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Re: Meri- titanium rib

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Hi ,

Thanks for your comments. I will take a look at the veptr.com site.

I know of many children with SMA (type 2, not type 1 like has) and all of them have had rods placed to manage their scoliosis. They return a few times a year to have the rods lengthened. I just wanted to see what other options are out there to try to prevent from getting any worse at this point, wanting to avoid the rod surgery in the future if at all possible.

has been seen by SMA specialists both at Stanford and Primary Children's in Salt Lake City. Since almost all type 1 SMA kids die so young, there are few examples to evaluate beyond 2 years of age. Those that do live are usually so weak that they cannot tolerate any sitting, and are on their back 100% of the time. They usually don't have problems with scoliosis. The type 2 kids are so much stronger from a respiratory standpoint, that it is very hard to do a comparison between what they would tolerate physically, and what could handle.

I appreciate all of your advice!

For anyone else reading, how bad does 's back look to all of you? I have nothing to compare it against, and with all of the other issues with his health that keep me busy, his curve ends up further down on the priority list.

Regards,Meri

To: infantile_scoliosis Sent: Thu, February 25, 2010 3:19:07 PMSubject: Meri- titanium rib

Hi Meri,Thanks for posting the pictures of your son. I have an old textbook about congenital spinal conditions that has a picture of a child with SMA. There was a mom who posted on veptr.com (the acronym for the titanium rib) whose son has SMA, but I have not really been on the site too much since we decided not to do the veptr for our daughter right now. There are other kids with the g tube or mickey button (I don't really know the difference) on the site also. I think this mom was in a support group for the SMA. Are you able to find other parents whose children also have it? I think she even went to a conference about it at one time. Are some doctors more experienced with the SMA diagnosis? I would imagine so.

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Hi Meri,

I don't know much about SMA, our daughter did have a bad case of pectus

carinatum (pigeon chest) when she was an infant. I searched near and far for a

treatment, but, when her muscles got stronger, it miraculously went away. I

found this article, you may have read it already, but it seems they feel the

pectus and skeletal deformity can be helped by assisted ventilation at night. I

wonder if this would help him sleep at night too, like I said, I really don't

know but just in case the info could be helpful, I pasted it below. Perhaps you

could contac the author of the article, his email is posted.

Pectus and Hypoventilation

Philip Glassner, MD and R. Bach, MD, FCCP

+ Author Affiliations

University of Medicine & Dentistry of New Jersey Newark, NJ

Correspondence to: R. Bach, MD, FCCP, Department of Physical Medicine and

Rehabilitation, University Hospital, B-403, 150 Bergen St, Newark, NJ

07103-2406; e-mail: bachjr@...

To the Editor:

We read with interest a recent article in CHEST (June 2001)1 that considered

pectus excavatum as the cause of hypercapnic respiratory failure. Patients with

generalized neuromuscular weakness that results in scoliosis, such as those with

Duchenne muscular dystrophy2 and those with pectus due to the generalized

weakness associated with spinal muscular atrophy type 1 (SMA1),3 develop

hypercapnic respiratory failure. Also, even when not associated with generalized

weakness, thoracic deformity from kyphoscoliosis, when severe enough, itself can

result in hypoventilation. However, in the cases of patients with Duchenne

muscular dystrophy and SMA1, the ultimate cause of the skeletal impairment is

muscular. Likewise, idiopathic scoliosis may be due to paraspinal muscle

weakness, just as the pectus in these patients may be due to, or at least may be

associated with, intercostal muscular impairment. Although diaphragmatic

function was tested in these patients with pectus, the values of the sniff test

were not given. An assessment of maximum inspiratory pressures would reflect the

strength of inspiratory muscles, including those of the rib cage. If the root

cause of the problem is an underlying muscular impairment, then the hypercapnia

may be due more to that condition, and the pectus deformity may be only

secondary, just as it is for patients with SMA1. The fact that the patient had a

history of being easily fatigued and frail as a child implies a possible

neurologic or muscular disorder that worsened with time.

It is also unclear when the pectus began. Just as for SMA1 patients whose

pectora resolve once they are placed on nocturnal high-span bilevel pressure

ventilation, this patient's pectus may have been milder had high-span bilevel

ventilation been instituted much earlier. It is of concern that, despite the

rigidity of the chest wall in this patient and the severity of the pectus, only

low bilevel spans were used. We suspect that, had this patient been introduced

to noninvasive ventilation at adequate levels long ago, hypercapnic respiratory

failure would not have developed. We are also concerned about oxygen

supplementation, and we suggest that the patient should use increasing periods

of daytime ventilatory support via a mouthpiece to normalize alveolar

ventilation rather than oxygen therapy, which entails a risk of developing

increasingly severe hypercapnia.

References

1. & #8629;Theerthakari, R, El-Halees, W, Jvadpoor, S, et al (2001) Severe pectus

excavatum associated with cor pulmonale and chronic respiratory acidosis in a

young woman. Chest 119,1957-1961Abstract/FREE Full Text2. & #8629;Bach, JR,

Ishikawa, Y, Kim, H Prevention of pulmonary morbidity for patients with Duchenne

muscular dystrophy. Chest 1997;112,1024-1028Abstract/FREE Full

Text3. & #8629;Bach, JR, Niranjan, V Spinal muscular atrophy type 1: a noninvasive

respiratory management approach. Chest 2000;117,1100-1105Abstract/FREE Full

TextAbbas Mansour, MD, Jenifer S. Khan and M. Anees Khan, MD, FCCP

+ Author Affiliations

Seton Hall University South Orange, NJ

Next SectionTo the Editor:

We appreciate the comments about our case report.1 Regarding the possibility of

a neuromuscular disease as the cause of our patient's pectus deformity, there is

no clinical evidence for any such disorder in our patient. Her neuromuscular

system is entirely normal. Muscle power and deep tendon reflexes are within

normal limits. She is able to generate a negative inspiratory force of 60 cm,

and the sniff test has excluded the possibility of diaphragmatic weakness, as

she could move both her diaphragms for at least one intercostal space. Advanced

neuromuscular disease during childhood usually leads to considerable deformity

of the spine and the trunk. Our patient is devoid of any other deformities

except for severe pectus excavatum.

As regards the onset of pectus, our patient had been aware of this abnormality

since approximately 12 years of age, but she denies having had any medical

checkup or follow-up while in her native country. Since her immigration to the

United States > 9 years ago, she had not had any medical evaluation until her

hospitalization last year, when she came to our attention for the first time.

Employing noninvasive, bilevel pressure ventilation (inspiratory pressure of 12

cm) and low-flow oxygen, we were able to improve her hypercapnia and hypoxemia

while maintaining a balanced arterial pH. Higher levels of inspiratory pressures

may be appropriate if the result from lower pressures is unsatisfactory.

Patients with acute-on-chronic respiratory acidosis develop posthypercapnic

metabolic alkalosis if alveolar ventilation is increased by overzealous

ventilatory support. Meticulous care was taken to prevent this complication. One

goal of therapy is to correct hypoxemia and balance the arterial pH.

Overcorrection of hypoxemia (Pao2 > 65 mm Hg), which is obviously the authors'

concern, was avoided by appropriate monitoring of gas exchange.

The authors are probably correct in the speculation that the patient would have

benefited from earlier intervention with bilevel pressure ventilation. We

successfully utilized an inspiratory pressure of 12 cm, which is within the

range used in most cases of hypercapnic respiratory failure treated with

noninvasive ventilation. Nevertheless, we feel that the recommendation to use

high-span bilevel pressure ventilation is an excellent idea, which we intend to

institute with this patient to see if it results in further improvement in

hypercapnia without inducing significant metabolic alkalosis, and to see if the

need for supplemental oxygen can be obviated.

Previous Section References

1. & #8629;Theerthakari, R, El-Halees, W, Javadpoor, S, et al Severe pectus

excavatum associated with cor pulmonale and chronic respiratory acidosis in a

young woman. Chest

2001;119,1957-1961CiteULikeComploreConnoteaDel.icio.usDiggFacebookTechnoratiTwit\

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doi: 10.1378/chest.121.2.664-b

CHEST February 2002 vol. 121 no. 2 664-665

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References

>

> Hi ,

>

> Thanks for your comments. I will take a look at the veptr.com site.

>

> I know of many children with SMA (type 2, not type 1 like has) and all

of them have had rods placed to manage their scoliosis. They return a few times

a year to have the rods lengthened. I just wanted to see what other options are

out there to try to prevent from getting any worse at this point, wanting

to avoid the rod surgery in the future if at all possible.

>

> has been seen by SMA specialists both at Stanford and Primary Children's

in Salt Lake City. Since almost all type 1 SMA kids die so young, there are few

examples to evaluate beyond 2 years of age. Those that do live are usually so

weak that they cannot tolerate any sitting, and are on their back 100% of the

time. They usually don't have problems with scoliosis. The type 2 kids are so

much stronger from a respiratory standpoint, that it is very hard to do a

comparison between what they would tolerate physically, and what could

handle.

>

> I appreciate all of your advice!

>

> For anyone else reading, how bad does 's back look to all of you? I have

nothing to compare it against, and with all of the other issues with his health

that keep me busy, his curve ends up further down on the priority list.

>

> Regards,

> Meri

>

>

>

>

> ________________________________

>

> To: infantile_scoliosis

> Sent: Thu, February 25, 2010 3:19:07 PM

> Subject: Meri- titanium rib

>

>  

> Hi Meri,

> Thanks for posting the pictures of your son. I have an old textbook about

congenital spinal conditions that has a picture of a child with SMA. There was a

mom who posted on veptr.com (the acronym for the titanium rib) whose son has

SMA, but I have not really been on the site too much since we decided not to do

the veptr for our daughter right now. There are other kids with the g tube or

mickey button (I don't really know the difference) on the site also.

> I think this mom was in a support group for the SMA. Are you able to find

other parents whose children also have it? I think she even went to a conference

about it at one time. Are some doctors more experienced with the SMA diagnosis?

I would imagine so.

>

>

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Guest guest

Hi Meri,

I am not familiar with SMA of either type, but I can see from your e-mail

thats its very serious. I am not aware of any children in the U.S. that

have experienced ET w/ EDF & the Mehta Method, but that doesnt mean

much.....Let me see what I can find out. Who knows, perhaps a proper

Mehta jacket could maintain his curve(s)and buy growth time...???? I

really dont know but will ask around and let you know what I find out. I

would definately recommend a consult with SLC Shriners.

Welcome to CAST.

HRH

> Hi ,

>

> Thanks for your comments. I will take a look at the veptr.com site.

>

> I know of many children with SMA (type 2, not type 1 like has) and

> all of them have had rods placed to manage their scoliosis. They return a

> few times a year to have the rods lengthened. I just wanted to see what

> other options are out there to try to prevent from getting any worse

> at this point, wanting to avoid the rod surgery in the future if at all

> possible.

>

> has been seen by SMA specialists both at Stanford and Primary

> Children's in Salt Lake City. Since almost all type 1 SMA kids die so

> young, there are few examples to evaluate beyond 2 years of age. Those

> that do live are usually so weak that they cannot tolerate any sitting,

> and are on their back 100% of the time. They usually don't have problems

> with scoliosis. The type 2 kids are so much stronger from a respiratory

> standpoint, that it is very hard to do a comparison between what they

> would tolerate physically, and what could handle.

>

> I appreciate all of your advice!

>

> For anyone else reading, how bad does 's back look to all of you? I

> have nothing to compare it against, and with all of the other issues with

> his health that keep me busy, his curve ends up further down on the

> priority list.

>

> Regards,

> Meri

>

>

>

>

> ________________________________

>

> To: infantile_scoliosis

> Sent: Thu, February 25, 2010 3:19:07 PM

> Subject: Meri- titanium rib

>

>  

> Hi Meri,

> Thanks for posting the pictures of your son. I have an old textbook about

> congenital spinal conditions that has a picture of a child with SMA. There

> was a mom who posted on veptr.com (the acronym for the titanium rib) whose

> son has SMA, but I have not really been on the site too much since we

> decided not to do the veptr for our daughter right now. There are other

> kids with the g tube or mickey button (I don't really know the difference)

> on the site also.

> I think this mom was in a support group for the SMA. Are you able to find

> other parents whose children also have it? I think she even went to a

> conference about it at one time. Are some doctors more experienced with

> the SMA diagnosis? I would imagine so.

>

>

>

>

Share this post


Link to post
Share on other sites
Guest guest

I looked at his photos and his curve(s) dont seem too bad (to the naked

eye). When you have a chance take a look at the photo section of CAST....

HRH

> Hi ,

>

> Thanks for your comments. I will take a look at the veptr.com site.

>

> I know of many children with SMA (type 2, not type 1 like has) and

> all of them have had rods placed to manage their scoliosis. They return a

> few times a year to have the rods lengthened. I just wanted to see what

> other options are out there to try to prevent from getting any worse

> at this point, wanting to avoid the rod surgery in the future if at all

> possible.

>

> has been seen by SMA specialists both at Stanford and Primary

> Children's in Salt Lake City. Since almost all type 1 SMA kids die so

> young, there are few examples to evaluate beyond 2 years of age. Those

> that do live are usually so weak that they cannot tolerate any sitting,

> and are on their back 100% of the time. They usually don't have problems

> with scoliosis. The type 2 kids are so much stronger from a respiratory

> standpoint, that it is very hard to do a comparison between what they

> would tolerate physically, and what could handle.

>

> I appreciate all of your advice!

>

> For anyone else reading, how bad does 's back look to all of you? I

> have nothing to compare it against, and with all of the other issues with

> his health that keep me busy, his curve ends up further down on the

> priority list.

>

> Regards,

> Meri

>

>

>

>

> ________________________________

>

> To: infantile_scoliosis

> Sent: Thu, February 25, 2010 3:19:07 PM

> Subject: Meri- titanium rib

>

>  

> Hi Meri,

> Thanks for posting the pictures of your son. I have an old textbook about

> congenital spinal conditions that has a picture of a child with SMA. There

> was a mom who posted on veptr.com (the acronym for the titanium rib) whose

> son has SMA, but I have not really been on the site too much since we

> decided not to do the veptr for our daughter right now. There are other

> kids with the g tube or mickey button (I don't really know the difference)

> on the site also.

> I think this mom was in a support group for the SMA. Are you able to find

> other parents whose children also have it? I think she even went to a

> conference about it at one time. Are some doctors more experienced with

> the SMA diagnosis? I would imagine so.

>

>

>

>

Share this post


Link to post
Share on other sites
Guest guest

I looked at his photos and his curve(s) dont seem too bad (to the naked

eye). When you have a chance take a look at the photo section of CAST....

HRH

> Hi ,

>

> Thanks for your comments. I will take a look at the veptr.com site.

>

> I know of many children with SMA (type 2, not type 1 like has) and

> all of them have had rods placed to manage their scoliosis. They return a

> few times a year to have the rods lengthened. I just wanted to see what

> other options are out there to try to prevent from getting any worse

> at this point, wanting to avoid the rod surgery in the future if at all

> possible.

>

> has been seen by SMA specialists both at Stanford and Primary

> Children's in Salt Lake City. Since almost all type 1 SMA kids die so

> young, there are few examples to evaluate beyond 2 years of age. Those

> that do live are usually so weak that they cannot tolerate any sitting,

> and are on their back 100% of the time. They usually don't have problems

> with scoliosis. The type 2 kids are so much stronger from a respiratory

> standpoint, that it is very hard to do a comparison between what they

> would tolerate physically, and what could handle.

>

> I appreciate all of your advice!

>

> For anyone else reading, how bad does 's back look to all of you? I

> have nothing to compare it against, and with all of the other issues with

> his health that keep me busy, his curve ends up further down on the

> priority list.

>

> Regards,

> Meri

>

>

>

>

> ________________________________

>

> To: infantile_scoliosis

> Sent: Thu, February 25, 2010 3:19:07 PM

> Subject: Meri- titanium rib

>

>  

> Hi Meri,

> Thanks for posting the pictures of your son. I have an old textbook about

> congenital spinal conditions that has a picture of a child with SMA. There

> was a mom who posted on veptr.com (the acronym for the titanium rib) whose

> son has SMA, but I have not really been on the site too much since we

> decided not to do the veptr for our daughter right now. There are other

> kids with the g tube or mickey button (I don't really know the difference)

> on the site also.

> I think this mom was in a support group for the SMA. Are you able to find

> other parents whose children also have it? I think she even went to a

> conference about it at one time. Are some doctors more experienced with

> the SMA diagnosis? I would imagine so.

>

>

>

>

Share this post


Link to post
Share on other sites
Guest guest

I looked at his photos and his curve(s) dont seem too bad (to the naked

eye). When you have a chance take a look at the photo section of CAST....

HRH

> Hi ,

>

> Thanks for your comments. I will take a look at the veptr.com site.

>

> I know of many children with SMA (type 2, not type 1 like has) and

> all of them have had rods placed to manage their scoliosis. They return a

> few times a year to have the rods lengthened. I just wanted to see what

> other options are out there to try to prevent from getting any worse

> at this point, wanting to avoid the rod surgery in the future if at all

> possible.

>

> has been seen by SMA specialists both at Stanford and Primary

> Children's in Salt Lake City. Since almost all type 1 SMA kids die so

> young, there are few examples to evaluate beyond 2 years of age. Those

> that do live are usually so weak that they cannot tolerate any sitting,

> and are on their back 100% of the time. They usually don't have problems

> with scoliosis. The type 2 kids are so much stronger from a respiratory

> standpoint, that it is very hard to do a comparison between what they

> would tolerate physically, and what could handle.

>

> I appreciate all of your advice!

>

> For anyone else reading, how bad does 's back look to all of you? I

> have nothing to compare it against, and with all of the other issues with

> his health that keep me busy, his curve ends up further down on the

> priority list.

>

> Regards,

> Meri

>

>

>

>

> ________________________________

>

> To: infantile_scoliosis

> Sent: Thu, February 25, 2010 3:19:07 PM

> Subject: Meri- titanium rib

>

>  

> Hi Meri,

> Thanks for posting the pictures of your son. I have an old textbook about

> congenital spinal conditions that has a picture of a child with SMA. There

> was a mom who posted on veptr.com (the acronym for the titanium rib) whose

> son has SMA, but I have not really been on the site too much since we

> decided not to do the veptr for our daughter right now. There are other

> kids with the g tube or mickey button (I don't really know the difference)

> on the site also.

> I think this mom was in a support group for the SMA. Are you able to find

> other parents whose children also have it? I think she even went to a

> conference about it at one time. Are some doctors more experienced with

> the SMA diagnosis? I would imagine so.

>

>

>

>

Share this post


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Share on other sites
Guest guest

Meri,

I am in San right now with my daughter Moriah. Can you email me what SMA stands for and I can try and ask their office if they have ever done a VEPTR on a patient with that diagnosis. The VEPTR was invented here and they have a large range of kids they have seen.

shellie

Subject: Re: Meri- titanium ribTo: infantile_scoliosis Date: Thursday, February 25, 2010, 10:56 PM

Hi ,

Thanks for your comments. I will take a look at the veptr.com site.

I know of many children with SMA (type 2, not type 1 like has) and all of them have had rods placed to manage their scoliosis. They return a few times a year to have the rods lengthened. I just wanted to see what other options are out there to try to prevent from getting any worse at this point, wanting to avoid the rod surgery in the future if at all possible.

has been seen by SMA specialists both at Stanford and Primary Children's in Salt Lake City. Since almost all type 1 SMA kids die so young, there are few examples to evaluate beyond 2 years of age. Those that do live are usually so weak that they cannot tolerate any sitting, and are on their back 100% of the time. They usually don't have problems with scoliosis. The type 2 kids are so much stronger from a respiratory standpoint, that it is very hard to do a comparison between what they would tolerate physically, and what could handle.

I appreciate all of your advice!

For anyone else reading, how bad does 's back look to all of you? I have nothing to compare it against, and with all of the other issues with his health that keep me busy, his curve ends up further down on the priority list.

Regards,Meri

From: ansiosamjm <hemiverthotmail (DOT) com>To: infantile_scoliosis @yahoogroups. comSent: Thu, February 25, 2010 3:19:07 PMSubject: [infantile_scoliosi s] Meri- titanium rib

Hi Meri,Thanks for posting the pictures of your son. I have an old textbook about congenital spinal conditions that has a picture of a child with SMA. There was a mom who posted on veptr.com (the acronym for the titanium rib) whose son has SMA, but I have not really been on the site too much since we decided not to do the veptr for our daughter right now. There are other kids with the g tube or mickey button (I don't really know the difference) on the site also. I think this mom was in a support group for the SMA. Are you able to find other parents whose children also have it? I think she even went to a conference about it at one time. Are some doctors more experienced with the SMA diagnosis? I would imagine so.

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