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What is
ALD?
ALD is a progressive
condition that affects the adrenal glands and the insulation
sheath of the brain called myelin. The myelin sheath is
the fatty white matter that acts as an insulator on nerve fibers in
the brain. It has a similar function to the plastic covering
of electrical cord.
The movie "Lorenzo's
Oil" MCA/Universal City Studios 1992, is based on this
disease. Although it was very emotional for
loved ones of those
inflicted with ALD to watch, it is also educational. If you
would like a better understanding of ALD, we recommend you rent
it. While the movie implies a cure is found, the oil has not
been successful with all boys. Current studies do show
that lowering the very long chain fatty acids (VLCFA) before
age six and the onset of symptoms does improve the chances of
avoiding symptoms. However, Lorenzo's Oil is still not FDA
approved or available to the general public.
The symptoms of ALD are a
result of absent or malfunctioning enzymes
within the
peroxisomes. The peroxisomes are tiny structures in the cells
of the body that help to break down large molecules of fats into
smaller ones so that they can be used by the body. In ALD, the
peroxisomes cannot break down a type of fat called very long chain
fatty acids, (VLCFA). Because the VLCFA cannot be broken down,
they accumulate throughout the body in the plasma, especially in the
brain and the adrenal glands. While it is unclear if the VLCFA
cause the deterioration or are simply another symptom of the
disease, the destruction of the myelin sheath surrounding the nerves
leads to neurological problems, and the adrenal gland malfunction
leads to Addison's Disease.
Part of the job of the
adrenal glands is to use cholesterol to make steroids. These
steroids are used to help the body properly use sodium and potassium
and to break down proteins, carbohydrates and other fats.
Almost all males that are affected with ALD have problems with their
adrenal glands functioning correctly. One of the symptoms of
an adrenal problem or Addison's Disease is dark skin. Thus,
boys often appear healthy
with the appearance of a beautiful tan. One way to
differentiate between dark skin from Addison's and a tan is the even
darker markings in the creases of the skin, as well as in scratches
or scars.
How Do You Get ALD?
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