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DUCHENNE
MUSCULAR DYSTROPHY
By Jade
Cognetti
continued
from page
one
EXERCISE
THEREAPY AT AN EARLY AGE (0-7)
The provision
for all individuals with DMD is to encourage
activity as is tolerated without forcing
exhaustion. Boys with DMD at a young
age should be exposed to a wide range
of activities to promote well-rounded
development and contribute to his cognitive,
neuromuscular, social, emotional, and
organic maturity. Additionally, allowing
the long bones to bear weight helps
to improve bone density and strength.
Ball games and sensory motor games will
improve gross motor skills and encourage
future learning. Water exercises and
hydrotherapy
are highly encouraged; these activities
are concentric exercises that cause
muscle fibers to shorten when firing,
and also take place without the resistant
(damaging) force of gravity. As a base
guideline, participation in normal,
developmentally appropriate activities,
and the ability to keep up with peers,
provide sufficient exercise for children
with DMD (PPMD,
2008).
It is of
upmost importance to initiate a flexibility
routine from an early age. Calf muscles
are likely to be the first to tighten,
and thus heel-cord or tendon-Achilles
stretches should be incorporated. Other
areas of focus at this age should be
hamstrings,
the iliotibial
band, and ankle joints. Stretching
should follow a warm up exercise, and
the intensity of the stretch should
always be sub maximal (PPMD,
2008).
EXERCISE
THERAPY FOR MIDDLE AGES (7- early teens)
At this age,
the child should choose activities at
his own level of participation, and
all activities should be balanced with
appropriate rests. Swimming is encouraged
and provides respiratory therapy in
the form of lung expansion and controlled
exhalation (PPMD,
2008). The buoyancy of the water
helps assist weak muscles, and breath
holding underwater exercises respiratory
muscles (MD A,
2009).
The stretching
regime from early years should continue
and be extended to include hip flexors
and stomach muscles. Research illustrates
that combining orthotics
with indicated stretching is one of
the best methods to reduce contracture
development (PPMD,
2008).
EXERCISE
THERAPY FOR MIDDLE AGES ( LATE TEENS
+)
At this stage,
people with DMD start to feel the degenerative
effects in the upper limbs. As before,
active exercise in the pool affords
an excellent opportunity for easy-movement
exercise and freedom from a wheelchair.
Time lying on a wedge also grants liberty
from the chair and provides stretching
for hips and knees. The regular flexibility
routine should now be extended to include
the tibialis posterior muscle and elbow
flexors (PPMD,
2008).
Through the
transition from manual to electric wheelchair,
the boy may choose to participate in
wheel-chair sports and become involved
in an organized team. Many people with
DMD also enjoy water sports well into
their 20s (MDA,
2009).
There is
no cure for DMD, only tremendous amounts
of research that offer hope for the
near future. Research supported by the
National Institute of Arthritis and
Skin Diseases (NIAMS)
has demonstrated that injecting a fragment
of protein called heregulin
(HRG peptide) into mice with a disease
similar to DMD improves the structure
and function of the mice muscles. Scientists
showed that heregulin was able to increase
the production of another muscle protein,
utrophin,
which is structurally and functionally
similar to dystrophin.
Although only tested on animals, this
study provides hope for using the body's
own cells to produce and regulate utrophin
to improve the dystrophic
phenotype
(Krag, et al.,
2004).
In 2009,
the National Institute of Health (NIH)
announced the results of a study that
relied on an intravenous cocktail of
morpholinos.
Morpholinos are artificial molecules
that mimic and bind to DNA.
Through the process of exon skipping,
these molecules act as patches that
forward the production of dystrophin
that would have otherwise failed due
to genetic mutation. The study was carried
out on dogs, and the results demonstrated
sufficient dystrophin production to
halt deterioration and "make a
real difference." A draw-back to
this method is that it failed to restore
dystrophin production in the heart;
however, further research suggests that
this may be possible with the inclusion
of a cell-penetrating peptide.
The intravenous nature of morpholinos
uses the blood stream to circulate the
treatments, which is a significant and
practical technique in the hunt for
a cure (Browne,
2009).
Other research
is currently being focused on methods
such as gene replacement therapy, drug
intervention, and myoblast
transplants. Scientists hope to
produce a synthetic gene which can be
introduced into the body and replace
the job of producing the missing protein
dystrophin. Recent science has also
led to development of the drug PTC
124 which is currently in clinical
trials. This drug attempts to 'read
through' and ignore the change in the
dystrophin gene that causes DMD and
BMD.
Myoblast transplant is a procedure in
which donor cells are injected into
damaged muscle in the hope that they
will fuse with the degenerating muscle
and create new, normal muscle fibers.
This treatment has not yet proven effective
(MDA, 2009).
The cure
for DMD is greatly a funding problem
which is making strides through efforts
of awareness and the auspicious prospect
of a cure. The 2007 multi-award-winning
documentary, "Darius
Goes West," followed 15 year
old Darius Weems (a boy with DMD) and
his friends as they traveled west from
Athens, Georgia in hopes of "pimping"
Darius' wheelchair on the MTV show "Pimp
My Ride" (DariusGoesWest.org,
2008). The story captures the
every-day life of living with DMD while
celebrating his cross-country voyage
and raising awareness for the disease;
the documentary has since expanded into
a web-based resource and donation forum.
Initiatives such as this advance strides
towards a cure, yet serve as a poignant
reminder of living courageously and
gratefully despite a degenerative illness.
references
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about the author,
Jade Cognetti:
Jade graduated from
the University of Florida in 2009 with
a B.S. in Health Education & Behavior
as well as a B.A. in Spanish. She is
currently applying to master's degree
programs in Human Genetics, and hopes
to work within the public health arena.
Her interests include science, travel,
learning foreign languages, and coaching
lacrosse.
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