By Mara Koven-Gelman
When Jimmy Kimmel powerfully shared the intimate fact that his newborn son was born with a congenital heart defect (CHD) called Tetralogy of Fallot (ToF), every person, parent or loved one of someone with a CHD silently cheered. Not out of cruelty or lack of empathy, but because now ToF has a public champion. Every disease should be so lucky.
That night and for several after, Kimmel was a champion for health care and coverage for pre-existing conditions. He was also a champion for parents shocked into reality (congenital heart defects are discovered right after birth) and those who have a long “zipper” on their chests (surgery’s souvenir.)
I had a different reaction to Kimmel’s announcement. I am a ToF widow. My first husband, Jonathan Koven, was born in 1963 in Toronto with ToF. As a baby, he had a Blalock-Taussig shunt put in to keep oxygen flowing to his body. He had to wait until he was 7 years old to have a full repair to the four defects with his heart.
Billy Kimmel had two of four repairs done moments after birth. That’s how far medicine has come. Just last year, the American Medical Board of Specialties created a new subspecialty in adult congenital heart disease because these kids are growing into adults and need to be followed.
As a person ages, new problems can develop. In fact, there are more adult than pediatric CHD patients, with the number of adult cases in the United States estimated at 1.3 million.
Jonathan died at age 39, a sudden death due to scar tissue from the original surgery. We had two young children, ages 3 and 6.
I still see his life as a miracle – had he been born 25 years earlier, he may not have lived. Did I suspect he would die so young? Not really. His doctors called his surgery a full repair. But clearly there is always the risk. He was in an adult clinical study and was followed by some of the field’s giants in CHD. But he still died because life is fragile and not all defects are fixable.
Jonathan’s cutting-edge surgery was performed by Dr. William Mustard, who pioneered the now-outdated “Mustard Procedure.” Under the Ontario Health Insurance Plan, the operation and monthlong stay in Sick Children’s Hospital cost his immigrant parents only the train fare to go back and forth between Toronto and the nearby town of Oakville.
It must have been a frightening time for his working parents, who were without family supports and couldn’t take time off to be with their son. Despite not having financial reserves, my in-laws demanded Jonathan have round-the-clock care.
“We had no idea of the cost, but it didn’t matter,” his mother told me recently. The doctor told her not to worry, that Jonathan already had a private nurse and all costs were covered.
The Adult Congenital Heart Association’s website offers free help and resources. Its Facebook page has numerous posts from people with ToF. They post their age, year of repair and subsequent procedures. “Hey, I had my repair in 1990 and doing great!”
Billy, like Jonathan, will have a life of follow-up care and vigilance by experts in pediatric and adult CHD. Hopefully, there will be fewer deaths like Jonathan’s, especially if people work with their ACHD centers. Buffalo’s closest one is either Strong Memorial in Rochester or the Cleveland Clinic.
Why was my reaction about Kimmel’s famous sharing different from most people? It’s this: Take it from me – if you have CHD, get followed like your life depends on it.