Monday, June 24, 2013

BWS - Enlarged Tongue (macroglossia)

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!).

A before and after comparison of her natural expression

Before surgery her mouth was always open.  It was amazing to us to see how different she looked after surgery!

A before and after comparison of her smile

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!

1 comment:

Anonymous said...

Jennifer, thank you so much for posting this! I have so many questions, as I am thinking of getting the surgery for my daughter. could I email you?