This article contains references to specific patients and therefore has been written, reviewed, and published with their full permission. Originally published July 26, 2023. Updated August 2, 2023.
Congenital heart defects are the most common birth defect in humans, affecting 1% of the world’s population. In the United States alone, 40,000 babies are born with CHD every year.1. Of those babies, about 25% will have a critical CHD, requiring surgery in the first year of life.1 There is no cure.
In the last few decades, advances in surgical techniques and research mean that today, about 95% of those born with non-critical CHD and 69% of those born with critical CHD will live to see adulthood.1 Those medical advances were largely in the pediatric space and focused heavily on survival.
In recent years, estimates show that over 1.4 million adults are living with CHD in our country.1 But for this population, the U.S. medical system is woefully underprepared to treat them. You may have seen stories from actors, politicians, or athletes living with CHD who seem to have no issues receiving appropriate care, but what about everyone else?
Meet Julie, a 53-year-old woman living with critical congenital heart defects. She is a daughter, twin sister, and loving mother. Julie has miraculously outlived every grim prediction doctors made in her early childhood, despite the odds being overwhelmingly stacked against her.
For babies like Julie, born with congenital heart defects between 1970-1974, the biggest hurdle was surviving the first year of life. If they did reach their first birthday, the chances of surviving to adulthood were just 77-83%.2
Heart surgery on infants did not become commonplace in the United States until the early 1970s. After a year of infant CHD surgery at Boston Children’s Hospital in 1972, surgeons had some data to work with. They realized that babies with critical CHD who died generally did so in the first few weeks of life.2 The rest of that decade saw a shift from surgery in infancy to surgery in the newborn period for these babies, with the first successful procedure coming in 1983 on an 11-day-old.2
Julie had her first open-heart surgery at the age of 5 1/2 in 1976. Her childhood was filled with summer vacations, school, birthdays, and lots of visits to the pediatric cardiologist. Because there were no specialists for adults with congenital heart defects until 2015, Julie continued to see her pediatric cardiologist as an adult. Her heart remained stable until her mid-20s.
In 1995, at age 25, Julie gave birth to a beautiful, healthy baby girl. Researchers had barely begun to study adults with CHD and no one told Julie she should have additional cardiac care and monitoring during pregnancy (probably because they didn’t know yet). Standards of care for adults with CHD were not issued until 2008 4 with the next update coming in 2018. 5
After the birth of her daughter, Julie’s heart really struggled. By age 26, in 1996, she was back in the hospital for her second open-heart surgery. There was no road map for Julie’s medical team to follow and they were dealing with a long list of heart-related issues in her body. Not to mention, Julie lived in Nevada, where the healthcare system could barely serve its healthy population.6
The surgery did not go as planned and took far longer than anticipated. As the procedure entered its seventh hour, extensive bleeding and the length of time on the bypass machine forced the surgeons to place Julie into a coma, with an open chest. She wouldn’t wake up for almost two weeks.
What happened during her 13-day coma is largely unknown. Medical records were not easy to access, and communication from doctors to family members was limited. Julie woke from the coma completely paralyzed on her right side, unable to hold, feed, or care for her young daughter. She didn’t know it at the time, but this would be a turning point for her health.
Facing yet another uphill battle in her young life, Julie persevered and recovered function on her right side with months of intensive therapies. She would go on living life, raising her daughter, and continuing to follow up with her pediatric cardiologist.
As she aged, additional health conditions arose and everyday tasks became more difficult. Julie could feel that something was happening in her body, but unfortunately, Nevada still did not have any adult CHD specialists that could investigate. Despite the herculean efforts of her pediatric cardiologists, Julie declined into heart failure and would require a full evaluation by a specialized adult CHD center.
The team at UCLA’s Adult Congenital Heart Disease Center confirmed Julie’s heart failure diagnosis, but she was blindsided by the severity of her disease. Without knowing they even existed, Julie never had the chance to use the ACHD care standards for her anatomy,5 and was now looking at evaluation for a heart transplant.
If Julie’s life were a feature film, it would have been at that moment when the screeching tires sound filled the room. A heart transplant?!
While processing that earth-shattering news, Julie was reminded that her home state did not perform heart transplants. In fact, with only one transplant center, Nevada could only perform kidney and liver transplants. She would have to leave her home, her family, and her support system to survive. And she is not the only one in this terrible position.
In October of last year, UCLA physicians told her there was no more time; Julie needed to start the transplant process as soon as possible. They already knew what she would learn quickly: out-of-state organ transplants take considerable time and finances before the clinical evaluation ever begins.
From that day in October 2022 until last month, June 2023, Julie spent hours every day completing paperwork and making calls to her insurance company, all while managing her failing heart.
Part of that paperwork required Julie to prove to the transplant center that she could afford to move to California before the procedure, and also live there for an estimated 3-12 months afterwards. As she crunched numbers, Julie felt hopeless.
- According to Rent Cafe7, the average monthly rent in LA is $2,781
- Transportation to/from medical visits, food, and medical costs not covered by health insurance could easily run $2,000 per month
- Her monthly bills at home wouldn’t stop, so there was also that to consider
Rough estimates put the total she would have to cover around $85,000. Julie couldn’t even consider what her co-insurance might be after the policy paid for the transplant and hospital stay. $500,000? $750,000? $1,000,000? Present estimates for a heart transplant and related hospital stay is $1.8 million.
Even though Nevada cannot perform heart transplants, there is no state funding earmarked for patients like Julie. Health insurance policies won’t cover services at a higher rate based on having to leave the state. Public programs where the transplant will take place are not an option because Julie is not a resident of California. Like thousands of other individuals awaiting an organ transplant, Julie was advised to fundraise.
How You Can Help
❤️ Please share Julie’s story!
❤️ Contact your politicians and ask them to provide healthcare for all Nevadans.
❤️ Contribute to Julie’s medical expense fundraiser on GoFundMe.
How is Julie Doing?
Julie was listed for a heart transplant at status 2E on July 11, 2023. She remains hospitalized at UCLA Ronald Reagan Medical Center until an organ becomes available.
On July 31, our Advocate visited Julie at the hospital. Later that evening, the call came – she had been matched. Around 7:30pm on August 1, Julie was wheeled into the operating room to receive her new heart. Surgery was successful and now Julie begins the long recovery.
- Data and Statistics on Congenital Heart Defects, Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities
- Temporal Trends in Survival to Adulthood Among Patients Born With Congenital Heart Disease, P. Moons et al, November 2010
- Congenital Heart Disease: A Surgical-Historical Perspective, A. Castañeda, June 2005
- Guidelines for the Management of Adults with Congenital Heart Disease, American College of Cardiology & American Heart Association, November 2008
- Guidelines for the Management of Adults with Congenital Heart Disease: A Report on Clinical Practice Guidelines, American College of Cardiology & American Heart Association, April 2019
- Continual Evolution: Healthcare in Nevada, Nevada Business Magazine, April 2019
- Cost of Living in Los Angeles California, Rent Cafe