Knoah and Dr. Pauli!
Last Thursday, I made the yearly pilgrimage up to Grand Rapids for Knoah’s annual visit with Dr. Pauli.
The verdict: It went fairly well….not as many issues as previous times, however recurrent issues are still present and new ones popped up.
So, Knoah’s Good Report!
1. His legs are still straight! YEAH!!! Typically some children with Achondroplasia will have bowing of the lower legs. This is due in part to the Tibia and Fibula. The Tibia, while not directly affected by Achondroplasia is still shorter in an LP than an AH person. The Fibula, continues to grow at a “normal” pace. At the point when the Tibia and Fibula start to pass each other in length, the Fibula starts to bow and causes the Tibia to bow along with it. The usual fix to this is surgery to brake the bones, set them in pins and rods to straighten out the legs.
So, no issues there!
2. Head size is good. He is at 56.7 cms, which puts him on the Achon growth chart in the 65% percentile. Which is good! Hopefully his head size has started to slow in growth.
3. His weight is good. He is 30 lbs, which still puts him on the smaller end of the spectrum, but compared to weight and height he is just under the 50% percentile, so he has caught up a lot!
Now, issues, which still remain plentiful…
1. Wrists: Knoah’s wrists are still very flexiable. Which means you can take his hand and bend it back toward his forearm and have the back of his hand touch his arm….BLAH. Dr. Pauli said that while this can happen it’s uncommon especially at Knoah’s age. We have to monitor this as he gets older and as he enters school. With writing, this can cause an issue with fatique of the wrists and hands. We may have to look at accomdedating him with less writing assignemnts to prevent the stress on his wrists. Worst case senerio is we can brace his wrists to prevent them from over extending.
2. Agh! He just had sugery June 22 to have his T-Tubes or ear tubes replaced for the 2nd time. We know get to do it again. One of then have already fallen out and the other is on it’s way….Nice.
3. Breathing. He is a snorter. Dr. Pauli said it at our first visit and he still hasn’t grown out of it. Even with his Tonsils removed and Adenoides removed twice, he is still a very loud sleeper. Doctor Pauli said that if his breathing starts to become worse or I start noticing an increase in his sleep apnea, to obviously get him into to have a sleep study, but for right now, as long as his sleep is not affected to not worry. His coloring has looked very good and I haven’t has any concerns other than the Achons snore, which can be quite loud.
4. Finally, his back. Agh. The “short version”. We can continue bracing therapy, with the expeectation that to see any type of measurable improvement, he will be in a brace at minimum another 2 to 3 years. I explained that *if* I saw some improvement during the last three bracing therapies, I would be more apt to continue bracing. However, with the last X-ray only showing a correction of 2 degrees, I fel I am tourturing him more than helping him. Even if we continue to brace there is no guarentee he will avoid surgery. If we do not brace, as he is doing quite fine mobility wise, he will have surgery by the time he’s 8. So, we have some decisions to make. His curve right now is hovering about 68 degrees or so with some correction of 20 degrees lying on his tummy. Personally, I think that’s the way to go, but I will leave that for another time….
So, other than that, Knoah is Knoah, just as ornary, just as bossy, just as adorable!





This post has 7 comments
January 19th, 2010
Sounds like a pretty good report overall! I’ve been waiting to hear that Trace has to have new tubes put in, but so far, so good. And I think I’m with you, if the bracing isn’t helping, what’s the point. Deal with the surgery when it comes up. I also agree that Knoah is absolutely adorable!
January 19th, 2010
I was just going to say the same as Destini! Sounds pretty darn good overall! I hope spinal surgery stays in the distant distant future or not at all! Yay for those legs being straight! We know all too well about bowing with Sonya, not fun! I totally agree, he is such an adorable cutie!!!
January 19th, 2010
Sounds like a good report to me too. I know the snorting/snoring thing too well. Aidan’s is still quite loud but not as bad as before his T & A. Knoah is handsome as well as adorable!
January 21st, 2010
We have to finish our phone date and we can talk more about Knoah’s appointment. You know that I support any decision you make and will be there for you both. Love you!
January 23rd, 2010
I’m happy to hear that Knoah’s appointment went fairly well with an overall good report! Good luck making a decision with his bracing therapy! I would lean towards not doing so too if it isn’t helping. Knoah is so adorable!!
February 1st, 2010
P.S. You have been nominated for the Kreativ Blogger Award (check out my latest post!)
February 9th, 2010
I just wanted to take this time to say thank you.. thank you.. THANK YOU!! Thank your for creating this site. You have been my most valuable resource for info on Achons. I am in my 33rd week of pregnancy and my son will be born with Achondroplasia (Just like his Daddy!!) When ever I have a question about anything I know I can turn to your page for help. THANK YOU