Born with a
Broken Heart
My husband, Terry, and I were to arrive
at the hospital at 5:30am. My caesarian was scheduled for 7:30. We
were expecting our third child but this would be my first caesarian.
There was something else different about this delivery as well. In one
corner of the operating room that day was a Neonatal Intensive Care team
awaiting the arrival of our baby.
The Diagnosis
Months earlier, a routine anatomy ultra
sound, performed during the fifth month of pregnancy, had revealed that
all was not well. Having had two daughters previously, we were
wondering if perhaps we would be painting the nursery blue this time.
What we learned was that our baby had a congenital heart defect known as
Hypoplastic Left Heart Syndrome (HLHS). We
were also told that a
Cystic Hygroma had been found. Finally, we
learned that our baby was a little girl.
After the ultra sound, Terry and I were
ushered into the office of a Genetic Counselor. The Counselor discussed
some of the possibilities of what had gone wrong and what the prognoses
were.
Down's syndrome,
Trisomy 13 and
Trisomy 18 were some of the possibilities that
were discussed. We learned that the Maternal Fetal Specialist, who had
performed the ultra sound, most strongly suspected that our baby might
have
Turner's Syndrome as left-sided heart defects
and Cystic Hygroma can both be associated with Turners.
Each prognosis discussed that afternoon all lead to the same outcome - death. We were told that even if the genetic defect was not fatal (Turner's Syndrome and Down's Syndrome for example, are not), the heart defect was in almost every case. We were told that there was a high probability that she would not even survive to term. My tears had turned to sobs by this point.
The Tests:
I consented to an Amniocentesis and had
it performed that very afternoon. We were also scheduled to see a
Pediatric Cardiologist (PC), the next morning, for a Fetal
Echocardiogram (Echo) to confirm the heart diagnosis.
My husband and I arrived at
Texas Children's Hospital the next morning tired and scared. Neither of us had slept much the night before. As we walked through the hospital, I noticed that it was decorated with Children's artwork. For a moment a sense of panic washed over me as I realized this hospital was full of sick children. I had this momentary urge to turn and run out the doors and never look back.
The PC, who performed the Fetal Echo, was a soft-spoken and gentle man. I began to cry again when he confirmed the diagnosis - HLHS. Then he spoke some of the most beautiful words I have ever heard. We had options. Children with HLHS were surviving. I felt like dancing.
Options:
First, he showed us detailed drawings;
one that depicted a healthy heart and one that showed our babies heart.
That morning the doctor thoroughly discussed with us the three options
available for HLHS. (1) Compassionate Care; (2) Surgical
Reconstruction; and (3) Heart Transplant.
Compassionate Care simply means choosing not to
intervene. Basically, we would take her home to die. Without any
intervention, HLHS is always fatal; usually within a few weeks.
Surgical Reconstruction, we were told, involves a
series of three open-heart surgeries to redirect the baby’s blood flow.
The surgeries would not ‘fix’ her heart. Instead they could enable her
heart to work in a manner that would sustain her. The first one, called
the Norwood and usually performed around one week after birth, is the
most difficult. We were told that at Texas Children’s Hospital, about
70% of children undergoing this surgery survive until the next surgery.
The next two surgeries, the Bi-directional Glenn,
performed at around 6 months, and the Fontan, performed at around 2 ½
years, were not as difficult. The survival rates for each of them were around 95%.
We were told that at Texas Children’s the
overall chance of surviving all three surgeries was about 65%.
The final option explained to us was a heart transplant. Texas Children's Hospital does not regularly perform these surgeries, but we were told that they would refer us to a center that does if this was our choice.
I was scheduled to see my Obstetrician
(OB) right after the Fetal Echo. I felt like I was walking on air as
Terry and I walked to her office. We could not wait to tell the Genetic
Counselor what we had learned. We thought she would be as excited as we
were to learn that her information on HLHS was outdated. Instead, she
was concerned that my husband and I were getting our hopes up too high.
I felt a little deflated by the Genetic Counselors manner but was still riding pretty high on the news we had gotten at Texas Children’s, when my OB came into the room. Terry and I were completely unprepared for what she had to say to us. She stated that she had received the PC's faxed report confirming HLHS and was therefore strongly encouraging termination of the pregnancy. She explained that my husband and I were still young (30 and 29 respectively) and so we could try again and have a healthy baby. She was also worried that this could be difficult for our older daughters. Finally, she let me know that she was aware from the Genetic Counselor that I had stated that abortion was not an option for me. She wanted me to reconsider. She would meet with me in a week for my decision.
As my husband and I drove home that afternoon, we named our daughter 'Lauren Esther'. Friends and family encouraged us to find a new OB. I prayed diligently about this and felt like the Lord wanted me to stay where I was.
The Meeting:
The week passed rather quickly and I was
pretty nervous about the meeting with my OB. I was trusting God to lead
me to say the right things. I explained to her that I had believed in
God my whole life and I was placing this baby in His Hands. I further
explained that under no circumstances could I be responsible for
stopping this baby’s heart from beating. My OB stated that she would
respect my wishes and we would proceed to term with this pregnancy.
Termination was never mentioned again.
My OB did however; wish to discuss her plan of not
monitoring the baby during the delivery process. She explained that she
did not want to put me through the additional risk of an emergency
caesarean if they detected that the baby was going into distress. We
told her that we wanted this pregnancy to be treated the same as any
healthy one and she respected and carried out our wishes.
A Little Good News:
Two weeks after the Amniocentesis, we
received a call from the Genetic Counselor. The results of the Amnio
were in and all tests had come back normal. No Genetic defects had been
detected and because they had looked at a lot of cells, the test had a
high accuracy probability. Also, later ultrasounds showed that the
Cystic Hygroma had completely dissipated.
The Birth:
Lauren arrived at 8:22am on August 30, 2000 at St. Luke’s Episcopal Hospital, which is physically connected, to Texas Children's Hospital. My OB handed her to a member of the Neonatal Team. Generally, children born with HLHS are stable at birth. The plan was to get Lauren's IV's in place and get the Prostaglandin drip started. We were then going to be allowed to hold her for a few minutes before she was transported to Texas Children’s Neonatal Intensive Care Unit.
Complications:
My husband was asked to step out of the
OR. My caesarian was completed and they began to wheel me out to
recovery. I explained again that I was supposed to get to hold Lauren.
A nurse gently whispered that Lauren was a little blue and that the
Neonatal Team was still very busy with her so it would be best if I went
on to Recovery. I had only gotten a quick glance at her. Surely I
could wait a little longer. My bed was pushed to Recovery.
Shortly after I was transferred from
Recovery to a regular hospital room the Chief of Congenital Heart
Surgery and Cardiac Surgeon In-Charge
at Texas Children's Hospital,
Dr. Charles Fraser, walked into my room. My
husband knelt by my bed to let me know that what the Surgeon had to tell
me was going to upset me. My heart sank.
Dr. Fraser explained that in addition to
HLHS, Lauren had been born with an intact Atrial Septum. Basically, the
blood from her lungs was being pumped into the tiny left side of her
heart where it had no way to get out. Blood was backing up into her
lungs, which had been badly damaged by this.
I learned that while I had been in recovery, Lauren had been taken to the Cath lab where they had hoped to open up the Atrial Septum thus allowing the blood to get from the left-side of the heart to the right side. The Cath procedure had failed. Dr. Fraser needed to get Lauren into emergency open-heart surgery, where an Atrial Septectomy would be performed. We were told that without this surgery, Lauren could only survive for another hour or two. We learned that Lauren was very sick, so Dr. Fraser thought there was only about a 20% chance he could get her through the surgery. Finally, because of how sick she was, even if she did survive the Atrial Septectomy, her chance of surviving the first of the Reconstructive Surgeries, the Norwood, had been compromised. How badly compromised, no one could say for sure at this point. Dr. Fraser stood at the end of my hospital bed waiting for our decision. We told him to go for the surgery. Putting Lauren through an additional heart surgery, knowing that the odds were not favorable was a tough decision. My husband said it best when he stated that our thoughts moved quickly from "How can we put her through all of this?" to "How can we not give her a fighting chance?".
At my request, my nurses quickly helped place me in a wheelchair so I could make a mad dash to Texas Children's. I wanted to see Lauren before her surgery. When we arrived, a Cardiology Fellow, came out to let me know that Lauren was already in the OR. Without a doubt, that was one of the darkest moments of my life. I was so afraid that after nine months together, Lauren would think that I had abandoned her. Beth Hess, the surgical nurse clinician who accompanied Dr. Fraser to my room and who helped escort me to see Lauren, bent down and hugged me and that was the first time that I cried on the shoulder of a stranger. I remember that Beth cried too.
When I got resettled into my hospital
room, I prayed. I then had this deep sense of peace. I felt like God
was telling me to be strong and to trust Him because this would not end
in death.
At 8:45 pm, when she was a little over 12
hours old, Terry and I met Lauren. She had a chest incision and was
very swollen, a ventilator was breathing for her, and she had more tubes
and wires connected to her than I had ever thought possible but she was
ALIVE!!!!
Waiting for her to recover enough from this surgery to undergo another one was very difficult. We seemed to take two steps forward and one and a half back. We watched as other babies recovered from their surgeries and moved from the ICU to a regular room in the hospital. During the waiting, we got to know several families, some whose children also had HLHS. One particular family, whose son Gabriel, had had the Norwood two weeks prior to Lauren's birth, were great support and comfort to Terry and I.
The Norwood:
When Lauren was ten days old, she was
again wheeled into the OR, where Dr. Fraser performed the Norwood. The
first time I saw her after the Norwood, I was shocked by how swollen she
was. I tried not to be squeamish when I saw that her chest had been
left open to allow some of the swelling to go down.
Dr. John, a Cardiology Fellow that we had gotten to know during Lauren's recovery thus far, was on duty in the ICU that night. It did not go unnoticed to Terry and I that he had his chair sitting right next to Lauren's bed. I asked him if we were hour by hour at this point (a place we had been before). He replied, Nola, at this point, we are minute by minute.
The recovery from the Norwood was filled
with ups and downs. Some days she made great progress, while others she
would take a setback. During this time, we saw more families pass
through. We were also there when two other babies with HLHS, including
Gabriel, went to be with Jesus.
Seventeen days after her Norwood, 28 days
after her birth, we got to hold Lauren for the first time. At 42 days
old, she came home.
Between Surgeries:
Lauren remained pretty frail between her
Norwood and the second stage of the Reconstructive Surgeries, the
Bi-directional Glenn. Eating difficulties were a daily struggle and
hospital visits were frequent. Lauren was fed through an NG tube.
Sucking a bottle proved too tiring for her. Lauren was also diagnosed
with severe acid reflux. She vomited about six to eight times a day.
The Bi-directional Glenn:
When Lauren was five months old, she woke up screaming one morning. Nothing seemed to console her. She was sweating profusely and turning very blue. I called 911 and we headed to Texas Children's in an ambulance.
An Echo and then Catheterization revealed that Lauren's Atrial Septum was narrowing. Measurements and pressures taken during the Cath indicated that Lauren was ready for her Glenn. In addition to the Glenn, Dr. Fraser performed another Atrial Septectomy. Lauren was in the hospital for 21 days this time.
At Home:
Lauren now seemed to blossom. She came
off the NG Tube completely one month after the surgery. She never
vomited from reflux. She began to hit her developmental milestones and
we discovered that she has the most beautiful smile.
Today, Lauren is 16 months old. She is
happy and a great joy in our lives. Developmentally, she is on target
socially, cognitively and with her fine motor skills. She is slightly
delayed with her gross motor skills so she sees a Physical Therapist one
time weekly. She has never had to go back on a feeding tube but weight
gain remains a struggle.
A Word of Encouragement:
To anyone reading this story that will soon be beginning a Congenital Heart Defect journey, I pray you find encouragement from this story and that you always believe that with God all things are possible. (Matt 19:26)
LAUREN'S STORY
