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NEWSLETTER. VOL-6. 9/1/01

Welcome to the sixth edition of our Newsletter. In this months publication

you will find the following:

1. News Flash's 2. 2002 Get Together 3. Spotlite Website 4. Irritable

Bowel Syndrome

5. Special Welcome 6. Opio-phobia

7. Tid-Bits 8. Neuromuscular Pain 9. LOL 10. That's Good! 11. Ask Dr. Vinci.

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

....................NEWS FLASH!!......................

In early August Founder (Gretchen Glick)was approached by (Deejay)

Delorenzo, Coordinator of Media and Market Research and Executive

Producer of 'Show You Care " (a Gulf-California Broadcast Company owned

by ABC) for up to date CMT information and a press packet.

(Deejay) wants to help further CMT awareness by producing a /CMT

Public Service Announcement on air The information Gretchen provided in

the

press packet is currently in the hands of the VP of News for the story.

Foundation Founder's Board Member Debra (at Disney) made the

initial contact. A

big thanks to Debra!

.................................................................................\

...................

........................News Flash #

2................................................

Gulf Broadcasting Corporate Offices, a division of ABC, has given

their aprroval for the use of their corporate name and logos for the

Foundation site! ABC, Fox 11, WB Chanel 5 and

KESQ - Chanel 3, Palm Springs. All these logos will soon appear

on the .org website.

..................GetTogether..................................

Save the Dates: August 23,24, 25, 2002 members will meet in Santa

Barbara California. Book your reservations now - all price ranges at

many diverse hotels and motels 1 - 800 - 793 - 7666 HotSpots OR Coastal

Escape 1 800 - 292 - 2222. SB Visitors Bureau 805 - 966 - 9222 or try

http://www.santabarbara.com to order a guide book to do your planning.

More information will be forthcoming. But, PLAN NOW!

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

................Spotlite Website.....................

This is our September Spotlite Website

<http://www.neurologylinx.com/thearts.cfm?readurl= & artid=198725 & specid=11> " >

<http://www.neurologylinx.com/thearts.cfm?readurl= & artid=198725 & specid=11>

Journal: Canadian Journal of Neurological Sciences

Headline: Charcot-Marie-Tooth Disease and Related Inherited Peripheral

Neuropathies

Abstract: This article reviews clinical, electrophysiological, pathological and

molecular aspects of hereditary motor and sensory neuropathies.

Advances in understanding the many faces of CMT have been rapid, fueled by the

progress in correlating clinical presentation with molecular defect. Some of the

CMT phenotypic variability clinicians detect can be explained by abnormalities

in different target genes, or differences in gene target dosing.

*Note by Becky, this article is long, but well worth reading.

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

.............Irritable Bowel Syndrome.....................

<http://webmd.com/condition_center_contentibs/article/1728.80302>

Real Help for Irritable Bowel Syndrome

New Drugs and Old Therapies Look Promising in Clinical Trials

By Liza Jane Maltin

WebMD Medical News

May 23, 2001 (Atlanta) -- It seems it's been all bad news lately for people with

irritable bowel syndrome. Lotronex, the drug with so much hope, was voluntarily

removed from the market this past November after some patients experienced

life-threatening side effects, and before that Propulsid -- which was linked to

serious heart problems -- became unavailable this past July. But now, things are

looking up.

This week, researchers at the Digestive Disease Week conference here presented

new findings that could mean relief for the one in five Americans suffering from

the pain, bloating, diarrhea, and constipation of IBS.

According to Lefkowitz, MD, " there is no proven effective therapy for the

group of IBS patients with abdominal pain, bloating, and constipation. " But

within a week of being on a new drug called Zelnorm, " these patients had

significantly less discomfort and improved quality of life, " he says.

After a four-week observational period, Lefkowitz' team randomly assigned more

than 1,500 female volunteers with IBS to a 12-week course of twice-daily Zelnorm

or placebo. The women all had moderate to severe abdominal pain and three or

more bowel movements per week, usually with straining.

Zelnorm " stimulates motility or movement of the digestive tract, stimulates

intestinal secretions, and inhibits visceral sensitivity or the perception of

pain, " says Lefkowitz. He is director of clinical research at Zelnorm

manufacturer Novartis Pharmaceuticals Corporation, in East Hanover, N.J.

The new drug works by mimicking the effects of the naturally occurring chemical

called serotonin, says Lefkowitz. Lotronex, in contrast, did essentially the

opposite. It inhibited the action of serotonin and was recommended for IBS

patients whose primary complaint is diarrhea.

Considerably more patients in the Zelnorm group than in the placebo group

reported complete or considerable relief from IBS symptoms, and a significant

increase in their overall sense of well-being. " Zelnorm twice a day results in

rapid improvement of multiple IBS symptoms, " and the effect lasted as long as

they continued the medication, he tells WebMD.

So far, extensive research has revealed no worrisome side effects with the drug.

The most common problems have been headache and nausea. " There was a two-fold

increase in diarrhea in about 6% of those taking Zelnorm, but it resolved by

itself, did not cause dehydration, and most patients were able to continue in

the study, " says Lefkowitz. Even so, he cautions that anyone whose IBS symptoms

include diarrhea should not take the drug.

Zelnorm is currently under FDA review with approval expected by summer, says

Lefkowitz.

Help is also on the way for those suffering mainly with diarrhea. A new drug,

cilansetron, is now in the final stages of clinical trial, and approval is right

around the corner, says IBS researcher Camilleri, MD, professor of

medicine and physiology at the Mayo Clinic in Rochester, Minn. This drug falls

into the same serotonin-inhibiting class as the now-defunct drug Lotronex, he

says.

And in other drug development, researchers are trying to modulate the

pain-messaging pathway, and a compound known only as substance P in particular.

" P stands for pain, " says Camilleri, " and the theory is that in IBS, something

has gone wrong with the transmission of pain signals from the gut to the brain. "

These new compounds will try to short-circuit those faulty signals, he explains.

Even with these promising new drugs on the horizon, there will likely be " some

IBS patients who do not respond to any available treatment, " says researcher

Francis Creed, MD, of Manchester University in England. " About 50% of them have

depression or anxiety in addition to their IBS symptoms. "

Creed's team looked specifically at this hardest-to-treat group, randomly

assigning them to receive eight weeks of standard medical treatment from a

gastroenterologist, eight weeks of Prozac, or eight weeks of one-on-one

psychotherapy session. They followed the patients for a full year after

treatment.

Overall, psychotherapy was more effective than Prozac, and Prozac was more

effective than standard medical treatment at improving overall quality of life.

Interestingly, although the one-on-one counseling was most expensive in the

short term, it was least expensive in the long term, due to fewer doctor visits,

drug prescriptions, and lost work time, says Creed.

" Psychotherapy really worked in about two-thirds of these patients, " he says.

This was true regardless of the patient's specific IBS symptoms, and especially

for those patients with a history of childhood abuse.

Most important, says Creed, " patients in the psychotherapy group reported

significant improvement in their quality of life, even if their IBS symptoms did

not change. They still had pain, " he says, " but they were better able to cope

with it. " He advises all IBS patients who are experiencing depression or anxiety

in addition to their physical symptoms to seek counseling.

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

..............Welcome New Members...................

A great BIG Welcome to all new members! You have just joined the fastest

growing CMT group! :)

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

.....................Opio-phobia..................................

*Note by Becky, this article speakes of cancer patients but applies to all who

are in pain and fear the use of opiates. Good article.

<http://msnbc.com/news/613494.asp?0dm=N224H>

Some unexpected casualties in the war on drugs: pain patients who avoid potent

opioids out of fear of addiction doctors who won’t prescribe them because of red

tape pharmacies that won’t stock them because of theft worries. Is OxyContin the

latest example of a much-needed drug that’s fallen victim to ‘opio-phobia’?

ONCOLOGY NURSE specialist Carol Blecher knows the face of pain and the face of

fear.

Cancer, says Blecher, is not a gentle, silent enemy but rather a painful, raging

foe, which must be fought with powerful weapons that often cause their own

unremitting pain. So easing or eliminating a patient’s pain is often Blecher’s

primary concern.

" But every day patients and families come to me filled with fear about taking

opioids, " she says - narcotic drugs like methadone, morphine, and OxyContin.

That fear, called " opio-phobia, " can stand in the way of relief for many

patients.

At her office at Valley Hospital System in Ridgewood, N.J., Blecher says the

media frenzy surrounding abuse of the long-acting painkiller called OxyContin

has fueled patients’ fears. " Now patients and families are asking: Does this

drug make you an addict? I have to just tell them over and over that they are

taking the drug for pain, not for addictive reasons, " says Blecher, a

spokesperson for the Oncology Nursing Society.

DRUG A ‘LIFELINE’ FOR CANCER PATIENTS

The furor surrounding use of opioid painkillers is very frustrating for pain

management specialists like Dr. Syed Nasir. " I take care of people who have

cancer, and for these people [OxyContin] is a lifeline, " says Nasir, a

neuro-oncologist at the Culichia Neurological Clinic in New Orleans.

Both patients and physicians have traditionally been wary about the use of

narcotics for pain relief, he says, because of fears it could trigger addiction.

It makes for a great movie-of-the-week plot - traumatic injury leads to

unrelenting pain that can only be eased with morphine, turning an unsuspecting

housewife or grandmother into a raving junkie - but such tales have little basis

in medical reality, says Nasir. In fact, he says, only about 1 percent of people

who take drugs such as OxyContin for treatment of chronic pain will become

addicted.

HOW IT’S ABUSED

s Hopkins University cancer expert Dr. Carducci tells WebMD that

some cases of OxyContin abuse may be related to confusion about how the drug

should be given. Doses of older long-acting opioids, such as MS-Contin, could be

increased from two times a day to three, four, or more times a day. OxyContin,

on the other hand, is " a twice-a-day drug, not three times, not four times a

day, " he says.

The drug’s special formulation allows for an immediate release into the

bloodstream followed by " 12 hours of slow release, so each pill lasts for 12

hours, " says Carducci.

Abusers of the drug discovered that if extended-release OxyContin pills were

ground up and snorted or injected, the user could, in effect, get the entire 12

hours’ worth of drug at one time, resulting in a much more intense high. Such

use has been blamed for around 100 deaths nationwide and prompted the FDA last

month to strengthen warnings on the drug’s label, likening it to morphine. The

agency also mailed letters to doctors, pharmacists, and other healthcare

providers alerting them of its potential for abuse.

And just last week, manufacturer Purdue Pharma announced its plans to

reformulate the drug in an effort to discourage such abuse. The new form of

OxyContin - available in three to five years - will come mixed with tiny beads

of naltrexone, a drug that counteracts the effects of narcotics and is used to

treat heroin addiction. The naltrexone is designed to be inactive as long as the

pill is intact - crush it, however, and the high-busting naltrexone is released.

MEDIA OVERKILL?

While the torrent of news stories about OxyContin abuse has certainly raised

public awareness of this deadly new drug trend, it’s also fanned the flames of

opio-phobia, say critics.

As the point man in implementing new federally mandated pain-control measures at

s Hopkins, Carducci says he deals daily with the results of painkiller

paranoia.

" I am implementing this plan in which all patients are asked if they have pain,

and then a pain care plan is started, " he says. " Now it makes that job even

harder because people are afraid to take drugs for pain. "

NEW DRUG, OLD FEARS

Many pain experts are concerned that scary headlines are making opio-phobia

worse, says Dr. , a Denver-based pain management specialist.

, co-founder of the National Pain Foundation, recently joined other pain

specialists for an international symposium on the problem of irrational fear of

opioid drugs.

Much of the attention being paid to OxyContin abuse is silly because very

similar drugs like " MS-Contin have been around for 10 years or longer, " he says,

with no attendant bad media.

The U.S. has a history of opio-phobia that stretches back to legendary newspaper

publisher Randolph Hearst, says , who used his newspapers to

campaign against the dangers of opium almost 100 years ago.

In the current environment, opio-phobia is flourishing because both physicians

and patients are uneducated about pain and pain treatment.

" The average physician has less than two hours of formal training in the

treatment of pain, " says , assistant clinical professor at the University

of Colorado Health Sciences Center in Denver. " Yet, the number one reason for

visiting a doctor is because of some painful problem. "

DEPENDENCE DOESN’T EQUAL ADDICTION

Even though pain leads people to seek medical help, too many patients suffer

needlessly because they have misplaced fears about the use of opioid medicines,

says Dr. Akshay Vakharia, a pain management specialist at the University of

Texas Southwestern Medical Center in Dallas. Those fears often stem from

confusion about the difference between dependence and addiction.

Patients who are treated for long periods with opioid medicines like OxyContin -

meaning more than two weeks - will experience physiological dependence on the

drug. That means, simply put, that if the patients abruptly stopped the drug

they would have symptoms of withdrawal, such as tremors, nausea, diarrhea, and

sweating. In many cases the symptoms are mild and not like Ross’ bathroom

histrionics in " Mahogany. " And if the patient is gradually tapered off the drug,

there are no symptoms and, most importantly, there " is no relapse, no

drug-seeking behavior, " says Vakharia.

says he and other pain experts want to get the message out that

addiction is not a significant risk when drugs like OxyContin are used to treat

pain. Moreover, he says that the whole concept of tolerance, meaning that

patients get used to the drug at a low dose and then need higher and higher

doses to overcome pain, is flat-out wrong.

" If the patient is started on an opioid and the dose is adjusted to a level

where pain is adequately treated, the patient can be maintained on that same

dose for the long-term, " says . When a patient complains that pain has

returned " it usually means that either the disease has progressed or there is

something else, another condition, " he says.

Moreover, says, even after years on opioids, patients can be taken off

the drugs without fear of relapse. He points to one of his patients who took

methadone for a painful hip defect. After many years the patient had hip

replacement surgery, which freed him from the pain.

" We weaned him off the methadone and he has been methadone free for two years,

no problem. Taking the drug did not make him an addict, " says .

Why such a low risk of addiction with such powerful narcotics? It seems the body

processes drugs differently when they’re taken for genuine pain and when they’re

taken for recreational purposes.

" Patients without a history of addiction who actually have bona fide pain don’t

get high when they take these drugs for pain, " says psychiatrist and addiction

specialist Dr. Wallace. For most pain patients OxyContin " relieves the

pain but doesn’t give the buzz, " says Wallace, director of professional services

at Professional Renewal Center, a drug treatment center in Lawrence, Kan.

It’s yet another of the missing pieces of information that contributes to the

opio-phobia phenomenon. Yet until both physicians and patients are educated

about the real opioid story, such misinformation and fear will continue to stand

in the way of " getting the job done: treating patients and their pain, " says

.

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

.............Tid-Bits..........................

As of Today, membership is now at 525!

Submitted by: List Owner and Founder, Gretchen Glick:

" IF ers had to PAY for this free

service of a CMT 24/7 global email group, each er would need to pay

$125.00 for what we currently offer! So, THANKS to for the free

service " !

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

.............Neuromuscular Pain Article MDA....................

http://www.mdausa.org/publications/Quest/

QUEST Volume 8, Number 4, August 2001

Living With - and Without - Pain

Years of living with a neuromuscular disease can take their toll in pain.

by June Price

" A Percodan, a Pepsi and a cigarette ... "

These were 's demands each morning even before she fully opened her eyes.

These habits took the edge off the intense pain she felt, so she could be

dressed and transferred into her power chair each morning.

As the day progressed, so did her self-medication. By midday, she was in a

pharmaceutical whirlwind; and by nighttime, she was crashing. This was her daily

ritual - all in the attempt to escape the ravages of pain and discomfort of

living for more than 30 years with an undetermined neuromuscular disease.

Then it happened. Following routine surgery, slipped into a coma. Nurses

said her vital signs wouldn't stabilize. Days later, she was dead.

When I helped her family clean her apartment after her death, we found pill

bottles in, on and under everything: table, counter, bed, floor, medicine

cabinet, refrigerator, drawers. Labels reflected a myriad of doctors, hospitals

and pharmacies spanning years. I no longer asked why had died but,

instead, why she hadn't died sooner.

I never had much sympathy for back then, often wondering if the pain she

claimed wasn't " all in her head " - a cry for attention. But 20 years later,

has come back to haunt me with a vengeance.

Pain now controls my days, much as it once did 's. Finally, I understand

her.

Understanding Pain

" Does it hurt? "

When I was growing up with spinal muscular atrophy, people often asked me that

question. Today, parents still ask, as they attempt to understand whether their

son or daughter with a new SMA diagnosis feels discomfort.

I reassure them that SMA, as a disease, doesn't " hurt. " Sure, growing up, I

experienced the inevitable sprained ankles and muscle aches (my mom used to call

these " growing pains " ), but there's no pain per se with this disease.

So why, then, do I, and many of my peers, have so much pain now, in middle age?

And where does this pain originate?

" Pain is one of the more enigmatic symptoms that all physicians confront, in one

form or another, every day, " explained P. McQuillen, professor of

neurology and medical humanities at the University of Rochester in New York.

" Since its origin may be multiple and varied, one has to start with the context

- character, origin, location, behavior, aggravating and ameliorating factors,

and the physical findings - to determine why the pain is there. "

McQuillen said a number of conditions common in middle age, such as degenerative

joint disease or inflammatory conditions such as polymyalgia rheumatica, can

contribute to increased pain in those with neuromuscular disease. The loss of

limb movement over time can also contribute, he said.

I asked McQuillen, a former MDA clinic director in Wisconsin, about what I call

" nerve pain, " or phantom pain. Be it throbbing, shooting, dull or burning,

almost every adult with neuromuscular disease I've spoken to seems to experience

this type of pain at times. As an example, I might feel that my foot is pressed

up against the bed sheets, accompanied by excruciating pain and elevated blood

pressure. But my caregiver assures me that nothing is touching it, and after a

short time the pain subsides.

McQuillen explained, " The neural basis of phantom pain is complex and poorly

understood. It probably has something to do with spontaneously activated

pathways at various levels of the central nervous system. It can be seen at

almost any age. "

Greg , clinical associate professor of rehabilitation medicine at the

University of Washington School of Medicine, offered some reasons for our pain.

" The first is simply biomechanical, " he said. " People who have an imposed

mobility problem will often develop secondary musculoskeletal conditions that

can be painful. These include low back pain, frozen joints, stiff necks,

arthritic joints from walking abnormally or sitting in a wheelchair all day,

etc. "

E. Csuka, associate professor of medicine, Division of Rheumatology, at

Froedtert Memorial Lutheran Hospital and the Medical College of Wisconsin, said

arthritic pain may be common in middle-aged people with muscle diseases.

Csuka, a specialist in geriatrics, osteoporosis, rheumatology and scleroderma,

pointed out that muscle is an important stabilizer to the joint. In people

without muscle problems, " even a joint with severe degeneration can remain

functional if the muscles can be strengthened. "

However, she explained, " Patients with muscle-wasting diseases lose the

stability factor of normal muscle and, hence, the joint capsules and ligaments

are under greater strain even to maintain neutral position. "

Immobility, by reducing blood supply to the cartilage of the bones, can also

contribute to the symptoms of aching and stiffness, she added.

Acknowledging Pain

Knowing all of this, you'd think we'd all be complaining to doctors about our

pain, yet this hardly seems the case. Peltier, assistant professor at the

Medical College of Wisconsin in Milwaukee who serves as the MDA clinic director

there, reported, " Pain is actually not a frequent complaint. "

[Hand full of pills]

, co-director of MDA's clinics in Olympia and Tacoma, Wash., suggested

that the subject of pain may take a bit more prodding. developed an

interest in chronic pain and the way it affects physical and emotional

functioning, so he started asking more neuromuscular disease patients if they

have pain. He was " amazed at how many said yes. "

On the basis of his conversations with patients, began doing pain

research on people with neuromuscular disease. He's working with psychologist

Mark P. Jensen, an associate professor in the University of Washington's

Department of Rehabilitation Medicine and a world-renowned expert on pain, and

Ted Abresch, director of research at the University of California at in

the Neuromuscular Disease Research and Training Center.

I suggested to that perhaps people with neuromuscular diseases are less

apt to complain about pain, because they believe it comes with being disabled -

just one more inconvenience or discomfort we must learn to accept.

" I absolutely agree, " he responded, " and that is exactly why I think pain in NMD

has been overlooked for so long. The good news is that there are great, new,

effective physical and pharmaceutical ways to treat pain. Thus, I encourage

patients to talk to their health care providers about their pain. "

Talking About It

Establishing a dialogue with your physician, along with obtaining a thorough

physical evaluation, will help determine the most effective treatment routes for

pain associated with neuromuscular disease. The complaints can be many and

varied: stiff joints from immobility, sore butt from sitting, pressure sores

from orthotics and so on. Identifying elements of your discomfort, such as joint

pain, is an essential start.

Sometimes pain diminishes when these problems are solved at the root - by

correcting the fit of a brace, adjusting positions more frequently or being

turned more often in the night. It's also important to treat pressure sores to

prevent major skin breakdown and infection, and to be ex-amined for possible

blood clots.

Your doctor may also recommend nonpharmaceutical treatments, including heat,

cold or massage. These techniques can actually change blood flow and reduce

inflammation. Some people have even found that psychological techniques, such as

meditation and relaxation exercises, can help to alleviate pain, or at least

relieve the tension that may be making pain worse.

But oftentimes, the " fix, " that is, " Leave your brace off till your sores heal, "

or " Exercise more, " isn't feasible. Psychological and mechanical treatments may

only go so far, and your doctor may agree that you need medication for the pain

(analgesics).

Nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin are the most

frequently prescribed for pain, but they can cause gastric problems in some

people. Csuka said she generally prescribes acetaminophen (Tylenol) for stiff,

inflamed joints. This is also the recommendation of the American College of

Rheumatology as the first medication to try for the treatment of

noninflammatory, or degenerative, arthritis pain. When compared to NSAIDs,

acetaminophen has been found both effective and safe. (See " Understanding

NSAIDs " .)

She noted that Tylenol is available in a variety of forms, including syrup.

" Arthritis Strength Tylenol is formulated so that patients only need dosing

three times a day, rather than four times, when the medication is taken

regularly, " Csuka said.

If you take acetaminophen regularly, you should be monitored for possible liver

toxicity, especially if you're also taking combination narcotic analgesics such

as acetaminophen and codeine phosphate (Tylenol 3), propoxyphene and

acetaminophen (Darvocet-N 100), hydrocodone bitartrate and acetaminophen

(Vicodin), or oxycodone hydrochloride and acetaminophen (Percocet).

I grew up with aspirin being the strongest pain pills in our family medicine

cabinet, so I asked , " Is it just me, or do you find older neuromuscular

disease patients are especially resistant to trying pain medication, especially

narcotics? "

" Yes, indeed, " he agreed. " That is why it is so important for the health care

provider to spend time talking to the patient, providing education about the

usefulness and side effects of pain medication.

" There is good evidence in the medical literature indicating that pain

medications are hugely underprescribed in the United States and people suffer

needlessly, " pointed out. " Plus, there are so many new, non-narcotic

types of pain relievers available. "

Tolerance vs. Addiction

acknowledged that pain in neuromuscular disease may reach the stage of

requiring narcotic treatment. He said that long-term medical use of narcotics

can lead to tolerance, but not necessarily to addiction.

" There is 'tolerance' and then there is 'addiction,' " he explained. " All

patients who take narcotic pain medication on a regular basis for a long period

of time will develop tolerance. This is much like the guy who drinks a six-pack

of beer a night for years. Soon, he feels no effect from the beer, and if he

suddenly stops drinking he may have DTs [delirium tremens or alcohol

withdrawal].

" Addiction really involves the psychological aspects of the drug, "

further explained. " If someone is taking the pills for a feeling of euphoria,

then they are at risk for addiction. If they are taking the medication to treat

pain, then the risk for addiction should be minimal, although they will develop

tolerance. "

emphasized: " Anyone using chronic narcotic medication needs ongoing

medical supervision. If they decide to stop, they should be weaned off slowly to

avoid physical withdrawal symptoms as noted above with our beer drinker. "

Although they can offer tremendous pain relief and don't usually result in

addiction, narcotic medications still have their caveats. A primary concern in

neuromuscular disease is that they're respiratory suppressants.

said, " There is a direct effect of the drug on the central nervous system

breathing centers. That doesn't mean you can't take it if you have breathing

problems, it just means you need close medical supervision. "

In my situation, I found that starting with a low dose of narcotic allowed me to

evaluate its effects on my breathing, which were minimal. Of more immediate

concern was constipation, which Csuka called " a major complication of

narcotics. " Awareness and preventive measures can minimize this problem, as

well.

Pain and Depression

Untreated pain in neuromuscular disease can begin a chain of events leading to

depression. The pattern is clear: Chronic pain leads to sleeplessness, which

reduces levels of endorphin (the body's natural painkillers). That increases

pain sensation, which lowers serotonin levels, which leads to depression, which

makes many of us begin to have " dark thoughts. " There are no simple solutions to

these issues.

That doesn't mean every case of depression or sleeplessness can be linked to

pain. Those symptoms should be examined separately and may be treatable without

pain medication. At the same time, antidepressants may not only lift your mood;

in some cases they may also ease the underlying physical pain.

McQuillen, who specializes in ethical issues in medicine, including

quality-of-life and right-to-die questions, said, " Pain is a very strong

determinant of depression and of the desire to do anything - even end one's life

- to get rid of pain. The literature on assisted suicide and the hospice

movement is rife with examples of this interconnection and how recognizing,

respecting and relieving pain can make all the difference in the world. "

" Some antidepressants have an analgesic effect, " Peltier added, " because they

also modulate neurotransmitters in the brain that may play a role in pain

regulation. Many patients who are depressed also become more 'tuned in' to

internal signals, and this may explain why pain seems amplified in depression.

Some of the newer seratonergic medicines (nefazadone [serzone], citalopram

[Celexa]) may be better for musculoskeletal pain, and I have used them even in

patients who do not have depression. "

Csuka agreed. " It is only logical that controlling pain by whatever means will

improve a patient's sense of well-being. However, in doing so, the approach

needs to be broad, taking in all factors related to pain, both physical and

psychological. "

[Hand full of pills]

A Hard Pill to Swallow

For some people whose neuromuscular disease has interfered with swallowing,

finding medication that will result in pain reduction is only half the battle.

Getting it into your system is the other.

" There are a lot of different ways to take pain medication, " said. He

listed pain patches (morphine, fentanyl, lidocaine); pain elixirs for under the

tongue (morphine); and inhaled pain medication (morphine inhaler, marijuana

smoked or vaporized). The cannabinoids in marijuana are analgesics, he said.

explained that medicinal marijuana is very strong compared to the street

variety and that " cannabinoids (active ingredients also found in chocolate) are

fat-soluble, rapidly diffusing compounds. The cannabinoids can be 'vaporized'

(as in aromatherapy) at a temperature much lower than combustion. Then, you

simply inhale hot air, which eliminates the health hazards of smoking. " It isn't

necessary to hold the smoke from medical marijuana in the lungs.

Several states have enacted laws permitting medicinal marijuana use, including

Washington, where prescribes it for his ALS patients.

" It works well for pain, spasticity and loss of appetite, " he said. " If used

properly, it is remarkably safe with very few untoward side effects. "

Although federal laws prohibit marijuana use for any reason, those states that

have enacted laws permitting medical use of marijuana offer some legal

protection against state prosecution.

Blessed Relief

Whether acetaminophen, morphine or marijuana, we all have a right to adequate

pain relief - without guilt, shame, fear or begging.

McQuillen best summed it up: " Medicine and society at large are beginning to

recognize the complexity of pain, with various medical societies taking a stand

on the need for adequate recognition and treatment of pain; the federal

government and the Supreme Court, as well. Sadly, a lot more remains to be

done. "

Understanding NSAIDs

Nonsteroidal anti-inflammatory drugs (NSAIDs) are the class of drugs most

frequently prescribed. The original NSAID is aspirin, which was first marketed

in 1898. It wasn't until the 1970s that scientists began to understand how

aspirin worked. We now know that aspirin and the NSAIDs developed to make a

safer more effective aspirin in the past three decades inhibit the production of

chemicals called prostaglandins. There are both " good " and " bad " prostaglandins.

The " bad " prostaglandins are produced in response to injury, and act as

mediators of inflammation and, hence, of pain. Inhibiting the production of

these prostaglandins has demonstrated a reduction in inflammation and pain in

both animal and human studies.

The " good " prostaglandins help to maintain the integrity of the lining of the

stomach, promote clotting by platelets to prevent excessive bleeding and

maintain kidney blood flow. Until recently, all NSAIDs were nonselective. In

order to inhibit the " bad " prostaglandins responsible for pain, one had to

accept some inhibition of the " good " prostaglandins. Fortunately, most patients

experience relief of pain without severe side effects, most commonly ulcers of

the stomach.

In 1999, two new NSAIDs were introduced (celecoxib [Celebrex] and rofecoxib

[Vioxx]), which, at least theoretically, were designed to inhibit the " bad "

prostaglandins of inflammation, while sparing the " good " prostaglandins that

protect the stomach. They seem to work as well as the older NSAIDs (e.g.,

naproxen, ibuprofen [Motrin], etodolac, diclofenac sodium [Voltaren],

indomethacin [indocin], etc.), and early studies have supported an improved

safety profile with respect to gastrointestinal bleeding.

Decreased blood flow to the kidneys remains a problem even with the newer

NSAIDs, so patients with impaired kidney function should not take any NSAID.

-M.E. Csuka, M.D.

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>

....................................LOL..........................................\

....

Why I fired my secretary

Two weeks ago, was my forty-fifth birthday, and I

wasn't feeling too hot that morning anyway. I went into

breakfast, knowing my wife would be pleasant and say

Happy Birthday and probably have a present for me. She

didn't even say Good Morning, let alone any Happy

Birthday. I said, well, that's wives for you. The

children will remember. The children came into

breakfast and didn't say a word. When I started to the

office I was feeling pretty low and despondent.

As I walked into my office, my secretary, Janet said,

" Good Morning, Boss, Happy Birthday. " And I felt a

little better; someone had remembered. I worked until

noon. About noon Janet knocked on my door and said,

" You know it's such a beautiful day outside and it's

your birthday, let's go to lunch, just you and me. "

I said, " By , that's the greatest thing I've

heard all day. Let's go. " We went to lunch. We didn't

go where we normally go; we went out into the country

to a little private place. We had two martinis and

enjoyed lunch tremendously.

On the way back to the office, she said, " You know,

it's such a beautiful day. We don't need to go back to

the office. Do we? "

I said, " No, I guess not. "

She said, " Let's go to my apartment. "

After arriving at her apartment, we had another martini

and smoked a cigarette and she said, " Boss, if you

don't mind, I think I'll go into the bedroom and slip

into something more comfortable. "

" Sure, " I excitedly replied.

She went into the bedroom and in about six minutes, she

came out . . .

.. . . carrying a big birthday cake, followed by my wife

and children. All were singing Happy Birthday.

.. . . and there on the couch I sat . . .

.. . . with nothing on but my socks . . . .

.............................................................

Classes for Dog to Talk and Read

A young man goes off to college, but about a third of

the way through the semester, he has foolishly

squandered what money his parents gave him. " Hmm, " he

wonders, " How am I gonna get more dough? " Then he gets

an idea. He calls his father.

" Dad, " he says, " you won't believe the wonders that

modern education are coming up with! Why, they actually

have a program here that will teach Fido how to talk! "

" That's absolutely amazing! " his father says. " How do I

get him in that program? "

" Just send him down here with $1000, " the boy says,

" I'll get him into the course. "

So, his father sends the dog and the $1000. About two-

thirds of the way through the semester, the money runs

out. The boy calls his father again.

" So how's Fido doing, son? " his father asks.

" Awesome, dad, he's talking up a storm, " he says, " but

you just won't believe this. They've had such good

results with this program, that they've implemented a

new one to teach the animals to read! "

" READ!? " says his father, " No kidding! What do I have

to do to get him in that program? "

" Just send $2,500, I'll get him in the class. " So his

father sends the money.

At the end of the semester, the boy has a problem. When

he gets home, his father will find out that the dog can

neither talk nor read. So he shoots the dog. When he

gets home, his father is all excited. " Where's Fido? I

just can't wait to hear him talk and listen to him read

something! "

" Dad, " the boy says, " I have some grim news. This

morning when I got out of the shower, Fido was in the

living room kicked back in the recliner, reading the

morning paper, like he usually does. Then he turned to

me and asked, 'So, is your daddy still messin around

with that little redhead who lives on Oak Street?' "

The father yells, " Oh, crap! I hope you SHOT that lyin'

son-of a biscuit!

" Sure did, Dad! "

" That's my boy!!! "

...............................................................

Philosophic Questions

Ponder these questions when you don't want to think

about important stuff!

If you choke a Smurf, what color does it turn?

Is it OK to use the AM radio after noon?

What do chickens think we taste like?

What do people in China call their good plates?

What do you call a male ladybug?

What hair color do they put on the driver's license of

a bald man?

When dog food is new and improved tasting, who tests

it?

Why didn't Noah swat those two mosquitoes?

Why do they sterilize the needle for lethal injections?

Why doesn't glue stick to the inside of the bottle?

Why is it called tourist season if we can't shoot at

them?

Why do you need a driver's license to buy liquor when

you can't drink and drive?

Why isn't phonetic spelled the way it sounds?

Why are there Interstates in Hawaii?

Why are there flotation devices in the seats of planes

instead of parachutes?

Why are cigarettes sold at gas stations where smoking

is prohibited?

Have you ever imagined a world without hypothetical

situations?

How does the guy who drives the snowplow get to work?

If the 7-11 is open 24 hours a day, 365 days a year,

why does it have locks on the door?

Why is a bra singular and panties plural?

You know that indestructible black box that is used on

airplanes? Why don't they make the whole plane out of

that stuff?

If a firefighter fights fire and a crime fighter fights

crime, what does a freedom fighter fight?

If they squeeze olives to get olive oil, how do they

get baby oil?

If a cow laughs, does milk come out of her nose?

If you are driving at the speed of light and you turn

your headlights on, what happens?

Why do they put Braille dots on the keypad of a drive-

up ATM?

Why is it that when you transport something by car it

is called shipment, but when you transport something by

ship it's called cargo?

Why don't sheep shrink when it rains?

What would Geronimo say if he jumped out of an

airplane?

Why are they called apartments when they are all stuck

together?

If con is the opposite of pro, is Congress the opposite

of progress?

If flying is so safe, why do they call the airport the

terminal?

If you throw a cat out of the house, does it become

kitty litter?

If aspirins are always " Take Two, " why not increase the

size of ONE?

>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>>....

.................Ummm So Good...............

Submitted by:

CORNBREAD CASSEROLE

1 (16 oz.) can cream corn

1 (16 oz.) can whole kernel corn---DO NOT DRAIN

1 ( 8 oz.) carton sour cream

1 stick margarine--melted

2 eggs--beaten

2 dashes of black pepper

1 box Jiffy Corn Bread Mix

In a large bowl mix corn, sour cream, melted margarine, black pepper, and beaten

eggs. Add corn bread mix. Place mixture in a greased 9X12 inch pan. Bake at 350

degrees for 45 to 50 minutes. The top should be golden brown. Enjoy!

.........................................................

CHERRY SALAD

1 can Cherry Pie Filling

1 can (medium size) crushed pineapple--drained

1 cup chopped pecans

1/2 cup sugar

1-1/2 cups miniature marshmallows

3 bananas--sliced

In a medium size mix all of the ingredients, making sure to drain the excess

juice from the pineapple. Mix at least 12 hours before serving. Place in

airtight container before serving and refrigerate. Enjoy!

*I tried the Cherry Salad , ummmmm, so good! ~>Becky

.................................................................................\

...............

******NEW*****NEWS******NEW******NEWS*****

>>>>New Column: Ask The Dr! (Dr. Paolo Vinci)<<<<<

*I saved the best for last!

*All answers by Dr. Vinci are personal opinions only and in no way intended to

replace the the medical advice of your medical providers.

Submitted by Anon:

1. You have mentioned your scale to classify CMT is seven stages several times,

as well as the fact that it was published (J Periph Nerv Sys 1999;4:279-304).

You include that there is different management for each stage. Since most of us

do not have access to the information would you share it with us? We, in turn,

could assist in disseminating it with our physicians. Since the list will not

accept attachments, you could either copy paste it or place it in the files area

where all would have access to it. I am aware that the UK has an excellent tool

to classify the extent of disability/handicap, but it is not limited to or

specific for CMT.

The classification was presented and discussed at the 8th Annual Symposium of

the European CMT Consortium, held in Antwerpen, Belgium, July 2-4, 1999.

This is the abstract.

Title: Classification of Charcot-Marie-Tooth disease for rehabilitative

purposes.

Authors: P.Vinci, S.L.Perelli

Charcot-Marie-Tooth (CMT) disease is a progressive neuropathy which causes

muscle weakness and wasting, starting from the intrinsic foot muscles and

spreading to the more proximal ones.

From a rehabilitative standpoint, the loss of functionality, which is related to

the progressive involvement of new muscle groups and to biomechanical and

postural problems, generally follows a well-defined order, so that we can grade

the disease in seven stages, for the lower limbs:

Stage 1: forefoot-drop

Stage 2: foot rotation

Stage 3: footdrop

Stage 4: plantarflexion failure

Stage 5: knee flexion failure

Stage 6: knee extension failure

Stage 7: hip extension failure

In every stage there is a new problem, typical of the stage, which causes a

different pattern of gait and requires a different rehabilitative intervention.

Although we cannot prevent patients from passing to the next stage, setting the

stage is useful to give the most appropriate solutions to the problems of the

present stage. Besides, as the first symptoms of the next stage appear, it is

possible to reduce some of its biomechanical consequences: for example, in a

patient at the first stage (inability to lift the forefoot), as the pronator

muscles start to weaken (2nd stage), appropriate foot orthoses can be prescribed

to prevent sprains and deformities, typical of the second stage.

Or for a patient in the third stage, as the plantarflexor muscles weaken (4th

stage), the heel of the shoe can be reduced to avoid stressing the quadriceps

and to prevent falls.

In our presentation, the gait of patients in each of the seven stages is shown

(videotape) and the rehabilitative intervention is discussed.

Based on the experience in the last 2 years entirely dedicated to CMT, I would

add that the classification is also useful to compare patients in a more

objective way and to monitor the overtime progression in the single patients.

[ A detailed and updated description of the classification with method of

evaluation for grading patients in each stage and a practical guide to a

step-by-step management has been recently published in the illustrated book

" Rehabilitation management of Charcot-Marie-Tooth disease " . ]

2. You also refer to your design for a boot that allows some to avoid AFO's. I

recall information was posted on the net a couple of years ago about it, but

can't locate it now. Since most of the group are not familiar with it, I feel

sure the information would be welcomed and possibly beneficial to some. As I

recall, they did aid in foot drop but were not sufficient for those with lateral

ankle instability, but am not sure of this. Could you share the information or

site where it is posted?

The " aesthetic in-shoe device for footdrop " was presented first at the 3rd

International Conference on CMT Disorders held in Canada, October 21-24, 1998.

When I came back to Italy, I sent the whole presentation to Mrs.Dorothy Gosling,

who put it on her website dedicated to CMT disease.

Since then, many patients affected with CMT or other diseases causing footdrop

took advantage of this solution. In April-May 1999, I was invited to the US to

train a pedorthist to make it. The results of the trial with the device have

been presented to many congresses in Europe and in the US.

The boots modified with the device are also effective in correcting the

supination associated with footdrop but, for severe supination, the best

treatment is surgical (muscle rebalancing by tendon transfers).

[ The device too is described the book.]

Submitted by :

What do you think about Stem Cell research . Do you find any hope for a CMT

cure/treatment by using Stem cells?

Since I am not a biologist, I am not the right person to reply to this question.

Sorry.

Submitted by Anon #2:

Do you think its necessary to test children of known CMT parents for CMT when

the child clearly show's signs of having CMT?

It is not necessary to test them, but it is important to know what genetic

defect the family has. So one member per family should be tested. This is

important also for prognosis and for making better rehabilitative decisions,

since different mutations are often associated with different progression and

severity. For example, patients with duplication often remain stationary at

stage 2 for decades and anyway do not generally progress beyond stage 4. On the

contrary, people with some point mutations in P0 or with early-onset CMT2 can

reach stage 6 or 7.

Submitted by Anon: #3:

How important is it to have the DNA test for CMT when EMG/NCV tests and symptoms

plus family history show CMT?

CMT is a disorder which can be due to many genetic defects. Of course, in each

family it is due only to one type of genetic alteration (e.g. duplication of

PMP22 gene or a specific mutation in one of the several genes responsible for

CMT), so it is not necessary to test all the family members having clear

clinical or EMG signs of CMT: one member per family suffices and the result is

valid for all of them.

However somebody might want to know if his/her fetus or child has the CMT gene

and therefore will have the disease in the future, no matter what degree. In

case of a child not yet having symptoms, it is a painless method to ascertain if

he/she has his/her family's CMT gene, in order to avoid heavy sports and jobs

which might trigger the symptoms.

Submitted by Gretchen: Would A CMT mother need to be taking EXTRA Folic

Acid aid in minimizing the genetic transmission of CMT to her offspring?

NO. There is no way to avoid the transmission of the chromosome carrying the CMT

gene instead of the normal one: it is due to chance.

.................................................................................\

...................................

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..............................................................

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