Princess Isabelle's Story: Birth and life Pre-Fontan

Isabelle was born on Feb 4th, 2005 via c-section. That day was a magical day for us and it was the day we were introduced to our miracle baby! Isabelle was diagnosed in-utero with a heart defect and it wasn't until 3 days after her birth that we found out that she had a set of complex heart defects that put her into the "single ventricle" classification. She was ultimately diagnosed with ASD (Atrial Septal Defect), VSD (Ventricle Septal Defect), HLV (Hypo-plastic Left Ventricle) and Mitral Stenosis. Because she was so small at birth (5 lbs), we had to wait until she was larger before we could consider surgery. We were told that our only option was to go with the 3-stage Fontan Series.

The First Operation: The Norwood
The medical team cared for Isabelle in the NICU at Children's Mercy for nearly five weeks before scheduling her for her first operation: the Norwood. The surgery performed the DKS/modified Norwood procedure on March 8, 2005. Dr. O’Brien and his team performed the surgery. Afterwards, she went to the PICU, where she stayed for two weeks. Before we could take her home, we needed to make sure she was stable and could keep food down. Unfortunately, due to her condition (which frequently caused loss of appetite), she had to be fitted with an NG tube. To our delight, we were able to take her home on March 24. We already had her nursery ready for her when she got home from the hospital, and we were thrilled to have her home.

The Second Operation: The Glenn
Isabelle’s hospitalizations and surgeries delayed her gross motor skills. Fortunately, she developed well during the four months following her first surgery. Despite a battle with the flu in July 2005, the doctors deemed her healthy enough to handle her next operation: the Glenn. On Aug. 17, 2005, the surgeons performed the second operation. The surgery went very smoothly, and she stayed in the PICU for only two weeks before coming home.

Recovering from Surgery
After Isabelle had her first two surgeries, she stayed in good health except for a few colds. To prevent Isabelle from getting RSV, her doctor prescribed Synagis. The medicine worked, and Isabelle managed to make it through the cold season without contracting this virus.

Following her surgeries, her gross motor skills fell behind. At 13 months, however, she started walking.

Her most daunting challenge was eating. After a lot of occupational therapy, we got her eating issues under control. The doctors then weaned her off the NG tube in early June, and we never looked back.

Fall, Winter, Spring passed without event (a few colds, etc). Isabelle was on Synagis each month to prevent RSV. We were lucky to not have to deal with RSV. Isabelle learned to walk around 13 months and her overall gross motor skills were only a little behind. It was eating that was still giving her the most challenges.

Sisters Forever
In February 2006, we got some great news: Isabelle was going to be a big sister! Our second daughter, Elorah, was born in September 2006. Elorah and Isabelle have been buddies ever since. Elorah "Sissy" goes wherever Isabelle goes and does whatever Isabelle is doing. They are best friends and "partners in crime". :)

The Third Operation: The Fontan
As summer faded into fall and winter, Isabelle stayed in good health and sidestepped RSV thanks to another round of Synagis. Isabelle had a few minor colds, but for the most part, she was healthy enough for her third procedure. Isabelle made it through the summer of 2007 without the Fontan procedure, and by the time fall rolled around, the doctors told us that they would evaluate Isabelle in the spring. This delayed our worrying for a few months, and we were able to focus on enjoying our girls and watching them grow.

In January 2008, we met with Isabelle’s pediatric cardiologist who said Isabelle would need to have a new cath done soon. She had the cardiac catheterization done in March 2008, and we found her cardiac output was very good. The doctor didn’t discover any serious leaks in her valves, and her resistances were low, which made her a good candidate for the Fontan.

The doctors told us Isabelle’s heart rhythm was off, so she may be in need of a pacemaker. She had to wear a Holter monitor for one day to test her rhythm. The results showed that her rhythm was good, but that it would be best to fit her with a pacemaker at that point instead of doing another surgery at a later time.

The Fontan and life afterwards

In the Spring of 2008, we set a date for the final operation: the Fontan. Isabelle had pre-op on August 22, and her surgeon performed the Fontan procedure on Aug. 25.

Post-operatively, Isabelle began experiencing arrythmias and difficulties managing her blood pressure. She experienced a myriad of complications including Atrial Fibrillation, Tachycardia, Acute Renal Failure, Ventricular Fibrillation, Anasarca and eventually Diastolic Heart Failure.

Isabelle underwent more treatments that we care to mention. On Oct. 11, a surgeon reversed the Fontan in a "takedown" procedure. Isabelle's poor situation made the surgery very risky. The medical team tried to get Isabelle back to a cardiac state that "worked for her" previously. The surgery went well and Isabelle "sailed" through it so to speak. Unfortunately, after two weeks, she showed no signs of significant improvement, so Children’s Mercy referred us to St. Louis Children’s Hospital to meet with Dr. Canter and Dr. Gandhi to get Isabelle's condition evaluated (to see if required a heart transplant).

Isabelle | Photos © Daddy
Isabelle, 1 month old, holding Daddy's finger (3/4/05)

Heart Transplant

When she arrived at the St. Louis hospital, the medical team evaluated Isabelle’s heart for a transplant. The medical team ran many tests to determine whether or not she would be a good candidate for a heart transplant. While we waited to hear the news, we thought about how devastated we would be if she didn’t qualify for a heart transplant. We came so far and survived so many events, and we would have just been crushed.

On Oct. 30, the doctors told us our Princess’ heart was “too tired,” and she needed a transplant. The angels must have been looking out for Isabelle because Isabelle still qualified for transplant (her health was still good enough). Our daughter was immediately listed in the United Network for Organ Sharing and was considered critical priority due to her state. However, because of her type of heart failure she did not qualify for any kind of mechanical assistance. (e.g. Ventricular Assist Device or a Berlin Heart).

Isabelle waited 6 weeks for a donor and sadly a donor didn't come in time. Isabelle's heart had been weakening and she suffered multiple cardiac arrest events in her final days. She passed away while in her parent's arms just after 8 pm on December 11th. She is the most wonderful, sweet and beautiful person we've ever known. So many people adored her. We'll miss our dear Princess Isabelle, but we know she's now in the arms of Jesus and in God's care for all eternity.

Isabelle, Mommy and Elorah, A Good Day, Isabelle putting a sticker on her sis (11/28/08)