Sarah, born 1990
Sarah was born at Hartford Hospital on December 10, 1990. She was a very sleepy baby, but otherwise seemed fine. At her two week checkup, the pediatricians detected what they thought might be a serious heart defect and had us drive directly to the pediatric cardiologists' office at Hartford Hospital.
The pediatric cardiologist determined that Sarah had Hypoplastic Left Heart Syndrome. Her left ventricle was 30% of normal size and was badly deformed. Sarah's aortic valve was extremely small, she had coarctation of the aorta, and her mitral valve was also deformed. Sarah was in congestive heart failure and was immediately admitted to Hartford Hospital's ICU.
After three days in the ICU, Sarah was stabilized, but her prospects for survival looked bleak. The Hartford cardiologist told us that there were three options for Sarah: 1) pursue a heart transplant at Loma Linda Hospital in California; 2) pursue the new three step Norwood surgery for HLHS at Children's Hospital Boston or Children's Hospital of Philadelphia; or 3) provide comfort care, with the understanding that Sarah would only live 6-8 weeks.
Our Hartford cardiologist recommended comfort care. He told us that the neonatal heart transplant program at Loma Linda was not viewed positively by the vast majority of US pediatric cardiologists. Further, our doctor had sent three children to Boston for the three step surgeries. Not only had all three children died, but they had died after a great deal of suffering.
Even though Hartford patients had not fared well with the three step surgery in Boston, our doctor wanted us to talk to a particular cardiologist at Children's Hospital, Dr. Michael Freed. Because it was Christmas time, Dr. Freed was away on vacation. So we took Sarah home and waited for Dr. Freed to return.
While we were waiting , my husband and I (both lawyers) set to work doing research. This was before the internet, so I spent hours in the UConn Medical School library printing out every article I could find about HLHS. Meanwhile, my husband talked to everyone he could find who knew someone who had had a child with HLHS.
The results of our research were sobering. Our research confirmed that Loma Linda's neonatal transplant program was not well regarded and had a poor survival record. Similarly, the three step surgeries for HLHS were just in their infancy. Less than one third of the children survived the three surgeries, and those who did survive often had significant disabilities.
It was at this time that we established the philosophy that would guide our decisions regarding Sarah's health. Our goal was not to have Sarah survive at all costs, but rather to have her survive only if she could have a good quality of life without suffering.
Armed with our research and our philosophy, we took Sarah to Boston on an outpatient basis. Dr. Freed examined all of the test results and walked us through the details of the three part surgery. We told Dr. Freed that we had serious concerns about the survival rates for the surgeries and the suffering that our Hartford doctor's patients had endured. We asked Dr. Freed to consider whether another less drastic approach might be possible , one that did not require the complete replumbing of the heart.
Dr. Freed suggested that performing an aortic valvotomy (the opening up of the narrowed aortic valve with a balloon catheter) might possibly allow the left ventricle to grow. We decided to pursue this option on the theory that, if the ventricle didn't grow,we could still consider the three part surgery in the future. We scheduled the procedure for three days later.
We took Sarah home to Connecticut to spend the weekend with family. On the way back to Children's Hospital on Sunday, we stopped at our synagogue and held a naming ceremony for Sarah in the Rabbi's study.
The balloon valvotomy was performed the next morning. The doctors were extremely pleased with the result, they had succeeded in opening up the aortic valve significantly without causing any leaking. Sarah stayed at Children's Hospital for the next ten days and then we took her home to see if the ventricle would grow.
Sarah suffered from failure to thrive. She was simply incapable of taking in enough calories to maintain her weight. She received almost all of her nutrition through a nasogastric tube for the first year of her life.
Despite the failure to thrive, the left ventricle grew and by the time Sarah was nine months old, we began to believe that she would survive long term. In a relatively short period of time, the ventricle grew to 100% of normal size and was functioning normally.
When Sarah turned one, her gastroenterologist inserted a gastric tube because it became obvious that she was not going to be able to gain weight on her own for some time. At first, she received both daytime and nighttime feedings through the gastric tube. Sarah attended "feeding lessons" at Hartford Hospital for many months in an effort to teach her how to eat. As Sarah began to eat on her own, we eliminated the daytime tube feedings, but she continued to receive nighttime tube feedings until she was five years old. The tube was finally removed when Sarah was six years old.
Sarah has had four additional cardiac catheterizations over the years, including two more balloon valvotomies in fifth grade and eighth grade. Sarah has never had open heart surgery. Her aortic stenosis prevents her from running, but she is an avid hiker and rock scrambler, and she walks faster than anyone I know.
Sarah is now nineteen years old. She attends the University of Connecticut on a full merit scholarship and plans to become a high school history teacher. You can see a picture of Sarah on the December page of the 2010 Little Hearts calendar.
Story by Sarah's mom, Alison - CONNECTICUT
2018 UPDATE from SARAH
After completing undergraduate degrees in history, psychology, and secondary education in 2012, I taught in an adolescent psychiatric unit in London, England and graduated from UCONN with a MA in Curriculum and Instruction in 2013. To address progression of aortic stenosis, I had an aortic valve replacement at Boston Children's Hospital in 2014 and am feeling great. I have traveled to six continents, and enjoy active outdoor activities, including hiking, biking, and kayaking. As of August 2018, I will be entering my sixth year as a middle school social studies teacher and also the Pre-K Social Studies Curriculum Specialist for the Portland, CT schools. To read about my most recent adverture in Antarctica, visit www.educatorexplorer.com