I am John Grayhack. I work at the Children's Memorial Hospital here in Chicago. And I am on the medical advisory board or panel for SMA.

Obviously you guys know there is a neurological dysfunction with muscular consequences. The thing that I focus on are the greatest things to forming contractures. They come up at different time spans of life and certainly is more or less degrees with different people. Most of you all have a pediatrician, and again I am a pediatric surgeon myself. They may have there own styles or prejudices or totally unfounded opinions and I have to say that we had a discussion last night in the medical board meeting we had and the majority of my opinions are unfounded except for that I have seen a lot of kids with SMA which is not a very large percentage of kids with SMA, but you do this for their joint and 10 years later they are fine and you do this for their spine and this one after that. Some times we go about with general conditions about the joints and spine. My opinion, I feel, is better than theirs. It may not be true.

The whole point is the spine and joints or whatever should be part of life's function. And that means to be pain free, be very active, and to be as moveable as possible. So, it is all about function. There is nothing wrong with having a straight spine or a curved spine, if your function is well then that is great.

Around the joint, therapeutic concerns are with the contractors. What you really want to do is to have a pain free joint to move the limbs at hand and it lines up for what you want to do. For some people, the joint can be out of the socket and it is pain free and it moves well enough and you can sit when you need to sit and if you happen to be able to stand you can well enough. There is very little evidence to say that a joint can, for instance the hip is the most common for people to worry about, that the hip being out of the socket would necessarily become painful. A hip that is sort of half way out of the socket is no more likely to become painful than one that is all the way out or all the way in. It just changes the mechanics. But just thinking of this as an example, people who have no other choice but to accept a dislocated hip, if you have a dislocated hip and this is the way you walk and you don't change the mechanics of it then it doesn't necessarily become painful well into your 30s or 40s and these are people who are up and around and then get soar when there hips are high. Now if they may get soar, they may get fatigued, they may not walk well, but as far as pain goes they are very determined to stand up or sit down if it is out of joint you can still do that. If have spasticity of the muscle around that area where the joint is out and the muscles are tight, then that there is another issue, there are a couple of articles following other types of spastic dislocation that they both tend to be painful, but recently as people look at this they are not for sure if the joint is out or if the muscle is spastic and painful. But what they have done is look at adults who have a dislocated hip or some form of spasticity and about 50 to 75% of them appear to be painful. A lot of the studies are based on people who have severe cerebral palsy and they can't really tell you if you move my hips, then my hips hurt. Or if it is just my muscle is too tight. So it is not clear that you have to run around putting  hips into the socket, now if they are out and painful, that may be true. If your doctor says you know what you hip is out but we are not going to correct them, that is not crazy. I have had people email me in fear of their doctors fell over the edge. No, a lot of doctors have kind of gone over the edge but not with the hip question. You are okay. So if the hip just doesn't look perfect, it is not a big issue.

The other issues around here are the tightness of the muscles, those contractures and such. Those can interfere with your daily living in putting shoes on, standing in a stander, or stand independently. Even to sit sometimes, it gets too tight, or somebody was complaining about their hamstrings. With the hamstrings are a bit tight but your hamstrings would have to be awful tight to prevent you from sitting well. If you imagine yourself in different positions that they would have to pull back very hard. It may be difficult to do standing once you're knees are contracted at about 30 degrees. But you can still do some things at 90 or even 100 degrees of contractures. So you have to keep track of where your goal is for the range of motion. You have to stick with what your goal is with all of this. You don't necessarily have to have your hip in the socket. You don't necessarily have to have full range of motion and again you can do stretches when you can or use splinting to help with what you can, but you don't need that surgery to get full range of motion. Most of the time you don't. It is not going to be a huge benefit. You have to keep track of where your goal is.

The most important thing in the evaluation is the exam. There are times when you get an x-ray, but with most you get a sort of a baseline x-ray of the spinal column at some point and it maybe worth while to get a base line x-ray of the hip. But to get one every time is not necessarily helping. If you are watching the spine, it doesn't necessarily matter what the x-ray shows, what matters is: is the kid sitting up straight enough? And the spine is not curved so much that I am worried about it. If it is not going to change the direction you are going to take, then you don't have to go get an x-ray. The thought behind x-rays is that you just want to know. The curve of the spine isn't so bad, but every one sitting in the room would like to know if the curve is actually getting worse. And it is not that you are thinking at all that you are going to operate on it, but it may help every body there keep track of where it is, particularly if you spend the whole year wondering how the spine was doing. So you don't need to get an x-ray done every time you see somebody to necessarily keep track of it. So I tend to get an x-ray either when everybody in the room would like to know an answer or when that answer is going to change what I am going to do. And a lot of times an x-ray is not going to change what I am going to do, particularly with the spinal column where the change in 6 months is not going to be that much.

With early intervention it is meant to try to avoid contractures or treat them if they are there and anything from SMOs to an AFO to a KAFO to a lot of other alphabet letters like RGOs can be used. We will talk more about how often you use them and so forth later. It also may be a functional aspect to which you need to consider, where a higher braces for a child who stands with them who wouldn't do much standing otherwise.

Again, if the hip is in the socket and it is just that the muscles are tight, then that is just a contracture. If it is part of the way out of the socket, but the two joint surfaces are still touching, then that is subluxation. Or that the hip is subluxed. So if the two joint surfaces are not touching, then it is dislocated. But one thing you need to keep in mind with the younger children, as in a infant, when you get an x-ray, a lot of the "bone" in the pelvic area is still cartilage and will not show up on the x-ray and may give the impression that the hip is dislocated or subluxed when it is not. So the x-ray is not going to be very helpful.

Another issue we talk about with hip x-rays is coxa. Coxa is Latin or Greek; I am not sure which one, for hip. They also use the terms "valga" or "valgus" and all that means is the hip is pointing up or down. In people who have to stand for the most part tend to be developing coming down and you may be told that your child has coxa valga or coxa valgus or that the hip is displaced. It doesn't matter which direction the angle of the neck is pointing as long as it is in front of the balls and socket of the pelvis. It is a common thing for kids who haven't been standing for a normal sort of weight bearing position and a normal muscle pull, their hips have been thrown more upright than down.

There are 2 aspects that you can look at: the ball side and the cup side. When they're looking at that on the ball side they are going to watch and see if it is gradually working its way out. But the ball, if it is in the hip or if it is out of the hip, or if it is in another county, when the ball grows up it is going to be a round ball. Maybe a little oval or something like that but it is going to be a round ball. The cup side, the pelvic side, if the ball is not sitting in the cup as you grow, it won't grow nice and round. It will grow kind of flat. They will use terms like dysplasia, but what they are really talking about is when you are an infant and you have the ball and the cup, the way that the cup gets its depth for some reason is by feeling the ball. Nobody knows exactly how it works, it is not really that the ball is crushed against it but whatever it is, if the ball is not there or if the ball is sitting away from the cup, instead of being really round and coming over the top it will be flatter and the further out the ball is and the longer it has been out of the socket, the flatter the cup will be. So that you will have less depth in the cup. So if you did say at the age of four, and the hip has never been in and now we are going to put it in, you have another issue to overcome. It doesn't mean you can't, I scheduled next week for a kid who both hips are out and the cup is going to be very shallow, when I go I am going to tilt the cup way over to and tilt the ball way down and kind