I know I just started this blog out of no where and most of you have no idea what is exactly wrong with Maxson. He was born with a few special attributes that only made him cuter :))))
Maxson's right ear is a unilateral grade II Microtia. Microtia means "little ear." I've never heard of it until Maxson was born, but apparently it's the 2nd most common birth defect after a cleft palate/lip. It's more common in boys and more common on the right side. Girls can be born with it and both boys and girls can have it on either side. They can be born with bilateral microtia as well....but it's more common to just have it on only one side. There are 4 different grades of it....grade 1 is just a slightly smaller ear or a slightly deformed ear. Grade II is an ear that is smaller, and slightly more deformed... but an ear canal can be present. In his case it is. He has his full ear...it's just folded. When you unfold it, an entire ear is present....almost like it has no cartilage to hold it in place. Grade III is the most common....there's no ear canal and the ear resembles a little peanut. Grade IV is when there is no ear present at all.
He did have a CT scan done when he was a week old (was not happy about that and we were not aware of it until it was done). He does have a middle ear! Each one of your ears has a middle ear and an inner ear. I was talking to someone who thought a middle ear was an ear in the middle of your head that connected the two :))))) You have an ear canal which leads to an ear drum. When you hear something your ear drum sends vibrations to the bones in the middle ear. Those bones vibrate to your cochlea....making it spin....which makes you hear! Maxson's ear drum doesn't work correctly and his stapes (a bone in the middle ear) is embedded in the soft tissue. His cochlea does work though!! He can hear loud sounds....it has to be loud enough to make everything vibrate to make his cochlea spin. An hearing aide would help fix that problem. (We're working on getting one...that'll be a whole other post). So since his middle ear is underdeveloped....he's know for having "conductive hearing loss." You're considered deaf when your inner ear is affected and doesn't work properly.
Maxson was also born with a Tessier 7 cleft (soft tissue) on his right side as well. Most people don't know this but you have 14 clefts in your face. A cleft palate is cleft 14. "Tessier" is just named after the guy who figured it out....Paul Tessier :)))) The cleft he has makes a "joker" like appearance in the face....there's an indentation from the corner of his mouth to his ear. Out of all the facial clefts.....this one is the most common (cleft palate is not considered a facial cleft). You can see the line in his cheek in some pictures...depends on the angle. The older he gets the more prominent it's becoming.
Usually accompanying a facial cleft 7 is macrostomia....which is also known as having a wide mouth. Pretty much the inside corner of his lips didn't meet where they were supposed to. His is very mild. Usually this inhibits babies from being able to thrive because they can't close their mouth fully. When we try to feed Maxson with a bottle, his mouth on the right side stays open so all the milk just pours out. We have to squeeze his cheeks together...almost making him pucker to help him eat. He hates it...almost as much as we hate doing it. Imagine eating like that!! Breastfeeding is just honestly easier for both of us. He can eat comfortably :))))) And he clearly likes to eat! Here is an extreme case....before and after her repair. You can see his in this picture...
Microtia is around 1 in 8,000 kids.....and his face is 1 in 80,000! They still don't know what causes it, but they believe from studying rats...it's a lack of blood flow to the face during the first 4-8 weeks of your pregnancy. Something as small as a blood vessel bursting causes this.
He was also born with two hemivertebrae in his lower back. His are like the top right picture. The two balance each other out....so it doesn't make his spine crooked! Clever little guy :))
Maxson was also born with a few holes in his heart. We did see a pediatrics cardiologist recently and they're 60% closed...so they're on their way to being closed fully :)))) We've very happy about that! Apparently that happens to 1 in 5 babies born full term or premature! Crazy!
They suspected he has Goldenhar Syndrome....but after researching and speaking to a geneticist....one of the main characteristics of Goldenhar is having dermoids in your eyes. His eyes are perfect. Goldenhar babies usually have kidney issues and problems with their vertebrae in their neck as well. Maxson does not have Goldenhar syndrome....and is on the most mild end of the spectrum for all of his "issues." We couldn't be more thankful. After being in the NICU...we saw babies who were in far worse conditions! His lungs, eyes, brain, ect are all perfect and that's all we could ask for! After all....it is just an ear :)))))))
Maxson's right ear is a unilateral grade II Microtia. Microtia means "little ear." I've never heard of it until Maxson was born, but apparently it's the 2nd most common birth defect after a cleft palate/lip. It's more common in boys and more common on the right side. Girls can be born with it and both boys and girls can have it on either side. They can be born with bilateral microtia as well....but it's more common to just have it on only one side. There are 4 different grades of it....grade 1 is just a slightly smaller ear or a slightly deformed ear. Grade II is an ear that is smaller, and slightly more deformed... but an ear canal can be present. In his case it is. He has his full ear...it's just folded. When you unfold it, an entire ear is present....almost like it has no cartilage to hold it in place. Grade III is the most common....there's no ear canal and the ear resembles a little peanut. Grade IV is when there is no ear present at all.
He did have a CT scan done when he was a week old (was not happy about that and we were not aware of it until it was done). He does have a middle ear! Each one of your ears has a middle ear and an inner ear. I was talking to someone who thought a middle ear was an ear in the middle of your head that connected the two :))))) You have an ear canal which leads to an ear drum. When you hear something your ear drum sends vibrations to the bones in the middle ear. Those bones vibrate to your cochlea....making it spin....which makes you hear! Maxson's ear drum doesn't work correctly and his stapes (a bone in the middle ear) is embedded in the soft tissue. His cochlea does work though!! He can hear loud sounds....it has to be loud enough to make everything vibrate to make his cochlea spin. An hearing aide would help fix that problem. (We're working on getting one...that'll be a whole other post). So since his middle ear is underdeveloped....he's know for having "conductive hearing loss." You're considered deaf when your inner ear is affected and doesn't work properly.
Maxson was also born with a Tessier 7 cleft (soft tissue) on his right side as well. Most people don't know this but you have 14 clefts in your face. A cleft palate is cleft 14. "Tessier" is just named after the guy who figured it out....Paul Tessier :)))) The cleft he has makes a "joker" like appearance in the face....there's an indentation from the corner of his mouth to his ear. Out of all the facial clefts.....this one is the most common (cleft palate is not considered a facial cleft). You can see the line in his cheek in some pictures...depends on the angle. The older he gets the more prominent it's becoming.
Microtia is around 1 in 8,000 kids.....and his face is 1 in 80,000! They still don't know what causes it, but they believe from studying rats...it's a lack of blood flow to the face during the first 4-8 weeks of your pregnancy. Something as small as a blood vessel bursting causes this.
He was also born with two hemivertebrae in his lower back. His are like the top right picture. The two balance each other out....so it doesn't make his spine crooked! Clever little guy :))
They suspected he has Goldenhar Syndrome....but after researching and speaking to a geneticist....one of the main characteristics of Goldenhar is having dermoids in your eyes. His eyes are perfect. Goldenhar babies usually have kidney issues and problems with their vertebrae in their neck as well. Maxson does not have Goldenhar syndrome....and is on the most mild end of the spectrum for all of his "issues." We couldn't be more thankful. After being in the NICU...we saw babies who were in far worse conditions! His lungs, eyes, brain, ect are all perfect and that's all we could ask for! After all....it is just an ear :)))))))
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