CALIFORNIA STATE UNIVERSITY, NORTHRIDGE
AN EDUCATIONAL FILM
,,
"EARLY DUCHENNE MUSCULAR DYSTROPHY
FOR TEACHERS AND AIDES"
A Graduate Project submitted in partial satisfaction of
the requirements for the degree of Master of Science in
Health Science
by
Randall Stuart Benson
June, 1976
The Graduate Project of Randall Stuart Benson is
approved:
Dorothy Hash
California State University, Northridge
ii
I would like to acknowledge the assistance of the following
people without whose cooperation this film would not have been made.
George and Norma Bennett
Executive Committee Members
Muscular Dystrophy Association, Inc.
Los Angeles County Chapter, California
John D. Hsu, M.D.
Chief, Muscle Disease Clinic
Orthopedic Surgery
Rancho Los Amigos Hospital
Downey, California
Muscular Dystrophy Association, Inc.
Los Angeles County Chapter, California
Los Angeles County Crippled Children's Service
North Hollywood Medical Treatment Unit
Los Angeles, California
Cathy Schmidt, R.P.T.
Department of Neurology
USC - Los Angeles County Medical Center
Los Angeles, California
David M. Shoop
Instructional Media Specialist
USC School of Medicine
Los Angeles, California
The "Boys" who made this film possible
iii
TABLE OF CONTENTS
Page
Abstract ....•............................................. v
I. Introduction .......................................•...... 1
What is Duchenne Muscular Dystrophy?
II. Script ...........•........................................ 2
III. References ....•........................................•.. 10
iv
ABSTRACT
"Early Duchenne Muscular Dystrophy for Teachers and Aides"
by
Randall Stuart Benson
Master of Science in Health Science
The idea for this educational film is the result of my own
experiences, and from observing and talking to other persons who
worked with physically handicapped individuals.
Many people were not
prepared to identify boys with Muscular Dystrophy.
Some people could
not discern the difference between children who had Cerebral Palsy,
and those who had Muscular Dystrophy.
In some cases, people assumed
that all physically handicapped children they were observing, or
working with, had Cerebral Palsy.
In order to try to prevent occurrences such as these from happening, this educational film about Duchenne Muscular Dystrophy has been
prepared.
The objective of this film is to enable teachers and aides to
identify boys in the early stages of Duchenne Muscular Dystrophy.
v
INTRODUCTION
What is Duchenne Muscular Dystrophy?
(Pseudohypertrophic Muscular Dystrophy)
A Neuromuscular Disease
"The child appears normal at birth.
The clinical signs become
evident only after the upright posture has been achieved.
Weakness
of the muscles which support and motivate the pelvic girdle becomes
apparent by the patient's lack of agility and impaired performance in
climbing stairs.
Gait becomes· broadbased and later waddling.
Lordosis
develops and becomes increasingly accentuated over a period of years,
ultimately resulting in a pronounced swayback.
enlarge.
Calf and other muscles
Muscle stretch reflexes gradually disappear, following a
general pattern, the ankle reflex being the most persistent.
gradually become distorted into a posture of equinovarus.
The feet
Differential
wasting of the pectoralis major muscles occurs as other trunk and
shoulder girdle muscles deteriorate.
mandatory.
Wheelchair existence becomes
Contractures form at ankles, knees, hips, and later at
the elbows, shoulders and wrists.
becomes reduced.
Scoliosis appears.
Vital capacity
With continuing enfeeblement the patient becomes
bedfast, and eventually the disease is terminated, commonly by a
respiratory infection" ( 2:19-20).
l
SCRIPT
"Early Duchenne Muscular Dystrophy for Teachers and Aides"
Written by Randall S. Benson
Scene
Narrator
Time
l.
Credits
(28 seconds)
Music
2.
Boy walking down a hill, up a ramp
and down a ramp.
(40 seconds)
This film was developed for teachers and
aides in the area of Special Education who
plan to work with Duchenne muscular dystrophy
children. This film is intended to assist
one to gain an insight into "how" children
function physically. By utilizing this film
as an educational tool, teachers and aides
can be better prepared to identify and work
with these children more effectively.
The children you will see in this film are
in the early stages of Duchenne muscular
dystrophy.
Once confined to wheelchairs boys with Duchenne's MD resemble many other children
with handicapping conditions who are also in
wheelchairs.
1\)
Scene
3.
Time
Boy in a wheelchair in an
animal pen.
(25 seconds)
4.
Boy walking with the help of a
young man.
(37 seconds)
5.
50%
(8 seconds)
6.
Boy walking with the help of a
young man.
(11 seconds)
Narrator
In the later stages of Duchenne's MD you may
have difficulty in identifying those with the
disease from other boys with similar diseases, and therefore will have to ask the
boys about their condition, or check your
school's medical records, or contact parents
to confirm the type of handicap.
Pseudohypertrophic muscular dystrophy, also
known as Duchenne muscular dystrophy, is a
neuromuscular disease. The symptoms of
Duchenne's usually manifests themselves in
children between the ages of two and six
years. However, it sometimes attacks soon
after birth. This disease is a hereditary
condition. Genetically, it has a high spontaneous mutation rate (5:3). A large number
of victims are stricken without previous
family history. Duchenne dystrophy is transmitted by the female to the male offspring.
Mothers who are carriers have up to 50 percent of their male offspring develop the
disease, while 50 percent of their female
offspring may carry the defective Duchenne's
MD gene.
At this time, some evidence suggests a
relationship between mental retardation or
learning problems with some of the affected
boys.
w
Scene
Time
7.
Mother talking to a doctor.
(18 seconds)
8.
Boy walking with the help of a
young man.
(25 seconds)
Boy swimming.
(28 seconds)
9.
Narrator
Diagnosis of Duchenne dystrophy can be made
prior to the onset of clinical signs by
means of the CPK serum enzyme blood test,
muscle biopsy analysis, and electromyographic readings. The blood test can also
be given to females to determine whether
they are carriers of the disease.
The progression of this type of muscular
dystrophy is rapid with no remission. With
this the most common and severe type of
dystrophy, death usually occurs within 15
to 20 ·years of the onset of clinical symptoms. Today, life is prolonged in many
cases due to recent advances in medical
science.
While viewing this film, it is important for
you, as teachers, or aides, to be concerned
with what Duchenne's MD boys can do in their
handicapped condition. You will have to
adapt their classroom and outdoor activities
to the progressive degeneration of the individual being, so that they may experience
success and hence develop positive self
images.
While first observing Duchenne's MD boys ...
.::::--
Scene
10.
Boy out of focus running,
to him in focus walking.
Time
(8 seconds)
Narrator
... they may appear to be "normal" when
engaging in physical activities.
As the youngsters begin to lose muscular
strength .....
11.
Same boy as in scene 10, placing
his hands on knees as he walks
up steps.
(8 seconds)
Shots of calf muscles from
rear and front.
(14 seconds)
Boy being released by a young
man and walking by himself.
(19 seconds)
14.
Boy standing up by himself.
(8 seconds)
15.
Close-up of a boy's face and
then him standing up.
(6 seconds)
12.
13.
... they will place their hands upon their
knees for support when walking up steps.
Many boys will appear to be healthy during
the initial stages of the disease. Their
calf muscles will appear extremely large,
but in reality the muscle tissue is being
replaced by fatty tissue.
In the early stages of the disease, the
ability to walk deteriorates as the disease
progresses. Note the lordosis and the loss
of balance which sets in, causing the boys
to waddle while walking.
As the disease progresses, the boys will
experience more difficulty standing up.
When standing up they will lose the fluid
motion .....
... that non-handicapped persons exhibit
and as a result will have to work harder to
stand themselves up.
Vl
',>,".
Scene
16.
Boy on all four limbs standing
up and then walking.
Time
(39 seconds)
Narrator
As the children lose the ability to stand
up, they finally reach the stage where they
demonstrate the Gower's Syndrome. The
Gower's Syndrome is observed when the boys
have to "walk" up their bodies in order to
stand up.
Subsequently the boys also lose the ability
to run.
17.
18.
Boy crawling to pool deck ladder
and pulling himself up to standing
position.
(23 seconds)
Boy walking, then he receives help
from a young woman to stand, and they
both walk away.
(42 seconds)
When the children are unable to stand by
"walking up their bodies," they will require
assistance from external sources.
The upper body eventually becomes so weakened that the boys can no longer pull themselves up under their own power and will
require assistance from others in order to
stand. Because of this lack of strength
they may also require assistance when
setting their bodies down.
Be sure when lifting the children that they
have their balance before letting go. Also,
be sure that you are lifting properly to
prevent back injuries to yourself.
0'\
Scene
19.
20.
21.
Boy walking down an incline,
up a ramp, and in a door with
support from a young man.
Boy standing with his chest
being stretched.
Boy in a wheelchair with his
chest being stretched, and then
a young woman attempts to lift
him from the chair.
Time
(21 seconds)
(5 seconds)
(34 seconds)
Narrator
The children become progressively weaker
and often require support to keep their
balance when walking. They will maintain
their balance by support from objects and
people.
When Duchenne's MD children become weaker,
their chest muscles become flaccid and
stretch when the boys are being lifted.
... by their arms and chest. This might
remind you of lifting a big water balloon;
you have to gently, but firmly, cradle
their chests to prevent them from slipping
through your hands.
In order to prevent the children from being
injured, don't lift them by their arms.
If you are not sure about the correct procedure for lifting the children, without
causing injury to them, ask them, for they
probably can tell you a way of lifting
them successfully.
22.
Boy walking with a walker.
(5 seconds)
Walkers are used to prevent the boys from
falling and as a means to keep them walking
longer .....
23.
Boy walking wearing a helmet.
(5 seconds)
. .. Many boys wear helmets to protect
themselves from head injuries when falling.
~
Scene
24.
25.
26.
Time
Boy swinging a whiffleball
bat while using a walker.
(11 seconds)
A young man and a boy
using a walker, walking.
( 8 s·econds)
Boy wearing braces, walking.
(23 seconds)
Narrator
... There is a danger of fractures to the
legs and feet of the boys, as the disease
progresses. Their play areas should be
kept safe in order to minimize the risks of
injuries .
... If the boys break a foot or leg while
they are still mobile and are placed in
wheelchairs, they may never be able to walk
again.
Prior to, or shortly after the children can
no longer walk by themselves, an operation
on the lower extremities may be performed
to release the muscle contractures which
inhibit standing and walking. This procedure in conjunction with the use of full
leg braces prolongs ambulation for about
two years.
Here again, a walker may be used to prevent
falling, and a helmet may be worn to prevent injury.
27.
Boy in a wheelchair wheeling
himself.
(13 seconds)
If the surgery is not performed, the boys
will have to be placed in wheelchairs in
order to maintain any degree of mobility.
():)
Scene
Time
Narrator
28.
Close-up of ankles and feet.
(14 seconds)
29.
Boy in a wheelchair trying to
swing a baseball bat.
(12 seconds)
30.'
Boy swimming.
(43 seconds)
31.
Boy wheeling himself away from
the camera in his wheelchair.
(19 seconds)
32.
Trees and sky.
(13 seconds)
Music
33.
THE END
(10 seconds)
Music
Ankles and feet show contractures in
Duchenne's muscular dystrophy. Hands and
arms lose strength as the children become
weaker. Observe how the boy is trying ..•
... to swing a regular baseball bat, and
cannot.
It is not believed that exercise would be
of value for making muscles stronger.
However, exercise does assist boys to
maintain their existing muscle tissue.
Exercise prevents atrophy due to lack of
muscle use in addition to assisting to keep
their lung capacity greater.
The progression of the disease is such that
the boys will ultimately be placed in
wheelchairs, then be bed-ridden, and finally
expiration of life due to such causes as
pneumonia or heart failure.
v
\0
10
REFERENCES
l.
Around the Clock Aids for the Child lvith Muscular Dystrophy.
Muscular Dystrophy Association, Inc., New York, N.Y.,
Pamphlet No. Pl46 50M 2/75.
2.
Bonsett, Charles A. Studies of: Pseudohypertrophic Muscular
Dystrophy. Charles C. Thomas, Springfield, Illinois, 1969.
3.
Chart of Differential Diagnostic Characteristics of the Primary
Diseases Affecting the Neuromuscular Unit. Muscular
Dystrophy Associations of America, Inc., New York, N.Y.
4.
For the Detection of Female Carriers of Duchenne Muscular
Dystrophy: The CPK Test. Muscular Dystrophy Association,
Inc., New York, N.Y., Pamphlet No. Pl92 25M 4/75.
5.
Muscular Dystrophy: Fact Sheet. Muscular Dystrophy Association,
Inc., New York, N.Y., Pamphlet No. Pl87-l00-2/75.
6.
Ogg, Elizabeth. Milestones in Muscle Disease Research.
Muscular Dystrophy Associations of America, Inc., New York,
N.Y., Pamphlet No. Pl90-500M-87l.
7.
Patient & Community Services. Muscular Dystrophy Association,
Inc., New York, N.Y., Pamphlet No. Pl05 lOOM l/75.
8.
Pearson, Carl M. Muscular Dystrophy: Review and Recent
Observations. Muscular Dystrophy Associations of America,
Inc., New York, N.Y., Pamphlet No. Pl59-3M-8/67.
9.
Sherwin, Albert C. Psychological and Emotional Aspects of
Muscular Dystrophy. Muscular Dystrophy Associations of
America, Inc., New York, N.Y.
10.
The Muscular Dystrophies. Muscular Dystrophy Associations of
America, New York, N.Y., Pamphlet No. Pl30-lOM-6/70.