One of the most common symptoms of BWS
is an enlarged tongue. Sometimes the enlargement is mild enough to
be hardly noticeable except to medical professionals. Other times
the tongue is large enough to interfere with the child's ability to
close his/her mouth and cause problems with feeding, teeth and jaw
development, and even the ability to breathe, particularly during
sleep. When we got Micaelyn at 3 ½ years old her enlarged tongue
was immediately obvious to us. The only time she ever actually
closed her mouth was when she swallowed. Her mouth hung open all the
time, often times with the tongue visibly protruding. You almost
never saw her teeth, even when she smiled, because her tongue covered
them up. Of greater concern than the fact she never closed her mouth
(though that alone would have caused social problems once she started
school) was the fact she was developing a severe underbite as her
lower jaw was growing forward to accommodate her enlarged tongue.
Perhaps the greatest concern was the many signs of sleep apnea she
displayed.
Though we hated the idea of putting
Micaelyn through so much pain we knew she needed surgery to reduce
the size of her tongue. While her tongue was certainly longer than
it should have been, it was also much wider and thicker than a normal
tongue. In fact, before her surgery the surgeon had actually
expected based upon the thickness of Micaelyn's tongue that after she
had recovered from her first surgery he would have to do another
surgery using a laser to further reduce the thickness, but thank
goodness he managed to do such an incredible job with the first
surgery there was no need for the second.
The surgery was very hard on Micaelyn.
She was in the PICU for 4 days, on a ventilator for 3 days. She was
supposed to remain sedated while on the ventilator, but her body had
an amazing tolerance to the sedation drugs (the possibility of
prenatal exposure to drugs was proposed to us as a possible
explanation for such a high tolerance to the drugs). She not only
had to deal with the extreme pain from the surgery, the ventilator,
NG tube, catheter, and multiple IV's, she also had to be physically
restrained to prevent her from pulling out all the tubes. She was
constantly being pumped full of drugs that caused her to be combative
and even more miserable. Once she finally got off the ventilator and
many of the drugs she started having withdrawal attacks that made it
difficult to even hold her. Beyond the initial hospitalization and
recovery period she continued to experience great pain for several
weeks (eating and drinking was torturous for her), and she continued
to complain about the pain for several months after the surgery. To
make matters worse when we first brought her home from the hospital she nearly
completely shut down emotionally. I could see obvious signs of
attachment issues (this was why we waited a full year to do the
surgery after bringing her home – so she could learn to trust us
before we put her through such a painful procedure). Thankfully as
she recovered physically she also recovered emotionally, and today
she has no lasting effects from the surgery (other than an eagerness
to show off her “princess tongue” to anyone and everyone!).
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| A before and after comparison of her natural expression |
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| Before surgery her mouth was always open. It was amazing to us to see how different she looked after surgery! |
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| A before and after comparison of her smile |
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| It was amazing to see how different her smile looked. Before surgery her smile was very open-mouthed, and even with her mouth so wide open you still didn't notice teeth due to her enlarged tongue. We never expected to see such a visible difference from the surgery! |
