New Here with Recently Diagnosed Toddler

[AndiS]

y'all brought up some great questions! thank you! we are in the process of making a list of things to ask at her post-mri appointment, so this is very helpful. i did talk to Dr. Lark today, he called me after a round table discussion he had about Odella with his colleagues. they laid out a plan starting with surgery in about a year due to her small size and the size of the implant they would use to replace t9. so basically they want to do the excision/implantation to t9 and then instead of unfusing her ribs at t4/t5 and excising that hemi they would fuse her spine covering the hemi at t5 and preventing the fused ribs from moving/growing and causing further problems. i have a ton of questions regarding this approach with the fusion.

i think the time frame is actually the best news. i feel like they aren't rushing it and i have time to ask all the questions and then go elsewhere if i think we should. it is scary too though because i know both curves are progressing, hopefully they go slow for just a bit longer!

thanks again for all of your insight!
andi

[leahdragonfly]

Hi Andi,

I would really caution you to ask enough questions to gain a complete understanding of what surgery they are proposing. I am very concerned to hear about fusing the spine of a two year old--when that is done, the portion of the spine that is fused will stop growing forever, and she will end up with an unnaturally shortened trunk. This also means her lungs will not grow to their full capacity, and that could cause permanent lung and heart problems for her later in life. Of course I don't know all the details of your daughter's situation and I am not a doctor, but I do know that there are other approaches which are growth-preserving. VEPTR is the first one that comes to mind. I would specifically ask about the VEPTR, as well as educate yourself about it. (VEPTR is vertical expanding prosthetic titanium rib).

I would be extremely hesitant to allow this surgery on your precious daughter without getting another opinion from a top center that routinely sees complex congenital scoliosis cases. I think it has serious long-term consequences that might be avoided. Please consider getting another opinion from Shriner's Philadelphia. You will not be sorry you did.

I hope you are not offended by my candor. I am a parent of a young child with scoliosis too, so I know how difficult it is to make choices. That said, I have been a student of scoliosis for over 4 years now since my daughter's diagnosis, and I have learned a lot over those 4 years. I would hate for you to get your daughter a surgery that con't be undone without knowing all of her options.

[mariaf]

Hi Andi,

Not to scare you, but I have to echo Gayle's concerns about fusing any part of the spine of a child so young for the reasons Gayle stated - that portion of the spine will no longer grow. I have heard of the approach to 'unfuse' the ribs which seems to be more common.

I am not in the medical profession but since my son's diagnosis over a decade ago, I have learned quite a bit about scoliosis through researching, reading, and talking to doctors and others in the medical profession. I would get another opinion no matter what, maybe more than one, if it were my child. Even if you feel comfortable with your current doctor and his plan, you have absolutely nothing to lose by getting another opinion. I would strongly recommend seeing Dr. Betz in Philadelphia. As Gayle pointed out, there are fusionless techniques such as VEPTR that allow the doctors to avoid fusing the spine and also new procedures being developed each day.

Think of it this way - when my son had VBS in 2004, the only place that particular surgical procedure was available was at Shriners in Philadelphia. Today, it is available at dozens of hospitals around the country and is performed by some of the top doctors in the field - but it took several years to 'catch on'. You need to be sure your daughter is seen someplace where they are up on all the latest techniques because she doesn't have several years to wait :-)

I hope you don't mind my candor and that you ake my post in the tone and nature it was intended - as friendly, helpful advice and most importantly with your daughter's best interest at heart.

[Pooka1]

Case Report: L5 Hemivertebra Resection and T12-S1 Fusion in A 14 Year-Old Female with A 36 Year Follow-Up

Winter, Robert B.; Burger, Evalina L.

Spine., POST ACCEPTANCE, 18 January 2012


Abstract:

Structured Abstract: Study Design. Case report.

Objective. To demonstrate a 36-year follow-up of a rare operation.

Summary of Background Data. There have been no reports of follow-up of pediatric hemivertebra excision and fusion into mid-adult life.

Methods. A chart and radiological review at 36 years following surgery.

Results. The patient is alive and well and leading a normal life. Her ODI (Oswestry Disability Index) is zero. Mild degenerative radiological signs are evident at the adjacent level above (T10-T12) and below (sacroiliac joints).

Conclusion. Earlier excision of the L5 hemivertebra would have been preferable, but the long term results are good.

(C) 2012 Lippincott Williams & Wilkins, Inc.

A case study.

[AndiS]

i have the same concerns as you ladies...i haven't the slightest idea why he wants to fuse as opposed to excision and unfusing the ribs. her mri is scheduled for may 31 with an appointment with Dr. Lark the following day to discuss. i'm going to have a lot of questions, i would like to think there is a reason why he thinks this is the best course.

thank you sharon (right?) for the case study. i am doing some research. it really is my husband that needs to be convinced to get a 3rd opinion...

thanks to all of you for caring and offering your advice, i truly appreciate it!

andi

[Pooka1]

thank you sharon (right?) for the case study. i am doing some research. it really is my husband that needs to be convinced to get a 3rd opinion...

It is impossible to get too many opinions on this. But make sure it is from people who have done a number of cases. The other opinions don't count as much.

[mariaf]

i am doing some research. it really is my husband that needs to be convinced to get a 3rd opinion...

Hi Andi,

I have to ask, why does your husband need to be convinced? What is there to lose by seeking another opinion? I guess that sort of hit a nerve with some of us here - I'm just not following his thought process at all.

I agree with Sharon whole-heartedly - that if the opinions you have gotten so far aren't the opinions of true experts who do this sort of thing almost routinely, and have done so for years, then they aren't worth that much. I would also throw out that first doctor you saw, so basically you have one opinion (Dr. Lark) that may or may not be the best course of action for your child.

I'm not trying to give you a hard time, really - there's just so much at stake that I can't understand why anyone would be against getting another opinion. As Sharon said, it is impossible to get too many opinions. I know folks who have gotten as many as 5 opinions until they were comfortable with the plan of action.

Best of luck to you and please keep us posted. I hope you don't mind my candor.

[Pooka1]

If this was a common condition and practically any surgeon you went to would have done a million cases then yes it might be possible to get too many opinions.

That's not even close to what you are dealing with here. It's not in the same ballpark, country, or universe.

I suggest there simply are not enough congenital cases around to account for large numbers of surgeons who have great experience. That's what makes it physically impossible to get too many opinions.

Rather than getting too many opinions, the likely situation here is not being able to get enough experienced opinions for lack of caseload. It's the exact opposite of what your husband seems to be suggesting.

[AndiS]

he trusts them. he doesn't understand why i'm so leary. he doesn't understand why i spend hours reading studies. he has asked me twice what my threshold number of cases is. i can't answer that, i really don't know. he didn't realize how awful the first appointment was until we went to Dr. Lark and now he can't imagine how i could not be satisfied with our current "plan". i don't know.

i did get the radiologists report today that goes with the xray from 3/5 and they measured both curves. they had them at 36 for the major and 32 for the compensatory.

i guess right now, we are for sure doing the MRI here and asking a ton of questions and then i'll have to have a serious talk with my husband if i still feel unsure. i know you guys are just trying to be helpful. i'm just trying to hold it all together. thanks again for caring and listening and suggesting.

andi

[leahdragonfly]

Dear Andi,

I am sorry to hear your husband is not getting how serious and extremely rare Odella's condition is. It always is devastating to a parent to learn their child has a physical problem, so I can not even imagine how difficult it must be to learn your young child has a rare and difficult congenital problem. However, I think your husband is seriously putting on his blinders and jumping with the first doctor who has offered a solution, without even critically evaluating whether that is the best choice for your daughter, not just now, but for her future.

I believe Dr Lark has offered what solution he has based on his probably very limited experience with congenital scoliosis. That does not make it the best choice by any means, and I think it has the very real possibility of robbing your daughter of stature and especially lung capacity later in life. You will not be able to undo the fusion once it is done. Imagine how sick you would feel if you went ahead with that, only to have Odella develop potentially avoidable problems later. I am sure your husband is hoping for a "once and done" surgery so that this can be put behind your family. Unfortunately, I think Odella's problems are more complex than that, and since she is too little to make choices, you must advocate strongly for her. Even if he does not agree.

There is another mom I have come to know on this board, her name is Carmell. She is a remarkable mom whose son Braydon was born with a multitude of severe congenital anomalies, including multiple hemivert's and fused ribs. He is now about 15 or so, and she is not on this board much, but I want to share with you a link to her pages about Braydon. http://carmellb-ivil.tripod.com/myfamily/ . Please visit them, and look along the top of the page for "Spine Page", "Expansion page", and "VEPTR" info. There are lots of photos and x-rays, and if your husband doesn't think you need another opinion, show him those pages. Braydon had part of his spine fused at 11 months old, before Carmell knew all of the options. Maria and I are moms, too, and we just want to show you our support and make sure you become fully informed about your options for Odella's care.

My best to you, and please accept this advice with the care I am sending it with. We are here for you.

[Pooka1]

Maria and Gayle have made excellent points.

Depending on Lark's experience base, and considering the first guy, you may not technically have gotten an opinion yet from someone who has done enough of these cases. I don't know that but I am saying it seems possible given the exquisite rarity of this and how different all these cases must be.

You might consider just calling Shriners and asking who out there (besides Shriners) has done the most cases. I think Shriners would know the answer to that. I would then get Shriner's opinion and any opinion from someone they name as having loads of experience. Those are actual bone fide opinions based on experience and not just potentially very spotty training. If Lark or anyone can't reel off the literature on these types of cases in this age group then they are not necessarily the right group to handle the case. Lark might know the literature though. I would just ask if Duke is known for drawing in congenital cases from afar. They may be able to handle it but there may be better options.

You need cutting edge, not convenience. I would ask Lark what is cutting edge in this field and see what he says w.r.t. his surgical plan.

Just some thoughts.

[mariaf]

Hi Andi,

I thought I would tell you a little of David's story. He was diagnosed around the very same age as your daughter. For the first few years, he was under the care of a doctor who would be considered excellent if we were talking about AIS (adolescent idiopathic scoliosis) and who was affiliated with the Hospital for Special Surgery in NY (considered an excellent hospital). However, this doctor had no experience with a child David's age, as evidenced by the fact that all I would see in the waiting room at every appointment were older children. (This was more than 10 years ago and I did not even know at the time that there were doctors who were experienced with this very rare condition we call infantile scoliosis.) Fortunately, in David's case, surgery was not something being considered. However, this doctor, like most, did not offer the most cutting-edge treatments for infantile/juvenile patients.

Anyway, few years later, in late 2003, we found Dr. Betz and Shriners Hospital for Children in Philadelphia. The first thing I noticed when we walked in were that there were TONS of babies and toddlers there, not just older children.

To make a long story short, we never returned to the first doctor and I try not to think about where David would be today if we hadn't made the switch.

Bottom line, there is NO COMPARISON between a very good, even an excellent doctor who has no real experience with infantile cases - and one that does. There is another doctor who also comes to mind in terms of infantile cases and that is Dr. Vitale in NY. Like Dr. Betz and the staff in Philly, Dr. Vitale has a particular interest and expertise in early onset cases.

It should tell you something that in the 10 or 11 years I have been educating myself about this condition, I can only rattle off a couple of names of guys who are expereinced in treating scoliosis in an infant or toddler.

I hope you take this post in the friendly, helpful manner it was intended.

[AndiS]

hello again!

we had Odella's echo and mri and both came back normal (YAY)! the mri was an awful experience though, no need to go into details, just a really long and messed up day. we met back up with Dr. Lark the next day and he offered the "wait and see for now" approach. she will have xrays again in september so now it's all about the progression. he is hoping it doesn't (how could it not when both hemi's are left?). arggggggg. i am so so so glad that it appears as though all of her problems are confined to the bones of her spine, but...

so (i expect a happy dance from all of you), i emailed Janet...yep, i did it. i haven't told my husband yet and that is kinda killing me, but she said everything i wanted and needed to hear. so now i have to tell him. she suggested i send xrays so that Dr. Betz could take a look!

i guess i feel like since it is "just bone" everyone breathed this huge sigh of relief and decided it wasn't so bad. it still feels so bad to me though. also, i mentioned that i thought her right shoulder was down more and that i didn't like the idea of fusing t5 and i was met with head nods and smiles...

so there is my current update.
thanks for listening.
andi

and here is a funny little view of O's back, don't mind the rug burn, she fell off the couch

[leahdragonfly]

Hi Andi,

I am doing the happy dance here in Oregon that you contacted Janet! Your mama-bear instinct is correct and you should follow it, regardless of what anyone else thinks. You daughter's spine problem is rare and she deserves an expert opinion! It is in her best interest for her health now and in the future. I truly hope your husband will come to see that more information is a very good thing, and that he should not close his mind to expert opinions on his daughter's spine just because he is fearful.

Good news about the MRI results, too.

Thanks for updating, and please stay strong and true to your mama-bear instincts!

[mariaf]

Hi Andi,

Gayle said everything I was going to say, so I will just add a big DITTO!!

I'm so glad you contacted Janet. I am thrilled the MRI and echo came back normal - yay!! And, yes it is 'just bones', but scoliosis in a toddler - while very treatable - is still a matter that requries the attention of a doctor experienced in such cases - and since these cases are rare, there aren't too many doctors with tons of experience in handling them - however you hit the jackpot in Philly.

Please keep us posted...and most importantly, as Gayle said, stay true to your Mama Bear instincts!!