Supertraining

--- Ian <[EMAIL PROTECTED]> wrote:

> Hi all
> I have a new client who has McArdle's syndrome who
> wants to start using 
> the gym. Any pointers or help from anyone would be
> appreciated. I know 
> what the syndrome is, and that they should avoid
> prolonged or intense 
> exercise. However, is it possible for them to do
> strength work if they 
> use very short sets and long breaks for instance?
> Any ideas for what 
> sort of cardio protocol they might be able to
> tolerate would be 
> appreciated aswell
> 
> Ian Pateyjohns B.Appl.Sc 
> Adelaide Australia

I don't know much about the disease other than what I
have read in the medical literature. This disease is
one of many inherited diseases known as glycogen
storage diseases.

<<The rare muscle diseases in which an enzyme
deficiency interferes with substrate utilization as
fuel for exercise (e.g., McArdle's disease) are often
associated with severe, exercise-provoked muscle
contractures. These contractures are electrically
silent by electromyography, in contrast to the intense
motor unit activity seen with cramps. Contractures
must not be confused with the limitation of joint
range of motion resulting from long-standing joint
disease or long-standing weakness—also termed
contractures

Chapter 213 – GLYCOGEN STORAGE DISEASES  Goldman:
Cecil Textbook of Medicine, 22nd ed., Copyright © 2004
W. B. Saunders Company .>>

I would imagine that the best type of exercise for
this individual might be low intensity endurance work
were the major fuel source is fat rather than glucose
or glycogen.  

<<ATP is the intracellular currency of life; it is
used for muscular contractions, to maintain the
integrity of the cell membrane, and for many other
vital functions. ATP is generated by two primary
mechanisms: oxidative fatty acid oxidation and
anaerobic glycolysis ( Fig. 109-2 ). 

Failure in either of these pathways can lead to ATP
depletion and can lead to muscle necrosis. 

The integrity of each of these pathways depends on
many different enzymes, and defects in these enzymes
can cause rhabdomyolysis that is associated with
depletion of ATP during exercise ( Table 109-3 ). 

Of these defects, carnitine palmitoyltransferase
deficiency is the most common hereditary aerobic
disorder causing rhabdomyolysis, and myophosphorylase
deficiency (McArdle's disease) is the most common
anaerobic disorder causing rhabdomyolysis

Chapter 109 Rhabdomyolisys-Goldman: Cecil Textbook of
Medicine, 22nd ed., Copyright © 2004 W. B. Saunders
Company >>

High intensity exercise requiring replenishement of
ATP via the glycogen pathway will lead to
rhabdomyolysis which is  muscle damage and extreme
rhabdomyolysis can cause kidney shutdown and in rare
cases death. 

Several years ago there was a cholesterol lowering
drug which was taken off the market because in rare
cases it caused severe rhabdomyolysis and there were
several fatalities due to acute kidney failure.

Unfortunately for this individual no amount of
training will improve glycogen storage. 

- I would discuss this patients condition with his/her
physician and get recommendations from specialists in
this disease.

Ralph Giarnella MD 
Southington Ct USA