We are so excited to announce the newest member of our family, our precious little Jonah! Jonah is 3 1/2 years old, lives in China, and has Down syndrome. He had been listed on the shared list since 2012, but he is no longer waiting to be chosen! We can't wait to bring him home. Because Jonah was featured on Reece's Rainbow we have a family sponsorship page there where you can make a tax deductible donation to help get little Breck home if you so wish. Please head over there and take a peek at our little sweetie!
http://reecesrainbow.org/75910/sponsormurray
Monday, April 7, 2014
Thursday, April 3, 2014
Welcome home, Hannah!
Since my computer was fried by a lightening strike to our house I wasn't able to blog for many months, and now that I am back to it I wanted to finish out the series on BWS before moving on to current news. Now that I've finished the series I can happily announce our little Hannah is now HOME where she belongs!
The process to get Hannah seemed to take forever. It's incredibly frustrating waiting on other people to do their jobs so that your daughter can come home, and we seemed to continuously find ourselves waiting much longer than expected. There was 17 very long months from the time we were matched with Hannah to the time we got her home. Needless to say her arrival was long anticipated and full of great happiness and excitement!
We chose to have only one parent travel to save money, and since we felt the kids at home would do best with mommy being with them (especially with one of them being a 1 year old extremely attached to mom) Ernie got to be the one to travel. It's a good thing he has "been there done that" before because Hannah was NOT happy with the transition. She screamed for a long time but soon settled in and attached to her new daddy, and as an added benefit Ernie gained new found respect for my role as a mom. :)
After 2 weeks it was FINALLY time for Daddy and Hannah to come HOME!!!! We have an incredibly wonderful church family, and we were so excited many of our friends from church showed up to welcome them home.
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| My kids were happy to have their best friends show up at the airport to help pass the time while waiting for Daddy to arrive! |
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| Joshua doesn't like to have his picture taken. Apparently his friend doesn't either, so this picture is more amazing than it looks! |
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| Looking for Daddy |
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| The plane has landed. Daddy and our little Hannah are HERE!!!!! |
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| Watching for Daddy and Hannah to walk off the plane. They were the last ones off. :) |
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| Waiting for them to appear. |
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| THERE THEY ARE!!! THEY'RE HERE!!! THEY'RE HERE!!! |
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| One of the happiest moments of my entire life!!!!!!!!!!!! |
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| I love this picture - the way my husband is looking into my eyes! We are so focused on each other, I'm holding little Hannah for the first time ever, and Ernie is holding Elianna for the first time in over 2 weeks. Such a special moment! Naturally little Hannah was ready to go back to her Daddy since she had gotten so used to him over the last 2 weeks, but I couldn't wait any longer to hold her and Elianna was ready to let Daddy hold her. (Once we were home Hannah took right to me.) |
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| I was feeding Hannah little fruit snacks to help her get accustomed to me, but Elianna wanted to feed her new sister herself. It was the sweetest thing ever to watch the first moments of bonding between the two girls who will grow up together as "virtual twins." |
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| Micaelyn's first moments with her new little sister. |
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| Even at her young age Elianna understood that Hannah was scared and upset and wanted to make her feel better. Such love! |
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| Our first family photo with Hannah (but missing Christina). |
Friday, March 28, 2014
BWS - Other considerations
There are a number of other conditions that can occur as a result of BWS. I'm not going to attempt to list them all, just some of the ones we have to be most concerned about.
Hypotonia/ developmental delays - Hypotonia (reduced muscle tone) is a symptom of many different genetic syndromes, including BWS. Many kids have developmental delays as a result of the hypotonia. We have no idea if Micaelyn had any early developmental delays since we didn't get her until she was 3 1/2. It's quite possible she had some early delays but had mostly caught up by the time we got her. Elianna did have some delays but not severe. She was later to roll over than expected, later to sit up, later to walk, etc., but her delays were not so severe as to cause any great concern. I knew to expect some delays, so we just gave her a lot of extra help. All her older siblings have been great at encouraging her, and they provide a lot of motivation for her to keep trying new things because she is so eager to keep up with them.
Hyperactivity/ stubbornness/ aggressiveness/ other difficult behaviors - It's hard to know how much of a young child's difficult behavior is normal immaturity combined with personality versus how much of it is a result of an abnormal disorder. Nonetheless difficult behaviors are another concern with BWS. Truthfully neither of our girls with BWS could be considered an "easy" child. Micaelyn is very sweet and loving but still requires a lot of discipline to keep her in control. Likewise, Elianna can be rather difficult at times. She and Hannah are both 2 years old but she is FAR more difficult. At the last appointment with the geneticist she was asking about Elianna's behaviors, and while Hannah was acting like a normal 2 year old Elianna was proving just how "wild" she could be. I am sometimes shocked by the aggressiveness both Elianna and Micaelyn show at times. Elianna can be particularly rough with Hannah, and Micaelyn can be downright awful towards Malachi at times for no reason. Both girls are extra active, stubborn, very strong-willed, and constantly pushing the limits with their behaviors.
Seizures - Seizures are another risk associated with BWS. Neither Elianna nor Micaelyn have had a seizure that we know of (we can't know for sure about Micaelyn since she was older at adoption). However, because I know they are at risk for them I am always extra careful at keeping temperatures under control when they are sick (Elianna has had many episodes of high fever). One seizure is concerning enough, but I also think about the possibility of a febrile seizure leading to a recurring seizure problem.
Kidney problems - People with BWS are at higher risk for a number of different kidney problems. Our girls get regular ultrasounds and kidney function tests to screen for kidney problems. We have had extra concern about Micaelyn since the formula she drank in China was tainted with melamine, but so far she hasn't had any problems.
Cardiac abnormalities - Cardiac abnormalities are another concern with BWS. So far we haven't had any concerns with Micaelyn. It was recommended to us by the geneticist she be evaluated at some point by a cardiologist, but since she has no signs or symptoms we have decided to wait until she is old enough to participate in sports and have the cardiologist evaluate her at that time. Elianna was born with a murmur, probably caused by a "hole in the heart" type defect. She was referred to a cardiologist before we adopted her, but she never got seen. By the time we got her the doctor here didn't feel there was a reason for concern, and her murmur is no longer audible so most likely she was born with a heart defect that corrected itself. Once she is older and participating in sports we will have her evaluated by a cardiologist just to make sure there are no more cardiac abnormalities. There is always the risk the girls could be fine and then develop abnormalities later such as cardiomegaly (an enlarged heart), so by holding off the cardiac testing until they are older we can catch any issues that arise while they are growing (the risk for heart problems isn't high enough for repeated testing each year). Obviously if they were to start having any signs or symptoms of cardiac problems we would get them tested immediately.
Teeth problems - Although there are several problems with the teeth related to an enlarged tongue (e.g. underbite, teeth splaying), I've read there can also be a problem with the enamel on the teeth. At her most recent dental exam the dentist said Micaelyn was starting to develop places on many of her teeth where the enamel was wearing away. He referred us to another dentist who will help us determine the best course of treatment.
Frequent Illnesses - Kids with genetic disorders typically have weaker immune systems and deal with more illnesses than other kids. It seems likely this is the case with both Elianna and Micaelyn. They both get sick more often than the rest of their siblings and take longer to recover from illness. It seems like Elianna especially deals with an excessive amount of sickness, and although she and Hannah are constantly together and always sharing drinks, pacifiers, spoons, etc. Hannah doesn't deal with near the sickness Elianna does. Likewise, Micaelyn deals with far more sickness than Malachi and Caleb with whom she goes to the same school, and also despite the fact she frequently shares drinks and food with Malachi.
Here is an image I got from a BWS group that lists some of the features of BWS. There are certainly many features not listed, and I have no idea what the source of the image is but thought it was quite interesting.
Hypotonia/ developmental delays - Hypotonia (reduced muscle tone) is a symptom of many different genetic syndromes, including BWS. Many kids have developmental delays as a result of the hypotonia. We have no idea if Micaelyn had any early developmental delays since we didn't get her until she was 3 1/2. It's quite possible she had some early delays but had mostly caught up by the time we got her. Elianna did have some delays but not severe. She was later to roll over than expected, later to sit up, later to walk, etc., but her delays were not so severe as to cause any great concern. I knew to expect some delays, so we just gave her a lot of extra help. All her older siblings have been great at encouraging her, and they provide a lot of motivation for her to keep trying new things because she is so eager to keep up with them.
Hyperactivity/ stubbornness/ aggressiveness/ other difficult behaviors - It's hard to know how much of a young child's difficult behavior is normal immaturity combined with personality versus how much of it is a result of an abnormal disorder. Nonetheless difficult behaviors are another concern with BWS. Truthfully neither of our girls with BWS could be considered an "easy" child. Micaelyn is very sweet and loving but still requires a lot of discipline to keep her in control. Likewise, Elianna can be rather difficult at times. She and Hannah are both 2 years old but she is FAR more difficult. At the last appointment with the geneticist she was asking about Elianna's behaviors, and while Hannah was acting like a normal 2 year old Elianna was proving just how "wild" she could be. I am sometimes shocked by the aggressiveness both Elianna and Micaelyn show at times. Elianna can be particularly rough with Hannah, and Micaelyn can be downright awful towards Malachi at times for no reason. Both girls are extra active, stubborn, very strong-willed, and constantly pushing the limits with their behaviors.
Seizures - Seizures are another risk associated with BWS. Neither Elianna nor Micaelyn have had a seizure that we know of (we can't know for sure about Micaelyn since she was older at adoption). However, because I know they are at risk for them I am always extra careful at keeping temperatures under control when they are sick (Elianna has had many episodes of high fever). One seizure is concerning enough, but I also think about the possibility of a febrile seizure leading to a recurring seizure problem.
Kidney problems - People with BWS are at higher risk for a number of different kidney problems. Our girls get regular ultrasounds and kidney function tests to screen for kidney problems. We have had extra concern about Micaelyn since the formula she drank in China was tainted with melamine, but so far she hasn't had any problems.
Cardiac abnormalities - Cardiac abnormalities are another concern with BWS. So far we haven't had any concerns with Micaelyn. It was recommended to us by the geneticist she be evaluated at some point by a cardiologist, but since she has no signs or symptoms we have decided to wait until she is old enough to participate in sports and have the cardiologist evaluate her at that time. Elianna was born with a murmur, probably caused by a "hole in the heart" type defect. She was referred to a cardiologist before we adopted her, but she never got seen. By the time we got her the doctor here didn't feel there was a reason for concern, and her murmur is no longer audible so most likely she was born with a heart defect that corrected itself. Once she is older and participating in sports we will have her evaluated by a cardiologist just to make sure there are no more cardiac abnormalities. There is always the risk the girls could be fine and then develop abnormalities later such as cardiomegaly (an enlarged heart), so by holding off the cardiac testing until they are older we can catch any issues that arise while they are growing (the risk for heart problems isn't high enough for repeated testing each year). Obviously if they were to start having any signs or symptoms of cardiac problems we would get them tested immediately.
Teeth problems - Although there are several problems with the teeth related to an enlarged tongue (e.g. underbite, teeth splaying), I've read there can also be a problem with the enamel on the teeth. At her most recent dental exam the dentist said Micaelyn was starting to develop places on many of her teeth where the enamel was wearing away. He referred us to another dentist who will help us determine the best course of treatment.
Frequent Illnesses - Kids with genetic disorders typically have weaker immune systems and deal with more illnesses than other kids. It seems likely this is the case with both Elianna and Micaelyn. They both get sick more often than the rest of their siblings and take longer to recover from illness. It seems like Elianna especially deals with an excessive amount of sickness, and although she and Hannah are constantly together and always sharing drinks, pacifiers, spoons, etc. Hannah doesn't deal with near the sickness Elianna does. Likewise, Micaelyn deals with far more sickness than Malachi and Caleb with whom she goes to the same school, and also despite the fact she frequently shares drinks and food with Malachi.
Here is an image I got from a BWS group that lists some of the features of BWS. There are certainly many features not listed, and I have no idea what the source of the image is but thought it was quite interesting.
Thursday, March 27, 2014
BWS - Other physical features
There are a number of other physical features that are common in kids with BWS. These include:
Ear creases - Many kids with BWS have ear creases or pits. In fact, this is so common it's even one of the signs used in diagnosing a patient. A patient with BWS may also present with low set ears. Here are some pictures of Micaelyn and Elianna's ears to demonstrate the ear creases and pits. Note, however, it's not easy to get good pictures to show these features, especially on a 2 year old, so they are a bit difficult to see in the photos.
Prominent Occiput - The back part of the head is known as the occipital region, so a prominent occiput means the back part of the head is more prominent than it should be. This is is only mildly noticeable in Elianna but very noticeable in Micaelyn. It is most noticeable when washing her hair as the back of her head bulges outward much more than normal so it feels different than the shape of my other kids' heads. The good thing is her long hair obscures the shape of the back of her head so it's doubtful anyone will ever notice it.
Nevus Flammeus - This is a red birthmark, also called a stork bite, that most often occurs between the eyebrows but can also appear on the eyelids or other parts of the face. It usually fades within the first few years of life. They also appear regularly on kids without any genetic syndrome (our son Caleb had one on each of his eyelids) so don't worry if your kid had one!
Epicanthal folds - Epicanthal folds of the eyes are sometimes seen in kids with BWS. They are also seen in kids with Down syndrome, and this combined with the enlarged/protruding tongue and mid-face hypoplasia common with both syndromes (and also the pot-bellied appearance caused by abdominal hernia and/or diastasis recti (abdominal muscle separation) seen with both BWS and Down syndrome) may be the reason why so many babies with BWS are mistaken to have Down syndrome (this is occasionally brought up on the BWS groups). Both of our daughters with BWS would have epicanthal folds even without BWS due to their ethnicity, but admittedly when we first saw Micaelyn she did remind us of a child with Down syndrome (Today I'm sure she would never be mistaken for a kid with Down syndrome - it's usually just very young kids with BWS that are mistaken to have Down syndrome.).
Other Orthopedic Issues - In being a part of a worldwide group of families affected by BWS one comes to realize there are many more issues faced by kids with BWS than the ones listed as the common features. It seems orthopedic issues are more common among these kids than among most kids. Micaelyn was born with a club foot on her right side, and interestingly enough Elianna's right foot also turns inward. At this point it's not severe enough for us to correct it, but we will continue to monitor it as she grows.
Ear creases - Many kids with BWS have ear creases or pits. In fact, this is so common it's even one of the signs used in diagnosing a patient. A patient with BWS may also present with low set ears. Here are some pictures of Micaelyn and Elianna's ears to demonstrate the ear creases and pits. Note, however, it's not easy to get good pictures to show these features, especially on a 2 year old, so they are a bit difficult to see in the photos.
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| Creases on the backside of Elianna's ears (some of them are hidden by my thumb, but you can see some to the left of my thumb in the area of her ear lobe) |
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| You can see a crease on Elianna's ear lobe (there is a single hair running over it). Micaelyn has more noticeable creases in her ear lobe, but her ear piercing hides them pretty well. |
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| Creases on the backside of Micaelyn's ears (look above her earring). |
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| A preauricular ear pit on Micaelyn's ear (look near the center of the photograph for the reddish colored dot, there are a few stray pieces of hair near it with one just barely touching the pit) |
Prominent Occiput - The back part of the head is known as the occipital region, so a prominent occiput means the back part of the head is more prominent than it should be. This is is only mildly noticeable in Elianna but very noticeable in Micaelyn. It is most noticeable when washing her hair as the back of her head bulges outward much more than normal so it feels different than the shape of my other kids' heads. The good thing is her long hair obscures the shape of the back of her head so it's doubtful anyone will ever notice it.
Nevus Flammeus - This is a red birthmark, also called a stork bite, that most often occurs between the eyebrows but can also appear on the eyelids or other parts of the face. It usually fades within the first few years of life. They also appear regularly on kids without any genetic syndrome (our son Caleb had one on each of his eyelids) so don't worry if your kid had one!
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| Since we didn't get Micaelyn until she was 3 1/2 years old and these marks usually fade within the first couple of years we really weren't expecting to see one on Micaelyn, especially with her darker skin. Nonetheless one was still visible between her eyebrows. |
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| An enlarged copy of the same picture. She was 5 years old in this picture, and even today (at 7 years old) in the right lighting the mark is still visible so obviously hers is one that didn't completely fade within the first few years. |
Epicanthal folds - Epicanthal folds of the eyes are sometimes seen in kids with BWS. They are also seen in kids with Down syndrome, and this combined with the enlarged/protruding tongue and mid-face hypoplasia common with both syndromes (and also the pot-bellied appearance caused by abdominal hernia and/or diastasis recti (abdominal muscle separation) seen with both BWS and Down syndrome) may be the reason why so many babies with BWS are mistaken to have Down syndrome (this is occasionally brought up on the BWS groups). Both of our daughters with BWS would have epicanthal folds even without BWS due to their ethnicity, but admittedly when we first saw Micaelyn she did remind us of a child with Down syndrome (Today I'm sure she would never be mistaken for a kid with Down syndrome - it's usually just very young kids with BWS that are mistaken to have Down syndrome.).
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| Our second day with Micaelyn just after we completed her adoption, 3 years old. |
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| It's not her whole leg that turns inward, only her foot. |
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| Her foot always turns inward like this, even when standing. It's noticeable when she walks also. |
Wednesday, March 26, 2014
BWS - Intellectual Effects
When we first reviewed Micaelyn's file and started researching all her individual diagnoses we pretty quickly realized she almost certainly had BWS (and Dr. Beckwith himself, for whom BWS is named, agreed). In discovering she had BWS we had to be prepared for her to have additional problems related to the syndrome but not recorded in the very limited information we had on her, so we started learning about all the other possible issues we might have to deal with once we got her home. There were a number of sources that listed "mental retardation" as a symptom (many, including wikipedia, have now removed this wording), and there were others that talked about learning disabilities. Some sources just listed these things as a feature of BWS, while the better sources described the possibility that any significant intellectual deficiencies were the result of untreated hypoglycemia (hypoglycemia during infancy is another sign of BWS).
We knew that as an orphan in the poorest province in China there was very little chance Micaelyn had been treated for hypoglycemia, so we also knew there was a significant possibility she would have noticeable intellectual deficiencies. Thus we weren't surprised as we started trying to teach her different things (e.g. colors, letters) that she was having difficulty. I first started trying to formally teach her a preschool curriculum at 4 years old, but it quickly became obvious she just wasn't ready for the material. We eventually enrolled her in a really good preschool, and it didn't take long before the teachers suggested to us she had some learning disabilities. We ended up holding off on sending her to kindergarten, so she didn't start until this school year (she is 7 years old right now). Thankfully my prayers for her to have a good teacher were answered, and she not only has a wonderful teacher but also has some great tutors as well. They even enrolled her in an after school enrichment program where she gets further help in her academic material. Overall she is doing well, and we are very pleased with her progress. It may involve more work and take her a little longer but we feel confident that with time, extra help, and the right motivation Micaelyn will do fine in school.
At this point Elianna does not appear to have any learning disabilities, though since she just turned 2 it would be nearly impossible to rule them out. However, because she was born in the US, was diagnosed with BWS as a newborn, and received all the appropriate medical treatment we have no concerns about intellectual deficiencies resulting from untreated hypoglycemia.
Here are a few pictures from last fall (and late summer). My computer got fried as a result of lightening striking our house so I am just now getting back to blogging and figured I'd include some older pictures.
We knew that as an orphan in the poorest province in China there was very little chance Micaelyn had been treated for hypoglycemia, so we also knew there was a significant possibility she would have noticeable intellectual deficiencies. Thus we weren't surprised as we started trying to teach her different things (e.g. colors, letters) that she was having difficulty. I first started trying to formally teach her a preschool curriculum at 4 years old, but it quickly became obvious she just wasn't ready for the material. We eventually enrolled her in a really good preschool, and it didn't take long before the teachers suggested to us she had some learning disabilities. We ended up holding off on sending her to kindergarten, so she didn't start until this school year (she is 7 years old right now). Thankfully my prayers for her to have a good teacher were answered, and she not only has a wonderful teacher but also has some great tutors as well. They even enrolled her in an after school enrichment program where she gets further help in her academic material. Overall she is doing well, and we are very pleased with her progress. It may involve more work and take her a little longer but we feel confident that with time, extra help, and the right motivation Micaelyn will do fine in school.
At this point Elianna does not appear to have any learning disabilities, though since she just turned 2 it would be nearly impossible to rule them out. However, because she was born in the US, was diagnosed with BWS as a newborn, and received all the appropriate medical treatment we have no concerns about intellectual deficiencies resulting from untreated hypoglycemia.
Here are a few pictures from last fall (and late summer). My computer got fried as a result of lightening striking our house so I am just now getting back to blogging and figured I'd include some older pictures.
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| Last September, a rare glimpse of Elianna with her mouth closed. |
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| Pirate day at Krispy Kreme (free dozen donuts for everyone dressed as a pirate). |
Tuesday, March 25, 2014
BWS - Hearing Loss
Since we are all looking forward to warmer weather here are some random pictures from last summer.
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| At the hospital for another ultrasound |
Monday, March 24, 2014
BWS - Hemihypertrophy/Hemihyperplasia
Another common symptom seen in kids with BWS is hemihypertrophy (also called hemihyperplasia). This is an abnormal asymmetry of the body in which one side or part of the body grows bigger than the other. It could be an entire side of the body that is bigger than the other, or it could be one part, e.g. one arm that is larger, one leg that is larger, one side of the face that is larger. Micaelyn has A LOT of the signs of BWS, but this is one she doesn't have (as with any medical issue a patient rarely presents with all of the signs). Elianna does have some hemihypertrophy (one leg and arm is bigger than the other), but it seems pretty minor so far so we will just continue to monitor it.
Sunday, March 23, 2014
BWS - Abdominal Wall Defects
Most kids with BWS have some sort of abdominal wall defects. Micaelyn was born with an omphalocele which had been surgically corrected by the time she was found as an orphan. Unfortunately she was from a very poor remote part of China, and the repair that was done was not to the standards that would be expected here in America. At her most recent visit with the geneticist it was suggested that we see a surgeon for an evaluation of her abdomen and possibly more surgery. We have always thought her scar seemed odd, and the geneticist felt not only did the scar look bad but also that it looked very likely her abdominal muscles were never correctly joined. Her exact words were, "It looks they just opened her up and shoved everything back in then closed her." It is not an emergency situation so we are going to wait until summer to see the doctor since she has already missed a lot of school this year due to frequent sickness, doctor's appointments, ultrasounds, etc.
Elianna was born with only a mild umbilical hernia. We do not believe it to be an issue as it has pretty much corrected itself, but we are closely monitoring it as she is developing a very noticeable dimple above her belly button.
The best way to get an understanding of what an omphalocele is is to do an image search on google. Click here to take you there.
In lieu of pictures of my girls abdomens, here are a few random pictures of them.
Elianna was born with only a mild umbilical hernia. We do not believe it to be an issue as it has pretty much corrected itself, but we are closely monitoring it as she is developing a very noticeable dimple above her belly button.
The best way to get an understanding of what an omphalocele is is to do an image search on google. Click here to take you there.
In lieu of pictures of my girls abdomens, here are a few random pictures of them.
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