We went for our 20 week ultrasound in December 2004. The position of the baby made it difficult to view all four quadrants of the heart, so we rescheduled for January 2005. Since I have a history of Coarctation of the Aorta (COA), our visit would be with a pediatric cardiologist … just in case. January 10th is a day we will never forget. The pediatric cardiologist diagnosed and explained the condition Hypoplastic Left Heart Syndrome (HLHS). We were devastated.
We began researching the disease, treatment options, facilities, and more. We learned that HLHS can be managed with a series of three open heart procedures during the first three to five years of life. Later in life, hopefully much later, the decision regarding the need for a heart transplant will also need to be made. We learned that there is no cure for HLHS and that our baby would never be “okay”. She would only be managed. We also learned that not long ago babies with this condition were sent home to die. We knew this was not an option and we would give our baby every chance at life.
We finally decided on delivering in Boston in order for our baby to have surgery at Children’s Hospital Boston. This was not an easy decision since we live so far away, over 1500 miles. This would mean multiple lengthy trips to Boston to receive care.
Our baby girl, Hannah Susanne, was born on May 4, 2005 at Brigham and Women’s Hospital in Boston, Massachusetts. She was a beautiful pink baby. Looking at her, you would never know something was so wrong with her heart.
Two days later, Hannah had her first open heart surgery known as the Modified Norwood Sano Shunt Surgery. She had some difficulties with fluid balance and required a tiny amount of oxygen. She finally overcame those obstacles and was discharged on May 24th.
We had a few scares in the following months. Hannah was admitted to Our Lady of the Lake Regional Medical Center (OLOLRMC) Pediatric Intensive Care Unit (PICU) twice and to the Emergency Room once. Most of the issues were related to her oxygen saturations dropping into the 50 – 60 percentiles. As a reference, a healthy person has saturations around 95-100 percent. Hannah was expected to be in the 70’s.
On September 22, 2005, at four-and-a-half months of age, Hannah had her second open heart surgery known as The Bidirectional Glenn Shunt Surgery. She did well following her surgery and we were home within two weeks. However, complications became apparent less than 24 hours after returning home. She was admitted to OLOLRMC with oxygen saturations in the 40’s to 50’s percentile. She was diagnosed with having fluid around her heart and left lung. The doctors drained the fluid around her heart and the following day it had accumulated once again. The following day, Hannah was medically transported back to Children’s Hospital Boston. Hannah had an occluded Left Pulmonary Artery that required interventions during her heart catheterization. We spent three and a half weeks in the Cardiac ICU with many ups and downs. Hannah had some complicated lung issues that were never understood. She spent many days on a ventilator before her lungs healed enough to support her on their own. We finally returned home November 3, 2005 after nearly 6 weeks in Boston.
Two years later, Hannah returned to Boston for the third of the staged procedures, known as the Intracardiac Fontan Surgery. This surgery took place on September 24, 2007. Two days following surgery, Hannah continued to show signs of distress. She was in “ARDS” (acute respiratory distress syndrome) and showed signs of DIC (Disseminated Intravascular Coagulation). It was unclear as to why this was happening and talks of reversing her surgery were mentioned. On September 26th, Hannah was placed on ECMO (extracorporeal membrane oxygenation), which is also known as a “heart-lung” machine, in order to give her lungs a much needed break. Just as the time before, the reason for her lung complications was never completely understood. However, we do know that with each surgery, Hannah’s complications are more severe.
Five days later, Hannah was taken off of ECMO and the following week she was taken off of the ventilator. We soon moved to the “step-down” unit in the hopes of going home soon. However, Hannah developed a left lung effusion that kept us in the hospital for another four weeks. Once the chylous effusion seemed to be under control, Hannah was medically lifted back home to OLOLRMC’s PICU where she remained until she was discharged on November 8,2007.
Since then, Hannah has been a thriving little girl. She is a thriving 5 year old who enjoys kindergarten, playing with her friends and dancing. She is currently on two medications, aspirin and a blood pressure medication. We have routine chest x-rays and echocardiograms with our local pediatric cardiologist, Dr. Hixon. We are so blessed to have incredible pediatric cardiologists in Baton Rouge. We are also so blessed that Children’s Hospital Boston was an option for us.
Summary of Medical Treatments
- 5 trips to Boston
- 9 hospital admits totaling 122 days
- 6 ambulance trips
- 3 open heart surgeries
- 7 heart cateterizations
- 1 ECMO placement
- 1 emergent medical flight to Children’s Hospital Boston Cardiac Intensive Care Unit
- 1 urgent medical flight to Our Lady of the Lake Pediatric Intensive Care Unit
Surgeries and Heart Catheterizations
- May 6, 2005: Stage 1: Norwood Procedure Modified Sano Version
- September 20, 2005: Heart catheterization prior to surgery #2
- September 22, 2005: Stage 2: Bidrectional Glenn
- October 7, 2005: Heart catheterization due to complications
- October 18, 2005: Heart catheterization due to complications
- August 1, 2006: Heart catheterization post Glenn follow up
- September 21, 2007: Heart catheterization prior to surgery #3
- September 24, 2007: Stage 3: Intracardiac Fontan
- September 26, 2007: Heart catheterization due to complications
- September 26, 2007: Placed on ECMO
- October 15, 2007: Heart catheterization due to complications