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Katelynn

October 10, 1997
My pregnancy with Katelynn, like my first, was uneventful and enjoyable.    On October 10, 1997, I was four days past due, and readily agreed with my OB to go in early the next morning to be induced.   Katelynn decided to take matters into her own hands however, and I went into labor around midnight.   Our beautiful daughter was born at 7:30 AM, weighing 7 lbs, 15 ounces and scoring a 10 on the APGAR scale.   The next day, the pediatrician mentioned casually that she had a heart murmur and was mildly jaundiced, but that both conditions were seen frequently in babies and would likely go away on their own.    We went home the next day.

We had a well baby  visit when she was three days old.   The pediatrician said he did not hear the murmur and that the jaundice was not a concern.   He handed Katelynn to me and said “Congratulations, you have a perfect baby”.   I went home greatly relieved, as the heart murmur issue had really bothered me.   That night, Katelynn was up constantly, wanting to eat and be comforted.    She ate a big breakfast in the morning and after that, life was never the same.   For the rest of the day, she was lethargic and wouldn't eat.  She would wake up when I picked her up, but just wanted to go back to sleep.  Her color did not change due the jaundice, she just look like she had a great tan.

To make a long story short,  we found ourselves in the ER that afternoon and she was tested for everything from meningitis to pneumonia.  Finally, an X-ray revealed that her heart was enlarged and they transported her by ambulance to RUSH Saint Luke’s Presbyterian Hospital in Chicago (we were in a suburb).  By the time we got to the hospital (at 3:30 AM), a cardiologist met us in the pediatric ICU and gave us the grim news that she had  coarctation of the aorta which would require surgery as soon as her vitals were stabilized.    The jaundice was out of hand by now and her bilirubin count had to be brought under control.   They reopened her ductis with prostaglandin treatments.  We were also told at the time that she has a bicuspid aortic valve and that 50-70% of kids with coarctation also have this valve malformation.  The valve would not require immediate surgery.

The coarctation was repaired when Katelynn was nine days old.  We went home two weeks later, scared to death!    The doctors were pretty optimistic with us from the get go about her future.    The coarctation repair should last a lifetime and we would have to monitor the bicuspid valve.     Apparently 2% of the population has a bicuspid aortic valve and that some people live to be quite old before they even know they have one.   We realized of course that Katelynn’s valve was narrower than it should be (aortic stenosis) and that some sort of surgery would probably eventually be needed.  She would just need to be monitored   to see how her valve was keeping up with  her growth.

My husband was transferred to Connecticut in 1997.   Our Chicago cardiologist had been planning a cardiac cathetarization but felt that it would best be performed by  the new cardiac team in Connecticut.    Katelynn had her first cath at YALE in February of 1999.   The cardiologist at first thought he would be able to balloon the narrowed area of  the valve to ease the flow (so that her heart would never be overworked in any way), but unfortunately, they made a new discovery -- Katelynn had was is known as a sub-aortic membrane, a flap or ring of skin under the aortic valve which would need to be removed.   No one knows if she was born with this flap or if it grew after birth.   On April 29, 1999 Katelynn underwent surgery at Yale to remove the membrane.   After the operation, the surgeon advised us that Katie also has a “parachuting” mitral valve and for the first time we heard the term Shone’s Complex to describe her collection of left side heart flow defects.   We asked what other “surprises” could unexpectedly  show up with her heart and the surgeon said, “this should be about it” .

All is going well so far.  There was a two day re-admission to Yale after surgery due to fluid accumulation in the lungs, but Katie just turned two and is completely normal in every other way.    She is an extremely bright and active two year old and you would never guess in a million years that she has been through these terrible ordeals.   She is off the charts for height and is in the 50% range for weight.  She  says her ABCs,  counts to ten, loves to sing and do everything that her older sister does!  She is a TREMENDOUS joy to us.

There is a 15-20% chance that the membrane will grow back.   This possible re-growth  and the bicuspid aortic valve are the major concerns at this point.  We pray that the bicuspid valve will sustain her at least until she is a full grown teen and that she will only have to undergo one surgery to replace the valve if necessary.    No one seems overly concerned about the mitral valve.     I will never relax about any of this, I just try to enjoy each day I have with her and count my blessings that she is with me.

I am very concerned about possible genetic causes of Shones.  I want my daughters to be as educated as possible about this when their times come to have children.    There isn’t much information out there regarding genetic studies, but what I have indicates that left flow defects tend to run in families.    I am about to send a sample of  Katie’s blood to a doctor  whose research shows that there may be a gene or genes that causes Shone’s complex (hypoplastic heart syndrome would be one of the defects that could be part of this “syndrome” and caused by this gene).    I am trying to find out everything I can about this!