Orthopedics: Dr. Grayhack

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 of get them back together and hopefully they will do well. But for the longer it is out that is what you can expect. So those are the things that they are going to talk about when they are looking at a hip x-ray. They are not all necessarily bad, again, you have to keep track of what your goals are and how well that hip is going to work during your life for that. If you know that you are not going to really be up and around too much as you get older, it is not so bad to have a little bit of a shallow hip as long as it is in a good place. But if you are going to be extremely active and you have a shallow hip and a poorly formed cup that is going to wear out overtime. So most of the kids involved with SMA, you don't need a really deep cup and a really perfect fit. You need one that is going to be pain free, that you are going to be able to go through the functional things that you have. So it may be a little bit different when you go from one doctor to another, they may see things a little differently, that is kind of what I am trying to help you with, but a lot of you have seen 3, 4, 5 doctors and everyone of them use different languages and everybody tells you something different. But sometimes they are not that far off; they just sort of use different languages and view it from a different angle. So those are the things you look for on the x-ray: where the hip is, how it is developing, and which direction you are going. The goal is to find comfort, sitting or standing and then pelvic alignment. Now this is an issue that different doctors disagree about. Some people feel that if the hip is out of place on one side and not on the other, you pelvis is necessarily going to tilt. That is usually true. If your pelvis is tilting that does not necessarily mean that your spine is going to curve more. So the tilt of the spine and the tendency for your spine is to tilt over that aptitude of scoliosis so people have tried to look at is there a big tie between having a pelvical tilt and developing more scoliosis. Most people believe there is but until recently somebody did a study where they looked if you had the right hip out you developed a scoliosis to the left, so if this hip is out and the pelvis is down a little bit, you expect yourself to develop this curve going in that direction and it turns out that if your right hip is out you have about a 50/50 shot that your curve is going to go one direction or the other, but that doesn't necessarily mean it is tied together. So again, it is not necessarily a reason to put the hip back in all the time. So if both hips are out you shouldn't have any affect on your development. But that may be something that people discuss with you, but if in doubt will that affect the pelvical alignment? Will that give you more risks for scoliosis? I personally don't think that is true in SMA. I think that scoliosis is a bit more independent than that. If somebody came up and said well is it going to cause more progression of the scoliosis and make it more severe and harder to handle, I couldn't argue that it can't possibly theoretically do that, but nobody has ever shown that it does. So again, you may not decide to put your hip in just because the pelvis is oblique or alignment. When you are looking around the joint, the first thing you want to do is match up and splint them. You need to do more than that if your hips or knees or ankles are tight, you may consider doing the surgery. One of the things that you need to remember is surgery is usually a lengthening of the muscle or a release of muscle that has gotten tight. The joint capsule underneath that may be tight also. Around the ankle it is really easy to release the joint capsule if it is tight. Around the hip it is not that hard but it is kind of uncommon to go and release it the joint capsule. The placement is often times more of an issue than the need, where if you are lengthening the hamstrings in the back, the joint capsule at the back of the knee has gotten very tight. That is a bigger deal trying to release that. I am sure that everybody has a doctor who would be comfortable doing it, it is just goes from being a small muscle surgery to a much bigger surgery, with a lot more swelling, takes a lot more time, and you are definitely right next to the nerves and arteries and veins that go down to your lower leg. And it is a lot more of a maticulate surgery. So it may be that even though you have done a lengthening of the muscles around the knee or the hip, you don't get all of the motion back. The motion you could get back may be related to either the bone is a bit deformed to a point or is more likely that the joint capsule is still tight. And I know that there is any number of kids who have gone through hamstring lengthening and they don't flex well, only about 15 or 20 degrees and then you start to weigh you know does it matter if you have to deal with a 15 degree contracture versus doing a pretty big surgery and getting a lot deeper and taking a lot more risks, and most of the time it is that it is okay to have 10 to 15 degrees less and that is alright just so that you understand that that's a an issue too.

Q: Can you address the activities that would be improved by having surgery?

A: If it is a very severe contracture, so severe that the knees are bent beyond 90 degrees, it may actually help sitting. It may go two ways, one is when you are sitting if your feet pull back too far, it is hard to get in your wheelchair if it is more than a 90 degree tilt then it may be true that it will pull them forward and the other thing is your hamstring is attached to your pelvis and if they are really tight then when they pull they will make your pelvic tuck sort of. If it pulls too much that your pelvis and your spine sort of goes to compensate one way or another and it becomes a little bit of an issue of trying to line everything up. Now, that is just for a sitting issue. More commonly, it is how easy can you stand or be braced to stand or be braced to take steps or things like that, you know or you work with somebody who is taking steps with an RGO with a high brace. In general, people say that they can brace you if you still have a 25, maybe even a 30-degree bend in your knee. But a bit more than that it is hard, and the other thing is that it affects the alignment of the other joints. If you are bending at your knee, you either have to be flexed at your ankle or you have to go up on your ankle. But if you're bending your knee and you have to flex at your hips then that makes you a little tighter in the hamstrings. So if you are getting really tight at your hamstrings that may be a worthwhile thing to do just to kind of keep your overall alignment. It may help you with some functional stuff; it may just be helping you with things you do in therapy. But those are some of the issues.

Is there a down side to this? Well, it is surgery. There are a lot of down sides with surgery. There is an old joke; "You know how you can tell the surgeon in the locker room? He is the guy with no scars." You know, there is anesthesia, there is pulmonary compressions which are more pronounced, getting infections, you know you could hurt the nerve or the artery, you are near there, but the other thing is if it is a really tight contracture, when you stretch it out, sometimes the nerve and artery can become contracted. You get them in a better position and then when they wake up, there feet are tingly or burning and I did that twice that I can think of neither one of those kids had spinal muscular atrophy. So then you had to just sort of like take the caps off that you put on and let them bend back and over time you can stretch them back out. But those are some of the things that can go on. There are other things that go on with surgery, you know, you are going to loose some blood, and may need a transfusion although that is really rare. And then there is the recovery period and one of the things is that anytime you do muscle surgery, everything else being equal but muscles always reject. Now for a lot of these kids they are not using their hamstrings really anyway so it is okay if they are weakened but it does throw off the balance between your quadriceps and your hamstrings or your hips extensors versus the flexors and things so you have to work in therapy some to maintain what you have got and to get back whatever strength that you had. In that period of time when you are down after the surgery, you know I am living proof that if you stop lifting weights, you atrophy pretty quick (Dr. Grayhack is somewhat overweight). I have been testing that theory now for like 25 years. You don't just atrophy quickly, but it keeps going. So you need to kind of get into really intensive therapy and you need to try and do some tricks in therapy where you use the muscles that you can even when you are laying down or even while you are recovering, even when you are soar get people moving to move their quadriceps as much as they can. One of the things I always warn people about before the surgery, I have not had this happen, but it is possible to take somebody who was standing before and do everything and it should line them up better and they just can't stand now or they just can't walk as well and in particular somebody who is barely taking steps with their RGOs on and it was all dependent upon their balance and what you did should have made them straighter and everything but somehow their balance isn't quite what it was. You know when you get up too quick early in the morning and your balance isn't quite what it was, it takes you a minute to catch and these kids don't have the opportunity to catch. They can't catch. So it can throw you off and I know that someday I am going to do surgery on somebody who was standing or was taking some steps and they are not going to be able to that anymore. It hasn't happened but I know it is going to happen. You think you are really smart. You think, "Oh yeah, I have done this 20 times and I know it is going to work." and it makes sense but even things that make sense that you can't understand everything that is going on in somebody's body. Was that a long enough list of stuff that can go in surgery?

The adductors and the iliopsoas muscles, the adductors are the groin muscles and that is what your scissors are pulling in and the iliopsoas closes the hip flexure and each of those kind of help aid to line you up a little bit better and the other thing is that if your adductors are really tight or your iliopsoas muscle is really tight, those will be involved in that hip subluxation. You can actually get it to slide out of the joint if those are too tight. Usually, that is not the issue here. The issue here is that they are tight and you are trying to stand up or you can't come up straight and that is another reason that the adductors and the iliopsoas might be tight like.

I kind of described this earlier when we were looking at the slides too but you can work on both sides of the hip joint if you are trying to get things better lined up, besides doing the lengthening of the muscle. Sometimes you can rotate a little bit to get the sides in a little better and on the pelvic side you can do something to bring it over the top a little bit more. Sometimes you will actually make a cup, involved with tilting pelvis side over. So if you really are trying to reconstruct or get the bone in a better place usually the bony surfaces that you use will do. Is there any kind of worry of the hip coming back out? Usually if you can get the bone kind of lined up well, but also the muscles that you lengthen, you didn't take them out, and there is a tendency for them to contract again overtime. Even though you have lined things up, what you want to do obtain a good position of the joint but to maintain it you need good muscle balance. The muscle balance that wasn't so good before may still not be so good, even though you have lengthened them once, it may come back and be an issue overtime. Probably the older you are the less likely your hips are to come back out or that you are going to have a recurrence of what you had. That would not be a reason to wait. Just thinking if you happen to do the surgery when you are 8 years old as apposed to when you are 4 years old, you are better off doing it at 4 if that is what you need because you get better hip development. But the more you are growing the more there is a chance that you are going to get a recurrent tightness of those muscles. And once you bend them you will still need to do the stretching and stuff. I know that you get tighter even when you get older.

There is another issue that I am going to touch on and that is about scoliosis. You guys all know what scoliosis is; it is the curve to the side of the spine. It is sometimes accompanied by what is called kyphosis or a bend forward. It is normal to have a kyphosis. It is normal to bend forward. It is normal to have a lordosis; the bottom of your back should bend in. Where you get the trouble is if you have excessive kyphosis or excessive lordosis and I will come back to those in a minute because the main focus is on scoliosis, the bend to the side. It is very common, the numbers range anywhere from 80 to 90% of the kids get it to 50%, but there is no question that it is really very common. It is probably progressive in almost all the time. A really small curve, 10 to 15 degrees, it is a scoliosis because it is a curve to the side, but it may just be sort of postural. It may just be the way somebody is leaning in particular if you take an x-ray of your 3-year-old and they have a 10 or 15-degree curve, yeah, maybe that is real, but it may also just be that that is just kind of the way they stood. You know if you ask a 3-year-old to sit or stand or whatever, straight as they can, it does not necessarily mean that they are going to be perfectly straight. If I ran around and did x-rays of all the kids in preschool, I am sure that half of them will have a 10-degree curve, just because they are not standing up so straight. But still you want to know if the curve is going to be progressive. The issues with progression are that it progresses with age and it progresses with involvement, maybe the weaker your calf muscles are the more you are worried about it. When you first see it, or if it is just a flexible curve, often times people will just do a wheelchair modification, you know lateral posts. And you are probably not really going to be treating scoliosis; you just treat the fact that their posture is bad. Just like I would like to do for my 12-year-old. He just doesn't stand straight. With scoliosis what you worry about is the rigid curve that is going to be progressive, it keeps getting larger. The worry of the curve is more that is it going to affect the way you sit up or whatever, but that is not what really drives you to become more aggressive necessarily so that if somebody had a 60 degree curve and it is really flexible, you know when they are sitting up there definitely curved over and then lying down you can get it down to 0 degrees. I am not particularly worried about that, that is just posture and you can maybe support them in some ways. But if somebody has a 60 degree curve and they are sitting up and you lay them down and it only goes down to 50 degrees, then that is a real curve and that is the one that is going to get worse and that is the one that you are going to start talking about whether or not surgery would be a benefit. So you have to kind of separate out that postural curve and the rigid curve and sometimes your doctors will get an x-ray sometimes laying down and sometimes sitting up, so they are looking at a little bit different issues in those. There will be times that they want to see how good your spine can look when you are laying down and then when you are sitting up you are looking at how bad your spine looks.

The function part is to just free up your hands. If you have to use your hands to support you all the time, you can't do too much with them. And then bracing is another way to get support. Bracing doesn't prevent the neuromuscular curve. I think that if you pull a hundred pediatric spine surgeons, that probably 99 of them will tell you that it doesn't stop the curve from getting worse. The brace is meant to support them and the function stuff that we talked about before, hopefully, is gonna kind of slow the progression, get more growth, get more height, which has two benefits that I will talk about in a minute. And then they are sitting down, the brace can be a big benefit, again, it frees up your hands and it gives you more function. So you need to always keep in mind that the brace is not actually going to stop the curve, you don't have to put somebody in a brace when they have a 10-degree curve thinking that you are going to miss out. It is not like you are missing the boat. There is not a boat to be at, okay. If there was something that could stop the curve, and we knew of this, then we all would do it. But the bracing is to help out with that not necessarily to prevent it. The good thing about bracing if we can besides the balance and stuff is that we hope it slows the progression. Getting older or before your curve gets bigger has two benefits. One is you get more height, so if you are 6 years old and you have to fuse your spine, that is kind of the height that part of your spine is going to be. The rest of it, above, below it, arms will grow, your legs will grow and so all of that stuff, but to get more height would be wonderful. The second one is you don't want so much growth left when you do it. One of the big concerns of everybody is that if you fuse the back of the spine with somebody that has a lot of growth left, then some of the spine keeps growing, the term that is used is crank shaft, which is not a very good choice of words, but what happens is the back part becomes a tether and the front part grows and kind of has to rotate around it cause it has got to grow somewhere. There are a bunch of issues with that, but one of them is the curve can progress even though the back is fused. Besides the progression we also see a twisting with someone who is forming, it pushes your ribs back more and your sides back more and everything and it throws your balance off a little bit. So that is how everybody would like to get the child from age 6 up to age 10. Now, one of the hard parts is what is an older age? I don't know the age that this helps, what you need to know is when have you got enough growth so that you are not worried about those issues, that you are not worried about crankshaft. The other doctors who are here tell me that there is less of a few little growth spurts in kids with SMA. So that much more of their growth will take place before that, so you will probably don't need to worry about it as much when you get up to around 10 or 11 years old where as if you say the average boy who grows until he is 17, that is a lot of growth left from age 10. If you look at a 4th grader and look at an 8th grader especially in high school, that is a huge difference in height. That could be a big issue. It may not be as big an issue that the kids with SMA have fused at age 10,11, or 12. So that may be a benefit as one of the things you don't have to worry about as much. We don't really know the answer to that per say, as we are trying to design a little discussion or study for a good spine surgeons who can find out more about it and help people more with that. When you are using a brace, make sure that it is helping the function more than it is hurting it. That is not just the sitting and moving function, it is can a child still do what they want to do that they could do without the brace. There are also is pulmonary function. The pulmonary function with a little bit by chest wall motion, the muscles of the chest wall is important in SMA so you have the issue of putting a brace around that and sort of holding it outward. You don't want the brace to squeeze so hard the ribs down. And you have to be careful about what it does. A very tight brace, so tight that it can actually affect the shape of the ribs. I don't think that anybody uses that kind of brace anymore but it is a really sort of cone shaped brace, and you will end up with more of a cone shaped rib cage. So you need a brace that is sort of like if you take a can and you take a water balloon and the idea is that you drop the water balloon and it will take up the shape of that can. So you are really using the hydraulics more to kind of push all around it. Same thing is true here, your not really trying to push really hard on this side and that side, necessarily to push the curve back, but you are kind of just want to push the whole body together with sort of hydraulic forces and that has less affect on the ribs.

What causes the ribs to flare out toward the bottom of the rib cage? That is kind of common, because the kids are more abdominal breathers. There are people who don't have SMA who have the same kind of form but I think that is real common. Is that a negative? No, it is just going to happen and there is not much you can do about it as far as I know. You can ask the pulmonary doctor if they have a problem with it. What sort of progression would be unacceptable? If the brace isn't sort of reducing the curve by 30 to 50% it is probably not doing much. Now having said that, you would maybe try to modify it or whatever, but the thing is that child who had you know 45 degree curve and they were not even 7-years-old and I am trying to use the brace just like for support, the fact that the brace only comes down to 35 degrees or something like that, I would still use the brace, but when I got the x-ray of the brace I would see if there was someway to modify it to accomplish that.

So when you actually end up doing the surgery, you know one of the things I left out is the age and that is the key to the whole thing like we talked about. But usually you decide with how big the curve is and how big the number gets as far as they give you a cob angle or fixed angle of degrees. The flexible curve again as I said, if I have a kid who has a 60 degree curve and bends down to 20, what I care about is how well it bends down, because that sort of pushes me to do surgery because that is probably the correction I am probably going to get or my correction is going to be related to that. So how well I can correct the balance of the spine is related not necessarily to how big the number is when they sitting and bent way over, but how small the number is when they bend to the side. So you might have an 80 degree curve and it bends down to 20 degrees, or if I have a 40 degree curve and it bends down to 20 degrees, both will probably correct about the same, you know when I do the surgery. So what you are looking for in the surgery is to get some correction, to prevent progression, but mostly to maintain balance and to get your pelvis and your shoulders kind of level with each other. I got a child here next week, they have a 120 degree curve, the biggest curve I have ever taken on in an otherwise healthy kid, and I am not going to get that down to the 30 or 20 degrees that I usually do, but if I get it balanced and it doesn't get worse, he will go through his life a little bit better than kind of being of bent. So again, you are really looking for balance, it is not necessarily for a given number.

Then you want to look at the pulmonary function. So one of the factors that the surgery has is on pulmonary function and what kind of pulmonary function do you have before you start. These guys are all focused on that anyway, so it is not going to slip by anybody in this room. So you need to have a good evaluation of the pulmonary function before you go into surgery. You really need to have a good plan of what you are going to do about the pulmonary function because in the surgery both the anesthesiologists is involved in the intensive care unit or where ever they are going spend the days time after and they try to get everybody sort of get everybody on board. They will consult with each other. So the real thing you want to do with surgery is you want to improve function, not just the next day, you are talking about compared to having not done the surgery. So if you do surgery on a child who has a 50 or 60 degree curve, then they are going to be a little better if they are sitting and everything, that would be better than they would have been sitting there in a brace or using lateral posts. But you are also trying to improve their function 10 years from now, when they would have had a 90 or 100-degree curve. So you can also improve their life in that way. The kids I have been in touch with are pretty happy afterwards because they sit more independently and that is what they have been trying to do their whole life, is to sit up better and now they can sit a little better or with less support. And they feel a little bit better about even though you can barely see that they were wearing a brace before, they were a little self conscious about it a lot. You know anything that you get rid of; they don't, thinking of 12 years, they don't want to wear AFOs. Or you know, like it is a huge deal to wear AFOs but to a 12 year old it is a big deal. To a kid to get rid of their braces and they feel like they are more a part of things. I think it helps out their self-esteem in that way and makes them feel like they are a little more independent. Often times they will help the caregiver too, because if you are trying to move somebody and you have a big rigid curve, it is a little awkward.

I did want to talk a little bit about the lordosis, which is the second curve you get at the bottom of your spine. Anytime you are sitting there may be a tendency to have more lordosis. Most common cause of increase in lordosis or more curve to the bottom of your spine, is not really that your spine is curving; it is that your hips are tight. If you get a hip flexion contracture, when you go to sit down you have to bend your back a lot, you have to add a lot of lordosis when you sit. You need to be careful if somebody is looking at that and saying oh gees his back is so bent up so we need to do surgery on it. Make sure that it is not that the hips flexors aren't too tight. One of the issues when we actually do the fusion is that all people use kinds of different rods, screws, and wires and hooks and stuff. We sit around for 2 or 3 days every year and argue about who wires, rods, and screws and such are the best. You can go to the Pontiac dealer or Buick dealer and they would like to tell you that the two cars are totally different but when you are sitting in traffic waiting to move about 4 feet, it doesn't matter. It is the same thing. And it is the same thing true here; don't get too fixed on that. Now there are more and more people using titanium as apposed to stainless steel or other types of metal. The titanium alloys are actually very strong now. The advantages they have are that you can do an MRI with them. They have a little bit more elastic or a little bit more bend, which goes along with the elasticity of bone. Some people think that they fuse a little bit better. But that is kind of again dealer's choice. I still use, at least half the time, the stainless steel and the other half titanium. Largely, because I sort of look at the kids and think you know they need something that is just a little bit stronger. The real issue that people talk about as far as different things that they do is whether they fuse all the way to the pelvis or not. The good thing about fusing to the pelvis is that it is a nice solid base and then you can get to the start of the spine and I think most of us do that. The bad thing is that it does tie up your pelvis a little bit more so if you have somebody who likes to do sports like wheelchair hockey or wheelchair bowling or something like that then they can't bend quite as easily at their waste and everything and you need to make sure that they have good motion at their hips if you are going to fuse up their pelvis because otherwise you have a nice solid base from thin to stern and no place to move.

The biggest issue is that a young child who has a curve how are you helping and mostly it is the seating and bracing and trying to wait for surgery and then when you actually say okay they are old enough that we can do surgery and I don't know that answer better than somebody else necessarily. There are other ways to deal with really small child who has a very big curve and they aren't ready for surgery, and those are ways that you place a rod but you don't fuse the spine. There are kind of two different versions of that. One is called the subcutaneous rodding and in that the rod is mapped down next to the bone but really rides right over the top of the muscles, so it is further away from the bone and at either end they are sort of hooked or screwed or something to grab the bone. Screw at the bottom and hook the top, something like that. And you use that just to kind of stand it like a stander. You will need to wear a brace over that too because you don't have anything solid. And over time some things are going to happen, one the child is going to grow, which is going to happen with a really small child so you are going to have to go in and kind of push the rod longer. The other thing is the rod is going to break. And I know the rod is going to break every time whether it is the first year or the first 4 years, sometime it is going to break. It is okay, the rod breaks, you just go back in and put in a new rod. Some people kind of got away from that, that just got to be a very frustrating problem because you know you have to go back in and do another surgery to put the longer rod in. That is one thing that can be used. Another one is this thing called a trolley, which is taken on, a few people tend to like this more. In that one you just hook the rod in at the bottom of the spine, wires, screws, hooks, whatever you use at the bottom and then the rods ride up along side the spine, but again you don't strip down, you don't go all the way down to the bone. You leave the bone alone and you run wires underneath the bone coming up you will have a series of wires up and down the rod wires and screwed in wires, and as you grow the spine is just going to kind of just grow off them like a sprout and then you go back in and change it because when you are big enough you change too and you fuse it. There are a million different ways people try and do this. It is a very difficult problem.

So there will be improvements in how we fuse the spine and there will be new material to help fuse it, not just the rods and hooks, but we are within a few months of having FDA approval for a mineral protein which is a kind of stuff you can put in, it is a genetically engineered stuff, the same as the thing that goes off your body that makes your bone heal after you break your arm or something and that may improve the rate of fusion. The thing is that the rate of fusion is not bad in kids. I mean if you are a 60-year-old smoker that is where you really need that. But in a child usually is heals itself pretty well. Sometimes it doesn't but those may be an issue. So we are starting like a quantum leap. The quantum leap we need to make is from sticking a rod in and fusing a person's spine, and figuring out how to just go ahead and just hold it straight or make it straight or at least make it flexible. I mean that is what I do, but it is not a very elegant way to treat somebody with what is supposed to be 24 jointed pieces who all move really intricately, and I just stick them all together and fuse them. I work really hard at it, but it is not a great thing. The quantum leap is not going to take place over the next 5 years. Maybe something else will happen. Will there be something different as you go on, so that you should kind of wait for? We may understand a little bit more about scoliosis in and things in another 4 or 5 years. We may know more about the genetics, but I don't think that there is going to be like a big shift of how we decide to treat it. We may no more about growth in kids with SMA and that may help us a lot in deciding if it is appropriate to do something on an 8-year-old or we just fuse a small segment and let them grow some more or something like that. That may be helpful.

Now with the trolley, it is best done when the child is young like 4 years old or 5 years old, but when you get up close to the 8 or 9 age, I know that my partner has done the trolley on 8 or 9 year olds to kind get them back. I don't know about the growing issue between kids that are 7 and 12. If you are over 12, then it is no issue. If you are under 7, then it is a huge issue. But somewhere in there it is unclear about the growth issue.

What actually causes the scoliosis? Is it a weakening of the muscle themselves that causes it to be progressive? It is probably more muscle related, now having said that, nobody knows for sure. The way you usually find that out is you have animal models. There shouldn't be any intrinsic problem with the bone in SMA, so then it is either that a neuron signal that makes you get a curve. Or it is the muscle imbalance. And I think with it happening so early, I think it is more likely the muscle imbalance.

What kind of studies have been done about the fusion all the way down to the pelvis? Does it make the fusion stronger or better? It doesn't make the fusion stronger. Usually what you say is that you can either stop the fusion just right at the level above the pelvis or go through the pelvis and the place where this has been studied the most is with Muscular Dystrophy, because they had a huge group of kids who have been fused so they were able to do comparison of both. One center said you should always go to the pelvis because when you have some of the muscle weakness in the trunk and pelvis area that if you stop at one level above you are just going to shift off over time. It just never works. And the other center said it works fine. So who knows? But I think that most people feel more comfortable going through the pelvis if there isn't a conflict indication. Because you are obviously sitting on your pelvis and it means that you kind of taken away that chance that things are going to shift right between the pelvis and the spine. And if it does, you can go back and fuse it, but you get kind of a leaning tower thing. Your pelvis, your lower spine and your hip all kind work as a unit which gives you motion throughout this area. So if you have locked up your lower spine to your pelvis, that leaves your hips to move and help you in sitting or standing positioning. So what you need to do is make sure you have good motion in your hips before you lock it up too much because if you do all of a sudden you have somebody who can't actually sit the way they used to and that is how you spend most of your time with them. If you want to get your knees to your chest, it would probably limit that. But certainly after the surgery, nobody is going to want you doing that because that puts too much pressure there. That would be bad. After healing it would kind of depend on the flexibility of the hip but you probably will lose some of it but if you look at sort of a functional range from being able to sit and being able to stand you want to make sure you have that much motion in your hip even with your pelvis fused. If you connect the hips to the rod then that is really going overboard. The reason I bring it up is because everybody seems to be focused down on one thing and then you get all done and you go "Oh, I never really checked the hips." and it turns out the hips are too tight and it becomes an issue then.

Is osteoporosis unavoidable with SMA? I heard people talking about using drugs to increase calcium content, is that a good idea? If you just take more calcium, your kidney which don't work too well will be working to get rid of the calcium you don't need so if you have enough of it in your diet anyway, then it doesn't necessarily help you to take extra. Now there are certain times in live when that is not true, if you are pregnant or breast-feeding or something like that, but that is not an issue with these kids. But as far as in order to increase the amount of calcium that is actually incorporated in your bones is kept, there are medications that can help with that. All of those are being developed for adult osteoporosis because that is a really big market. That is a huge market, which means that is a lot of money. There is actually a lot of money being poured into this for reasons other than singling out kids with SMA. It has just been in the last couple years there were a couple of articles of trying to use some of those medications to get stronger bones in kids that are less active and I have to say that those studies were really done in children with cerebral palsy. I don't think that any of them were done on SMA kids. But in those groups, what they did was they looked at the rate of fracture after they took these medications and they looked the rate of bone corporation and it was not really a significant shift. It wasn't enough that they could statistically say that this is a lot better. Now having said that, there was another study where they looked at kids before they did surgery to see if they could improve on nutrition and improve on calcium and that seemed to be better with kids that they have proved that have sort of depleted calcium and they gave it to them, then those things were beneficial. But if they had a normal calcium and they gave it to them it didn't seem to make much difference. In a sense their bone can be weaker than if they were standing and sitting normally, yeah probably they can be more at risk for osteoporosis. I mean doing therapy, doing more standing, more times upright, all those things help you develop more strength not just in your muscles but in your bone because your bones respond to forces involved. If you don't have any force to prop them they don't feel the urge to thicken up. So if you are doing some standing and things like that, that probably benefits and decreases the risk for developing osteoporosis. Is it unavoidable? Osteoporosis is a relative term, compared to the general population, you probably are going to have a little bit of weaker bone than if you were standing a lot more. Wouldn't that be called osteopenia then? Osteopenia and osteoporosis are used sort of interchangeably although they are not technically the same thing. One is sort of a thinning of the bone and the other one is less bone mineral content. So with osteopenia, the structure of the bone, like all the non-calcified areas will probably be about the same but it makes it a little bit weaker either way. So I would speak to the pediatrician, because I am not really a expert on ways that you can change that but there are other medications like I said that you could use and things that you can do like make sure you have normal calcium in your diet or a calcium supplement and then do more weight bearing. Those are the things that are going to thicken things up a little more.

Is Botox a good option with the release of muscle contractures? You want to get them in a shoe or a brace and you want to see what you can do as far as sort of putting some weight on it if that is possible, one of things to remember is that Botox is a used for a lot of different reasons. The thing about Botox is it is a way to paralyze the muscle so if you have a muscle that is over active and tight it will help with that, but if you have a muscle that is too short, then Botox doesn't do anything for that. I have seen once with a child who didn't have SMA where he had a severe affect on his breathing where he had a rough couple of months. In some kids who recently have become just a little bit tight, I recently thought that maybe what you get is a brace for at night too. Because that will help with that. I think that nighttime bracing thing is very helpful for a lot of kids. If you don't have enough motion to start with it not is going to be of help, but if enough motion to get in that position then it could be a lot of help. I think the night time splinting kind of half way replaces a lot of the stretching you spend you day doing. It is a good thing. Sometimes they go a little past the 90-degree angle for the ankle for example to stretch it out just a little bit more.

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