Hi everyone. My name is Sarah. I live in Illinois. I am dating a
male who has one of the diseases that fall under Muscular Dystrophy.
There are things that I am having trouble understanding, and he does
not like talking about it. I can tell he has a hard time accepting it
at times. I am joining this group to maybe learn a little more, and
get some support in his condition. Thanks. Sarah

Please talk to your docs about monitoring CRP levels in your children
since inflammation is a key in this disease. This info was extracted
from an article that appeared on the "naturemade.com" website.

When you hear about inflammation you probably think of redness in
skin, pain and swelling. Inflammation is a natural reaction of the
body to injury or infection, and is caused by release of chemicals
from tissues and migrating cells.

The inflammatory response is the body`s response to certain stimuli,
which may or may not be disease-related. For example, a splinter in
your finger would trigger an inflammatory response to a small degree,
which is not related to or a result of disease. On the other hand, a
fever during the flu is a clear example of a disease-related
inflammatory response. Inflammation existing within the body has the
potential to negatively affect health, especially when it is chronic
in nature.

Chronic inflammation is a major concern in medicine today. Long-term
adverse health effects occur when inflammation persists at low
levels. In fact, many researchers now believe that inflammation may
contribute to the cause of many chronic diseases, such as
cardiovascular disease, cancer, arthritis, diabetes and
Alzheimer's disease. It is well established that elevated levels
of inflammatory markers, such as C-reactive protein (CRP),
Interleukin-6 (IL-6), and others indicate significantly greater risks
of contracting specific diseases.

For example, researchers are trying to understand why some
individuals suffer heart attacks even though they do not have major
heart blockages. In addition, inflammation is a painful component to
arthritis. The pain, redness, heat, swelling and other harmful
effects of inflammation can be treated with medication, diet and
exercise. Some researchers have also characterized obesity as a
chronic, systemic low-grade state of inflammation. New research is
looking at nutrition as a powerful weapon against inflammation.

C-Reactive Protein: Should you get tested?

C-reactive protein (CRP) is a special type of protein produced by the
liver that is only present during episodes of acute inflammation.
Testing for CRP measures the concentration of a protein in serum that
gives a general indication of inflammation. Recently, new studies
have suggested that CRP may be elevated in those at risk for heart
disease. Researchers are uncertain of the role of CRP may play in
heart disease; it is not known whether it is merely a marker of
disease, or whether it actually plays a role in causing heart
disease.

Many consider elevated CRP to be a positive risk factor for coronary
artery disease. Some researchers have suggested that inflammation is
more important than cholesterol at triggering heart attacks, and
individuals with high levels of CRP have twice the risk of people
with elevated cholesterol. Many healthy, middle-aged Americans have
normal cholesterol but above average inflammation, putting them at an
unusual risk for heart attacks and stroke.

If an individual is at risk for heart disease or has any other
condition where inflammation may be present, it is important talk to
their health care provider about testing for CRP.

Diet and Supplementation Can Help

Choosing fresh whole foods, lots of fruits and vegetables, along with
healthy fats such as omega-3 fatty acids found in salmon, tuna,
walnuts, flaxseeds/oil, etc., may help keep inflammation at bay. It
is also important to stay hydrated.

Nutritional supplements can promote health and may aid in staving off
inflammation, especially if these "anti-inflammatory" foods are
lacking in the diet. Vitamin E may play a critical role in the
reducing elevated blood inflammatory markers, such as CRP and IL-6,
seen in inflammatory processes. Furthermore, some new research
suggests vitamin C may be beneficial for reducing CRP. In one study,
participants were either smokers or exposed to cigarette smoke, and
were randomized to receive either a daily dose of a vitamin C
supplement (500 mg), a mixture of antioxidant supplements (E,C, and
alpha lipoic acid), or a placebo (sugar pill).

Results showed a decrease in CRP by 24% among those taking vitamin C
when compared to the placebo group (Block et al. J Am Coll Nutr,
2004). Other antioxidants such as selenium may help fight
inflammation. The antioxidant and anti-inflammatory properties of
selenium may be enhanced when combined with vitamin E. A deficiency
in vitamin D is a risk factor for several disorders with inflammatory
components, such as heart disease, diabetes and automimmune disorders
such as rheumatoid arthritis. (Timms, PM et al.,QJM 2002; Hein, G and
Oelzner, P. Z Rheumatol, 2000.)

The essential fatty acid, omega-3, is also very important to prevent
or reduce chronic inflammation. Omega-3 fats provided through diet
and/or supplementation produce anti-inflammatory substances that
prevent chronic inflammation from occurring. Essentially, omega-3
fats prevent the formation of inflammatory substances which are
produced in abundance when the diet is lacking in omega-3 fats. (The
typical American diet is high in fat, but is lacking in nutritionally
essential omega-3 fatty acids).

Arnold

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How about the study they did to determine the effects of cow
flatulence(sp?) on the ozone layer.

In Him Forever,
Andrew

--- In MD-List@yahoogroups.com, AV <raybug10@y...> wrote:
> The war is only one example of possible wasted moeny.  How about
those studies on where people sit in restaraunts?  or studies on
what is the average size foot of an average sized man?  Or how about
3 billion to pop a hole into a comet?  yeah, a lot of money is
wasted for the sake of "good stuff".
>
> Anneliese
>
> JoyceGlass@a... wrote:
> I know some people who read this won't agree with me, but I just
wanted to say that when I hear of such promising research as this I
think of all the money wasted, for instance, the billions that have
gone into the war in Iraq, that could have been spent on researching
further these promising cures.  I don't want to stir up  a lot of
controversy and start a big political discussion but think of all
the wasted money that could have been used to help our boys and
girls with muscular dystrophy.
>
>
> ---------------------------------
> YAHOO! GROUPS LINKS
>
>
>     Visit your group "MD-List" on the web.
>
>     To unsubscribe from this group, send an email to:
>  MD-List-unsubscribe@yahoogroups.com
>
>     Your use of Yahoo! Groups is subject to the Yahoo! Terms of
Service.
>
>
> ---------------------------------
>
>
>
> __________________________________________________
> Do You Yahoo!?
> Tired of spam?  Yahoo! Mail has the best spam protection around
> http://mail.yahoo.com

New procedure allows quadriplegic to cough:

http://news.yahoo.com/s/ap/20050731/ap_on_he_me/experimental_coughing;_ylt=AoR4t\
NqGXUIReB.0NIuYjUus0NUE;_ylu=X3oDMTA3czJjNGZoBHNlYwM3NTE-

That sound like very good seating. The other thing we have some success
with is the Jay system. That is a line of cushions with countroured frim
foan bases and gel inserts on top. Roho cushions are super at releiving
pressure on your bottom, and used very commonly for adults with spinal
cord injuries and others who lack sensation, but they are also a very
unstable base of support, which may aggravate postural problems and
scoliosis particularly for children.

Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids) at UAMS
and Arkansas Children's Hospital.  Working Mom of Sarah T. and Michael C.,
and wife of Henry Stefans. Every mom is a working mom!- OK, dads too.

On Fri, 29 Jul 2005 JoyceGlass@... wrote:

> Beverly, my son used to have a Roho cushion and he was in pain.  The  latest
> one that I mentioned in the email is just a thick padded seat raised on  one
> side because he has a little bit of scoliosis and it helps him to sit
> straight.  Also the back  is foam and is cut to conform to his back  and gives him a
> lot of support.  You need a good physical therapist to take  their time and
> work with him.  I used Advanced Medical in Baltimore,  Maryland and the PT is
> Janice Urban.  She is very  good.
>

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Re the Botox - a while ago someone wrote to me abtou their son with
arthrogryposis who got Botox and probably, since there is such a lack of
muscle cells, got a very undesirable worsening of generalized weakness.
I'm thinking that could have been because of a lack of meuromuscular
junctions to actually bind with the Botox and "absorb" it. If you know of
this person, and hopefully some of their generalized weakness improved as
the Botox wore off, I would really like to get in touch with them and
possibly write up and publish the case to make sure more people know NOT
to use Botox in lower motor neuron diseases.

Thanks,

Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids) at UAMS
and Arkansas Children's Hospital.  Working Mom of Sarah T. and Michael C.,
and wife of Henry Stefans. Every mom is a working mom!- OK, dads too.

On Fri, 29 Jul 2005, Gapko, Andrea M. wrote:

> The contractures surgery was painful for a few days after, but Jacob got
> by with tylenol after 1 or 2 days.  Took percocet one day and thought it
> was too much.  I hope you saw Pat Furlong's message about the Botox.
> That stuff scares me and it isn't appropriate treatment for DMD.  I'd
> take my son to a PT and/or physiatrist to address the pain issue.  We
> have a physiatrist who also manages the local pain clinic.  That
> combination should be helpful to you.

That sound like very good seating. The other thing we have some success
with is the Jay system. That is a line of cushions with countroured frim
foan bases and gel inserts on top. Roho cushions are super at releiving
pressure on your bottom, and used very commonly for adults with spinal
cord injuries and others who lack sensation, but they are also a very
unstable base of support, which may aggravate postural problems and
scoliosis particularly for children.

Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids) at UAMS
and Arkansas Children's Hospital.  Working Mom of Sarah T. and Michael C.,
and wife of Henry Stefans. Every mom is a working mom!- OK, dads too.

On Fri, 29 Jul 2005 JoyceGlass@... wrote:

> Beverly, my son used to have a Roho cushion and he was in pain.  The  latest
> one that I mentioned in the email is just a thick padded seat raised on  one
> side because he has a little bit of scoliosis and it helps him to sit
> straight.  Also the back  is foam and is cut to conform to his back  and gives
him a
> lot of support.  You need a good physical therapist to take  their time and
> work with him.  I used Advanced Medical in Baltimore,  Maryland and the PT is
> Janice Urban.  She is very  good.
>

It is worth a try. Anyone with a frozen joint can get neuropathic pain,
which basically responds a little to narcotics and hopefully much better
to Neurontin (gabapentin). This is the same kind of pain as in what used
to be called RSD or Reflex Sympathetic Dystrophy, now known as complex
regional pain syndrome. Some people need, and most people find they can
tolerate, fairly high doses, sometimes even up to a few grams a day. It
takes a few days to kick in so try a given dose for a few days before
moving up.

Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids) at UAMS
and Arkansas Children's Hospital.  Working Mom of Sarah T. and Michael C.,
and wife of Henry Stefans. Every mom is a working mom!- OK, dads too.

On Thu, 28 Jul 2005, CJohnson wrote:

> Vikki, I will ask about neurontin. My son needs some sort of relief as
> we can barely touch him without him saying ouch. And his skin is shiny
> and tight like you mentioned. Is that due to the muscle tightness? Thank
> you, CJ
>  ----- Original Message -----
>  From: Vikki Stefans
>  To: MD-List@yahoogroups.com
>  Sent: Thursday, July 28, 2005 5:21 PM
>  Subject: Re: [MD-List] Contractures
>
>
>  With the kind of pain you are describing, we've also had some success with
>  Neurontin. WHen  joint is fixed or "frozen" it can become painful, skin
>  sniy and atrophic, tender just to touch etc.
>
>  I often see people try muscle relaxers as if it was spasticity they are
>  treating, and even consider Botox, which as noted is generally not
>  indicated in muscle disease.  I had one patient say that baclofen helped a
>  little.
>
>  Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids) at UAMS
>  and Arkansas Children's Hospital.  Working Mom of Sarah T. and Michael C.,
>  and wife of Henry Stefans. Every mom is a working mom!- OK, dads too.
>
>  On Thu, 28 Jul 2005, CJohnson wrote:
>
>  > Pat, Thank you for your response to my contractures post. I just wish
>  > there were more options (as with all issues regarding dmd) to help with
>  > the pain. We had a doctor prescribe valium due to the intense
>  > cramping-but I don't like that for the long term. I can deal with the
>  > rigidity that makes it difficult to dress,lift etc but the pain it is
>  > causing my son is worrisome to say the least. We have a PT consult and
>  > an appt with a physical medicine doctor in 6 wks. There was mention by
>  > the doctor of medications we can use. I noted in your response there was
>  > no reference to available medications to help with the tight muscles. Do
>  > you have any informations with regards to that? Thank you so much for
>  > taking the time to respond. CJ mom to Gage 11 w/dmd
>
>
>  SPONSORED LINKS Muscular dystrophy  Critical illness  Mental illness
>        Terminal illness  Muscular dystrophy association  Chronic illness
>
>
> ------------------------------------------------------------------------------
>  YAHOO! GROUPS LINKS
>
>    a..  Visit your group "MD-List" on the web.
>
>    b..  To unsubscribe from this group, send an email to:
>     MD-List-unsubscribe@yahoogroups.com
>
>    c..  Your use of Yahoo! Groups is subject to the Yahoo! Terms of Service.
>
>
> ------------------------------------------------------------------------------
>
>

I know some people who read this won't agree with me, but I just wanted to say that when I hear of such promising research as this I think of all the money wasted, for instance, the billions that have gone into the war in Iraq, that could have been spent on researching further these promising cures.  I don't want to stir up  a lot of controversy and start a big political discussion but think of all the wasted money that could have been used to help our boys and girls with muscular dystrophy. 

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Tired of spam? Yahoo! Mail has the best spam protection around
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http://news.bbc.co.uk/2/hi/health/4725633.stm

Body's own cells may mend muscles

Satellite cells repair muscle
A type of cell found naturally in the body holds hope of treatments for
muscle diseases such as muscular dystrophy, say UK scientists.
It has long been suspected but never proved that satellite cells which coat
muscles can make new muscle.

The Medical Research Council and experts at University College London have
now shown this is the case, at least in mice.

Similar repair cells may be present in other tissues, they told Cell journal.

Self-repair

Stem cells are at an early stage of development and have the ability to
become almost any tissue type in the body.

Professor Terence Partridge and colleagues took between five and 25 mouse
satellite cells and transplanted or grafted them into some muscle tissue in the
lab.

They then measured the amount of new muscle and satellite cells produced.

Each grafted satellite cell produced new muscle.

   This is a step forward which, if shown to be applicable to humans, might
point a way to investigate new therapies

Muscular dystrophy expert Professor Kate Bushby

Some of the grafted satellite cells also replicated more of themselves,
expanding greatly in number by at least 10 times.

These new satellite cells were robust, being able to sustain vigorous
regeneration over at least two subsequent rounds of muscle damage in the lab.

The researchers said this not only proves that these cells are responsible
for muscle repair but also suggests that they share the same renewing properties
of stem cells.

Type of stem cell

Professor Partridge said: "We have established that the satellite cell in
muscle is a sort of stem cell.

"This is the first evidence of such a thing in a solid stable adult tissue
and suggests that this type of system may exist in other stable tissues.

"If we can work out what controls these satellite cells and what goes wrong
in the various conditions in which they seem not to keep up our muscle size and
strength, then we can devise rational ways of modifying the system to our
advantage."

His colleague, Dr Jennifer Morgan from Imperial College London, said the next
step was to see if the same was true in humans.

"It will take some time because there are very difficult problems to be
solved. The technique is complicated," she said.

Professor Kate Bushby from the Institute of Human Genetics at the University
of Newcastle said: "This work is significant because this shows there are
cells that have the potential to promote regeneration of muscle.

"This is a step forward which, if shown to be applicable to humans, might
point a way to investigate new therapies."

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

From: JoyceGlass@...

To: MD-List@yahoogroups.com

Sent: Thursday, July 28, 2005 6:28 PM

Subject: Re: [MD-List] Contractures

My son was experiencing a lot of pain in his hip area.  One doctor gave him a shot of cortisone and it really helped.  Also, the representative from our wheelchair provider (I think she is a physical therapist) came over and fitted him with a better seat and back with a lot more support and it helped tremendously.

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

From: Gapko, Andrea M.

To: MD-List@yahoogroups.com

Sent: Friday, July 29, 2005 2:10 PM

Subject: RE: [MD-List] Contractures

The contractures surgery was painful for a few days after, but Jacob got by with tylenol after 1 or 2 days.  Took percocet one day and thought it was too much.  I hope you saw Pat Furlong's message about the Botox.  That stuff scares me and it isn't appropriate treatment for DMD.  I'd take my son to a PT and/or physiatrist to address the pain issue.  We have a physiatrist who also manages the local pain clinic.  That combination should be helpful to you. 

 

My Jacob had the release surgeries on his ankles at age 22.  His brother has more contractures (neck, arms, hands, plus the hips, knees and ankles, but no surgery.  But little pain. 

 

Hope you find some help.  Jacob occasionally take tylenol at night if he can't get comfortable.  We also use many pillow to prop both boys legs at night.

 

Andrea

-----Original Message-----
From: MD-List@yahoogroups.com [mailto:MD-List@yahoogroups.com]On Behalf Of CJohnson
Sent: Wednesday, July 27, 2005 6:31 PM
To: MD-List@yahoogroups.com
Subject: Re: [MD-List] Contractures

Hi Andrea thank you for responding so quickly. That being said why do you think Botox or meds is not a good idea? My son is very uncomfortable and stretching isn't even an option. He got PT for several years and once he stopped walking we continued at home- since insurance didn't deem it necessary- and it did not stop the contractures. He also wore ankle-foot orthotics up until about a year ago. He stopped being able to tolerate them,not sure why. I am just concerned because it is hard to move him because of the pain and he gets excruciating cramps where he is in tears. Just putting his pants on he complains of pain. I find it odd the contractures progressed so rapidly since we take him out of his chair every day to sit on the couch or recliner. He has contractures all over his body. Can the tendon release be done on all joints? I hear it is pretty painful hence the reason we are looking for alternatives. Thanks for your input.

CJ

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

From: Gapko, Andrea M.

To: MD-List@yahoogroups.com

Sent: Wednesday, July 27, 2005 5:22 PM

Subject: RE: [MD-List] Contractures

I would not try either Botox or other meds for this.  Are you doing regular stretching and/or does he go for physical therapy on a regular basis?  I think these, plus massage, are probably the best things to do.  My son had the release surgeries at age 22 because his feet were rolling over on their outsides so much he couldn't comforably wear shoes any more.  If he needs the surgery, I'd do it sooner rather than later and put him in AFOs, if he doesn't already use them.

Hope this is helpful.

Andrea

----Original Message-----
From: MD-List@yahoogroups.com on behalf of CJohnson
Sent: Wed 7/27/2005 4:23 PM
To: MD-List@yahoogroups.com
Subject: [MD-List] Contractures

Hello All-
Just a quick question for everyone regarding contractures and treatment of them. My son is 11 and has been wheelchair bound for about 2 1/2 yrs. His contractures have gotten increasingly worse over the last few months. He is in an almost fixed position and is having pain. He gets muscle cramps that are excruciating to him. We are looking for alternatives to the surgery that is offered. Anyone try a physical medicine doctor or more specifically Botox injections or medications? Any thoughts or advice appreciated.
CJ mom to Gage 11 w/dmd

-----Original Message-----
From: MD-List@yahoogroups.com [mailto:MD-List@yahoogroups.com]On Behalf Of CJohnson
Sent: Wednesday, July 27, 2005 6:31 PM
To: MD-List@yahoogroups.com
Subject: Re: [MD-List] Contractures

Hi Andrea thank you for responding so quickly. That being said why do you think Botox or meds is not a good idea? My son is very uncomfortable and stretching isn't even an option. He got PT for several years and once he stopped walking we continued at home- since insurance didn't deem it necessary- and it did not stop the contractures. He also wore ankle-foot orthotics up until about a year ago. He stopped being able to tolerate them,not sure why. I am just concerned because it is hard to move him because of the pain and he gets excruciating cramps where he is in tears. Just putting his pants on he complains of pain. I find it odd the contractures progressed so rapidly since we take him out of his chair every day to sit on the couch or recliner. He has contractures all over his body. Can the tendon release be done on all joints? I hear it is pretty painful hence the reason we are looking for alternatives. Thanks for your input.

CJ

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

From: Gapko, Andrea M.

To: MD-List@yahoogroups.com

Sent: Wednesday, July 27, 2005 5:22 PM

Subject: RE: [MD-List] Contractures

I would not try either Botox or other meds for this.  Are you doing regular stretching and/or does he go for physical therapy on a regular basis?  I think these, plus massage, are probably the best things to do.  My son had the release surgeries at age 22 because his feet were rolling over on their outsides so much he couldn't comforably wear shoes any more.  If he needs the surgery, I'd do it sooner rather than later and put him in AFOs, if he doesn't already use them.

Hope this is helpful.

Andrea

----Original Message-----
From: MD-List@yahoogroups.com on behalf of CJohnson
Sent: Wed 7/27/2005 4:23 PM
To: MD-List@yahoogroups.com
Subject: [MD-List] Contractures

Hello All-
Just a quick question for everyone regarding contractures and treatment of them. My son is 11 and has been wheelchair bound for about 2 1/2 yrs. His contractures have gotten increasingly worse over the last few months. He is in an almost fixed position and is having pain. He gets muscle cramps that are excruciating to him. We are looking for alternatives to the surgery that is offered. Anyone try a physical medicine doctor or more specifically Botox injections or medications? Any thoughts or advice appreciated.
CJ mom to Gage 11 w/dmd

Joyce,

What specifically did you do to change the your son's wheelchair?
Our son, age 12, often has a lot of hip pain too.  He has a Roho
cushion and a custom back.  What type of cushion does your son have
and what did they do the back to give it more support?

Thanks.

Beverly

--- In MD-List@yahoogroups.com, JoyceGlass@a... wrote:
> My son was experiencing a lot of pain in his hip area.  One
doctor  gave him
> a shot of cortisone and it really helped.  Also, the
representative from our
> wheelchair provider (I think she is a physical  therapist) came
over and
> fitted him with a better seat and back with a lot more  support
and it helped
> tremendously.

Justin has been on prednisone(20mg daily) for 3yrs now. He has been hospitalized
for severe diarreah(he had Rotovirus)twice. He was hospitalized for 16 days due
to not eating and had two surgeries for g-tube placements, and he had minor
surgery. He was never given an extra dose or more prednisone. He never did or
has suffered any effects from not having it.
                  SAMANTHA
                  SAHM to
                  Halee--6/19/92
                  Justin--4/14/96
         www.caringbridge.org/tx/justind
                  Kennedy--3/25/01
            "You Will Dream New Dreams"


--- "berit_fff" <berit_fff@...> wrote:
Thank  you for your reply. It's good to hear stories like this.
Just want to clear up something. When I talk about "stress
situations"  I  don't  mean  when  a  person  get  stressed (by
worrying).
When I talk about stress situations this is a term used when
you  talk  about  a  hormon produced in the adrenal gland. This
hormon
helps  the  body  to cope with "stress situations", infections,
injury,
vomiting, diarreah and so on. When our kids
start on daily steroids, the adrenal gland will stop to produce
this
hormon.
My  question was; does anyone give a coverage dosage when their
child
gets into these stress situations. Our pediatrician did suggest


_____________________________________________________________
Netscape. Just the Net You Need.

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

From: Vikki Stefans

To: MD-List@yahoogroups.com

Sent: Thursday, July 28, 2005 5:21 PM

Subject: Re: [MD-List] Contractures

With the kind of pain you are describing, we've also had some success with
Neurontin. WHen  joint is fixed or "frozen" it can become painful, skin
sniy and atrophic, tender just to touch etc.

I often see people try muscle relaxers as if it was spasticity they are
treating, and even consider Botox, which as noted is generally not
indicated in muscle disease.  I had one patient say that baclofen helped a
little.

Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids) at UAMS
and Arkansas Children's Hospital.  Working Mom of Sarah T. and Michael C.,
and wife of Henry Stefans. Every mom is a working mom!- OK, dads too.

On Thu, 28 Jul 2005, CJohnson wrote:

> Pat, Thank you for your response to my contractures post. I just wish
> there were more options (as with all issues regarding dmd) to help with
> the pain. We had a doctor prescribe valium due to the intense
> cramping-but I don't like that for the long term. I can deal with the
> rigidity that makes it difficult to dress,lift etc but the pain it is
> causing my son is worrisome to say the least. We have a PT consult and
> an appt with a physical medicine doctor in 6 wks. There was mention by
> the doctor of medications we can use. I noted in your response there was
> no reference to available medications to help with the tight muscles. Do
> you have any informations with regards to that? Thank you so much for
> taking the time to respond. CJ mom to Gage 11 w/dmd

With the kind of pain you are describing, we've also had some success with
Neurontin. WHen  joint is fixed or "frozen" it can become painful, skin
sniy and atrophic, tender just to touch etc.

I often see people try muscle relaxers as if it was spasticity they are
treating, and even consider Botox, which as noted is generally not
indicated in muscle disease.  I had one patient say that baclofen helped a
little.

Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids) at UAMS
and Arkansas Children's Hospital.  Working Mom of Sarah T. and Michael C.,
and wife of Henry Stefans. Every mom is a working mom!- OK, dads too.

On Thu, 28 Jul 2005, CJohnson wrote:

> Pat, Thank you for your response to my contractures post. I just wish
> there were more options (as with all issues regarding dmd) to help with
> the pain. We had a doctor prescribe valium due to the intense
> cramping-but I don't like that for the long term. I can deal with the
> rigidity that makes it difficult to dress,lift etc but the pain it is
> causing my son is worrisome to say the least. We have a PT consult and
> an appt with a physical medicine doctor in 6 wks. There was mention by
> the doctor of medications we can use. I noted in your response there was
> no reference to available medications to help with the tight muscles. Do
> you have any informations with regards to that? Thank you so much for
> taking the time to respond. CJ mom to Gage 11 w/dmd

Dear Arlene

Thank you for your reply. It's good to hear stories like this.

Just want to clear up something. When I talk about "stress
situations" I don't mean when a person get stressed (by worrying).

When I talk about stress situations this is a term used when
you talk about a hormon produced in the adrenal gland. This hormon
helps the body to cope with "stress situations", infections, injury,
vomiting, diarreah and so on. When our kids
start on daily steroids, the adrenal gland will stop to produce this
hormon.

My question was; does anyone give a coverage dosage when their child
gets into these stress situations. Our pediatrician did suggest
this. If he is right, I think all parents should be told.


We had this discussion recently on Fight for a Future, and here is a
bit from a article posted by Dr. David Feder:


""MARIANNE SHAW, MD
Department of Rheumatic and Immunologic Diseases, The Cleveland
Clinic

A: ALTHOUGH THE QUESTION is controversial, I still recommend
perioperative steroid coverage for patients who are receiving more
than 5 mg/day of prednisone or an equivalent and for patients who
have recently stopped long-term steroid therapy. The stress dose
should be proportionate to the severity of surgical stress and
should be given for no longer than 1 to 3 days perioperatively.

Traditionally, patients on long-term corticosteroid therapy are
given a brief dosage boost in situations that cause acute
physiologic stress, such as surgery, trauma, infection, or severe
illness. Although the need for such "steroid coverage" or "stress
dosing" has recently been questioned, it seems prudent to continue
it for the present.

Unfortunately, stress dosing in patients on long-term corticosteroid
therapy has never been studied in large, randomized trials, and some
observational studies and small trials suggest that it may be
unnecessary. In this situation of uncertainty, clinicians should
weigh the physiologic and clinical evidence about the normal
hormonal response to surgery, the response in steroid-treated
patients, and the risks of adrenal insufficiency.""

You can find the whole discussion here:
http://groups.msn.com/fightforafuture/paediatrician.msnw?
action=get_message&mview=0&ID_Message=5070&LastModified=4675532415298
425426

You will also find a protocol, information of side effects, written
by Douglas Biggar. My concern is that many parents never have been
told some of the things written here.

I do agree with you that our kids shouldn't be worried by all the
bad side effects that might occur. But WE MUST know about them,
know what to look for and how to handle them.

Again, thank you for your reply Arlene.

Best wishes
Berit
http://groups.msn.com/FightforaFuture


--- In MD-List@yahoogroups.com, "Arlene " <breezytimber@y...> wrote:
> I think the more aperson is aware of problems any med can have the
> more they worry and actually make things worse.  Have you ever
> watched something on TV and began to worry if you may have that
> illness.  I have.  Therefore it is better not to really make the
> child aware of the problem with steroids.  Otherwise every time he
> gets a minor injury he may actually get sick because of worry
about
> it. Mike has always been treated normal, I can't think of one time
he
> was sick because of stress, injury or other minor reason because
of
> prednisone. Other than recently he felt sick when he had a stomach
> problem. Even with the operation and 2 other breaks in his life it
> didn't create a problem, being on prednisone and I didn't give him
> predniisone.  Don't remember if at the hospital the doctors did.
He
> had pneumonia,and before we took him to the emergency room, and no
> prednisone problem.
> Arlene
>
> --- In MD-List@yahoogroups.com, Vikki Stefans <vstefans@g...>
wrote:
> > I am sorry, I answered as best I knew how and I'm not sure what
to
> add! I
> > will ask one of our endocrinologists here for more information
when
> I get
> > a chance. In English, they call it a gland rather than glandule
too.
> >
> > I am sure the information applies to everyone who is taking
> steroids for
> > musclar dystrophy, not just you. I was only trying to give
> information
> > that complete shutdown of the adrenal gland will not ALWAYS or
> universally
> > occur with the dosage patterns we use.
> >
> > Actually, can we open the question to the whole list as to what
> they have
> > been advised re need for stress replacement doses while on
steroids
> if
> > any, after steroid tapering if any, and what evaluations have
been
> done
> > after steroid tapering if any??
> >
> > Thanks,
> >
> > Vikki Stefans, M.D., pediatric physiatrist (rehab doc for kids)
at
> UAMS
> > and Arkansas Children's Hospital.  Working Mom of Sarah T. and
> Michael C.,
> > and wife of Henry Stefans. Every mom is a working mom!- OK, dads
> too.
> >
> > On Thu, 14 Jul 2005, berit_fff wrote:
> >
> > > Dear Vikki
> > >
> > > Thank you for your answer. I still have some questions,
because if
> > > there is a thiny little posibility that our pediatrician is
right,
> > > this should be told to all parents.
> > >
> > > My concern is if a child use steroids daily, for several
years,
> the
> > > adrenal glandule will stop to produce this hormon which makes
the
> > > body capable to handle stress reactions. To have fever can
give
> the
> > > body a stress reaction, to have an injury (brake a leg,
> > > caraccident...) can also give the body a stress reaction. To
give
> > > double dose of steroids will then make the body more capable to
> > > handle the stress reaction.
> > >
> > > I am aware that once our children starts taking steorids on a
> daily
> > > basis, we cannot suddenly stop. We have to do this under the
> guidens
> > > of a pediatrician, slowly for several weeks.
> > >
> > > My question is how to handle stress reactions (fever, diarreah,
> > > vomit, injury) when the child starts taking steroids daily.
> > >
> > > Isn't it so that if a child use daily Deflazacort
> > > (0,9mg/kg/weight/day) for several years, the adrenal glandule
will
> > > stop produce this hormon - it will be insufficient.
> > >
> > > To be absulutely sure our children will not have any dangerous
> > > situations while they use daily doses of steroids, do you
think
> our
> > > pediatricians suggestion is something to consider, not just
for
> us -
> > > but for other parents too ?
> > >
> > > Best wishes
> > > Berit
> > > http://groups.msn.com/fightforafuture
> > >
> > > --- In MD-List@yahoogroups.com, Vikki Stefans <vstefans@g...>
> wrote:
> >
> > >> Berit, this is very important to think about. Steroid
medication
> should
> > >> not be stopped abruptly unles it has been used only a short
> time. The
> > >> dosages we are using are a little less than what is commonly
> used for
> > >> autoimmune diseases, but the potential for some degree of
> adrenal
> > >> suppression is there due to the long term use. If I have a
> patient who
> > >> tries steroids and they don't tolerate them well enough to
> continue,
> > >> they usually know this in less than a week or two and usually
> just stop
> > >> them. The other thing that reduces risks is that we often do
> weekend
> > >> dosing or other non-daily dosing patterns, and use
deflazacort
> or
> > >> prednisolone if possible instead of prednisone.
> > >>
> > >> We have not been doing stress doses of hydrocortisone for
fever
> > >> routinely, but it could be considered for major surgery,
> preferably
> > >> evaluated in advance. On the other hand, evidence is rolling
in
> that
> > >> the need for major surgery may be much less frequent (at
least
> > >> scoliosis surgery) in steroid-treated children.
> > >>
> > >> That's the thing - steroids, even the best ones with the best
> dosing
> > >> regimens, have side effects; when the evidence was less
> impressive for
> > >> benefits, a lot of clinics and clinicians, including me,
> hesitated to
> > >> use them. I even met a family who was basically thrown out of
> their
> > >> local MDA clinic for wanting to try them at all, no more than
5
> or 6
> > >> years ago.. Their child has become one of our success stories
of
> > >> treatment. But as Pat Furlong and others pointed out, a
little
> > >> impatiently, dystrophinopathy is a bad disease with a known
> prognosis
> > >> for progression, worse than several other conditions they are
> > >> practically considered mandatory for.
> > >>
> > >> I did have one patient who came to my clinic from another who
> was
> > >> stopping Prednisone and developed the withdrawal syndrome
with
> nausea,
> > >> and had already been referred to GI then Endocrinoology for
it
> when I
> > >> saw him; I haven't generated any of my own cases so far (hope
> that
> > >> continues..!)
> > >>
> > >> Besides slow tapering, a key is recognizing symptoms of
steroid
> > >> withdrawal and slowing down a tapering schedule if need be.
Here
> are
> > >> some excerpts from
> > >> http://www.endotext.org/adrenal/adrenal14/adrenal14.htm that
> seems like
> > >> pretty good information on the subject, reflecting the real
> complexity
> > >> of the situation quite well also (though not all of it
applies
> since
> > >> dystrophinopathy is not actually an autoimmune disease):
> > >>
> > >> ACUTE ADRENAL CRISIS
> > >>
> > >> Recovery of the hypothalamic-pituitary-adrenal axis can take
12
> months
> > >> or longer. Abrupt cessation of glucocorticoid treatment or
quick
> > >> tapering can precipitate an acute adrenal insufficiency
crisis.
> The
> > >> main symptoms range from anorexia, fatigue, nausea, vomiting,
> dyspnea,
> > >> fever, arthralgia, myalgia, and orthostatic hypotension to
> dizziness,
> > >> fainting, and circulatory collapse. Hypoglycemia is
occasionally
> > >> observed in children and very thin adult individuals. The
> diagnosis is
> > >> a medical emergency, and treatment should be immediate
> administration
> > >> of fluids, electrolytes, glucose, and parenteral
glucocorticoids.
> > >>
> > >> GLUCOCORTICOID WITHDRAWAL SYNDROME
> > >>
> > >> Glucocorticoid withdrawal can present as an acute adrenal
crisis
> or
> > >> with symptoms of chronic glucocorticoid deficiency. Thus,
> patients may
> > >> suffer from anorexia, myalgia, nausea, emesis, lethargy,
> headache,
> > >> fever, skin desquamation, arthralgias, weight loss, and
postural
> > >> hypotension. In addition, they may experience exacerbation of
> > >> previously present autoimmune disease (eg, rheumatoid
arthritis,
> atopic
> > >> dermatitis, or asthma) or have new autoimmune disease (eg,
> Hashimoto's
> > >> thyroiditis or Graves' disease). Amatruda et al. first
defined
> the
> > >> steroid withdrawal syndrome as a symptom complex resembling
true
> > >> adrenal insufficiency, with nonspecific symptoms like
weakness,
> nausea,
> > >> and arthralgias, occurring in patients who have finished a
> dosage
> > >> reduction of glucocorticoid therapy and who respond normally
to
> HPA
> > >> axis testing.
> > >>
> > >> The occurrence of the subjective component of the steroid
> withdrawal
> > >> syndrome does not depend on the absence of cortisol from the
> > >> circulation or an impairment of the hypothalamic-pituitary-
> adrenal
> > >> axis, because many of these symptoms may occur while on
proper
> > >> glucocorticoid replacement or while the patient has a normal
> cortisol
> > >> response to Cortrosyn (Organon Inc). In this instance, the
> steroid
> > >> withdrawal syndrome may be a result of difficulties in
> withdrawing from
> > >> the high levels of glucocorticoids - a phenomenon that
appears
> to be
> > >> idiosyncratic.
> > >>
> > >> However, when patients become ill after a dosage reduction,
the
> > >> physician should consider a differential diagnosis that
includes
> true
> > >> adrenal insufficiency, a flare-up of the disease being
treated,
> and
> > >> steroid withdrawal syndrome. All three conditions resolve
after
> > >> patients are restarted on the glucocorticoid regimen that has
> > >> previously controlled their symptoms.
> > >>
> > >> BIOCHEMICAL DIAGNOSIS OF ADRENAL INSUFFICIENCY
> > >>
> > >> Glucocorticoid treatment may not suppress the HPA axis at
all,
> or it
> > >> may cause central suppression or complete adrenal gland
atrophy.
> > >>
> > >>
> > >>
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
> > >>
> > >> So basically, it is not so black-and-white or absolute that
the
> adrenal
> > >> gland shuts down totally or even partially in two weeks of
> steroid
> > >> treatment. But your information from Ana Lucia is otherwise
very
> good
> > >> and very important to realize. Immunologic compromise is also
> variable
> > >> for every individual, and should be conisdered re
vaccinations;
> again,
> > >> though, I have not seen it being so severe that kids catch
every
> virus
> > >> that goes around or can't go to school or out in the
community
> because
> > >> of it.

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

From: pat

To: MD-List@yahoogroups.com

Sent: Thursday, July 28, 2005 6:42 AM

Subject: Re: [MD-List] Contractures

Dear Friends,

 

Botox is not recommended in DMD.  In an effort to provide appropriate information, I have consulted with experts in the field - posting their comments.

 

Dear Pat,

             Botox will not help for Duchene contractures because it works by paralyzing muscles. The nerve supply to that muscle is temporarily knocked out, using the botulinum toxin.  In CP this is very effective as it targets those spastic muscles which prevent other muscles from working, because the pull of the spasm is too great. When the spastic muscle is paralyzed the physical therapist can work to re-educate the other muscles and gain a more normal pattern of movement.  

 

  In Duchenne the contractures are caused by a completely different process and paralyzing the muscle even temporarily will have a detrimental effect on the boy’s already waning strength.   In DMD as the muscle cells die they are replaced by inelastic scar tissue similar to the scars you see on skin (as it is the same collagen material). Scar tissue needs to be constantly mobilized and stretched or it will firm up in a shortened position. When boys are sitting in a chair all day their hips knees and ankles are fixed in that position so the tightening occurs in the hip flexors, knee flexors, and the ankles. ( be careful of the upper limb also as contractures occur here also ( Elbows ,the ability to turn the palm upwards and the long finger flexors )   Your PT will have encouraged you to do a set routine of stretches every day to keep that scar tissue from fixing in a shortened position.    Once the contractures are there it is a little difficult to know what to do.  I would not contemplate surgery for hips and knees as the process is fraught with problems . Feet too can be a minefield as the small bones in the feet are very osteoporotic and the manipulations done after tendons are snipped to get the right position, can cause the bones  to break and the pain is severe.   If I find boys with severe contractures my intervention is palliative and directed to providing comfortable positioning in chair and bed and ensuring that the clothes are made to open up freely so that they do not have to be dragged over joints that will not move.  (Helen Posselt)

Sincerely,

Pat Furlong
PPMD

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

From: CJohnson

To: MD-List@yahoogroups.com

Sent: Wednesday, July 27, 2005 5:23 PM

Subject: [MD-List] Contractures

Hello All-

Just a quick question for everyone regarding contractures and treatment of them. My son is 11 and has been wheelchair bound for about 2 1/2 yrs. His contractures have gotten increasingly worse over the last few months. He is in an almost fixed position and is having pain. He gets muscle cramps that are excruciating to him. We are looking for alternatives to the surgery that is offered. Anyone try a physical medicine doctor or more specifically Botox injections or medications? Any thoughts or advice appreciated.

CJ mom to Gage 11 w/dmd

 

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

From: Adele

To: MD-List@yahoogroups.com

Sent: Thursday, July 28, 2005 7:03 AM

Subject: RE: [MD-List] Contractures

CJ,  The only “cure” for these contractures is intense P/T.  Stretching is the most important thing to do.  As to the surgery, is that the tendon release surgery?  We did this for Jason when he was 8-9.

 

My personal opinion is to stay away from Botox.  Think about it.  Does or has Gage been on prednisone?  If so, this increases the risk of infections.  Botox, is a bacteria, no matter how diluted it may be, it’s not something I want to enter the system of someone with a higher risk of infections.

 

Adele

 

---

From: MD-List@yahoogroups.com [mailto:MD-List@yahoogroups.com] On Behalf Of CJohnson
Sent: Wednesday, July 27, 2005 5:23 PM
To: MD-List@yahoogroups.com
Subject: [MD-List] Contractures

 

Hello All-

Just a quick question for everyone regarding contractures and treatment of them. My son is 11 and has been wheelchair bound for about 2 1/2 yrs. His contractures have gotten increasingly worse over the last few months. He is in an almost fixed position and is having pain. He gets muscle cramps that are excruciating to him. We are looking for alternatives to the surgery that is offered. Anyone try a physical medicine doctor or more specifically Botox injections or medications? Any thoughts or advice appreciated.

CJ mom to Gage 11 w/dmd

 

 

Prasad, yes, by all means the inactive vaccine.  Many times, after Jason was
DX, it was recommended that he have vaccines that are inactive.

Adele

-----Original Message-----
From: MD-List@yahoogroups.com [mailto:MD-List@yahoogroups.com] On Behalf Of
plsurampudi
Sent: Wednesday, July 27, 2005 9:35 PM
To: MD-List@yahoogroups.com
Subject: [MD-List] inactivated (not-live) vaccacine and its usefulness

I am planning to go to India and I am thinking about Typhoid
vaccination for my DMD son who is on deflazacort. I understand there
are two types of vaccinations: live and inactivated. I am thinking
about inactivated.

Since steriods dampen antibody response, do these vaccinations help?
What do the people do when they go on cruise to countries like Mexico.

Thank you for yor help

Prasad





Yahoo! Groups Links

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

From: CJohnson

To: MD-List@yahoogroups.com

Sent: Wednesday, July 27, 2005 5:23 PM

Subject: [MD-List] Contractures

Hello All-

Just a quick question for everyone regarding contractures and treatment of them. My son is 11 and has been wheelchair bound for about 2 1/2 yrs. His contractures have gotten increasingly worse over the last few months. He is in an almost fixed position and is having pain. He gets muscle cramps that are excruciating to him. We are looking for alternatives to the surgery that is offered. Anyone try a physical medicine doctor or more specifically Botox injections or medications? Any thoughts or advice appreciated.

CJ mom to Gage 11 w/dmd

 

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

From: CJohnson

To: MD-List@yahoogroups.com

Sent: Wednesday, July 27, 2005 6:31 PM

Subject: Re: [MD-List] Contractures

Hi Andrea thank you for responding so quickly. That being said why do you think Botox or meds is not a good idea? My son is very uncomfortable and stretching isn't even an option. He got PT for several years and once he stopped walking we continued at home- since insurance didn't deem it necessary- and it did not stop the contractures. He also wore ankle-foot orthotics up until about a year ago. He stopped being able to tolerate them,not sure why. I am just concerned because it is hard to move him because of the pain and he gets excruciating cramps where he is in tears. Just putting his pants on he complains of pain. I find it odd the contractures progressed so rapidly since we take him out of his chair every day to sit on the couch or recliner. He has contractures all over his body. Can the tendon release be done on all joints? I hear it is pretty painful hence the reason we are looking for alternatives. Thanks for your input.

CJ

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

From: Gapko, Andrea M.

To: MD-List@yahoogroups.com

Sent: Wednesday, July 27, 2005 5:22 PM

Subject: RE: [MD-List] Contractures

I would not try either Botox or other meds for this.  Are you doing regular stretching and/or does he go for physical therapy on a regular basis?  I think these, plus massage, are probably the best things to do.  My son had the release surgeries at age 22 because his feet were rolling over on their outsides so much he couldn't comforably wear shoes any more.  If he needs the surgery, I'd do it sooner rather than later and put him in AFOs, if he doesn't already use them.

Hope this is helpful.

Andrea

----Original Message-----
From: MD-List@yahoogroups.com on behalf of CJohnson
Sent: Wed 7/27/2005 4:23 PM
To: MD-List@yahoogroups.com
Subject: [MD-List] Contractures

Hello All-
Just a quick question for everyone regarding contractures and treatment of them. My son is 11 and has been wheelchair bound for about 2 1/2 yrs. His contractures have gotten increasingly worse over the last few months. He is in an almost fixed position and is having pain. He gets muscle cramps that are excruciating to him. We are looking for alternatives to the surgery that is offered. Anyone try a physical medicine doctor or more specifically Botox injections or medications? Any thoughts or advice appreciated.
CJ mom to Gage 11 w/dmd

I am planning to go to India and I am thinking about Typhoid
vaccination for my DMD son who is on deflazacort. I understand there
are two types of vaccinations: live and inactivated. I am thinking
about inactivated.

Since steriods dampen antibody response, do these vaccinations help?
What do the people do when they go on cruise to countries like Mexico.

Thank you for yor help

Prasad

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

From: Gapko, Andrea M.

To: MD-List@yahoogroups.com

Sent: Wednesday, July 27, 2005 5:22 PM

Subject: RE: [MD-List] Contractures

I would not try either Botox or other meds for this.  Are you doing regular stretching and/or does he go for physical therapy on a regular basis?  I think these, plus massage, are probably the best things to do.  My son had the release surgeries at age 22 because his feet were rolling over on their outsides so much he couldn't comforably wear shoes any more.  If he needs the surgery, I'd do it sooner rather than later and put him in AFOs, if he doesn't already use them.

Hope this is helpful.

Andrea

----Original Message-----
From: MD-List@yahoogroups.com on behalf of CJohnson
Sent: Wed 7/27/2005 4:23 PM
To: MD-List@yahoogroups.com
Subject: [MD-List] Contractures

Hello All-
Just a quick question for everyone regarding contractures and treatment of them. My son is 11 and has been wheelchair bound for about 2 1/2 yrs. His contractures have gotten increasingly worse over the last few months. He is in an almost fixed position and is having pain. He gets muscle cramps that are excruciating to him. We are looking for alternatives to the surgery that is offered. Anyone try a physical medicine doctor or more specifically Botox injections or medications? Any thoughts or advice appreciated.
CJ mom to Gage 11 w/dmd