Treatment: PT using MedX per Mooney & later, McIntire research

[Pooka1]

Wow you found some extremely interesting abstracts, AMom.

One of them includes some material that I think should be considered in any future bracing design studies...

All 13 patients with a curve magnitude>30 degrees at the time of peak height velocity had progression of their scoliosis to>45 degrees despite bracing. Four of 29 patients (14%) with curves<or = 30 degrees at peak height velocity progressed to 45 degrees.

Overt inclusion of thinks like this and Scoliscore and the like in the experimental protocol will help to clear the muddy waters of bracing study results. As things stand now, they appear to be hopelessly confounded by known unknowns and unknown unknowns. The bracing literature still has not really achieved liftoff in any meaningful way due to all these confounders. AIS looks variable but in fact if all the key indicators were identified, it might be totally predictable or at least much more so than at present.

[AMom]

triradiate cartilage

If this is accurate, then I could have been reading her x-rays all along. (Very clear photo examples) Her triradiate cartilages appear closed. I had thought the ortho had to take a special set of x-rays (certain angle or view) to see it. I wonder why he never mentioned it during her regular visits. This would seem to imply that her growth spurt is slowing down. Hmmm, I also wonder why he said he expected her curve to have increased at the next visit if things are slowing down per this, her Tanner, and the fact she had started her menses.

Maybe it is because the size of the last increase in the curve and the size of her structural curve that is leading him to think it will progress. Now, I have another question to ask....

http://cdn.intechopen.com/pdfs/36614..._of_growth.pdf

Predicting Curve Progression in Adolescent
Idiopathic Scoliosis - An Outline of Different
Indicators of Growth
Iris Busscher and Albert G. Veldhuizen
University Medical Center Groningen
The Netherlands

"...Sanders et al.13,14 found the status of the triradiate cartilage (Figure 2) to be more predictive
for the timing of the peak growth velocity of total body height, though it is only predictive
whether the patient is before or after the pubertal growth spurt. Once the patient has closed
triradiate cartilages, he or she is very likely past the pubertal growth spurt. However, the
predictive value of open triradiate cartilages is less clear...."

[rohrer01]

I am focusing on the heart, kidney, and diabetic concerns that may come with Turners. I do know that her grandparents on both side had diabetes and one sibling had heart problems (died in infancy). We have always discussed adoption, so the pregnancy concerns may not be as stressful to her as it would be to others--we'll see how she feels about it when the time comes to start her family.
__________________________________________________ __________________________________________________ ___________________________________

Amom,
Has your daughter been diagnosed with Turner's Syndrome? If so, this is a whole different ballgame that you are dealing with. As you probably already know, Turner's Syndrome is caused by the absence of one of her X chromosomes. I'm a little confused because you say that she's started menstruating. I find that a little unusual, since Turner's affects the reproductive organs and the normal physical development of female features, such as breast growth and other secondary sex traits that go along with puberty. This condition is also usually followed very closely throughout childhood and treated with hormone therapy, especially during the pubescent period so that she will develop the secondary sex traits that I mentioned above. I'm not sure if scoliosis is more prevelant among Turner's females or not, but I do know that small stature is. So I would be very surprised to hear that your pediatrician would not have sent you to an endocrinologist right away, like as soon as she was born. If you are not sure if she has Turner's, there are several physical characteristics that are almost always present, such as a strapped upper trapezius and small stature. There is also a genetic test that can tell you for sure. They just need to do a karyotype to determine her chromosome count. I wish you all the best and hope you get things sorted through.

[AMom]

Amom,
Has your daughter been diagnosed with Turner's Syndrome? If so, this is a whole different ballgame that you are dealing with. As you probably already know, Turner's Syndrome is caused by the absence of one of her X chromosomes. I'm a little confused because you say that she's started menstruating. I find that a little unusual, since Turner's affects the reproductive organs and the normal physical development of female features, such as breast growth and other secondary sex traits that go along with puberty. This condition is also usually followed very closely throughout childhood and treated with hormone therapy, especially during the pubescent period so that she will develop the secondary sex traits that I mentioned above. I'm not sure if scoliosis is more prevelant among Turner's females or not, but I do know that small stature is. So I would be very surprised to hear that your pediatrician would not have sent you to an endocrinologist right away, like as soon as she was born. If you are not sure if she has Turner's, there are several physical characteristics that are almost always present, such as a strapped upper trapezius and small stature. There is also a genetic test that can tell you for sure. They just need to do a karyotype to determine her chromosome count. I wish you all the best and hope you get things sorted through.

rohrer01,

The pediatrician is saying she has Turners, but we do not have a diagnosis yet.

I *argued against that dx because 1) she had a full 7 day period in Dec and spotting in Jan, though nothing since then; 2) Her growth was sluggish from the very beginning, rather than normal in the beginning and then falling behind; and 3) The ped said she is a 3-4 on the Tanner scale. These things seemed to say it couldn’t be TS. When I argued constitutional growth delay (CGD) was a better fit, she came back with “range/ spectrum of the disorder” comments. So I looked into Turners and found that girls can do all three of those things, though the menstrual cycle seldom continues. And, I had to agree she does match a lot of other things on the LONG Turner Syndrome list. One of the sites I was looking at is http://turners.nichd.nih.gov/clinical.html

BTW, TS girls are likely to have weak bones and there has been research into how GHT might impact their scoliosis. She does not have following features: neck, eye spacing, ears, hair line, or chest shape. CGD is much easier medically to live with, so I hope the tests r/o TS.

This late dx IS very surprising, but you are right, it will be sorted out once we get in to see a geneticist and endocrinologist. Once I know the dx, I won’t be oblivious if related TS symptoms show up. I just need to know WHAT I NEED TO KNOW so I can do my job as a mom.

A Mom

*Though I argued against the dx, I am not delaying a visit to the specialists.

[rohrer01]

Amom,
I understand that, as a mother, it's difficult to hear that there is anything wrong with our children. But your pediatrician can NOT say that she has Turner's without a genetic test. They usually have the neck thing going on as far as I know. I'm not a geneticist by any means, but that was my line of study in college. We had to learn about all kinds of genetic disorders. I hope, for her sake, that it is growth delay. Although, Turner's women can go on to lead very normal and fulfulling lives. The DNA test will tell you what you need to know. If it's undiagnosed Turner's, I myself would be very angry with the pediatricians that looked after her as a child, but that's just me.

My daughter was extremely small for a VERY long time and NO ONE said a word to me. She wasn't even in the "normal" range, meaning she wasn't even on the growth chart. She had a normal birth weight and then failed to thrive for some unknown reason. By the time she was about five, she had caught up to the "normal". But, like I said earlier, she is much smaller than I expected her to be. My son was the same way. He's a good 5' 9" now at 19 years old. But considering their dad's height and my height, I would have expected both of them to be much taller. My son was wearing a 3T when he was 5 years old. My daughter was wearing a size 6 months and a shoe size of 1 when she was a year old. They were tiny, but caught up. It took my son quite a bit longer to catch up, but he finally did.

Please keep us updated on the status of your daughter. You may not be dealing with JIS after all.

[AMom]

Is this the right test?

rohrer,

Do they use "high resolution karyotype" to determine if a child has TS? If so, she had this test in 2001 and the results were reported to be normal.

A Mom

[Pooka1]

rohrer,

Do they use "high resolution karyotype" to determine if a child has TS? If so, she had this test in 2001 and the results were reported to be normal.

A Mom

That's it then. No Turners. Karyotype is the definitive test for Turners as far as I know. They either see one or two X chromosomes. Your daughter apparently has two X chromosomes so no Turners.

[rohrer01]

rohrer,

Do they use "high resolution karyotype" to determine if a child has TS? If so, she had this test in 2001 and the results were reported to be normal.

A Mom

I'm glad to hear this news and I agree with Sharon. They are just taking a chromosome "count" and don't need to see the chromosomes up close and personal. They can also do a cheek swab. You can see the "deactivated" X chromosome under oil magnification as a speck on the side of the cell nucleus. You may still want to see a geneticist to determine if she has some other genetic disorder that slows growth. Otherwise, you just may have a very petite young lady. My step-mom never made it to 5". You have to remember that her scoliosis makes her shorter, too.

[AMom]

Not TS

I'm glad to hear this news and I agree with Sharon. They are just taking a chromosome "count" and don't need to see the chromosomes up close and personal. They can also do a cheek swab. You can see the "deactivated" X chromosome under oil magnification as a speck on the side of the cell nucleus. You may still want to see a geneticist to determine if she has some other genetic disorder that slows growth. Otherwise, you just may have a very petite young lady. My step-mom never made it to 5". You have to remember that her scoliosis makes her shorter, too.

GOOD NEWS: A quick note to say I received a call from Cedars-Sinai confirming she does not have Turners. They are going to see her at the beginning of next month to see if they can figure out what is going on with her growth.

BAD NEWS: They said CHLA is taking months and months to get kids into the endocrinology dept. I am going to look into a back up resource, I don't think she has that much time to wait around and then try to get the insurance dept to authorize the meds. (growth plates closing)

[AMom]

1st visit w/ the pediatric indocrinologist

Chronological age: 12.6

The pediatric endocrinologist looked at her, her bone age study, and her pelvic x-rays and stated her bone age is 14.5 yrs, she is roughly 98% done growing, she will be approximately 4'10" when she is done growing. He said we could have given her growth hormone therapy, but we should have started it years ago. He said if we give it to her now, we will have to halt the normal progress of estrogen before we can start the GHT and the most she will get in the time she has left is about an inch more of growth. He is sending her for lab work and will meet us at the end of the month with the results. If he finds anything concerning, then he will send her for more tests. He said he can only confirm it with certainty via lab work, but he does not believe she has Turners.

He was very good with my daughter and patiently answered her every question. He gave us his email address in case we have any more questions after we leave the office.

[Pooka1]

Well you have some answers. What causes the bone age to be that advanced compared to chrono age? That sounds like premature puberty per what I learned when my kids were being observed for that. Did the endo mention that?

[mariaf]

Chronological age: 12.6

The pediatric endocrinologist looked at her, her bone age study, and her pelvic x-rays and stated her bone age is 14.5 yrs, she is roughly 98% done growing, she will be approximately 4'10" when she is done growing. He said we could have given her growth hormone therapy, but we should have started it years ago. He said if we give it to her now, we will have to halt the normal progress of estrogen before we can start the GHT and the most she will get in the time she has left is about an inch more of growth. He is sending her for lab work and will meet us at the end of the month with the results. If he finds anything concerning, then he will send her for more tests. He said he can only confirm it with certainty via lab work, but he does not believe she has Turners.

He was very good with my daughter and patiently answered her every question. He gave us his email address in case we have any more questions after we leave the office.

Hi AMom,

As Sharon said, you have some answers now and in my experience it is always good to know where things stand vs. guessing, which can drive a parent crazy.

I'm confused about one thing though (and I probably read something wrong to please bear with me). In an earlier post, you said your daughter is 12.6 years old and 4'7" tall now. If I read correctly, the endocrinologist is saying she is 98% done growing but will end up at 4'10" - so does that mean she has 3 inches more to grow if you DON'T halt the normal progress of estgrogen, etc.? That doesn't really make sense to me if he is also saying she will only gain 1" with GH, but again, this is not my area of expertise - but I am trying to follow along :-)

Thanks!

[AMom]

We don't know the cause

Well you have some answers. What causes the bone age to be that advanced compared to chrono age? That sounds like premature puberty per what I learned when my kids were being observed for that. Did the endo mention that?

He did mention that she may have started puberty earlier than expected. I told him I had looked up the normal timing of this when I saw initial signs at 10 and the beginnings of recognizable features at 11. She didn't start her period until she was 12.1. None of that is "early." (The normal time frame is between 8 and 16. Literature says that breast development is occurring sooner, but menstrual cycle is not. Listening to some of the older teachers in the schools talk, they are both occurring earlier and more frequently. That isn't proof of anything--just an observation by staff that is responding to surprised little girls.) In any case, from a layman's perspective (and compared to her peers) she doesn't seem to have started this phase of her life sooner than expected.

He did say she that based on her known & estimated birth family's heights & allowing for scoliosis, she would be under the expected height range at an ending height of 4'10". Hopefully, between the endo and geneticist we will learn the cause.

[AMom]

growth with &amp; without GHT

Hi AMom,

As Sharon said, you have some answers now and in my experience it is always good to know where things stand vs. guessing, which can drive a parent crazy.

I'm confused about one thing though (and I probably read something wrong to please bear with me). In an earlier post, you said your daughter is 12.6 years old and 4'7" tall now. If I read correctly, the endocrinologist is saying she is 98% done growing but will end up at 4'10" - so does that mean she has 3 inches more to grow if you DON'T halt the normal progress of estgrogen, etc.? That doesn't really make sense to me if he is also saying she will only gain 1" with GH, but again, this is not my area of expertise - but I am trying to follow along :-)

Thanks!

Hello Mariaf,

--Sorry, your message came in while I was writing a response to Sharon so I missed your question.

The endo said she will slowly grow 3" more to reach an end height of 4'10" without using GHT. I was unclear when I wrote about using the GHT earlier--I meant to say she would end up 4'11" with GHT (only a one inch addition). Of course this is all an estimate. He said the hand finishes growing first, the long bones have the most growth left, and the spine has some growth left. She has almost nothing left in her hand.

I hope he is correct about having an additional three inches of growth left because her fingers & toes are super SHORT. Her hands and feet are tiny. Her skull is small/ narrow. All of this is in comparison to the relative portions of her body i.e. head to torso, limb to hand to fingers, limb to foot to toes. He didn't responded to my question about her proportions (we had so many questions, I'm not surprised I missed following up on one or two of them). Normal growth is cepheal-caudal and proximal-distal so something is wrong with her development. I noticed this when she was about a year old and it explains my initial questions about her height. The fact that she was petite simply re-enforced my questioning over the years.

Please continue to let me know whenever I am unclear.


A Mom

[AMom]

I took her in for the blood work this morning before summer school. The endo told her it would be 2, 3 vials at the most. It was somewhere between 13-15 vials. I wish doctors would be more careful--some kids don't tolerate a change from one stick to multiple sticks for 4x as many vials as they were told to expect. (It was one of her specific questions.) That leaves the mom and techs to deal with an upset child.

Anyway, they said most of the results will be ready in a couple of days, some of it will be finished in a week because it has to go to an outside lab.