Erica's Story
It was the early morning hours of Thursday, September 18, 2003. I was trying to roll out of bed to visit the bathroom, for the fifth time that night. As I was just about in the upright position I felt this "pop" in my lower abdomen. Suddenly there was gush of fluid running down my legs. Twelve hours later, at 3:32pm, my husband and I welcomed a beautiful baby girl into the world! She weighed in at a whopping eight pounds three ounces.
She was a slow eater and didn’t seem take much at a time. I was new to breast-feeding, even though this was my second child, as I was unsuccessful doing such with me son. The nurse continued to bring Erica in every two hours and assured me that she would eat when she was hungry. I found out later that the reason for Erica not eating much at a time was due to her defect. Most congenital heart defect babies eat until they are satisfied not until they are full as they tire quickly.
The memory of my doctor gently shaking my leg to wake me at 5:30 in the morning is as vivid as if it had happened yesterday. She informed us that Erica was not doing well. During the night the Obstetrics nurse had notice Erica’s lips and nails were turning bluish and her oxygen saturation was dangerously low. The doctor told us that Erica either had pneumonia or there was something wrong with her heart. We were taken to see Erica as she was being prepped to be life flighted to the University of Iowa Hospitals and Clinics.
After watching the helicopter disappear into the sky, Eric and I headed home to pack a bag and drive to Iowa City. When we arrived we were informed that Erica was in the Neonatal Intensive Care Unit and we would have to wait until it was "visiting time" to see her. The waiting was excruciating. When we were finally allowed to she Erica, our time was cut short do to a procedure being performed on the infant next to her. She looked like a giant compared to the other babies in the room. My baby looked healthy. Why was she here? I remember being so scared. Something was terribly wrong with me baby girl and all I wanted to do was hold her and cuddle with her and was not being allowed to do such.
The next thing I remember was sitting down in a conference room, my husband and I on one side and several doctors on the other. When they told us what Erica’s diagnosis was, my heart sank. Erica had double inlet of the left ventricle with a d-transposition of the great vessels. She also had a large ventricular septal defect. What does this mean? The positions of the great arteries (pulmonary artery and aorta) and the left and right ventricles are the reverse of a normal heart. Double Inlet Left Ventricle is one of the so-called Single Ventricle heart defects, as there is effectively only one pumping chamber in the heart. At the time I had no idea what that meant. It sounded like the doctors were speaking some foreign language. Now, I can say and use most congenital heart defect terminology like a second language. We were also told that about fifteen years ago children born with the defect were giving a 50/50 chance of survival. The medical technology has advanced greatly since then and we are forever grateful for this.
We were told Erica would need several surgeries and medications in order to make her heart into an organ that could function for the rest of her life. Erica spent the two weeks in the hospital before the doctors decided that everything they were doing for Erica at the hospital we could do for her at home with the help of a nurse. I was finally getting to take my baby home! She came home with six medications and a nasal gastric (ng) feeding tube because she was not eating enough to gain weight on her own. She was on a strict regimen for medications and feedings. Erica was put on a high calories formula diet and we had to hook her up to a kangaroo pump at night that continuously fed her through her ng tube for eight hours. We had a in-home nurse visit three times a week to check Erica’s oxygen saturation and to administer her Synagis shot (RSV prevention shot) and had monthly visits to the University for check-ups. This continued until Erica was eight months old and we were told that it was time for her first surgery.
Erica had been doing so well, with the exception of her weight gain, that we were told that it was a possibility that Erica could "skip" the first surgery normally required, which is the banding of the pulmonary artery, and would have the Glenn procedure done. However, one inside of Erica’s chest the surgeon decided the banding needing to be done after all as the artery had started to harden due to the amount of blood flow. Erica impressed her doctors and surgeon with her recovery time. She spent just under one week in the hospital.
Just after her first birthday, Erica was going back to the hospital to have the Glenn procedure done. Everything went smoothly and Erica again impressed the doctors with her recovery time. She did have an arrhythmia issue and had to were a holter monitor home for the first twenty-four hours. However, her arrhythmia came back a month after surgery.
It would be four years before Erica had her next surgery. While the third surgery, the Fontan, is usually performed around the age of three years, when the child is between 30-35 lbs., Erica’s was not done until the age of five. Erica was always tiny and petite and will continue to be. The doctors wanted her to be "bigger" before they performed the surgery, therefore it was much later.
We wanted the surgeon that performed Erica’s first two surgeries to also perform the third. It is not that we felt the surgeons at the University couldn’t perform Erica’s surgery, we just thought that since Dr. Burkhart had "been inside" Erica’s chest twice already, he had a better understanding of her anatomy. We tracked down Dr. Burkhart and found he was practicing at the Mayo Clinic in Rochester. While Rochester was approximately four hours from our home, we knew it was worth it to put Erica’s life in Dr. Burkhart’s hands.
As we were sitting in the waiting room waiting for the nurses to take Erica back to surgery, fear suddenly swept over me. I knew we were in good hands with Dr. Burkhart but I think realty finally had a chance to sink in that my princess was having open heart surgery and this could be my last hug, my last kiss, the last time I see her precious smile. Of course the waiting was excruciating, but at last we were called down to see Dr. Burkhart to get an update. I remember sitting in astonishment as he explained that the procedure went fairly well but instead of repairing the tricuspid valve in her right atrium it had to be capped and a huge blood clot attached to it removed. Erica had several complications following this surgery including fluid buildup, fevers, another irregular arrhythmia. After two and a half weeks in the hospital, Erica was finally released.
Erica was on several medications when we brought her home, including Coumadin, because of the capped valve and blood clot found. This medication required us to have Erica’s blood tested three times a week to make sure her blood was not too thick or not too thin. Luckily we found a place near by that did finger pokes instead of full blood draws.
Erica’s arrhythmia never regulated itself like it did after her last surgery and she started to show signs of heart block. We made another trip back up to Rochester to have a pacemaker implanted. While the risks of this surgery are not as high as compared to the Fontan, the recovery was a lot more painful for Erica.
Erica had her last cardiology checkup on April 16th of this year. She was able to stop her lasix, a diuretic, as her x-rays showed no excess fluid whatsoever. This was a huge feat for Erica after all the complications she had after her Fontan, plus the fact that she was just six months post op and she was now done to just three medicine, two for her heart, which she will remain on for the rest of her life.
On the outside Erica appears to be a normal, happy-go-lucky little five year old. She will start kindergarten this fall and will continue her dance lessons, the one activity she can enjoy without exhausting herself. However, every night when I help her get her pajamas on, I am reminded of all she has been through when I look at her scars. We will continue to have checkups every six months, rotating between her cardiologist and electro physiologist. Her pacemaker is currently set at range for an average battery life of 7-9 years. At that time she will have to undergo another surgery to have the battery replace and this will continue to be done throughout her lifetime. She will also more than likely have to have the wires, connecting the pacemaker to her heart, replaced at least once.
While the past five years have been difficult, there isn’t anything I would change, with the exception of Erica’s pain. No parent wants to see their child suffer. Our family is stronger because of everything we have been through. We have been blessed with a wonderful family. We are not asking for sympathy just your help to spread the awareness of CHD and encourage others to support CHD research.
Sincerely,
Mandi Rees (Erica’s mom)