Congenital Scoliosis

[stees]

Hello,
I was just searching through this new forum for congenital scoliosis information. I am the mother of a 35 month old boy with severe congenital scoliosis. He has about ten vertebrae which are not completely formed, ie hemivertebrae. So far we are just bracing as our doctor is monitoring research on the Titanium rib procedure that Carmel, also of this forum, has talked about. I think with my son that more than the rib will be necessary. He is, to make things harder, extreeeeeemely small for his age. I mean, we're talking right off the lowest percentile scale! Anyway, there's not much flesh to stick a rod or a rib into and that was one of the reasons that our doctor and the Seattle Titanium Rib team didn't think my son was a good candidate at that time.

I'm going to post in the research forum if I can, but I am interested in the very leading edge therapies including transplanting bone growth tissue to the affected vertebrae, building up the affected vertebrae with synthetic supporting materials, variations on external support for the spine, etc. I think my son may need a multipronged approach.

I'm interested in other parents' experiences, fears, thoughts, etc.

Susan

[Carmell]

Hey Susan!

Good to see you posting here. I hope this forum becomes active and popular - I miss the congenital email list.

I know how much you are following any improvements in technology to help your little guy. He is a unique case. I wish there was a magic wand to wave to make all his problems disappear. Sadly, that isn't reality... I'd give you a big hug if I knew it would help!

Question - what is his oxygen sats? Has he had a pulminary function test done lately? I ask, because Braydon's lung function has improved dramatically since his TRP implants 2 years ago. Just after the implantation, his lung capacity was 51%. In September 2003 his lung capacity had improved to 71%. Since his body is oxygenating much better, his overall growth is GREATLY improved. He is healthier overall and he has grown so much - finally thriving. I can't explain it very well, but there is a huge benefit to having your body oxygenate well. The entire body and all the internal organs are healthier when your lungs are working well. I wish I knew how to get your little guy to have such a great improvement. Has your Seattle team discussed his case specifically with Dr. Campbell or Dr. Emans from Boston? Dr. Emans has worked miracles on children prior to the development of the TRP. I would trust him with my child's life. He is actually more experienced than Dr. Campbell because of his years of spine/congenital scoliosis cases for many years.

Sorry for rambling... I'm glad to see you here and hope that all is well (as well as it can be) at your place. Gentle hugs to your little man!

[Penny Rutledge]

I'm glad to see people posting. I have missed the MIT list too. We have had a wonderful repreive from surgeries this summer. Dylan is doing great. He began preschool and is loving it.

He had a doctors appointment last week and he told me he didn't want to go because he didn't want anymore surgeries on his back. I was able to reassure him it wasn't for his back, but it made me realize how much harder it is going to be next time he does have to have surgery. One day at a time!

It would be great to hear from more former MIT list members and new people too!

Penny

[Carmell]

Hey Penny!

Good to see you here. I'm so glad to hear Dylan is doing well. I understand how hard it is when they have to go to the hospital all the time, and they finally say enough. I've been very fortunate that Braydon has had good, caring, understanding teachers at school to recognize that he has little control over the medical decisions in his life. They allow him to make decisions in class that are HIS choice, and that HE controls. This has helped a lot. That, and him maturing and being able to understand more about the "whys" of having surgery every 6 months makes a big difference. He had his last rod expansion/exchange surgery on Sept. 22. This has been his easiest recovery yet (knock on wood!). I'm so pleased with his ability to "bounce back" from these procedures. Hopefully he continues to do so well. The anxiety these kids get is not fair, but who says life is fair? By helping them with their anxiety, they will feel better about the procedures they have to endure (IMHO).

Take care and I hope to see this messageboard do well!

[Penny Rutledge]

Susan -

Dylan is also extremely small for his age too (not even on the curve). Dr. Emans in Boston felt he was too thin for the Titanium Rib because it is so bulky. Dylan was able to have a "growth rod" put in though. You can see the rod bulge under his skin and he wears a brace full time to protect him. His orthopaedic surgeon is always concerned about his weight - she wants more fat on him!

I know how frustrating it is to see them so thin - we have friends with 18 month olds who weigh as much or more than Dylan at 37 months! We supplement his food intake with Pedisure or Ensure drinks. He gets 1-2 a day, depending on how well he is eating that day. Have you tried these with your child? I also never say no if he wants a fat-filled snack over a more "healthy" one. I'm just happy to get calories in him!

[Penny Rutledge]

Carmell -

How exciting to hear that Braydon underwent his final expansion! It is wonderful to hear you have seen so much progress in his lung capacity and overall growth!

It's always nice to hear good news from other parents.

[Carmell]

Hi Penny,

um, Braydon hasn't had his final expansion - just his latest expansion... we'll be doing this expansion thing for another 8-9 years, every 6 months... some fun, huh!? LOL

My point is that Braydon is adjusting very well and takes these surgeries in stride. He's dealing very well with his anxiety and that has made a huge improvement in his recovery lately. Hopefully he continues to do so well. Take care!

[stees]

Hey Penny!

We give Malcolm 1-2 Pediasures per day too. He eats the most fattening of foods too, like corn chips, chicken nuggets, etc. Oddly enough he doesn't like sugary foods much. When he gets surgical implants they are sure to show through!

Although I'm not glad that Dylan is also tiny, it is nice to have 'company' in this.

Susan
mum to Malcolm almost 3 years, congenital scoliosis diagnosed at birth, bracing and waiting for that time we HAVE to operate

[Carmell]

Just wanted to share that Braydon is small, proportionately small. He is also lean, but not unhealthy skinny. He has two vertical, adjustable rods which you can see and feel through his skin. At the bottom of one rod, he has a bursa (fluid filled sac) caused by the friction of the rod rubbing on the underside of the skin. "MY" biggest fear is that he will develop skin breakdown in this area because of the repeated bursas (doc drains the fluid every 6 months with each expansion surgery). Surgeon isn't worried about that too much. Hopefully he's right and I'm wrong.

I understand the worry about being too thin. I also know that there are added risks with children who have this problem. However, sometimes you have to weigh the benefits and risks and decide when the right time for implantation is (like Susan said). Sometimes the risks of waiting longer are worse than taking a chance now. Braydon was 6yrs old at the time of his impant surgery. If we would have waited much longer, his chances of having a good correction and lung function improvement would have been much less (because of the maturation of the lung tissue, etc.). Braydon was 31 pounds at 6 yrs old when he had his implant surgery. He is now 8yrs old and weighs a whopping 43 pounds - a much needed improvement that could only happen because his body is oxygenating well and healthy.

Sending healthy vibes to Malcolm and Dylan and all! Take care

[stees]

Hi Carmell!

Braydon sounds like he is doing great thanks to your being so "on top of" things. His weight gain is stupendous. Anyway, these kids will be glad later on to be light, as it is less weight to bear and less tissue to oxygenate. I wish I could just chill a little bit.

I hope Braydon's bursa is only a minor thing as the doc seems to think.


Susan

[Carmell]

Thanks for posting Susan!

You are absolutely right - I feel that Braydon needs to be on the lean side, rather than taking on the family trait of being overweight. I can't imagine having an unstable spine and trying to support unnecessary weight. That, and dealing with body oxygenation make it a very fine line to walk. Right now, Braydon is eating well and seems to be maintaining a good weight gain (not too much, not too little). I hope that continues. I hate to be the food police (i.e., don't eat so much, drink more milk, stop eating before bed, etc.).

Give Malcolm a big hug from us!

[stees]

Hi Carmel (and Penny) and any lurkers,

At Malcolm's last doctor visit we were surprised to hear that our doctor is making moves to get the Titanium rib established in Vancouver! He thinks that this is the best strategy for Malcolm.

Now I've done some more reading, since we will probably be entering the VEPTR surgical merry go round later this year, and I'm disappointed that the stats on kyphosis and scoliosis improvement are so poor. Vertebral growth seems to increase, yes, but the curves appear to progress. Carmel, any insight into this? I'm not sure if Braydon was one of the published cases (he would have had lots of CTs if he was). I'm disappointed because I think that Malcolm's kyphosis must be addressed to improve lung function.

Maybe continued bracing will achieve this goal. Our next doc appointment is in March.

Susan
mum to Malcolm, 3 years, congenital scoliosis with various vertebral shapes and problems, height 30 inches standing (longer when lying down), weight 20 lbs or so.

[Penny Rutledge]

Hi Susan -

Your concerns are exactly why we didn't pursue the Titanium Rib for Dylan. Dr. John Emans from Boston Children's whose been involved in the Titanium Rib project from early on, reviewed Dylan's case and said his kyphosis would not be adequately controlled by the rib. At that time Dylan's kyphosis was progressing quickly, it went from 40 degrees to over 85 degrees in 6 months. Emans also said he was concerned about Dylan's size because (according to him) the rib is quite bulky. Dylan was 20 lbs and about 30 inches long at the time. Emans agreed with our doctor that using the halo traction to straighten his kyphosis followed by putting in growth rods was the best approach. I can tell you more about that if you e-mail me directly.

I would strongly encourage you to contact Emans or someone like him. I was very impressed with him. He is highly respected yet very accessible. I e-mailed him directly (found his e-mail address on the web) and he responded within one day. He reviewed Dylan's x-rays and pictures and responded within 10 days. It was great because we were pressed on time. If we need to look into the rib again I will definitely contact him about it. He continues to ask our doc about how Dylan is doing when he sees her.

Right now we are pleased with the results we have had from the traction and growth rods.

My best to you,

Penny

[Carmell]

Hi Susan,

I agree with Penny - Dr. Emans will be able to give you both perspectives. He was very well experienced and respected in treatment of scoliosis prior to joining with Dr. Campbell and the TRP team. If anyone on this planet would know the best options for Malcolm, it would be Dr. Emans. I met him once, and from all the other parental input, he is FABULOUS. I would trust him with my child's life, if I needed to.

I know several children who have had TRP implants because of kyphosis. They have had very good improvements. One has been able to come off oxygen during the daytime! Each case is very unique, which makes comparing any two cases like comparing apples and oranges. The TRP has improved their design of the pelvic attachment device recently, which means the few problems with not holding a kyphosis improvement has been addressed in most cases. I wouldn't completely write off the TRP for Malcolm. You are absolutely right - his kyphosis needs to be the focus to improve his lung function.

I also know that when children wear braces at such a young age for extended periods, their bones (especially rib bone structure) doesn't fully develop. This makes the bones too soft to support more invasive treatments, like the TRP. My point is to be careful with bracing.

Also, our Shriners in SLC is one of the premiere places (I'm told) for halo traction and rod placement surgery. Maybe getting an opinion from someone who specializes in halo options wouldn't hurt either.

I know how hard this is for you. Malcolm deserves the very best. And so do you. Please keep us posted on how things go. Hopefully you'll find the magic answer very soon.

[jacki]

Hi Susan

I wanted to introduce us, though I'm kind of jumping in 'off topic'!

My 4 year old has Scoliosis (not congenital), and we live in Vancouver! We see Dr Tredwell at Children's and Robert Ford for my little girls brace.

I am always looking for parents in the area to connect with (there's not many of us around!).

I have 'spoken' with Carmell re the Titanium Rib Project, as its something I may look into in the longterm (I really hope to delay the spinal fusion for as long as possible). I was wondering if Malcolm sees Dr Tredwell? And if its him thats looking into the Titanium Rib Project?

Right now Siobhan's curves are 40/30, and they are progressing in the brace.

I look forward to 'chatting' when you have time.
And Happy New year.

Jacki