Natalie was born on December 25, 2002 and was diagnosed at birth with Down syndrome (Ds) and a congenital heart defect called tetralogy of Fallot (toF). She will be having her 7th heart surgery on Monday, April 21, 2014. They will be replacing her pulmonary valve and patching her narrowed pulmonary arteries. This is her heart story.
Tetralogy of Fallot is a rare, complex heart defect. It occurs in about 5 out of every 10,000 babies. Since approximately 50% of children with
Down syndrome have a congenital heart defect, all newborns with Ds are routinely
screened by cardiac ultrasound.
When Natalie was born, her oxygen level was 86% (normal is above 95%, and
usually 99%).
Tetralogy
means four, and Fallot was the doctor who gave a very detailed description of the four anatomical characteristics of the defect. There is nothing surgically that they do for the
overriding aorta, or the boot shape of her heart. The large hole and the pulmonary stenosis, however, require
surgical intervention.
Surgery #1
Natalie had her first heart surgery in March of
2003, while she was in the hospital with RSV(a lower respiratory infection).
Because Nat's oxygen levels were starting to decrease before she got
sick, there was some question if the trouble with her oxygen levels were caused by
the RSV or her heart. They decided to do a central shunt to
help the blood distribute more the way that it should. During this stay,
she developed a fungal bladder infection, a MRSA pneumonia and finally an
enterococcal blood infection. After 42 days in the PICU and 7 days
in a regular room....she was finally ready to go home. At this point, she
had a feeding tube that continuously provided nourishment, as she was too
weak to eat enough and we were watching every calorie that went in and every
calorie she expended. She would keep that feeding tube for about a year, and it
would turn out to be a blessing in disguise!
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| This is Natalie a few weeks before being released from the hospital. It was the first time I got to hold her in 19 days. |
During that next year, we would have 7-8 professionals we would see on a
weekly basis: a home health nurse, a dietician, a physical therapist, an
occupational therapist, a developmental therapist, a speech therapist, the
cardiologist and the pediatrician, in addition to routine ear nose and throat doctor visits, as well as the eye doctor. Finally, when Natalie was 9 months old, she was the size and age
that would provide the best outcome for her 'full repair'.
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| Natalie, age 9 months, the day before her first open heart surgery. |
Surgery #2
A 'full repair' for tetralogy of Fallot (toF) means
that she would have open heart surgery to repair the quarter sized hole in her
heart and put a patch in her pulmonary valve to make it a little larger. Her
dad and I donated blood directly for Natalie before this surgery. Unfortunately, one of the tests showed that
our blood was not able to be used for her, but thankfully there were three
units of blood available that could be used.
This surgery was very rough on her in many ways, and her lungs were
still weak from the previous illnesses. It took a couple of weeks in the
hospital and a lot of prayers before she was ready to come home. Never one to take the easy route, Natalie developed a chylothorax during this stay.
Needless to say, I had NO CLUE as to what that was. As I would learn, it basically means that lymphatic fluid was pooling in the lining around the lungs which can be a fairly rare complication of chest surgery. What it meant
for Natalie was a fat free liquid diet of some horrible tasting
'formula' for a full 6 weeks. Thank God for that feeding tube!
I can’t imagine any baby drinking it willingly, and she never had to
taste a drop!
Surgery #3
At 13 months old, Natalie started showing signs of heart failure once
again. She was sweating when eating, had labored breathing, and was just tired
all of the time. A visit to the cardiologist showed that there was a 1mm
'residual VSD'...which is a fancy way of saying that one of the stitches in the
patch for the hole in her heart came undone. Apparently, as I would
learn, the heart lining of children with Ds is not the same as the rest people without Ds, and residual VSD's 'aren't uncommon'. Back to the OR. This would be Natalie's third heart surgery. Again, it was a blessing to have that
feeding tube! I was able to keep her hydrated during the night before surgery while she
slept. The surgery was a cardiac catheterization (where they go in
through the groin) and they were going to take some measurements and see if
they could balloon her pulmonary arteries open as they were increasingly
narrowing without doing a full open heart surgery. Unfortunately, it was not successful. During this hospital stay, Nat developed an allergy to the antibiotic that was given to her to fend off any possible infection. Her 4th heart surgery would happen in February 2004 at 14
months old. This time it would be
another full open heart surgery.
Surgery #4
Before this surgery, I was once again able to keep her hydrated through
the night, which meant that getting the IV's started in the morning was not as difficult
as usual. The feeding tube was, once again, our friend. During this
surgery, it was determined that there were a lot of adhesions (bands of
scar tissue) that were basically pulling down on her arteries. Regardless, they were
still narrowed, and she required some gortex patches on the right and left sides. Happily, it was
determined that she was also big enough to have the smallest replacement valve
that they had, put in place. This valve was supposed to last 3-5 years. During
the surgery, they had difficulty finding the 1mm VSD and the electrical system in her heart ended up damaged in the process. A few days after surgery,
the home health nurse said her pulse seemed low, so we went back into the
hospital and an ECG revealed that she didn't have any P waves (Although I know nothing about reading ECG's, I could tell by
the look on the physicians assistant's faces that P waves are something that we all
need, and it wasn't good that Nat didn't have any! I was right.)
Her doctors and I monitored her for a few months, since this type of issue
can sometimes correct itself. Nat wasn't that lucky.
Surgery #5
About 6 months after surgery she
woke up one morning and looked like she hadn't gone to bed, even though she had
slept for over 12 hours. They put a 24hr heart monitor on her and we
found out that her heart rate was dropping to 22 in the middle of the night.
Nine days later, we were back in the OR for a pacemaker....that was
surgery #5. Natalie was only two and a half.
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| Natalie, age 2 1/2. Three days after her pacemaker implantation. |
Because of the RSV and MRSA when she was a baby, Nat developed asthma.
We spent a few weeks every 6 months or so in the hospital for pneumonia
or RSV (yes...RSV...again...when she was 5!) Around the age of 5, I started giving her
omega 3's/fish oil and her lungs improved markedly! We were able to
discontinue the maintenance asthma medicine, and haven't ended up in the hospital
again for lung issues.
Surgery #6
Finally, in 2012, the battery in her pacemaker was nearing the end of
its viability and needed to be replaced. It was supposed to be a 'simple'
surgery, but the pacemaker, which had been located in a pocket of muscle in her
abdomen, was pushed through her abdominal wall and was directly on her
intestines. Not good. So a whole new pocket needed to be created
and the pacemaker needed to be relocated. It turned into much more
complex surgery than originally anticipated. Natalie came through it like a trooper though!
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| Still a little groggy after having her pacemaker replaced. |
None of this slows Natalie down....
The valve that was implanted when she was 14 months kept on working for almost 10 years before her cardiologist decided it was time for it to be replaced. That was in February of 2014. That is where we are now.....heading toward her 7th heart surgery on April 21, 2014 which will be her 12th hospitalization.
Like Natalie always says, “Heart girls don’t quit and failure is not an option!”
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