| May 1, 2016
The History of HLHS (and an open letter to the parents of HLHS babies)
I came across a journal article today and couldn’t not write about it. It cuts right to the core of why we are so passionate about funding CHD research. The article was a semi-longitudinal study following 98 children born with Hypoplastic Left Heart Syndrome in the state of Oregon from 1971-1986. I say “semi-longitudinal” because sadly none of the babies in the study lived very long. Of the 98 participants 15 died the day they were born, 30 died within a week, and 91 died within 30 days. When I first read that I thought, “didn’t any of the babies have surgery?” At that time, the stage 1 mediation called the Norwood Procedure was in practice but still considered experimental. The article goes on to say that 20 parents elected to pursue the experimental surgery and the results were “significantly improved” for those children. Statistics don’t define “significance” the same way I would. They indicated there was a significant improvement over the course of the first 30 days of life. That means most of these babies that endured open-heart surgery only made it about 30 days longer in life than their peers who did not have surgery. The article summarizes by stating, “hypoplastic left heart syndrome is a lethal congenital heart defect in children and poses management and ethical dilemmas.” At the time the article was published in 1990, only one of the 98 babies was still living.
As the parent of a child that had the Norwood Procedure at 7 days old, this one hits close to home. Could you imagine being those parents? At least when my son’s diagnosis was made I had time to prepare myself for what would happen as I was still pregnant. Also, after the doctors described the horrors of infant heart surgery they told us our son was a good candidate for survival and a some-what normal life. I’m sure the parents of these babies would have loved to hear those words, however, they were faced with the choice of comfort care and a few precious hours or days with their baby or an experimental procedure with unknown outcomes.
So to the parents who handed their babies over to a surgeon not knowing if they would ever see them again, thank you. Thank you for pioneering this front for parents like me. Thank you for being a part of the history of this now life-saving procedure. Thank you for enduring the agony of infant heart surgery in the hopes that your child’s life would be better for it, because my child’s life is certainly better because of it. Because of you my child was home sleeping in his own bed just 6 weeks after surgery. Because of you he learned to walk on Easter Sunday at his grandparents’ house. Because of you he goes to school and looks just like any other kid in his class. I thank God every day for his good works in our son’s life and for blessing his surgeon’s hands with the ability to do nothing short of miracles. But without you there would have been no progress. No way for the surgeons to perfect this procedure into something better and more effective with every passing year.
Now approximately 69% of children with critical single ventricle CHDs, like HLHS, make it to adulthood. A diagnosis that was a death sentence just a few decades ago has so much hope. From 0-69% survival is a huge step, but as a parent it’s still not good enough for me. I want to see my son not just “make it” to adulthood, but thrive into adulthood. I want to see him graduate from college and become a cardiologist and marry a cute girl who adores her mother-in-law and gives me the cutest dang grandbabies I’ve ever laid eyes on! Or, you know, whatever he sets his mind to. So that’s why we’re in this. That’s why we do Project Heart. Let’s stomp this CHD thing and make an impact so that parents another 40 years from now will look back and say, “dang, look how far we’ve come.”
Morris, CD. Pediatrics, 1990 Jun;85(6):977-83 “Hypoplastic Left Heart Syndrome: natural history in a geographically defined population.” Dept of Med, Oregon Health Sci Univ, Portland.