Overheard while leaving the Doctors office

[concerned dad]

Well, I haven’t been around much recently but we had our follow-up appointment with my daughters’ doctor.

Just a quick history – She was diagnosed 10 months ago at age 14 with a 38 degree curve. A local doctor said she needed to be braced and wanted to put her in a Boston Brace for 2 years. She was braced briefly in Jan/Feb with the SpineCor but did not achieve a significant inbrace correction.

One of the things that bothered me about my daughters’ situation was the assessment of her maturity by way of Risser; she was Risser 0 at diagnosis however almost 1 year post menarche and had already grown 6 inches taller than my wife. We live near a large metropolitan area with several SRS doctors. We sought a doctor who was not only an SRS physician (suggesting a focus on scoliosis) but also knowledge of skeletal maturity indicators. We were fortunate to find one 80 miles away, indeed this fellow writes many papers on skeletal maturity and, in my opinion, is the perfect doctor to assess my daughters somewhat unique circumstances. (As an aside, he used to practice at a Shriners hospital and used to offer the SpineCor. He is now participating in the Braist trial). We decided in the spring after consultation with this doctor (and incorporating the information about her skeletal maturity based on her hand xray) to “watch and wait”. Yesterday, we went to our appointment with great anxiety (even more so after reading Sharon’s post).

In any event, we went into the consultation room after she had her xray. The doctor soon followed along with a student (intern? Resident? I don’t know, it is a university/teaching hospital). After talking with us about how things are going he pulled up her recent and (many) past xrays. Her curve now is 36 degrees – essentially the same as at diagnosis.

We chatted about what that implied and I pointed out that “had she been wearing a brace” ….. he finished my sentence “we would have attributed the stabilization to bracing”. He went on to say that the goal of bracing is to stabilize a curve and she appears to have a stable curve.
He suggested we come back in a year.

Had we followed our first instincts and listened to the first doctor we saw (who seemed like a competent fellow) my daughter would be wearing a Boston Brace right now (and for another year). After seeing how miserable she was wearing the SpineCor for just 6 weeks I can’t imagine what her life would have been like.

I am not suggesting that bracing doesn’t help or that folks should use our isolated case history as reason to abandon bracing. I am just offering our experience as food for thought. I am saying though that the issue is complex and we don’t know enough yet to be sure we are making the right decisions. In our case, it looks like we spared my daughter the burden of bracing and still achieved the results one would hope for by bracing.

I am indebted to ALL the forum participants here who helped me wade through the issues and literature. In particular those formerly braced folks who helped me understand that bracing is not a trivial path to follow. Sure, I would have "slept better" knowing (hoping) I was doing everything possible to help my daughter but instead I bore the burden of potential guilt and spared my daughter the burden of wearing the brace. Some may say I gambled with my daughters health. I would say I made an educated decision based on available information with no guarantee of a particular outcome.

As we were leaving the exam room I overheard our doctor say to his student …… “There is a reason I am skeptical about bracing”

[Ballet Mom]

CD!

I'm so happy to hear about your daughter's result! I have been praying this summer for her and hoping that the Spinecor incident would not have any effect on her. That would have been unjust. I am thrilled that her curve appears to be stable.

As for the doctor who made the skeptical comment about bracing, I would question his judgement, as obviously, your situation shows absolutely nothing about the efficacy of bracing. It shows that your daughter was fortunate that she was already probably physically mature when the scoliosis was diagnosed. I think your case and others on this forum show more of a problem with using Risser scores versus other measures to determine skeletal maturity. Of course, there's a reason they use Risser versus other methods which is to reduce radiation exposure.

Anyhow, great news CD, I'm very happy for your daughter's result.

[concerned dad]

Thanks for you thoughts and prayers.

I think you may have misunderstood his statement. He suggested the watch and wait approach because he determined my daughters skeletal age was advanced not because he is anti-bracing. He does brace some of his patients. But, in my daughters case, any efficacy of the bracing would be diminished due to her skeletal maturity.

I believe that, if pressed, he may have clarified his comment to his student by saying “there is a reason I am skeptical about the bracing literature”.

My daughter would have fit all the inclusion criteria for a bracing study.

So, when you say

as obviously, your situation shows absolutely nothing about the efficacy of bracing.

you are correct, but it does say something about the bracing literature.

Perhaps this goes to the whole issue of the Braist study. Anyone whose reads my posts over the past 9 months knows I’ve flip flopped all over the place on the topic. Any doctor who participates in the trial must believe that there is a significant doubt about bracing efficacy. That is to say, they recognize the limitations of the current evidence.

If they believed bracing didn’t work, they would be unethical participating in the trial because they would brace 50% of the kids.
If they believed bracing did work, they would be unethical because they would NOT brace 50% of the kids.
The only way they could ethically participate is if they felt that the issue was unresolved based on the current literature.

[concerned dad]

And I have to be careful about putting words in a doctors mouth

[Ballet Mom]

CD,

I think maybe my questioning your doctor's judgment was a little overreaction. I am very sensitive to these doctors and their bracing biases.

I think we can all agree that the bracing studies are extremely limited in their usefulness. I do not agree that the solution is to not brace children (i.e. the Braist study) who would have what appears to be a 50% to 80% chance that they would respond to bracing treatment of some sort if they were braced. As I have stated before, I believe the Braist study is unethical. And I believe there are ways to design studies that don’t require putting children at risk of major spine surgery unnecessarily.

Perhaps orthopedists should be x-raying the hand of those patients who are a year post-menarchal when diagnosed, in order to help some patients avoid bracing completely if those patients are shown to be more skeletally mature than what their Risser sign would suggest. That could be taken into consideration in regular bracing studies . So could physical attributes such as being overweight or having spinal flexibility that would be very beneficial knowledge for doctors to determine whether patients would respond to bracing or not. So could the sensors be used in regular bracing studies to determine brace use that does not put children at risk. I think much knowledge could be obtained while avoiding putting children at risk like the Braist study does. I’m sure the medical research community is well-attuned to creating studies that try not to put patients at risk as I would guess many, many diseases and conditions would be unethical to not treat the patient with current best practices. I think scoliosis is one of these conditions.

There would also be a lot more interest in participating in the study, if all children were to be treated in the study. I know that I would never put my child in that study, I would hope that most other parents would also feel the same way.

My daughter is almost nine-months post menarchal and she is still showing improvement in the look of her back with her night-time brace and working with her ballet teacher on posture. I don’t think anyone could tell at this point that she has scoliosis. Even her right scapula that was more pronounced than her left one has resolved to the point of being absolutely equal to the left in prominence. Perhaps she is very unusual, but maybe researchers should be looking at the success stories of bracing and determining what it is about those cases that makes them successful.

Sorry my response is so slow, but my son is heading off to college tomorrow and I’m having to run and take him to doctor’s appointments, etc. Wonderful that one of my kids is flying the nest!

[Pooka1]

Flip-flopping versus being open-minded

Very excellent news, CD! I'm very happy for you and your daughter.

Anyone whose reads my posts over the past 9 months knows I’ve flip flopped all over the place on the topic.

I would say you are open-minded and evidence-driven. That is a very good trait. Your daughter is very obviously lucky you are that way.

Hopefully she is out of the woods or will soon be so.

Best regards,
sharon

[concerned dad]

Going off to college, it must be an exciting time in your home.

Look, the Braist study is an interesting but dangerous thing to discuss.
It is interesting because it involves science and ethics.
It is dangerous because we who are discussing it here have a personal stake in scoliosis. Whether we admit it or not, we have biases based on our own life experience. Too often here at NSF intellectual disagreements turn personal.

Having said that, what the heck …….

You say

I do not agree that the solution is to not brace children (i.e. the Braist study) who would have what appears to be a 50% to 80% chance that they would respond to bracing treatment of some sort if they were braced.

But don’t forget the statement from the 2007 paper discussing the followup to the (now recognized as flawed) SRS bracing study. They ask (emphasis added)

As 70% of the observed patients during the original study period did not require any other treatment, 70% of the initially braced patients can therefore be regarded as having been treated unnecessarily. One major question is as follows: is it worth overtreating such a high percentage of patients to realize the goal of “saving” only 10% of the patients from surgery, the percentage in the group of observed patients?

If my kid were one of the unfortunate 10% I would probably say, yeah, you bet it’s worthwhile. But, if my kid was (and I think she may be) one of the 90% I would say, heck no.

A parent of a braced kid today has no way of guessing what group they are in. Maybe advances in DNA testing will help. Maybe the results of the current brace trial will help our grandchildren.

[Ballet Mom]

CD,

All I can say is, I'm glad my daughter wasn't sacrificed in the name of science. I'm also glad things worked out for your daughter, I was not questioning your decision not to brace, obviously your daughter was at a stage where it was reasonable to decide not to, due to her maturity and discomfort with wearing one.

[Pooka1]

As 70% of the observed patients during the original study period did not require any other treatment, 70% of the initially braced patients can therefore be regarded as having been treated unnecessarily. One major question is as follows: is it worth overtreating such a high percentage of patients to realize the goal of “saving” only 10% of the patients from surgery, the percentage in the group of observed patients?

If my kid were one of the unfortunate 10% I would probably say, yeah, you bet it’s worthwhile. But, if my kid was (and I think she may be) one of the 90% I would say, heck no.

A parent of a braced kid today has no way of guessing what group they are in. Maybe advances in DNA testing will help. Maybe the results of the current brace trial will help our grandchildren.

If 70% were perhaps unecessarily treated and only about 10% are saved from surgery (at least at the point of maturity), then that leaves about 20% are bracing failures.

And that is for AIS I assume. Scoliosis associated with connective tissue disorders may be a whole 'nother ballgame. I found one paper of a small (uncontrolled) study of bracing kids with Marfans claiming about a 20% chance that bracing avoids surgery as for AIS.

http://www.ncbi.nlm.nih.gov/pubmed/10984787

CONCLUSIONS: The success rate for brace treatment of Marfan scoliosis is 17%, which is lower than that reported for idiopathic scoliosis. Possible reasons include increased progressive forces, altered transmission of corrective pressure to the spine, and younger age at inception of bracing. Because there was no control group, it is unknown whether bracing slowed curve progression. Physicians should understand that most patients with Marfan syndrome who have a curve of more than 25 degrees and a Risser sign of 2 or less will reach the surgical range, even with brace treatment.

My kids have not been diagnosed with Marfans but one has a diagnosis of hypermobility. And they both fell into the category referred to in the last line of the Conclusions section quoted above.

That said, it's one uncontrolled study and, as such, has a high chance of being false. Nobody knows.

[leahdragonfly]

Great news CD!

Dear CD,

I've been missing you here lately. I am really thrilled to hear that your daughter had good news today, that's wonderful that she doesn't have to be checked for another year.

As you know I completely respect each parent's choices for treatment of their child's scoliosis. I think over the past year or so you did a tremendous job educating yourself and trying to understand the issues surrounding treatment of your daughter's scoliosis. It takes courage to "go against the grain" and decline bracing, but I think in many cases it can work out. I've been rooting for your daughter! And as a formerly braced child who found bracing to be personally pretty devastating, I have a unique perspective on this that others may lack. I personally have a really hard time with the possibility of overtreating 70% of braced children. It is not a benign treatment by any means. The more scientific evidence we have to guide treatment, the better. So I am glad things worked out well for you and your daughter. She is lucky to have such a caring and involved dad.

Now please don't leave us high and dry here. You are a great addition to the discussions here.

Take care,

[Snoopy]

Physicians should understand that most patients with Marfan syndrome who have a curve of more than 25 degrees and a Risser sign of 2 or less will reach the surgical range, even with brace treatment.

My kids have not been diagnosed with Marfans but one has a diagnosis of hypermobility. And they both fell into the category referred to in the last line of the Conclusions section quoted above.

Sharon,
My daughter doesn't have Marfans either, but she too falls into the same category as your girls. We've always thought because of the rate at which her Scoli progressed and the degree of her Kyphosis, that there is an underlying cause, which we believe is CMT (Charcot-Marie-Tooth). We put Jamie in two different braces, which she just would not wear. Like you, I agreed with her decision to not wear the brace and I don't regret that decision. I know she would have progressed with or without a brace. Makes me wonder just how many kids with underlying causes needlessly wear a brace.

Mary Lou

[Pooka1]

Sharon,
My daughter doesn't have Marfans either, but she too falls into the same category as your girls. We've always thought because of the rate at which her Scoli progressed and the degree of her Kyphosis, that there is an underlying cause, which we believe is CMT (Charcot-Marie-Tooth). We put Jamie in two different braces, which she just would not wear. Like you, I agreed with her decision to not wear the brace and I don't regret that decision. I know she would have progressed with or without a brace. Makes me wonder just how many kids with underlying causes needlessly wear a brace.

Mary Lou

Mary Lou,

Your last point haunts me. I think there should be more effort to try to identify the cases that might not be AIS of the sake of these kids.

I think the speed of curve movement might be used by some surgeons. Savannah's curve moved 5* a month (on average) for every month she was under observation. There was never any talk of bracing her. I haven't seen another testimonial like that (that speed over that amount of time) here at least. How fast did Jamie's curve move? Based on your comment, I'm guessing faster than the average testimonial here.

Willow's curve was stable for 6 months prior to the brace and then moved 8* while wearing the brace nightly. Again, I don't recall another testimonial like that. Willow's curve moved between 3-4 degrees a month over the last 6 months (on average). That adds up fast.

I found part of one discussion in a book (the next page wasn't shown) that started out with something like... while scoliosis associated with connective tissue disorders is sometimes held in brace during brace treatment due to flexibility.. and then ended. I am guessing they go on to say ... when they come out of brace, the curve continues to progress.

My kids have squirrelly spines. I accept that now. I am very grateful to have run into two surgeons who choose not to brace one and who only went with a night time brace with the other just just to try. He said he never would have put her in a 23 hour a day brace. He also doesn't think she has Marfans but the geneticist seems to think we should do yearly aortic monitoring for that.

I am glad I involved Willow in all this. It was her decision to keep wearing the brace after I said I didn't think it would matter. I LOVE that she didn't just blindly follow my thoughts at that time just as an exercise in independent thinking. And it was her decision to stop wearing it when it stopped fitting. It gives me hope I have raised a skeptical, critical thinker.

[concerned dad]

He also doesn't think she has Marfans but the geneticist seems to think we should do yearly aortic monitoring for that.

If it turns out that she does have Marfans her scoliosis would have been a blessing in disguise as it may have provided a critical clue to compel you to monitor for something much more dangerous.

Gayle, Thanks for your kind words. And thank you for sharing your life experience here which helped me make my decision. Were it not for yours and Pams (and others) contributions to the discussions my daughter might be wearing a brace now. The personal burden of wearing a brace may be difficult for a parent to fathom.

BalletMom, I know you werent questioning my decision (although it would be OK to question it, I sure did). We wouldnt participate in the Braist trial either but I dont fault those who do. In retrospect, had we caught my daughters scoliosis earlier we probably would have gone with a night brace like yourself and Sharon. It seems like a reasonable compromise between being proactive and not being too intrusive on a childhood.

I know we are not out of the woods. Entering adulthood with a curve in the high 30's is not a great thing. Perhaps it bought some time to hope for even more advances in surgical techniques should it come to that.

[Snoopy]

Mary Lou,

Your last point haunts me.

Sorry, I didn't mean to haunt you. I think we are both on the same page with this issue.

How fast did Jamie's curve move? Based on your comment, I'm guessing faster than the average testimonial here.

Wow! Your girls' curves moved quicker than Jamie's! Jamie's curve progressed about a degree per month AFTER she was skeletally mature.

Willow's curve was stable for 6 months prior to the brace and then moved 8* while wearing the brace nightly. Again, I don't recall another testimonial like that. Willow's curve moved between 3-4 degrees a month over the last 6 months (on average). That adds up fast.

When you see your girls' and Jamie's progression, it reminds me that it was time to do surgery sooner rather than later. At the time of her surgery, Jamie's Scoli curve was 46* and still going. Her Kyphosis was in the low 70's and still going.


He also doesn't think she has Marfans but the geneticist seems to think we should do yearly aortic monitoring for that.

Monitoring doesn't sound like a bad idea. I would compare it to the monitoring our doctor does on my youngest daughter who has a heart murmur. Better safe than sorry.

There is testing available for CMT (and Neurofibromotosis (sp?) which is another condition that Jamie might have) but there isn't a cure for either one. Jamie chose not to be tested. She wants to live her life as fullly as possible and not worry about or watch out for symptoms of either condition. She knows because of the strong family history, affecting mostly women in her dad's family, there's a chance she will develop CMT. She chooses not to dwell on that.

I am glad I involved Willow in all this. It was her decision to keep wearing the brace after I said I didn't think it would matter. I LOVE that she didn't just blindly follow my thoughts at that time just as an exercise in independent thinking. And it was her decision to stop wearing it when it stopped fitting. It gives me hope I have raised a skeptical, critical thinker.

I think you and I have both done a wonderful job raising our daughters! From the day Jamie was diagnosed, I told her this was her Scoliosis, but her dad and I would support her 100%. She was involved in all decisions as it was her body, not mine.

Mary Lou

[Pooka1]

I think you and I have both done a wonderful job raising our daughters! From the day Jamie was diagnosed, I told her this was her Scoliosis, but her dad and I would support her 100%. She was involved in all decisions as it was her body, not mine.

Mary Lou

I love your approach to this.

And with regard to "haunting," I have been haunted by that thought for a long time, not just your recent mention. I agree that you and I are on the same page. I just keep imagining the let down when these kids wear a brace and still need surgery, if not right away then later in life. It has to be crushing. I wanted to avoid that for Willow.

Luckily, she knew the brace only had a small chance of working from the start. There was some confusion on this point with me because I wasn't able to be at that appointment... I thought her chances were better than that at first based on what my husband reported. I later came to the conclusion she couldn't have a good chance of the brace working based on my reading. Anyway, Willow agrees that it was worth a try and it didn't interfere unduly with her life. She wore it for a year. I doubt she would have worn any non-night-time brace at all given the circumstances.

Jamie's curve progressed about a degree per month AFTER she was skeletally mature.

Okay that is the fastest I have read in a skeletally mature person. Wow.

My girls were not skeletally mature. Willow is now at a Risser of 4. In the last six months she moved from 36 to 57. That's 3.5 degrees a month on average. And as I have mentioned several times, Savannah's curve moved 5 degrees a month (on average) for at least 5 months. Someone correct me if I'm wrong by that seems quite different from the typical AIS case, no?

There is testing available for CMT (and Neurofibromotosis (sp?) which is another condition that Jamie might have) but there isn't a cure for either one. Jamie chose not to be tested. She wants to live her life as fullly as possible and not worry about or watch out for symptoms of either condition. She knows because of the strong family history, affecting mostly women in her dad's family, there's a chance she will develop CMT. She chooses not to dwell on that.

That's a very rational way to proceed.

Because the genetic test for Marfans has a small false negative rate, even if they had the test and it was negative, we would still have to do the monitoring. So we don't do it. There are no false positives. If there was a good test with no false negatives, I would do it in the hopes of being able to stop the monitoring if negative.

I still can't get over Jamie's progression AFTER skeletally maturity and when the curve is still in the 40's.