's a far cry from the environment of little more than a decade ago. Research and investment in gene therapy had been brought low for years after the death of teenager Jesse Gelsinger in 1999 while he had been enrolled in a clinical trial to treat a liver disease. Gene therapy was a completely novel concept back then, and his death created existential questions about whether it was a proper pathway to pursue. Cheruvu, a cardiologist, calls the years after Gelsinger's death an "ice age" for gene therapy.
However, those dark years eventually yielded to a thaw. And while there have been some recent stumbles, they are considered part of the trial-and-error that has often accompanied medical research as opposed to an ominous "stop" sign.
The deaths of three patients last year receiving gene therapy for myotubular myopathy – a degenerative disease that causes severe muscle weakness – promptly ended the clinical trial in which they were enrolled. However, the incident caused few ripples beyond that. Researchers linked the deaths to dosage sizes that caused liver toxicity, as opposed to the gene therapy itself being an automatic death sentence; younger patients who received lower doses due to a less advanced disease state experienced improvements.
The gene sequencing and editing that helped create vaccines for COVID-19 in record time also bolstered the argument for more investment in research and development. Cheruvu notes that the field has usually been the domain of investors with significant expertise in the field; these days, more money is flowing in from generalists.
The Challenges Ahead
What will be the next step in gene therapy's evolution? Many of Samulski's earliest innovations came in the laboratory, for example. Then that led to him forming a company called AskBio in collaboration with the Muscular Dystrophy Association. AskBio sold its gene therapy to Pfizer five years ago to assure that enough could be manufactured for stage 3 clinical trials and eventually reach the market.
Cheruvu suggests that many future gene therapy innovations will be the result of what he calls "congruent innovation." That means publicly funded laboratories and privately funded companies might develop treatments separately or in collaboration. Or, university scientists may depend on private ventures to solve one of gene therapy's most vexing issues: producing enough finished material to test and treat on a large scale. "Manufacturing is a real bottleneck right now," Brunn says.
The alternative is referred to in the sector as the "valley of death": a lab has found a promising treatment, but is not far enough along in development to submit an investigational new drug application with the FDA. The promise withers away as a result. But the new abundance of venture capital for gene therapy has made this scenario less of an issue for private firms, some of which have received hundreds of millions of dollars in funding.
There are also numerous clinical challenges. Many gene therapies use what are known as adeno-associated virus vectors (AAVs) to deliver treatments. They are hollowed-out husks of viruses that can cause a variety of mostly mild maladies ranging from colds to pink eye. They are modified to deliver the genetic material used in the therapy. Most of these vectors trigger an antibody reaction that limits treatments to a single does or a handful of smaller ones. That can limit the potential progress for patients – an issue referred to as treatment "durability."
Although vectors from animals such as horses trigger far less of an antibody reaction in patients -- and there has been significant work done on using artificial vectors -- both are likely years away from being used on a large scale. "For the foreseeable future, AAV is the delivery system of choice," Brunn says.
Also, there will likely be demand for concurrent gene therapies that can lead to a complete cure – not only halting the progress of Duchenne's in kids like Conner Curran, but regenerating their lost muscle cells, perhaps through some form of stem cell therapy or another treatment that has yet to be devised.
Nevertheless, Samulski believes demand for imperfect treatments will be high – particularly with a disease such as muscular dystrophy, where many patients are mere months from spending the remainder of their lives in wheelchairs. But Samulski believes those therapies will also inevitably evolve into something far more effective.
"We are watching something of a conditional evolution, like a dot-com, or cellphones that were sizes of shoeboxes that have now matured to the size of wafers," he says. "Our space will follow along very similarly."
Jessica Curran will remain forever grateful for what her son has received: "Jude gave us new hope. He gave us something that is priceless – a chance to watch Conner grow up and live out his own dreams."