Radial Dysplasia

I think it's pretty obvious to most people who read this blog or know our family that Asher came to us with some visible differences. When we excepted his referral, we were told her had bilateral upper limb differences. Which is just to say that his arms are different on both sides. We were also told that his left radius, the bone on the thumb side of the forearm, was absent.
Now that Asher is home and we can actually see what is going on for ourselves, we have a more detailed diagnosis. He was born with radial dysplasia or radial aplasia Type III or in common language, radial club hand (a term no longer used by medical professionals). All this really means is the bones on the radial, thumb side, of his arms, wrist, and hand have no developed normally. This is severe on the left side in Asher's case, as the radial bone is only partly there, which causes his arm to be short and bowed in, while his hand turns inward.  He also has
non-functioning thumb numbs on both sides. This is a very rare condition and you will find very few others out there. I've been lucky enough to find and make contact with three other mommies with young children who have radial dysplasia.

Almost 2 weeks ago we made the long trek to our Shriners Hospital for Children. It was a really good visit and we were able to talk with a pediatric orthopedist doctor and also an occupational therapist who works with children like Asher. We were very pleased with Dr. R. He spent a fair amount of time with us talking about things. He is conservative about surgery and inclined to take a wait and see approach. I can't say enough how happy I am to find a doctor like this on our first try! Surgeons tend to want to do surgery first and ask questions later! Anyway, not Dr R! He mentioned a number of things we can consider doing to try to improve function for Asher, but stressed that nothing needs to be decided any time soon. He did suggest the removal of the thumb numb on the left hand, but other then that surgeries can wait for years from now. In fact, he stressed that most kids with these differences do just fine with no surgical intervention. I already knew all that, but, yeah for a doc who will say it!! He agreed with our OTs
here that a brace to stretch the tendons was a good idea, but said that since this does the most good during the first year of life, we should not push it at the expense of other development. We were encouraged to only have him wear the brace at night and to do some stretching exercise with every diaper change. So that is the plan of care for the left arm for now.

For the right hand/arm, he also wants to take a wait and see approach. We will be returning for x-rays in 6 months at which time he will consider doing some reconstruction work on the right thumb or, if that is not possible (which is very likely), look at doing pollicization surgery to give him a thumb. At first I was a little unsure about waiting that long to consider doing the surgery as it is usually done before the child is 2, but can be done at anytime before 5. But the more I thought about Dr R's reasons, the more I think he is right.
Basically, he wants to give Asher 6 months to grow and catch up develomentally before putting his arm in a cast for 6 weeks followed by a splint/brace for 6 more. Three months of not being able to use his most functional arm would indeed slow down and possibly stop the huge gains we are seeing in gross motor. Also those 6 months will give him more time to bond to us and to get in a better place nutritionally. (Hey, he has already put on over 3 pounds in 7 weeks!!)  So that's the plan... reevaluate in six months at which time hopefully we will do surgery.

It's been a short two and a half weeks since I wrote an Asher update. Yet how far he has come in that short time is worthy of a post.

As little as 10 days ago our hero would stand rooted to the spot and fuss if you left him standing against something. Now he will cruise up and down the couch. and his speed and confidence grows by the day. He will now pull to stand on any low, steady item. He does not yet have enough upper body strength to reach too far above his head to pull up. His balance is getting better and better. He has now stood for 3 seconds twice. He really wants to learn to walk so I think he's going to get there soon as he is getting braver and braver.

He has learned "patty cake" with all the motions (it is TOO cute) and says "pat, pat" when he wants to play it. He now also says "boo" when playing peek-a-boo. In other language development, he now signs up, down, and all done in addition to "more". He has responded to a stranger calling his name. He now says "mama" to mean me when in distress. He has differentiating cries between scared/upset and hunger/want
something.

Bonding wise, he is starting to see me as "mama". He does not yet want to admit that unless he really needs me, but it is coming. :)

He is doing more and more for himself... helping me dress him, wanting to feed himself. He has successfully used a fork to get food in his mouth if I load it for him. He has also used a spoon with relative success when I load it.

We've been to see the occupational therapist and Asher has been fitted for his first brace. The brace is to help stretch the tenants in his hand/arm so that the hand does not pull in so much and has a greater range of motion. We will change the angle of the brace every month or two. He tolerates it fairly well, but we've had some issue with it coming off or shifting went he is crawling. Here are a few picture from the first day he had it. We have a consult with Shriner's Hospital today which we are excited about. Can't wait to hear what they say.