On January 30, 2008 God blessed
our family with the birth of our beautiful baby girl,
Khloe’ Julia. On January 31, 2008, not even
24 hours after one of the most joyous times in our life
we were blasted with the worst news of our life; Khloe’
has Tetralogy of Fallot as well as a disconnected
Left Pulmonary Artery.
How could this be? She looks perfect, she is pink, she
is crying, everything we expect of a newborn. The news
came to us as we were getting ready to go home; thank
God for the pediatrician on call for our regular pediatrician.
He saved her life. Every OB appointment brought
the same happy news, a beautiful heartbeat, good movement,
good growth. The ultrasound at 28 weeks told us the
news we had hoped for, it is a GIRL! What a wonderful
addition to our family, two boys and now a princess. The
ultrasound also brought good news, all systems functioning
and everything looked great. The delivery was picture
perfect, less than 7 hours, no real pain with contractions,
epidural was wonderful, 3 pushes and our baby girl made
her much anticipated debut into this world. My
OB was amazed at how Khloe’ delivered. As
she was crowning, she was moving her head from side
to side, something my OB said she had never experienced
before. Looking back I take it as Khloe’s
way of telling us to move things along; she has a job
to do in this life!
I remember feeling her body on mine for the first time.
She was so amazing, so real. All the kicking, moving,
and hiccups I had been feeling for those 9 months was
now outside of me and real. This is a true sign of God’s
grace and gift to us. Shortly after her birth,
the nurse caring for her heard a murmur in her heartbeat.
She assured us that this was common and that it was
likely caused by a valve that would close off in about
24 to 48 hours. It is the valve that keeps blood out
of the lungs while the baby is in utero. Once the
baby is born the blood begins to flow through the pulmonary
artery and that valve closes, getting rid of the murmur. OK,
so everything is fine. Khloe’ was nice and pink,
crying, doing everything they wanted her to do, except
for the murmur.
I remember so vividly putting her to my breast for the
first time; I was so nervous because it had been so
long since I breastfed, and I really, really wanted
to be successful with Khloe’. I had only breastfed
our twins for a month; it was so difficult with two. Khloe’
latched on and ate for the first time like an old pro.
In fact, she really taught me what to do. It was a wonderful
feeling. We had a good night; she ate and slept,
ate and slept. The next morning we were packing our
things to go home, waiting on my OB to discharge me
and waiting for the pediatrician to check Khloe’
once more, and then we would be off to start our life
at home with our newly expanded family. In the
next few moments that morning, our lives would change
forever with the news of Khloe’s congenital heart
defect. The pediatrician came in to check Khloe’,
and he spent a great deal of time just listening to
her heart and not really saying a word or making any
movements. I remember his words so vividly: “I
don’t like the way it sounds, I am going to order
the Echo.” Within literally a few minutes, the
NICU came and got Khloe’ and that was the start
of our journey.
No, this can’t be happening, we were going home,
everything is supposed to be fine I thought to myself,
I literally cried out to my husband, “I can’t
do this!!!” We stood in the hospital room in disbelief
that any of this was happening, all I could do was cry,
and pray. Within about an hour, the NICU nurse came
in to tell us the news; Khloe’ has Tetralogy of
Fallot, a congenital heart defect that causes 4 areas
of the heart to form incorrectly. We didn’t know
it at that specific time, but later learned that she
also had a fifth defect in her heart where the left
pulmonary artery was disconnected from the main pulmonary
artery. Her aortic arch also arches to the opposite
side, however later we learned this wasn’t causing
any problems nor would it be repaired. The murmur
we heard was literally keeping her alive because that
valve had made sort of a “bypass” and was
supplying the left pulmonary artery with blood so that
is why she was pink and things seemed to be fine in
the beginning. It was the shutting down of that valve,
or PDA, that the pediatrician heard that concerned him.
God bless him for saving Khloe’s life. If we had
come home with her there would have been very serious,
likely fatal, problems.
The first thing that I thought was that she was going
to die and that I must call my church to have her baptized.
Thankfully we are blessed with a wonderful parish and
wonderful priests. Father George was at the hospital
within minutes. Khloe’ was baptized into our Catholic
Faith on January 31, 2008, just one day old. Only my
husband and I were present, aside from two beautiful
nurses who prayed with us during the baptism. Father
George also anointed her and prayed over her for a long
time. It was truly beautiful despite the situation. My
dad arrived shortly after that, thank God because I
think my husband needed help in holding me up.
The NICU started her on a medicine called prostoglandin,
which was keeping the PDA open, thus keeping the blood
flowing to the Left pulmonary artery. The hospital
called for Life Flight to take Khloe’ to Texas
Children’s Hospital in Houston, but the weather
was too bad so they took her by ambulance. The hospital
quickly discharged me, and before we knew it we were
following behind the Kangaroo Krew ambulance that held
our precious baby inside. What a surreal moment that
was. It was only an hour drive, but I felt it was
a lifetime. Once we arrived at Texas Children’s
Hospital, they admitted Khloe’ to the NICU. They
hooked her up to every monitor available. As the day
and evening progressed, we learned more and more about
Khloe’s congenital heart defect. In fact, we have
learned way more than we ever wanted to know about the
heart in this life and the next.
During her time in the NICU, Khloe’ was stable,
requiring only an IV with the life saving medication
and oxygen through nasal canula. Still, it was a frightening
sight to see, our baby girl with all those wires, beeping
and alarming all the while. We spent 13 days in
the NICU while the entire cardiac team debated on which
approach was best for Khloe’: a full repair via
open heart surgery to correct the Tetralogy defects
as well as reconnecting the pulmonary artery OR a shunt
surgery (open heart) to correct the LPA now, followed
by another open heart surgery in several months to correct
the Tetralogy. Of course we were praying for only
one surgery, but we wanted the best for Khloe’.
It was decided that the full repair would be attempted
since Khloe’s Tetralogy was rare (in a good way)
in that there was not much scar tissue in the right
ventricle of the heart (common in Tetralogy) that would
require the heart muscle to be cut into in order to
remove the scar tissue. So, having this special condition
helped the surgeon decide that he could do the Tetralogy
repairs while he reconnected the LPA to the main pulmonary
OK, so we are set. Surgery is Monday morning. One
can never be prepared for this moment. I spent
the entire night at her bedside, nursing her up until
the last minute before she had to go NPO. The surgical
and anesthetic team came to get her early in the morning.
I remember walking with her to the elevator, up to the
Cardiac floor, then departing from her in that grey
hallway. I felt as though my heart and soul was being
ripped out. It was the hardest moment to face. I am
so thankful Ghaleb was there to hold me up, yet once
Khloe’s surgery was 9 hours. We received updates
every couple of hours, each one with good news, progressively
getting us to the end around 5pm. The surgeon came
out and briefed us on the surgery. He felt he made the
best repairs he could for Khloe’ and was pleased.
He did tell us the good tissue remaining of the LPA
was very short, he had to use part of her pericardium
to make the connection to the main pulmonary artery.
He told us there was a chance that she could have some
narrowing with the LPA that could require further surgery,
but hopefully it could be done via catheter with a balloon
or stint. The pulmonary valve would also become too
leaky, possibly by adolescence requiring a surgery to
replace the valve.
OK, so let us just see our baby girl. Another moment
you can never prepare for. It was quite a terrifying
sight. Khloe’ had wires, tubes, IV’s,
you name it, coming out of her body. And her lips. Her
beautiful, perfect lips. They were so swollen that I
couldn’t recognize her. I remember thinking MY
GOD, she will never nurse again. Once again Ghaleb had
to hold me up. Each day (which ended up being only
a miraculous 2 1⁄2 days) more and more tubes and
wires were removed. Khloe’ progressed so well
and so fast, we were out of the CVICU and into the step
down room before we knew it. Once in the step-down room,
I was able to room in with Khloe’ full time.
It was so wonderful to be able to be at her bedside
all the time. Here we learned how to care for her IV,
bathe her, basically do all care so that we would be
prepared to go home.
We were home within a week. OK, so finally we are in
the car, strapping Khloe’ into her car seat that
we had so proudly loaded into our car some three weeks
earlier expecting to come home in a day with our new
baby, our new family. What a joy and a blessing.
We love her as she is; perfect in every way. Her heart
is different, not weak. In fact, her heart is strong,
and will carry her through a lifetime of opportunities.
So we learned a few new things. Sometimes there
are kinks in the road, some bigger and scarier than
others. Sometimes life doesn’t go as we planned.
Sometimes we feel helpless and totally out of control
but deep down we know we never really have control in
the first place. We learned that faith is a real
word and that it can mean more to you in a crisis and
bring new meaning to Life. Despite all the hard
times, we made it. Khloe’ made it. She is
a fighter, she is strong. I think my entire pregnancy
Khloe’ was kicking me so hard just to let me know
she is a strong girl. Now I see it as her way of telling
me that she would be OK.
Today Khloe’ is 3 and 1⁄2 months old. We
had our cardiologist follow up visit and she is doing
well. We know that the possibility of heart surgery
again to possibly widen the LPA as well as surgery in
her adolescence years to replace the pulmonary valve
are very real, still we pray for a miracle. We also
pray that the medical technology will continue to progress
and new and easier procedure will be perfected so that
in the event she does need surgery it will be less invasive. We
will live our life together to the fullest; living,
laughing, loving all the while. God bless you on your
journey, let God bring you through!
to Congenital Heart Defects Stories
-The support, information and encouragement provided by the PPFL parents is not meant to take the place of medical advice by a medical professional. Any specific questions about care should be directed to a health care professional familiar with the situation.