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In a message dated 1/14/01 5:59:44 PM Eastern Standard Time, lls@...

writes:

<< In response to your question about heredity, males are not carriers, for

any known type >>

Lamar,

I must have missed something, what does the above refer to?

E

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Judy,

Lamar here,

I have occasional painful cramps that will draw a muscle up. They were bad

in my legs after exertion, but as my CMT progressed they disappeared. My

neurologist said that the reason was the nerve damage was now so extensive

that the signals were no longer there. I now have them in my hands after

long use in things such as painting, hammering, or driving. My fingers will

draw up in what is known as a " trigger finger " . Manually pulling them out

by stretching will usually stop it.

I have had general anesthesia 8 times and have noticed a rapid progression

of my CMT after each time. Many CMT'ers have reported this, and others have

seen no change. On my last anesthesia, I did have more problems waking up

and my oxygen level was lower than ideal for hours, but not at a dangerous

point. CMTI has a good publication on this. Things to remember are:

1: Always let the anesthesiologist know you have CMT and make sure he knows

what it is.

2. He should monitor both Carbon dioxide and Oxygen levels.

3. He should use as light a level of anesthesia and for as short a time as

possible

4. He should be cautious about the use of muscle relaxants because they

tend to have a more intense and longer reaction with individuals with

neuromuscular diseases. The one named SUXAMETHONIUM should be avoided as it

can cause an increase in potassium ions leading to possible cardiac failure

in a person with neuromuscular disease. (You can just say the muscle

relaxant SUX, and he will know what it is)

5. Keep warm during and after anesthesia, as people with neuromuscular

diseases tend to be more likely to develop a condition called " malignant

hypothermia " where the body temp drops to a very low and potentially

dangerous level.

In response to your question about heredity, males are not carriers, for

any known type, with the exception of some very rare types that are

autosomally recessive. In those both the mother and father must carry the

recessive gene to have a child with it. In X linked CMT a female can be a

carrier, but a male can not be. The male either has CMT or does not. If

the male has X linked CMT he will have carrier daughters but his sons will

not have CMT/ In the most common heredity pattern in CMT (autosomally

dominant), each child without regard to sex will have a 50% chance of having

the gene--and again they will either have or not have CMT. It is very

possible that some individuals have CMT and do not show symptoms. I have

met people that had essentially no symptoms until in their 70's. I showed

almost none until I was in my mid 40's. If your brother has children with

the same type of CMT that you have, he would have to have an almost 100%

probability of having CMT, but may show virtually no symptoms at this point

in life.

The same thing happened in my family. A great grandparent passed CMT on.

Two daughters had it and passed it on. One son did not show symptoms, BUT

he died in an accident in his 40's, and he had children and grandchildren

with CTM. Therefore he would have HAD to have CMT, and likely would have

shown symptoms if he had lived longer.

As to your brother, most prostrate surgery can be done with spinal

anesthesia which some consider to be a safer alternative if he has CMT.

I am currently working on a web page that will show diagrams of the various

heredity patterns found in CMT and hopefully it will be easier to visualize.

It is somewhat difficult to explain or understand without a diagram.

When completed, I hope that it will help you and others.

----- Original Message -----

From: john dawson

egroups

Sent: Sunday, January 14, 2001 12:13 PM

Subject: [] misc

Gretchen,

I think it was you talking about your body " locking " up. When I sleep my

arm

is under my pillow. When I awake I must pick up my arm with the opposite

hand. The part that hurts and can't move is the upper arm between shoulder

and elbow. Is this what you meant by locking up?

Anesthetic...What is the problem here? I once had thyroid surgery and

spent

an extra four hours in recovery unable to be awakened. When I did wake up

it

felt like the elephants had just left my chest.. I'm concerned as my

brother(skinny legs) but no other major problems is having surgery. He

had

seeds implanted for cancer of the prostate and they failed. Now he is

facing

major surgery. His son is the one of my family that has the CMT symptoms .

If my brother doesn't have CMT, is he a carrier being his son has it?

Would

a carrier be in just as much jeopardy.

I haven't received my packets yet from CMTI and CMTA that's why I'm asking

the experts.

I had mentioned that the past years progression of symptoms is what made

me

look again for answers. Since my bx in December my symptoms have really

progressed. My right leg has been most affected. Now my left feels very

weak

at the ankle. the right side of my torso(back) feels like it freezes up

upon

rising from a sitting position,(need to sit and try again) face and neck

twitches, swallowing has been a problem for awhile. Vision has gotten way

worse.Hands aren't affected but finger dexterity is awful. Is it possible

for these things to go into remission or slow down or do I need to start

drawing up plans for my ranch house with wide hallways quick? I know

everyone is different but what's the general consensus on this? I wonder

too

how the fatigue factor enters into this. Surgery, bad chemical burn on my

leg from something sprayed on during surgery, Holidays, back to work,

admittedly I'm tired. Also have a husband who had a brain injury and when

things get stressful his Obsessive compulsive behaviors get worse. To add

to

the stress. Before Christmas he had shopping money on him. He went to a

diner for lunch-long story short-he paid a waitress's electric bill $380.

It

was to be turned off the next day. Fortunately he went to the electric co.

and paid it but it sure wasn't in my budget.

Sorry this is so long, but just needing some answers.

Judi

eGroups Sponsor

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,

It was in response to the question Judi asked and was on the bottom of my

post, which was:

" I'm concerned as my

brother(skinny legs) but no other major problems is having surgery. He

had

seeds implanted for cancer of the prostate and they failed. Now he is

facing

major surgery. His son is the one of my family that has the CMT symptoms .

If my brother doesn't have CMT, is he a carrier being his son has it?

Would

a carrier be in just as much jeopardy. "

From: jege6666@...

egroups

Sent: Sunday, January 14, 2001 07:28 PM

Subject: Re: [] misc

In a message dated 1/14/01 5:59:44 PM Eastern Standard Time, lls@...

writes:

<< In response to your question about heredity, males are not carriers,

for

any known type >>

Lamar,

I must have missed something, what does the above refer to?

E

eGroups Sponsor

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In a message dated 1/14/2001 7:33:30 PM Eastern Standard Time,

egroups writes:

<< 5. Keep warm during and after anesthesia, as people with neuromuscular

diseases tend to be more likely to develop a condition called " malignant

hypothermia " where the body temp drops to a very low and potentially

dangerous level. >>

I wanted to tell you guys that this almost happened to me. After one of my

surgeries, the nurses were trying to wake me up and I kept trying to ignore

them because I thought I was really warm (asleep) and waking up felt really

cold. In reality, my body temperature was dropping rapidly.

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" Body Locking up " It's nice to know someone else has the same problems at

night!

I usually wake up in pain form either one arm or the other. It has gotton a lot

worse in the

last five years. Getting Old?!?

Jane

----- Original Message -----

From: john dawson

Sent: Sunday, January 14, 2001 11:16 AM

egroups

Subject: [] misc

Gretchen,

I think it was you talking about your body " locking " up. When I sleep my arm

is under my pillow. When I awake I must pick up my arm with the opposite

hand. The part that hurts and can't move is the upper arm between shoulder

and elbow. Is this what you meant by locking up?

Anesthetic...What is the problem here? I once had thyroid surgery and spent

an extra four hours in recovery unable to be awakened. When I did wake up it

felt like the elephants had just left my chest.. I'm concerned as my

brother(skinny legs) but no other major problems is having surgery. He had

seeds implanted for cancer of the prostate and they failed. Now he is facing

major surgery. His son is the one of my family that has the CMT symptoms .

If my brother doesn't have CMT, is he a carrier being his son has it? Would

a carrier be in just as much jeopardy.

I haven't received my packets yet from CMTI and CMTA that's why I'm asking

the experts.

I had mentioned that the past years progression of symptoms is what made me

look again for answers. Since my bx in December my symptoms have really

progressed. My right leg has been most affected. Now my left feels very weak

at the ankle. the right side of my torso(back) feels like it freezes up upon

rising from a sitting position,(need to sit and try again) face and neck

twitches, swallowing has been a problem for awhile. Vision has gotten way

worse.Hands aren't affected but finger dexterity is awful. Is it possible

for these things to go into remission or slow down or do I need to start

drawing up plans for my ranch house with wide hallways quick? I know

everyone is different but what's the general consensus on this? I wonder too

how the fatigue factor enters into this. Surgery, bad chemical burn on my

leg from something sprayed on during surgery, Holidays, back to work,

admittedly I'm tired. Also have a husband who had a brain injury and when

things get stressful his Obsessive compulsive behaviors get worse. To add to

the stress. Before Christmas he had shopping money on him. He went to a

diner for lunch-long story short-he paid a waitress's electric bill $380. It

was to be turned off the next day. Fortunately he went to the electric co.

and paid it but it sure wasn't in my budget.

Sorry this is so long, but just needing some answers.

Judi

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  • 2 years later...
Guest guest

wHEN i SIGNED UP I only wanted the daily digest-- I rechecked and that is

what I am supposed to get-- instead I am getting a dozen or so at at time--

is that the daily digest?

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