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!

Two years, Happy 16th Birthday!

Last month, just a few days after we celebrated 2 years with Micaelyn, we also celebrated 2 years with Joshua. Then immediately after that was his 16th birthday. Unfortunately I was too busy to blog about it at the time, but I didn't want to do anymore posting until I got a chance to write a post about Joshua. He deserves it!

Joshua is an AMAZING kid! He is very nearly a perfect child. It's hard to believe we almost didn't get to adopt him. We were already in the process to adopt Micaelyn (a child who herself had waited many months on the photolisting) when we decided to adopt Joshua, a teenage boy who was about to "age-out," i.e. turn 14 years old and be marked as "too old" for adoption. Very sadly, our agency was willing to let him age-out and live the rest of his life as an orphan rather than let us adopt him, despite our previous experience with older child adoption, because they were unwilling to make an exception to their rule of not ever allowing families to adopt two unrelated children at the same time.

It took a HUGE battle to finally get the agency's approval to adopt Joshua. It was many weeks of endless meetings with social workers, numerous committee meetings, and countless phone calls and emails (during which time Micaelyn's adoption was put completely on hold, a huge concern considering her risk for tumors was so high she needed blood work every 6 weeks and ultrasounds every 12 weeks to screen for tumors but was receiving NO medical treatment in China for her condition).

In the end I wrote about 40,000 words to defend our case - my arguments ranged from our knowledge and experience in adoption, particularly older child adoption and attachment issues, to their mission as a Christian organization "dedicated to carrying out God's plan for every child to have a permanent, loving family," Joshua's need for a family, their responsibility to find him a family, and the lack of any other families who were both willing and able to adopt him in time. I wrote about other older children who had been listed with their agency but for whom they had never found a family, and I even included pictures of some of the ones who had aged out most recently (We actually first inquired about Joshua months before we really started to fight for him, and as it started to get closer and closer to his 14th birthday I started watching the agency's photolisting more and more waiting for him to find a family. During this time period other kids were already aging out, and I kept their pictures knowing that if it came down to the line and we had to fight for Joshua I could use this to help him avoid the same sad fate the others were dealt, a fate I still feel could have been avoided had the agency fought for those kids to have a family like we fought for Joshua to be our son).

In the end, although we waged a great war, I truly believe it was God's hand in it all that finally led the agency to give us their approval to adopt Joshua along with Micaelyn.  I will never forget the call from our agency to give us the news, nor their words to keep quiet and not make a big deal out of it because they didn't want other people to think they might also be able to get approval.  I said that whenever I told people about it I would make sure to say we were the rare exception to their rule.  Their response was, "No, you are not the rare exception, you are the ONLY exception."  Ironically, shortly after our fight there were a couple of other families allowed to bring home two kids at one time, both involving kids who were about to turn 14.  It was really tough to accept the fact that others were given approval so much more easily after we were put through so much in our battle to adopt Joshua, but at the same time we knew that had we not fought that battle for Joshua, had we not won the fight with our agency, had we not "broken the mold" for them, those other families would never have gotten approval either.  So our fight not only allowed Joshua to know the love of a family, it also helped a couple of other kids as well.  I know there are people who do not like to use the phrase "save a child" when referring to adoption, but when you look at the fate these children would have faced without adoption and the fact they were so close to that fate one could definitely make the argument Joshua and these other children were in fact "saved."  So while I will never be able to think about our battle with the agency without having a lot of hard feelings, knowing we not only got Joshua but also helped those other kids makes it all worth while.

So while we feel very blessed to have all our children, we know Joshua is an extra special blessing, really a miracle.  We came so close to not getting him, he came so close to not being adopted.  We so often look at him and think about that, about how close he came to a very different fate, about how close we came to never experiencing all the joy he brings to our family.  It took a fight to get him home, but he is so worth the battle we fought.  We are so thankful to God for bringing us the miracle of this great son.  Happy 2 years, happy 16th birthday, Joshua!

More tumor screening

Micaelyn and Elianna had their routine trip to the hospital last week to screen for tumors.  We got word today that all was good, praise God!

Mostly I don't think about their risk for cancer, but I have to admit the thought has crossed my mind occasionally that with TWO precious daughters having such a high risk for tumors the chance that at least one of them will develop cancer is just plain scary.  There are times I look at Elianna's beautiful head of hair and pray she never loses it to chemotherapy.  Then there is Micaelyn, who so often complains about her short hair in China, how they shaved it all off "because it had bugs in it!"  She has been so happy to let her hair grow out, and after 2 years it is finally to the point of being long (though not as long as she wants it).  How awful it would be for her to lose it all again!  And obviously losing their hair is minor compared to all the other concerns of a child dealing with cancer.

However, thankfully God is in control, and I allow my faith in Him to guide my life, not my fear of what could happen.  I remember back when Micaelyn was in China being concerned about the fact that as an orphan she was NOT receiving any of the necessary screening for tumors.  There was a chance we could get her home only to find she had tumors that were too far progressed to be treatable.  It was troubling to consider that possibility, but we went on faith that the same God who so perfectly created her was still holding her in the palms of His great hands.

Now I have these TWO precious daughters, both Chinese, though born on the opposite sides of the world, both with the same rare genetic syndrome.  Many people with BWS will never know anybody else with the same syndrome, and yet these two girls will grow up together sharing not only a bond of adoption and of ethnicity but also of BWS.  They will never have to feel alone in having BWS, they will always have each other!  What an amazing bond these two sweet sisters share!

At the waiting area inside Children's Hospital.  When you have a child that has to have blood work taken every 6 weeks plus ultrasounds every 12 weeks you do all you can to minimize the pain and maximize the fun.  I do a lot to make our frequent visits to the hospital fun (thank goodness for a good gift shop!),  and one of the things I have always done to help Micaelyn is take pictures of her during her ultrasound.  She LOVES having her picture taken,  so this not only adds an element of fun but also allows her to really see the overall picture of what they are doing.  Since the ultrasounds just involve her abdomen, sides, and back she doesn't have to take any clothes off (for this reason I never put her in a dress on hospital days - lifting up her shirt a bit is much less revealing than having to lift a dress all the way up).  However, this time we weren't able to take any pictures.  The hospital instituted a policy of no pictures.  While I understand not taking pictures during certain procedures, particularly if there are safety/emergency concerns, (e.g. I don't take pictures during the blood work because it is a traumatizing event and I feel it would be inappropriate to take pictures of my daughters like this, rather I need to be there holding and comforting them) I really feel it is my right as the parent to choose when I take pictures of my children.  Micaelyn was quite disappointed!

Introducing.........!!!!!

We are so proud to announce the newest addition to our family, a special little princess who is turning 1 year old TODAY!

We weren't planning another adoption just yet, but as always God's plans are so much better than our own!  When an agency contacted us about a little girl they felt would be perfect for our family, how could we say no?  We definitely had some reservations, but only because of the financial strain we are in from having completed 5 special needs adoptions in 6 years time.  However, while there were lots of verses in the Bible to encourage us and motivated us to proceed with the adoption, there was one passage that prevailed more so than all the rest:

For I was hungry and you gave me nothing to eat, I was thirsty and you gave me nothing to drink, I was a stranger and you did not invite me in, I needed clothes and you did not clothe me, I was sick and in prison and you did not look after me.’  “They also will answer, ‘Lord, when did we see you hungry or thirsty or a stranger or needing clothes or sick or in prison, and did not help you?’

“He will reply, ‘Truly I tell you, whatever you did not do for one of the least of these, you did not do for me.’ - Matthew 25: 42-45

When presented with the opportunity to love and care for this precious little child, to make her our daughter, there were only 2 possible answers - yes or no.  To say no seemed to be saying no not just to this baby girl but also to God himself.  We have always said we go on faith, and since money was the ONLY reason we could find to say no in the end we realized we really had no reason!  We would just have to proceed on faith that God would provide.

So while we weren't planning to start another adoption until we were a bit more stable financially, once this precious angel was presented to us we knew that once again we were seeing God's plan in action, not our own!  While we are trying to figure out how we'll cover all the adoption expenses, knowing without doubt God will provide, we are also growing ever more excited about having another little girl in our home.  She is only 4 1/2 months older than Elianna (and her Chinese name is An Na!), so they will be "virtual twins."  Of course since we are just now starting the process it will be close to a year before we bring her home, but I am already eagerly awaiting the day I can finally hold her in my arms and give this sweet baby all the love she needs!

Happy Birthday, baby girl!  You are no longer an orphan waiting for a family, you are now our BELOVED DAUGHTER waiting to come HOME!!!!!!!!!!!

Our precious daughter, Zhou An Na.  Her special needs are listed as cleft lip & palate, hearing impairment, and an enlarged liver,  but we think her most special need is to be loved!  

TWO YEARS!!!

Two years ago today a little girl walked into our arms, and our lives were forever changed!!!

I will never forget the first time I saw her.  As soon as the car pulled up to the building I started searching everywhere for her, and then I saw her walking up.  I had known this girl as my daughter through nothing more than a few pictures for OVER A YEAR, and for many months before that I had looked at her picture daily praying for her to find a family.  All those many, many months of dreaming of my daughter, and finally there she was in person!  I tried to hold myself together for her sake, but once I picked her up the held-up emotions of so many months of waiting burst through and the tears began to flow.  Poor little girl didn't know what to think!

Our first days together were tough.  She grieved terribly.  She cried.  She was inconsolable.  She tried leaving and would even try to escape in the middle of the night (thankfully the sound of the door opening always woke me).  However, before we even left China she had attached to us, and within just a month it was as if she had always been with us.

We had planned to adopt a baby from China, but as always God's plans are so much better than our own.  We knew this little girl needed a family, and we knew even though we had wanted a baby we could love her just as much as a baby.  I am ever so glad that I was willing to give up my dream of adopting a baby girl from China to accept the blessing of adopting a preschooler instead.  It didn't take long to realize just how perfectly this little girl fit into our family!  Micaelyn has filled our hearts with great love, our lives with great happiness, and our home with great humor.

Happy Forever Family Day, sweet daughter!  We are so very blessed and honored to be your parents!  We love you!

Our first glimpse.  Love at first sight!

Daddy's first time holding her.

First evening with her back at the hotel.  Poor little angel was so sad.  :(  But she did like all her pretty new clothes and jewelry I had brought, and she liked it when I painted her nails.  :)

Caleb tried to make her feel better when she went to bed.

Up bright and early the next morning, bag packed and ready to leave.  Notice she is holding a shoe.  She really liked her new shoes I had brought so she put them on but still felt the need to take her others with her.  However, she could only get one to fit in her backpack so she resorted to carrying the other.

Her first smiles with us!  It was such a good thing we had taken Caleb and Malachi with us because they were the ones who got her laughing and playing.  She had a lot of fun with them!

Happily settled in to her new life.  Hair all grown out.  Showing off her new smile (before her tongue reduction surgery her smile was very open-mouthed with a lot of tongue showing).  Loving life with her new family in America, but still a Chinese princess, loves her Chinese clothes and Chinese food, and still a pro with her chopsticks!

Routine Hospital Visit

Last week Micaelyn had her routine ultrasound and blood work to screen for tumors.  She also had a kidney function test since BWS puts her at high risk for other kidney problems beyond just cancer (and unfortunately her orphanage in China was known to have used the melamine-tainted formula which led to kidney problems, and Micaelyn was probably drinking this all the way up until we got her at 3 1/2 years old since they often keep kids on formula longer there).

It was all very routine, but there was one huge difference this time around.

This time Micaelyn had a buddy along with her!  Yes, our little Elianna also has Beckwith-Weidemann Syndrome, just like Micaelyn.

What an adorable little patient she is!

Our indescribable gift!

Yes, our new daughter is Chinese.

No, she is not in China.

She is here in my arms!!!

At just 3 months old!!!!!

First moments with Mommy.

Indescribable joy in meeting our precious little angel!

First bath from Mommy.

Taking in all the action in a home full of kids!

This was an absolutely amazing whirlwind adoption experience, taking just 1 month from the time we found out about our baby girl to the time we held her in our arms.  In just 4 weeks we completed the home study and all other necessary paperwork, planned a trip, and traveled to meet our sweet little angel!  After 4 previous lengthy adoptions, all full of troubles, it's still impossible to believe we completed an adoption so quickly and easily!  Also, because we have always had a heart for the waiting children our previous adoptions have all been "older" children.  Caleb (our biological son) has been our only baby.  It is such a joy to have a baby in our home again!

Thanks be to God for His indescribable gift! - 2 Corinthians 9:15

Introducing...

our newest blessing, our precious little Elianna!