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Re: [Myotubular_Myopathy] Briar's Procedure/Lack of Vocal Cords

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  • ANN MILLER
    Hi Stacey, Kyle will be 4 years old in December. Mimicing is good, even if it s just some of the time. He will get better as he gets older. Kyle loves to
    Message 1 of 8 , Sep 10 4:47 AM
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      Hi Stacey,
      Kyle will be 4 years old in December.  Mimicing is good, even if it's just some of the time.  He will get better as he gets older.  Kyle loves to mimic his favorite P/T.  She can get him to do anything.   Don't worry about Briar not doing the signs the right way.  Kyle has trouble doing most of them exactly right because his fingers don't always do what he wants.  But people do understand what he is saying.  That
      makes him happy.  Sometimes, I have to say , do that sign again, before I know what he wants.  I've found that I do signing alot while I'm talking even to someone who doesn't know what I'm doing.  Kyle picked up on it and will sign back.  Kyle has even made a few signs of his own.  For example, he likes Scooby Doo, he will touch is nose (scooby has a big black nose) then signs dog.  Everyone he is around alot  knows what he wants.  He calls my husband (his grandfather) dad, but when he wants his father he signs, me then dad.  Just keep showing Briar the signs, he will pick up on it and start using it when he is ready.  All kids have milestones at different times. 
       
      Kyle takes 3 robinul pills a day.  We had better results with the pill then the liquid.  I don't know what Briar is taking, but that may be a thought.  The passey-muir valve also helped with decreasing the secretions. 
       
      Kyle has only had one MRI, the doctor never said anything was different or wrong.  He just said there
      was nothing to worry about.  The main thing with these boys are to keep them healthy.  Find out what their strong points and weak points are then work from there.  We just try to keep Kyle's life as normal as possible, and let him go from there as to what he can and can't do.  He will try everything the other kids do.  If he likes it he wants to do it again, if not he will sign finished.  If I know he is able to do something I will push him to keep trying. 
       
      Kyle has P/T and O/T 4 times a week.  He can now be put in the crawl position and hold himself up for
      4 minutes, his biggest problem is holding is head up.  He still has a problem with holding his head up when in a position other than sitting or standing.
       
      Take care,
      Darlene
       
       
       
      ----- Original Message -----
      Sent: Friday, September 09, 2005 9:00 PM
      Subject: Re: [Myotubular_Myopathy] Briar's Procedure/Lack of Vocal Cords

      Hello, thanks for the reply.  I have actually seen the photos from his procedure and Briar definately doesn't have vocal cords.  As for the recommended procedure I am not ready to make a decision without opinions from people like you and I will also seek opinions from Briar's other doctors.  He is being followed by several whose advice I value greatly.  As for the Robinul, Briar is on it but unfortunately we have not noticed a decrease at all in his secretions.
       
      How old is Kyle?  Briar's development is way behind and I think that may have something to do with his "unwillingness" to do any signing.  He mimics some things that we do but so far Mommy and Daddy are the only things he has signed and he doesn't do those exactly right and not on a regular basis.  Briar has had MRIs done that show his brain isn't as large as it should be and his previous neurologist had said that she wasn't sure if he would develop as quickly or how intelligent he would be.  We do not see her anymore for many reasons but mostly because she was one of the most negative and tactless human beings I have ever met in my life.  We haven't had the MRI repeated since he was about 9 months old and haven't seeked to get any second opinions because we are just blessed that he is alive and well and to us that is so much more than enough.  Has anyone else had MRIs that have shown a smaller brain than should be expected?
       
      Thank you for your response.
       
      Stacey 

      ANN MILLER <dm4462@...> wrote:
      Hi Stacey,
       
      I would get a second opinion.  Kyle never had a problem with his
      vocal cords.  The last bronchoscopy and larynoscopy he had
      done did not show anything wrong.  He is starting to make more
      sounds and trying to say more words everyday.  HE does wear
      a passey-muir valve, which helps.  His ENT says his biggest
      problem is the trach size.  He wants to try and down size Kyle's
      again.  He says the bigger the trach the less air gets around the
      vocal cords. 
       
      As far as signing.  Kyle does very well he knows around 150 signs. 
      We just sat him in front of the VCR and let him watch Signing Time
      over and over.  He started picking them up and using them on his
      own.  Now we sit down everyother day for about 1/2 hour and
      learn a new sign.   He wants to sign all the time now. 
       
      As far as secretions,  Is Briar on robinul or anything for drying
      up secretions.  Kyle did have a lot of secretions when he was
      younger and still now when he is sick.  The robinul helps alot.
      He is also on allergy medicines.  We think his allergies had 
      something to do with all the secretions.  We do exercises with
      his mouth and tongue to help with the secretions too.  Since
      he started doing this there is a big difference.
       
      Hope this is some help.  Well keep Briar in our prayers.
       
      Take care,
      Darlene 
      ----- Original Message -----
      Sent: Friday, September 09, 2005 10:00 AM
      Subject: [Myotubular_Myopathy] Briar's Procedure/Lack of Vocal Cords

      Hello all, Briar had a procedure Sept. 7th.  He had tubes placed in
      his ears along with a rigid bronchoscopy and direct larynoscopy. 
      The purpose of the latter two were because the EENT suspected that
      Briar had tissue build up around his vocal box that prevented him
      from vocalizing.  We have heard little noises from Briar on
      occasion, most of them when is on his vent and a couple of times
      during trach changes.  Briar's doctor felt this was due to the
      tissue and upon removal we were going to start working with Briar
      and a paci-muir (I think that's how it is spelled).  Well, Briar did
      have the tissue build-up and it was removed, however, the discovered
      that Briar's vocal cords never developed.  The voice box can
      generate noise but without the vocal cords it is unclear at this
      time what the clarity of Briar's voice will be or if he will only be
      able to cry, laugh, make noises or if the little growls and faint
      crys we've already heard will be more of what we can expect.  Upon
      asking Briar's doc about the lack of development, he told us that it
      was very rare, someting he had hardly ever seen and said it was
      possibly due to MTM.  Briar's EENT is not by any means familiar with
      MTM and so I ask all of you....have any of you heard of the lack of
      development of vocal cords being associated with MTM?

      Also, one of Briar's doctors had suggested at one time the
      possibility of closing off Briar's vocal box completely to prevent
      aspiration so that the amount of suctioning would be reduced when
      Briar is in an upright position.  Briar is unable to swallow
      effectively and therefore any time he is up, he requires almost
      constant suctioning.  At first, the idea was shot down, after all, I
      want to hear my baby's voice. I told his doc that if we had to start
      fighting fluid build-up in Briar's lungs and pneumonias due to
      chronic aspiration, then at that point, I would reconsider the
      possibilty.  However, now I have to rethink the pros and cons. If
      Briar will not be able to vocalize to communicate with us, the
      procedure doesn't sound like such a bad idea.  We can always teach
      Briar to sign (although, we've already tried to start with a little
      and he isn't cooperating). Anyway, I'd love any comments that you
      all may have in regards to this procedure.  How may of you have
      heard of it, and how many of you have considered it or had it done?

      Thanks for your time, Stacey




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    • connie guinn
      Stacey, I m sorry to hear that Briar s vocal cords have not developed. I have never heard of that being associated with MTM. I ve read a lot of articles on
      Message 2 of 8 , Sep 12 12:08 PM
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        Stacey,
        I'm sorry to hear that Briar's vocal cords have not developed.    I have never heard of that being associated with MTM.  I've read a lot of articles on MTM, and know that a lot of things are suspected as being linked to it, but I have not run across anything about vocal cords and MTM.
        As  far as advice on whether or not to do the surgery, here is my input:  
        Briar is making some sounds, and they don't really know what to expect in the future regarding his voice- that is very promising!   You are able to hear your son's voice-what a blessing!   It is very hard to not be able to hear their voice anymore (take, for instance, Shannon's recent post about replacing Javad's cuffed trach with a non-cuffed trach,  and being grateful to hear his voice once again!)   Another thing to consider is that it could be difficult for you to live with the fact that you made the decision to not hear your son's voice again.   As far as the secretions go, all of the boys have increased secretions when they are sitting up.   What I know from reading and from talking to others (i.e.the mothers of the boys) is that the oral secretions get better with time.   I remember when Benjamin was younger, he had a horrible amount of secretions.   We tried Robinul on several different occasions and found that he had just as many oral secretions as before, but the medicine dried out his lungs way too much.   Using a passey-muir valve does help a lot, too.   These days, [by the way, Benjamin turned 3 today- Happy Birthday, Benjamin!]  Benjamin has much better control of his secretions- way more than when he was younger.  He still has a way to go in this area, but I have definitely seen major improvements over the years.  Besides, they are likely to always need to be suctioned, right?  Give it some time with Briar.   I definitely would think twice before rushing into any decision.   Give him a hug for me!  Godspeed,
        -Connie 

        briars_mom <briars_mom@...> wrote:
        Hello all, Briar had a procedure Sept. 7th.  He had tubes placed in
        his ears along with a rigid bronchoscopy and direct larynoscopy. 
        The purpose of the latter two were because the EENT suspected that
        Briar had tissue build up around his vocal box that prevented him
        from vocalizing.  We have heard little noises from Briar on
        occasion, most of them when is on his vent and a couple of times
        during trach changes.  Briar's doctor felt this was due to the
        tissue and upon removal we were going to start working with Briar
        and a paci-muir (I think that's how it is spelled).  Well, Briar did
        have the tissue build-up and it was removed, however, the discovered
        that Briar's vocal cords never developed.  The voice box can
        generate noise but without the vocal cords it is unclear at this
        time what the clarity of Briar's voice will be or if he will only be
        able to cry, laugh, make noises or if the little growls and faint
        crys we've already heard will be more of what we can expect.  Upon
        asking Briar's doc about the lack of development, he told us that it
        was very rare, someting he had hardly ever seen and said it was
        possibly due to MTM.  Briar's EENT is not by any means familiar with
        MTM and so I ask all of you....have any of you heard of the lack of
        development of vocal cords being associated with MTM?

        Also, one of Briar's doctors had suggested at one time the
        possibility of closing off Briar's vocal box completely to prevent
        aspiration so that the amount of suctioning would be reduced when
        Briar is in an upright position.  Briar is unable to swallow
        effectively and therefore any time he is up, he requires almost
        constant suctioning.  At first, the idea was shot down, after all, I
        want to hear my baby's voice. I told his doc that if we had to start
        fighting fluid build-up in Briar's lungs and pneumonias due to
        chronic aspiration, then at that point, I would reconsider the
        possibilty.  However, now I have to rethink the pros and cons. If
        Briar will not be able to vocalize to communicate with us, the
        procedure doesn't sound like such a bad idea.  We can always teach
        Briar to sign (although, we've already tried to start with a little
        and he isn't cooperating). Anyway, I'd love any comments that you
        all may have in regards to this procedure.  How may of you have
        heard of it, and how many of you have considered it or had it done?

        Thanks for your time, Stacey



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      • connie guinn
        Stacey, As far as signing goes, Briar may just need some time with that also. Benjamin knows some signs and can sign them well, but he doesn t want to do it-
        Message 3 of 8 , Sep 12 12:18 PM
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          Stacey,
          As far as signing goes, Briar may just need some time with that also.  Benjamin knows some signs and can sign them well, but he doesn't want to do it- he is very resistant to it.  I know there are several of the boys who are this way, too.  Stubborn, is what I call it!!  Benjamin will only do the signs when we MAKE him, but, even still, he asserts a little passive-aggressiveness by making some sort of silliness in connection with the sign.  Then, he laughs to divert the attention away from the sign and more onto himself (of course, his accomplices are mommy and all the nurses who then go on and on about how clever he is!).   He'll get there- in his own time, not mine!  I'm just determined not to stress about it.  I hope this helps.   Godspeed,
          -Connie

          ANN MILLER <dm4462@...> wrote:
          Hi Stacey,
           
          I would get a second opinion.  Kyle never had a problem with his
          vocal cords.  The last bronchoscopy and larynoscopy he had
          done did not show anything wrong.  He is starting to make more
          sounds and trying to say more words everyday.  HE does wear
          a passey-muir valve, which helps.  His ENT says his biggest
          problem is the trach size.  He wants to try and down size Kyle's
          again.  He says the bigger the trach the less air gets around the
          vocal cords. 
           
          As far as signing.  Kyle does very well he knows around 150 signs. 
          We just sat him in front of the VCR and let him watch Signing Time
          over and over.  He started picking them up and using them on his
          own.  Now we sit down everyother day for about 1/2 hour and
          learn a new sign.   He wants to sign all the time now. 
           
          As far as secretions,  Is Briar on robinul or anything for drying
          up secretions.  Kyle did have a lot of secretions when he was
          younger and still now when he is sick.  The robinul helps alot.
          He is also on allergy medicines.  We think his allergies had 
          something to do with all the secretions.  We do exercises with
          his mouth and tongue to help with the secretions too.  Since
          he started doing this there is a big difference.
           
          Hope this is some help.  Well keep Briar in our prayers.
           
          Take care,
          Darlene 
          ----- Original Message -----
          Sent: Friday, September 09, 2005 10:00 AM
          Subject: [Myotubular_Myopathy] Briar's Procedure/Lack of Vocal Cords

          Hello all, Briar had a procedure Sept. 7th.  He had tubes placed in
          his ears along with a rigid bronchoscopy and direct larynoscopy. 
          The purpose of the latter two were because the EENT suspected that
          Briar had tissue build up around his vocal box that prevented him
          from vocalizing.  We have heard little noises from Briar on
          occasion, most of them when is on his vent and a couple of times
          during trach changes.  Briar's doctor felt this was due to the
          tissue and upon removal we were going to start working with Briar
          and a paci-muir (I think that's how it is spelled).  Well, Briar did
          have the tissue build-up and it was removed, however, the discovered
          that Briar's vocal cords never developed.  The voice box can
          generate noise but without the vocal cords it is unclear at this
          time what the clarity of Briar's voice will be or if he will only be
          able to cry, laugh, make noises or if the little growls and faint
          crys we've already heard will be more of what we can expect.  Upon
          asking Briar's doc about the lack of development, he told us that it
          was very rare, someting he had hardly ever seen and said it was
          possibly due to MTM.  Briar's EENT is not by any means familiar with
          MTM and so I ask all of you....have any of you heard of the lack of
          development of vocal cords being associated with MTM?

          Also, one of Briar's doctors had suggested at one time the
          possibility of closing off Briar's vocal box completely to prevent
          aspiration so that the amount of suctioning would be reduced when
          Briar is in an upright position.  Briar is unable to swallow
          effectively and therefore any time he is up, he requires almost
          constant suctioning.  At first, the idea was shot down, after all, I
          want to hear my baby's voice. I told his doc that if we had to start
          fighting fluid build-up in Briar's lungs and pneumonias due to
          chronic aspiration, then at that point, I would reconsider the
          possibilty.  However, now I have to rethink the pros and cons. If
          Briar will not be able to vocalize to communicate with us, the
          procedure doesn't sound like such a bad idea.  We can always teach
          Briar to sign (although, we've already tried to start with a little
          and he isn't cooperating). Anyway, I'd love any comments that you
          all may have in regards to this procedure.  How may of you have
          heard of it, and how many of you have considered it or had it done?

          Thanks for your time, Stacey




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        • kristin cypher
          Hi Stacey! I think that everyone has given you excellent advice in this area, but I thought I would share my experience, too. When Sebi was born, he wasn t
          Message 4 of 8 , Sep 12 9:51 PM
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            Hi Stacey!
             
            I think that everyone has given you excellent advice in this area, but I thought I would share my experience, too.
             
            When Sebi was born, he wasn't able to make any sounds, and a bronchoscopy (sorry about the spelling) showed that only one of his vocal chords had 'developed'.  At the time, not much was made of this.  We were basically told that he probably wouldn't be able to speak, and that we should count on learning/using sign language with Seb.  And for several months, we didn't hear a peep from him.  But then, right around his 4 month birthday, he began to make weak sounds when crying or really happy.  When we brought this to the attention of the doctors, they said that we should still not keep our hopes up, because when he was born, it looked like he didn't even HAVE one of his vocal chords.  Well, Sebi is now 2 1/2, and the doctors were so, so wrong!!  He now talks constantly, about everything, and has more than made up for his vocal delays.  We did do sign language with him 'till he was about 2, but after this time, he was just talking so much that he began to drop using the sings, and we did, too.
             
            I guess my point is:  Don't always listen to the doctors, because in many cases, they have very low expectations for our boys.  Sebi has a mild-er form of MTM, so his progress has been more rapid, but I still think that in time Briar may surprise everyone.
             
            Just my 2 cents!!  I know that you love him and any decision you make will reflect the desire to do what you think is best.
             
            Warmly,
            Kristin

            connie guinn <kookla_g@...> wrote:
            Stacey,
            I'm sorry to hear that Briar's vocal cords have not developed.    I have never heard of that being associated with MTM.  I've read a lot of articles on MTM, and know that a lot of things are suspected as being linked to it, but I have not run across anything about vocal cords and MTM.
            As  far as advice on whether or not to do the surgery, here is my input:  
            Briar is making some sounds, and they don't really know what to expect in the future regarding his voice- that is very promising!   You are able to hear your son's voice-what a blessing!   It is very hard to not be able to hear their voice anymore (take, for instance, Shannon's recent post about replacing Javad's cuffed trach with a non-cuffed trach,  and being grateful to hear his voice once again!)   Another thing to consider is that it could be difficult for you to live with the fact that you made the decision to not hear your son's voice again.   As far as the secretions go, all of the boys have increased secretions when they are sitting up.   What I know from reading and from talking to others (i.e.the mothers of the boys) is that the oral secretions get better with time.   I remember when Benjamin was younger, he had a horrible amount of secretions.   We tried Robinul on several different occasions and found that he had just as many oral secretions as before, but the medicine dried out his lungs way too much.   Using a passey-muir valve does help a lot, too.   These days, [by the way, Benjamin turned 3 today- Happy Birthday, Benjamin!]  Benjamin has much better control of his secretions- way more than when he was younger.  He still has a way to go in this area, but I have definitely seen major improvements over the years.  Besides, they are likely to always need to be suctioned, right?  Give it some time with Briar.   I definitely would think twice before rushing into any decision.   Give him a hug for me!  Godspeed,
            -Connie 

            briars_mom <briars_mom@...> wrote:
            Hello all, Briar had a procedure Sept. 7th.  He had tubes placed in
            his ears along with a rigid bronchoscopy and direct larynoscopy. 
            The purpose of the latter two were because the EENT suspected that
            Briar had tissue build up around his vocal box that prevented him
            from vocalizing.  We have heard little noises from Briar on
            occasion, most of them when is on his vent and a couple of times
            during trach changes.  Briar's doctor felt this was due to the
            tissue and upon removal we were going to start working with Briar
            and a paci-muir (I think that's how it is spelled).  Well, Briar did
            have the tissue build-up and it was removed, however, the discovered
            that Briar's vocal cords never developed.  The voice box can
            generate noise but without the vocal cords it is unclear at this
            time what the clarity of Briar's voice will be or if he will only be
            able to cry, laugh, make noises or if the little growls and faint
            crys we've already heard will be more of what we can expect.  Upon
            asking Briar's doc about the lack of development, he told us that it
            was very rare, someting he had hardly ever seen and said it was
            possibly due to MTM.  Briar's EENT is not by any means familiar with
            MTM and so I ask all of you....have any of you heard of the lack of
            development of vocal cords being associated with MTM?

            Also, one of Briar's doctors had suggested at one time the
            possibility of closing off Briar's vocal box completely to prevent
            aspiration so that the amount of suctioning would be reduced when
            Briar is in an upright position.  Briar is unable to swallow
            effectively and therefore any time he is up, he requires almost
            constant suctioning.  At first, the idea was shot down, after all, I
            want to hear my baby's voice. I told his doc that if we had to start
            fighting fluid build-up in Briar's lungs and pneumonias due to
            chronic aspiration, then at that point, I would reconsider the
            possibilty.  However, now I have to rethink the pros and cons. If
            Briar will not be able to vocalize to communicate with us, the
            procedure doesn't sound like such a bad idea.  We can always teach
            Briar to sign (although, we've already tried to start with a little
            and he isn't cooperating). Anyway, I'd love any comments that you
            all may have in regards to this procedure.  How may of you have
            heard of it, and how many of you have considered it or had it done?

            Thanks for your time, Stacey



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          • connie guinn
            Stacey, Hello again! Benjamin had a CT scan of his head (first time ever) in April of this year. The ER doctor said that the scan showed brain
            Message 5 of 8 , Sep 14 10:38 AM
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              Stacey,
              Hello again! 
              Benjamin had a CT scan of his head (first time ever) in April of this year.  The ER doctor said that the scan showed 'brain shrinkage'(hydroma). 
              At follow-ups with our neuro   (we love this man! he is so sweet- yes, I said "sweet" and "neuro" in the same sentence!),    we came to understand that Benjamin's brain is normal sized- it's his head that is not.  Because their heads grow at such accelerated speeds after birth, their body can't always keep up with it.  So, he has a normal (age-appropriate) sized brain in an oversized skull. 
              To compensate, the body cushions the 'space' that is left between the brain tissue and the skull with extra fluid- this prevents constant contusions and concussions from occurring- it sort of 'cushions' the brain from slapping around inside the skull.  All human bodies do this to protect the brain, but most people don't have such a big head to fill!! 
              Benjamin has been noted by several of his doctors to be on track mentally (for his age).   And we can all testify to how clever he is!!  
              I was talking to Benjamin's geneticist about this.  She said that a lot of times, kids who have dissabilities like these DO have a smaller brain, and that it is considered normal for kids with these problems to have smaller brains.  So, she said that a lot of the other families that I know may not know that the same thing is going on with their kid because the report comes back to them as being "normal" and what the doctor really means is "normal for him" (but compared to a healthy child, the scan would be considered abnormal).  Am I making any sense?  
              Also, Benjamin's pediatrician offered that if there really are parts of his brain that are shrinking, it may have to do with areas that he doesn't use (like the muscular use of and sensations of: running, jumping, climbing, flying a kite, catching a butterfly, etc). 
              We are going to be assessing this periodically in the future with repeat CT/MRI scans- then, we will know a little more about what is actually going on in this area (since we will have something to compare future results with).  
              I'm sorry I have to run.  Benjamin is coming home on the school bus and I have to meet him!!  Godspeed,
              -Connie
               
               

              Stacey Cummins <briars_mom@...> wrote:
              Hello, thanks for the reply.  I have actually seen the photos from his procedure and Briar definately doesn't have vocal cords.  As for the recommended procedure I am not ready to make a decision without opinions from people like you and I will also seek opinions from Briar's other doctors.  He is being followed by several whose advice I value greatly.  As for the Robinul, Briar is on it but unfortunately we have not noticed a decrease at all in his secretions.
               
              How old is Kyle?  Briar's development is way behind and I think that may have something to do with his "unwillingness" to do any signing.  He mimics some things that we do but so far Mommy and Daddy are the only things he has signed and he doesn't do those exactly right and not on a regular basis.  Briar has had MRIs done that show his brain isn't as large as it should be and his previous neurologist had said that she wasn't sure if he would develop as quickly or how intelligent he would be.  We do not see her anymore for many reasons but mostly because she was one of the most negative and tactless human beings I have ever met in my life.  We haven't had the MRI repeated since he was about 9 months old and haven't seeked to get any second opinions because we are just blessed that he is alive and well and to us that is so much more than enough.  Has anyone else had MRIs that have shown a smaller brain than should be expected?
               
              Thank you for your response.
               
              Stacey 

              ANN MILLER <dm4462@...> wrote:
              Hi Stacey,
               
              I would get a second opinion.  Kyle never had a problem with his
              vocal cords.  The last bronchoscopy and larynoscopy he had
              done did not show anything wrong.  He is starting to make more
              sounds and trying to say more words everyday.  HE does wear
              a passey-muir valve, which helps.  His ENT says his biggest
              problem is the trach size.  He wants to try and down size Kyle's
              again.  He says the bigger the trach the less air gets around the
              vocal cords. 
               
              As far as signing.  Kyle does very well he knows around 150 signs. 
              We just sat him in front of the VCR and let him watch Signing Time
              over and over.  He started picking them up and using them on his
              own.  Now we sit down everyother day for about 1/2 hour and
              learn a new sign.   He wants to sign all the time now. 
               
              As far as secretions,  Is Briar on robinul or anything for drying
              up secretions.  Kyle did have a lot of secretions when he was
              younger and still now when he is sick.  The robinul helps alot.
              He is also on allergy medicines.  We think his allergies had 
              something to do with all the secretions.  We do exercises with
              his mouth and tongue to help with the secretions too.  Since
              he started doing this there is a big difference.
               
              Hope this is some help.  Well keep Briar in our prayers.
               
              Take care,
              Darlene 
              ----- Original Message -----
              Sent: Friday, September 09, 2005 10:00 AM
              Subject: [Myotubular_Myopathy] Briar's Procedure/Lack of Vocal Cords

              Hello all, Briar had a procedure Sept. 7th.  He had tubes placed in
              his ears along with a rigid bronchoscopy and direct larynoscopy. 
              The purpose of the latter two were because the EENT suspected that
              Briar had tissue build up around his vocal box that prevented him
              from vocalizing.  We have heard little noises from Briar on
              occasion, most of them when is on his vent and a couple of times
              during trach changes.  Briar's doctor felt this was due to the
              tissue and upon removal we were going to start working with Briar
              and a paci-muir (I think that's how it is spelled).  Well, Briar did
              have the tissue build-up and it was removed, however, the discovered
              that Briar's vocal cords never developed.  The voice box can
              generate noise but without the vocal cords it is unclear at this
              time what the clarity of Briar's voice will be or if he will only be
              able to cry, laugh, make noises or if the little growls and faint
              crys we've already heard will be more of what we can expect.  Upon
              asking Briar's doc about the lack of development, he told us that it
              was very rare, someting he had hardly ever seen and said it was
              possibly due to MTM.  Briar's EENT is not by any means familiar with
              MTM and so I ask all of you....have any of you heard of the lack of
              development of vocal cords being associated with MTM?

              Also, one of Briar's doctors had suggested at one time the
              possibility of closing off Briar's vocal box completely to prevent
              aspiration so that the amount of suctioning would be reduced when
              Briar is in an upright position.  Briar is unable to swallow
              effectively and therefore any time he is up, he requires almost
              constant suctioning.  At first, the idea was shot down, after all, I
              want to hear my baby's voice. I told his doc that if we had to start
              fighting fluid build-up in Briar's lungs and pneumonias due to
              chronic aspiration, then at that point, I would reconsider the
              possibilty.  However, now I have to rethink the pros and cons. If
              Briar will not be able to vocalize to communicate with us, the
              procedure doesn't sound like such a bad idea.  We can always teach
              Briar to sign (although, we've already tried to start with a little
              and he isn't cooperating). Anyway, I'd love any comments that you
              all may have in regards to this procedure.  How may of you have
              heard of it, and how many of you have considered it or had it done?

              Thanks for your time, Stacey




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