Before and After Tongue Reduction Surgery

It is said a picture speaks a thousand words, and I think that is especially true in showing just how much Micaelyn needed the tongue reduction surgery.  There are different types of tongue enlargement - length, width, and depth.  Micaelyn had all 3 types with the depth being her most significant problem (and also the most difficult to deal with surgically).  Some people found it to hard to understand her need for surgery since her tongue did fit inside her mouth, but her tongue filled up her mouth so much she almost never closed her mouth other than to swallow.  Even at 4 1/2 years her mouth was always hanging open.  A major concern was the fact the pressure from her tongue was causing her lower jaw to be pushed forward so much that she had developed a significant underbite.  If she had not had the surgery the underbite would have continued to worsen so that by the time she was a teenager she would have needed major jaw reconstruction surgery along with the tongue reduction surgery.

Here are some pictures of Micaelyn before the surgery.  You can see how her mouth was always open. You couldn't even see her teeth because her tongue protruded beyond them.

 

 

 

 

Before the surgery Micaelyn very often had her tongue completely hanging out of her mouth, especially when she was busy doing something.  This next picture was taken completely spontaneously before she realized I was taking pictures (whenever she saw me with the camera she would pull her tongue in so even though she often looked like this it was hard to capture it in pictures).

Here is another one with her tongue stuck out.  It looks like she was sticking it out purposely for the picture, but it's just because she just happened to look towards me right as I was taking the picture.  We were at one of Joshua's basketball games, and because she thought I was still taking pictures of him I was able to get one of her without her pulling her tongue back in to pose for me.

This next picture has always been one of my favorite pictures because I just happened to catch her with her mouth completely closed (she had probably been swallowing).  We always thought she was beautiful, but whenever she had her mouth closed it was amazing to see just how beautiful she truly was without her tongue hanging out.  Getting an occasional glimpse of her with her mouth closed gave us a preview of what she might look like after surgery.

This is at the hospital 4 days after surgery, our first time to ever see her actually keeping her mouth closed.

This is when we were getting ready to go home from the hospital.  We were still in awe at how different she looked once she was able to keep her mouth closed.

 A few more pictures post-surgery...

I purposely chose non-smiling pictures for this post so as to show the difference in her natural expression.  I will do another post to show the difference in her smile.  However, since she loves to smile for the camera I don't have many of her not smiling (even in some of the ones above she has a small smile).  This is one of her in time-out, her least favorite place - no smiles for her here!  She can put on quite a drama scene when she gets sent to time-out!

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!

BWS - Weight Control

Beckwith-Weidemann Syndrome is an "overgrowth" syndrome so it's understandable and even expected for kids with BWS to be large for their age.  What's much harder to understand is at what point their size becomes unacceptable.  Should they be allowed to eat incessantly just because they have an overgrowth syndrome?  At what point do you consider a child's weight to be a problem when they have a syndrome that causes them to be big?

I have heard before that for a rough estimate you can take a child's age and add zero to it to get an idea of what they should weigh.  However, for the most part this has NOT been true for my kids.  Most of my kids have weighed considerably less than this.  Micaelyn is the exception.  She reached 40 pounds BEFORE she weighed 4 years old.  What makes that even more amazing is that the first 3 1/2 years of her life were spent in the very poorest province in all of China, a place where people are known to be small.  She was so large, her abdomen so big, that the Chinese physician performing her physical exam there in China commented on it as if it were not only abnormal but also a problem.  Because she had not been receiving any of the appropriate tumor screenings there in China we were actually concerned when we got Micaelyn her huge belly could have been the result of a tumor.  Thankfully it turned out to be nothing more than overeating.

As we quickly learned Micaelyn's appetite was truly insatiable, her desire to eat was incessant.  Since she was blessed enough to be placed in the care of a good foster family who loved her she was given a lot more opportunity to eat.  We feel she was in fact probably spoiled by her foster family (we are very grateful to them for the love they gave to her!).  Once we confirmed that Micaelyn had no tumors we felt we needed to get her weight under control.  It seemed to us that even with an "overgrowth" syndrome she was still a girl who would all to soon be self-conscious about her weight if we continued to let her eat and grow out of control.

Thus we were faced with the challenge of helping an overweight child with an overgrowth syndrome lose body fat while still allowing her to grow upwards as she aged.  To add to that challenge was the difficulty of controlling the diet of a preschool aged child with an insatiable appetite.  To make the situation even more challenging we had a son just 1 year older (Malachi) who was (and still is) tiny for his age, who has heart defects which make it very important we get him to grow as the goal is to get him as big as possible before he has to have surgery.  We had to get one child with an incessant desire for food to NOT eat as much while at the same time pushing another child nearly the same age to constantly eat more.

Through very close monitoring of her diet we have gotten Micaelyn to a much more healthy weight.  She will probably never be "skinny" but she looks healthy and for us that is what matters.  It hasn't been easy.  She constantly sneaks food behind our backs.  We have to be extra alert for food situations that wouldn't matter in most homes.  If somebody leaves an unfinished bowl of cereal on the kitchen counter in the morning and we don't get home until late that afternoon she will gladly start eating the nasty, mushy mess most other people would turn away from.  If someone drops food on the floor and she finds it later she will happily snatch it up to eat it.  No matter what I have told her about staying away from the stove, I have caught her on a number of occasions eating leftovers straight out of the pan as it sits on the stove (admittedly this has only happened AFTER dinner when the stove is off, but it still concerns me to know she was getting food directly from the stove).  So although the challenges of keeping Micaelyn's weight under control seem minor compared to some of the other issues related to BWS, on a day-to-day basis it's actually one of the more noticeable issues we deal with!

Sisters posing in our backyard.  You can see Micaelyn still has a little bit of a tummy, but it's now a much healthier size.

I think it's too early to know exactly what Elianna's eating habits will become, if she will have the same insatiable desire for food Micaelyn has, but she certainly shows a love of eating.  She loves to crawl around the floor looking through groceries that haven't been put away yet.  She could lay away a whole apple, skin and all, from the time she got her first few teeth.

Nothing better than a fruit smoothie while shopping!

BWS - Increased Appetite

Admittedly I have never seen "increased appetite" listed as a symptom of BWS, but being that it is an overgrowth condition I can't help but wonder if it is the cause of Micaelyn's voracious and insatiable appetite (I think it's probably too early to say what kind of appetite Elianna has - she certainly eats quite a bit, but I'm not sure it's overly excessive just yet).

Micaelyn seems to have no end to either her desire to eat or the amount she will eat.  For example, if taken out to a restaurant to eat she can eat an adult entree and when she is finished she will ask to eat the leftovers from everybody else's plates.  Then by the time we get home she will be asking for yet more food.  She wants to eat continuously all day long, not just nibble but eat full meals.  If given a big bowl of noodles she will eat the whole thing then ask to eat the leftovers from her siblings bowls.  She will ask for meat and vegetables to go with it.  Then when she is done she will ask for a banana.  After she has finished the banana she will ask for an apple.  Then she will ask for a treat.  If she doesn't have food available she will drink excessively.  Whenever we have places to go I always make sure the kids have a large cup of water to take along.  I learned pretty early with Micaelyn that I could not allow her to keep her cup in her car seat like the other kids.  She would have the whole thing drunk before we even got out of the driveway.

Along with Micaelyn's insatiable desire for food, she seems to have no reservations regarding what she eats.  She will eat pretty much anything, not just a wide variety of foods but even foods that are old.  We have seen her lay away a bag of chips, continuously declaring how wonderful the chips were, only to find out once we ate one ourselves they were disgustingly stale.  I have learned to be super cautious about any foods the boys don't finish in the morning, particularly if I will be gone for a long time before returning home after dropping them off at school because Micaelyn will come in and immediately start eating it.  For example, we can drop the boys off at school and then spend all morning at the hospital, have lunch while we are out, then when we return home that afternoon if there is any leftover food on the table or counter from breakfast she will start eating it.  It could be old soggy cereal that has been sitting in milk half the day, and she will gladly eat it as if it was a great treat.

Micaelyn's appetite is so exceptional even the kids at Caleb and Malachi's school talk about it.  Of course, even with an overgrowth disorder there is a limit to how much food a child should consume.  Needless to say, keeping Micaelyn's food consumption under control requires quite a bit more attention than with the average child!

BWS - Overgrowth

As I mentioned in my last post, BWS is an overgrowth syndrome.  Kids with this syndrome tend to be big, usually above the 90th percentile (though as I said kids typically only exhibit a few of the signs/symptoms, and the severity of those signs/symptoms can vary so a kid does not have to be big to have BWS).

It's always interesting to hear people's responses when I tell them how old Micaelyn and Elianna are.  Those who know they have an overgrowth disorder tend to say, "They don't really look very big."  Those who don't know it often say, "Wow, she's pretty big for her age!"  I think the difference comes with people who know they have BWS thinking they should look so huge as to look odd, and because my girls look completely "normal" it's hard for some people to see them as having a genetic "defect."

The truth is that neither Micaelyn nor Elianna will probably ever be very big in comparison to the average American child.  We don't know how big Micaelyn's birth parents were but we know she come from a part of China where the people tend to be quite small.  When we visited there I was bigger than most people, both men and women, and I am only 5'4" tall.  We do know how tall Elianna's birth parents were, and they were BOTH shorter than me, even her dad.  It's most likely that WITHOUT BWS both girls would be very petite compared to American kids.  Thus, even if BWS causes them to be "big" they will probably just be average in comparison to other kids here.

Micaelyn was really big even by American standards when we first got her (40 pounds at 3 years old).  After 2 years with us VERY closely controlling her food intake she has slimmed up significantly and now appears to range in the average/slightly above average range on the growth chart.  At last measurement she was near 50% for height and 75% for weight (so obviously while we are closely controlling her food intake she is not starving based on her height to weight ratio!).  Elianna also appears to fall in the average range for height and slightly above average range for weight (~70%).  So both girls are almost certainly a lot bigger than they would have been without BWS, but they are only slightly bigger than their peers here in America.

The fish Elianna was sitting on was vibrating so she was too fascinated with it to bother looking at the camera.

Beckwith-Weidemann Syndrome

Lots of people wonder about Micaelyn and Elianna's special needs.  They both have Beckwith-Weidemann Syndrome (BWS), a rare genetic syndrome caused by a defect with chromosome number 11.  Like most genetic syndromes, there are multiple issues related to the syndrome, and a child with BWS will generally only display a few of the signs and symptoms of the syndrome.  This is the first in a series of posts I plan to do to help our friends and family better understand BWS.

BWS is an "overgrowth" syndrome, so many of the signs and symptoms are related to overgrowth.  Being that tumors are composed of an abnormal overgrowth of cells, it's not surprising that kids with BWS  have a greatly increased risk of childhood cancer.  Because the liver and kidneys are the most likely (but not only) places for tumor growth in BWS kids they received abdominal ultrasounds every 12 weeks to screen for tumors in both the liver and kidneys as well as blood work every 6 weeks to further test for tumor growth in the liver.

So while there are many aspects of BWS, this increased risk for cancer is one of the most prominent features of the syndrome, and almost certainly the most concerning.

I know there are many people that end up on my blog because they are searching for info on BWS.  Because it is not very common many people with BWS (or parents of children with BWS) have a hard time finding others who understand the syndrome.  In fact, many people even have to educate their physicians on BWS.  There are lots of blogs on adoption, older child adoption, special needs adoptions, orphan ministries, etc.  However, it is much harder to find a blog about BWS.  I hope to use my blog not only to show others just how greatly our lives have been blessed through our adoptions in hopes that more orphans will find families of their own, but I also hope those families who have stumbled upon my blog because they have been affected by BWS will find my blog helpful to them as well.

Enjoying the hospital Christmas decorations in December.  When you have to have blood work every 6 weeks and ultrasounds every 12 weeks for two of your children you might as well make the most of each trip to the hospital.  The kids love the gift shop the best!

My beautiful little girls.  When I see them I do not see special needs, I see special blessings!