<Very well said Laimi

[Guest guest]

Very well told Laimi, great information and you are really well informed..Thanks for the info..

Sincerely,

Kelli and Santiago

De: L F-N <congenitalkyphosis@...>Para: infantile scoliosis treatment Enviado: dom,9 enero, 2011 06:12Asunto: Re: To Emma --Diagnosed with hemi

Emma,

Her curve may be too low for the VEPTRs as you say, but ask surgeons who use the instrument. You may be surprised to hear one saying 'no' and another one saying 'yes'. [Then you would need to see a 3rd surgeon ;-) ].

VEPTRs were designed for those patients with chest wall deformities and fused ribs, but then some surgeons realized it could control the spine as well.

Really, try to contact Dr. Betz in Shriners Philadelphia. Dr. Emans in Children's Hospital Boston also uses the VEPTRs. These guys would give you their opinion if you mail them the x-rays. They just love what they do. When I was looking for choices for my son, I used to send them a letter or e-mail explaining our situation and then would ask if they could help us. Leave it up to them, but most likely they will offer to give you their opinion at no charge if you send them the x-rays. They know what we are dealing with.

The VEPTRs migrate through the ribs (brake the ribs) sometimes. My son had bilateral VEPTR I and then VEPTR II, and both times the instrumentation migrated through the 2nd and 3rd ribs. In most cases, it is painless and by the time the surgeon notices the migration, the ribs are already strong to hold the instrument again. My son has kyphosis though. From what I have heard, scoliosis kids do better with the VEPTRs. It is important to keep in mind that kyphosis may develop also from the rotation of the spine in scoliosis patients. At that point, the VEPTRs may not be very reliable either. Even though the initial diagnosis is scoliosis, the resulting kyphosis complicates the job for the VEPTRs. Other times, like in the case of 's daughter, the metal "sinks" in the illiac crests. I have heard from a couple of parents already that these cases can get

really complicated, especially if some of the nerves in the area are damaged.

The screws of the Shilla (the one my son has now) and the screws of the growth rods can also pull out of the vertebrae. In fact, the upper screws of my son's Shilla are out. He developed a proximal kyphosis (a kyphosis above the fusion) but since he is not at risk of paralysis right now, we just have to let him grow and then do a more extended fusion when he is 16-17 years old. So that you have an idea of how badly his spine wants to keep the kyphosis... he spent 13 1/2 months in bed with a cast first and a brace later. We were waiting for the posterior fusion to get hard to avoid a second thoracotomy (getting in the chest through the ribs) to put bone in the front of the spine. After the 13 months in bed, he had an exploratory surgery and the fusion in the back was hard enough to hold all the weight without having to put bone in the front. At some point during those 13 months, the screws

of the Shilla came out even though he was kept at no more than 45 degrees during those 13 months. Needless to say he had to relearn to walk. He was allowed to walk again on January 6, 2010 (I will never forget that date). Two months later he could ride a tricycle.

Every instrumentation can fail at some point. Fortunately, in most cases the hardware can be reset again. Not to mention the risk of the screws not going through the pedicles or damaging peripheral nerves.

Of all the instrumentation available, the ones I don't like are growth rods because of the required fusion of levels at the attachment sites and because growth rods end up fusing everything in between sooner or later. However, like everything in medicine, no one can say they are completely wrong. Some kids have done great with those.

There are also Dr. McCarthy in Arkansas (U.S), Dr. Lenke in Saint Louis , and Dr. Skaggs in L.A. who have been using the Shilla. Our surgeon, Dr. Shufflebarger in Miami , is a man who does not talk too much but he cannot say no when it comes to helping a parent. He is also using the Shilla. I heard from a surgeon in Cleveland that Dr. Lenke is very good when it comes to removing hemivertebrae, but our guy here in Miami , removed my son's three problematic laminas 2 years after the vertebral bodies had been removed in Boston . In other words, he manipulated my son's spine with no bone structure at three levels, just metal rods running along the spinal cord in that area. I am talking about geniuses of the orthopedia. They are not only here in the U.S. They can be found in other countries too, but you have to look for them. Do not trust the first guy that gets on your

path. Many will tell you they can do this and that. Others will tell you you cannot have a certain procedure done because THEY do not know how to do it; those are the ones who can do more harm than good.

Avoid confronting a doctor with the opinion another doctor gave you. You may see a few closing their mouths and then you would lose a chance to learn.

There is still some cartilage tissue in the bones of a small child like your girl. The "game" is to let her grow until you see a sign of neurological loss, a lung is being too compromised (or the heart if the curve goes to the left), or the ribs are getting terribly out of shape. Stronger bones bring better chances for success with any hardware. As for the ribs to fuse, you need a very bad curve. I would not worry about this for now. Always keep in mind that your daughter's curve may not progress at all. It is good to be ready, but you have time.

My son's kyphosis got to be 100 degrees. We saw 5 neurosurgeons and 21 orthopedic surgeons (including those who spoke with us over the phone or by e-mail) before we decided what to do. My son's case was so complicated and scary that everyone told us something different. The surgeons who only offered to fuse 6 to 8 vertebrae kept telling us that we had no time, that he was going to be paralyzed, etc. One orthopedic surgeon and a neurosurgeon told us it was not going to happen in one day and that we could wait a little longer. Those two gave us some peace of mind and it was then when we started looking for other choices to avoid fusing 2/3 of his thoracic spine. By the time he had the first surgery, the vertebral bodies at T5, T6 and T7 were separating from the laminas, moving to the right. Due to the lack of pedicles, the front and back of those vertebrae were not

connected. But he was walking and potty trained. He was mildly hypotonic but this had been the case since he was born, when the curve was not even noticeable. The hypotonia never got worse. We chose the VEPTRs trying to avoid unnecessary fusions and keep the instrumentation as far from the spine as possible. But then, the short fusion in the front of his spine failed and the ribs didn't hold. We didn't know back then that the front of the spine holds 85-95% of the weight. Anyone who needs to face spinal cord decompression (removal of vertebral bodies) please, keep this in mind. It was a mistake to delay his fusion in the front and leave all the work to the VEPTRs. It was also a mistake to do the first extension 3 months later, instead of 6. At the time, we agreed thinking that we would be gaining chest growth before the fusion took place. But you do not move an area that is being fused. I don't blame any surgeon. I was the one who was not

supposed to be ignorant about this. At the end, it was my child the one who needed more surgeries, 2 of them were horrible. The second part of the story you already know it. It almost happened again, but we kept him in bed because we already knew about the front of the spine holding most of the weight. His life would be very different now because, with no bones at all, the spine would have collapsed and who knows what would have happened to the spinal cord.

Forgive my English mistakes, please. Even though I have lived in the U.S. since 1995, English is not my first language. I think I will be old and still trying to improve it. (:-P) I just hope you understand me. Keep asking and... why not...? Contact doctors. You have nothing to lose if they say they can't give you their opinion by e-mail or over the phone. Most of them helped us. Why wouldn't they help you?Laimi

From: L F-N <congenitalkyphosis@...>Subject: To Emma --Diagnosed with hemiinfantile scoliosis treatment Received: Saturday, 8 January, 2011, 4:45 AM

Dear Emma,

I have not read all your e-mails yet, but I see you are having a hard time to get your MRI. Please, contact Janet Cerrone at Shriners Philadelphia. Let her know what you are going through and ask if there is any way Dr. Betz could see your x-rays. Do you have them in CDs? Can the hospital put the x-rays in a CD? Or maybe sending copies of the films would be better just in case the computer systems are not compatible (???)

Betz will probably tell you to get an MRI. I am still wondering if they don't need a CT. Sometimes it looks like a hemi and there is something else/different. The CT has the radiation of ~20 x-rays though. This is why many surgeons wait to get the CT. However, putting her through anesthesia twice (for the MRI now and a CT 2 months later) is not a very intelligent thing to do. I would bring this up during the next appointments.

Dr. Betz is the head of the department in Philadelphia. I am not sure they can do the MRI right there, but you could ask. Needless to say the man has been doing this for years and is one of the best in the U.S. He is not doing surgery right now but his team is one of the best here.

There is something called Phoenix rod in France. Here in the US, there is Shilla, VEPTRs, growth rods, and vertebral stapling. Try to look for information about these.

Regarding waiting 6-8 months for an MRI is a long time, but it is not a bad thing in general terms. You will need a few months at least to see if her curve progresses. Even if it progresses slowly, it may not be good to rush into surgery.

In a small child you do not want to fuse so quickly. Fusing a couple of levels is not too bad but whatever you fuse, does not grow. They have to remove the growth plates of the vertebrae involved in the fusion. Again, losing the growth of a couple of levels is not even noticeable when the child is an adult. Your girl's anomaly is in the lumbar. There are only 5 vertebrae in the lumbar (I think). The lumbar has a lot of mobility. It is actually at the lumbar and the cervical spine where all movement takes place. She may lose some mobility in the area, but kids learn to compensate so well, that no one may even notice it later. However, for girls the pelvic and lumbar areas are very important. You have to ask many, many questions and unfortunately, all the questions don't come at the same time. Write questions in a notebook because you may forget some of them when you are in front of the doctors. It often happened to me

because I got overwhelmed with the new info they were giving me.

[speaking of growth plates, some hemivertebrae do not have growth plates and this, dear, would be the best that could happen because the hemi would not grow and it could be the end of your story with scoliosis. No progression, just a little piece of bone there that would not cause any more trouble].

Drs like to use long instrumentation. It is common to see curves developing above a fusion. In order to keep the entire spine under control, they try to fuse as little as possible, but instrument several vertebrae above and below the fusion. Be careful with the instrumentation they use. Growth rods run right over the bones and just exposing the vertebrae (uncovering them during surgery) causes involuntary fusions. VEPTRs are attached to the rib and do not touch the spine above. The lower attachment can go on the illiac crest or the lower spine. Attaching it to the spine requires the fusion of one more level. VEPTRS requires extension surgeries every 3-6 months (average in most cases). The Shilla runs on the muscles and the vertebral screws go in through the muscles so that the vertebrae are not exposed during surgery. Shilla does not requires extension surgeries. The Phoenix does not requires surgeries for the extensions,

parents can extend the rods with a special magnet. The rod is internal, you do not see it. It is just amazing! I am not sure whether vertebrae are exposed during the surgery of if it requires fusion at the attachment points.

Vertebral stapling is good when there are no vertebral anomalies, but at this point you never know what may work for each kid because every case is different.

Again, I have not heard of any case of success with casting when dealing with a hemi itself, but the guys in the infantile scoliosis group are saying it has helped and they are more into casting than me. The truth is that it was never a choice for us. My son had missing pedicles at 3 levels (a very weird anomaly). I did meet a boy in Boston whose spine rotated like a snake inside the cast because the parents waited too much. If you try casting to buy time, do not do it without having the MRI first. If you see the ribs are changing (start to stick out), the spine starts rotating, or she starts developing a mild kyphosis, STOP. I WOULD try casting before going to surgery, with a good doctor who knows the new systems mentioned in the infantile scoliosis group, but would keep my eyes very open. The more time you gain before the surgery, the better because she will be growing and getting stronger bones to hold any instrument she may

need; but you cannot delay too much to the point of making the surgery harder for the surgeon. Again, you need someone very qualified who knows how to use every kind of instrumentation available. When the time comes, do not let any surgeon choose the instrument he knows, but the one your daughter needs. You will know by then who is choosing the instrumentation because he/she does not know how to "install" the others. You are going the right way, asking and questioning everything.

Best of luck! Your daughter has YOU and she will be fine.

Laimi