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Celine's Story
A Prenatal Diagnosis of Complex
Heart Defects
If you are reading our story
because your baby has been diagnosed with complex heart
defects I have good news for you. The good news is that
now, in 2006, there are more resources available than
ever before. You can find online specialty message boards,
journal articles, lots of websites with chd survivors with
many varieties of defects. You can even email
many of the top ranked surgeons and pediatric heart
centers! The not-so good-news is that while access to
all this information will help you make important decisions
about your baby, it may also leave you feeling
conflicted, and all the more uncertain about the future.
Despite many advances in medicine, it is still the case
that many complex defects cannot be "fixed". I know
that every outcome is different because no child is
like another. Beyond all the caveats about
this and that defect, statistics, surgical approaches,
and every other possible unknown factor, I hope
our story will provide to you the same kind of
hope that reading about other children gave to us in
our hour of need.
We received our "bad news" about
baby in June of 2001. I was 22 weeks along and expected
only to find out the sex of the baby, and confirm what
I knew to be true, that baby was perfect! Wrong. The
OB left us in the exam room for a very long time. I
don't know if she had ever delivered bad news before.
I wonder what must have raced through her mind when
she looked over the ultrasound. Eventually she did return
to the little room where I sat with legs dangled over
the exam table and my husband bobbed our squirmy 10
month old son on his knees. There was no good way to
phrase it, I'm sure, but telling us that our baby had
an "incompatible with life" condition still seems boldly
absurd to me. We were told that our baby had hypoplastic
left heart syndrome and we were given our options. My
body, mind, and spirit were already rejecting this news.
With arms firmly crossed I asked, probably with some
misdirected defiance, "What about fetal surgery?"
The scene essentially repeated
itself again not many days after when a maternal-fetal
specialist confirmed the diagnosis. We were given our
options once again; termination, comfort care, surgeries,
or transplant. By then we had done some research and
had a better understanding of the situation. We knew
by then that "incompatible with life" was not entirely
accurate. We also knew that if our baby would survive,
it would require that we fight for her. We didn't know
yet exactly what we would do, although termination was
out of the question entirely. The mfm offered to set
us up with an appointment with a fetal cardiologist
to help us sort things out.
Dr. Cuneo of the Heart Institute
for Children determined that our baby did not have hlhs,
but instead hrhs, or hypoplastic right heart syndrome.
An extremely rare defect known as L-TGA (L-Transposition
of the Great Arteries), or ventricular inversion, had
fooled the mfm – he may never have even encountered
the defect before. In addition to that, our baby also
appeared to have Ebstein's anomaly, a coarctation of
the aorta, a few holes, and of course, the hypoplastic
right ventricle. The new diagnosis did not mean that
our baby was out of the woods. No. It simply meant that
the right sided issues vs. the left sided issues resulted
in a potentially more favorable prognosis. She would
still require the same surgeries we had read about when
we thought we were dealing with hlhs. We consulted with
Dr Cuneo, and Dr Ilbawi, a top-rate pediatric cardio-thoracic
surgeon, and researched as much as we could online.
Our insurance company assigned us an excellent case
manager to help us research surgeons and hospitals.
We were moving inch by inch away from despair and toward
the light.
Our hearts told us that we needed
to fight for our baby. We did not want to risk wondering
what might have happened. We did not want to know that
someone was missing from our family because we were
afraid of surgery. I certainly did not want our baby
to go through three successive surgeries and whatever
suffering that might involve. But given the fact that
it was her very life that was at stake, and that there
was as good a chance as any that she would survive and
do well, we regarded the surgeries as very much worth
it. In fact, instead of fearing the future, I began
to feel a sense of relief and gratitude. I was thankful
that we had an option, that we really might be bringing
a baby home after all, and that she might live a long
and happy life on top of it!
And so, in several months time,
Celine entered the world. She was only hours hold when
she began to turn blue and required her first intervention,
initially only some oxygen, but they gradually began
adding support in anticipation of her surgery, such
as an arterial line, an "ng" tube, and a ventilator.
Within two days things began to deteriorate and she
required a balloon catheter procedure to keep her alive.
And then, in several more days, it was time for her
first surgery, the Norwood. She did well, although it
was extremely hard on us, her parents. I was a basket
case and ill-prepared for the experience of seeing my
baby after open-heart surgery. During her three week
recovery period, I often had to be at home with our
son. The separation from my new baby was almost unbearable.
We muddled through.
When Celine came home, we weren't
quite sure what to do with her! She was technically
still medically fragile, and would be up until her second
surgery. She had some difficulties with feeding and
we were creative about figuring out how to make sure
she had more than the minimum required calories. Day
by day we understood what she needed from us and life
started to unwind from the tight little ball we were
all wrapped up in at the time of her birth and first
surgery. Her coarctation began to narrow, dangerously,
two months after she came home, which required a catheterization
to open it up . . . one that failed (I was heartbroken)
and would require a surgical resolution. Fortunately,
her surgeon was willing to wait to correct it during
her second scheduled surgery.
The time for her next surgery
came quickly, and again she did well, and we, the parents,
did slightly better than we did the first time around.
We sought out Early Intervention because Celine seemed
to have some developmental delays. EI determined her
primary delay was with speech. If you are unfamiliar
with EI, it is a state program that provided one on
one therapy in your own home. Many, many infants and
children make use of these services. Celine benefited
from speech therapy for several months and suddenly
it was time for her fontan (and hopefully final!) surgery.
She was 18 months old, so her recovery was a little
bit harder to bear. She did not enjoy a week restricted
to her hospital bed, the near dehydration (this is a
therapeutic issue required for a good recovery), the
IV's, the suture pain, the chest tube removal, the poking
and prodding, yes . . . the boredom. No, of course she
didn't like it, but it was a temporary period of discomfort
that ultimately saved her life!
Now she is a very normal
4 yr old. She is in preschool and has no known delays
to report. Her heart function has been excellent. She
had an oxygen saturation level of 100% several checkups
ago. She'll be monitored for the possibility of a pacemaker
beginning sometime in the near future. And just to throw
in a few details of her personality here, she wants
to be a doctor, a nurse, or a rock star when she grows
up. Her favorite movies at the time of writing include
"The Incredibles" and "Madagascar". She has a slap-stick
sense of humor and often has her parents and her three
siblings laughing (with/at) her. We have been extremely
blessed, this we know. But we had to take a chance to
receive our blessing. We know not to take it for granted
that tomorrow will be trouble free. I suspect that some
trouble will come, sometime down the road. Whatever
troubles may come (if they do), we are a family and
we will weather them as a family, just as we celebrate
our trouble-free times as a family. We think we will
do just fine, but as we will pray for you (anyone who
may be facing some very difficult decisions and circumstances
regarding their baby), please pray for us!
By Monica and Celine,
who inspired it all
Please visit www.benotafraid.net,
and take advantage of their support message board!
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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.
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