phrenic nerve

[Guest guest]

This is an update. (Re: shortness of breath problem. Not serious at this time;

mainly after exertion or a big meal. Stomach and small intestines have

encroached into chest cavity due to weak diaphragm muscle.) I just had an EMG

for the phrenic nerve and it apears to be dead, as there was no conduction of

the electric current. The needles into the diaphragm indicated about 20%

function of the diahraghm muscle. I am awaiting word from the internist, after

he reviews the EMG results and consorts with his professional peers, what if

anything can be done at this point. The MDA neurologist said he highly doubted

there would be phrenic nerve involvement with CMT because the nerve was so

short! I will make sure that he gets results from the x-rays and EMG so I can

further his CMT education!

I am wondering if Dr Paoli Vinci has run across this? And if so what has been

the management and prognosis?

Thanks, Ruth C

---------------------------------

[Guest guest]

Hello.

A phrenic nerve involvement has been reported as a feature of CMT 2C. None

of my patients has this symptom. To evaluate the respiratory failure caused

by the diaphragme weakening I'd suggest to undergo spirometry and

emogasanalysis. Since a big stomach hampers the diaphragm work, it is very

important to be slim, to avoid constipation and gas in the bowels. If the

intercostal muscles are healthy, they can be used as respiratory accessory

muscles. Exercises to breath better can be taught by a physiotherapist. In

the very rare cases of severe respiratory failure, assisted ventilation in

the night preserves life (and allows the diaphragme to be functional for the

day).

Best regards.

Paolo

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

From: Ruth Creasy <rfc40@...>

respond to forum < >

Sent: Monday, June 03, 2002 6:57 AM

Subject: phrenic nerve

>

> This is an update. (Re: shortness of breath problem. Not serious at this

time; mainly after exertion or a big meal. Stomach and small intestines

have encroached into chest cavity due to weak diaphragm muscle.) I just had

an EMG for the phrenic nerve and it apears to be dead, as there was no

conduction of the electric current. The needles into the diaphragm

indicated about 20% function of the diahraghm muscle. I am awaiting word

from the internist, after he reviews the EMG results and consorts with his

professional peers, what if anything can be done at this point. The MDA

neurologist said he highly doubted there would be phrenic nerve involvement

with CMT because the nerve was so short! I will make sure that he gets

results from the x-rays and EMG so I can further his CMT education!

>

> I am wondering if Dr Paoli Vinci has run across this? And if so what has

been the management and prognosis?

>

> Thanks, Ruth C

>

>

>

> ---------------------------------

>

[Guest guest]

Thank you, Paolo, for your response. I am seeing the internist

next week for a follow-up on the EMG and other tests. I will

share your reply with him and see what he suggests. My body is

not in major distress yet. The symptoms are definitely present

and I am aware of the progression over the last few years. I

just want to be informed as to what my options might be for

management. Other than night splints to keep foot bones

straight, daytime braces and AFOs and a short-lived ankle

fusion surgery, nature has " taken it's course " with my CMT

progression, and I have not dwelled on management, but rather

have learned to continuously adapt and function.

I have always been fairly healthy, except for the physical

limitations of CMT and have always had a lot of stamina. It was

through this forum that I heard about phrenic nerve involvement

with CMT and started wondering if that was the cause of the episodes

of shortness of breath that I had been experiencing over the past

several years.

After a bout of pneumonia in Dec 2000, I viewed a lung x-ray which

didn't look normal to me. I ASKED the Dr to follow up with lung

studies. He said nothing about the elevated diaphragm, only that

the pneumonia had cleared up.

I will not be visiting that " tunnel-vision " doctor again. I am

impressed with the internist and have confidence that he will

investigate and confer with colleagues to see what, if

anything, will be beneficial for me. Thanks again for your

reply.

Ruth C

[Guest guest]

Hi Kay,

Thanks for the welcome. I have my share of health issues, but a

positive attitude goes a long way in keeping me on track.

Sorry to hear about your sister, as well as your phrenic nerve

involvement. In addition to my sister, my aunt, who also has CMT, has

phrenic nerve involvment. I wonder if it's a lot more common than

originally thought?

Jeff

[Guest guest]

Hello Jeff,

Yes, It is a lot more common than originally thought. I know people with

CMT who have gone to their doctors for help, and the doctors tell them that

their breathing problems have nothing to do with CMT. They say it's

allergies, and send them on their way. That is what they told my sister,

and a friend of mine. I think doctors are unaware because many of the CMT

sites don't talk about the Phrenic nerve problem.

Around 1994, when my sister was in the hospital, they told her there were

only 3 people known with this problem. Now that we have the internet we are

really beginning to see just how many are affected with breathing problems.

I just pray the CMT sites and MDA become more aware so we can get more

funding for research. Hopefully the next generation can be helped.

I'm glad you stay positive. It only makes sense to enjoy life ...who wants

to waste energy being angry about having CMT, or anything else. I would

rather smell the roses ;-)

Have a great day!

Kay