| Approaches on DMD |
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A. Special Care |
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Physiotherapy |
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Physiotherapy involves a program of stretching exercises
to maintain muscle length and the flexibility of joints. Physiotherapists
also work with orthotists, who make or provide various pieces of equipment
to maintain posture and mobility. Night splints, calipers, swivel
walkers and braces are some of the aids used. Physiotherapists are
the main people involved in showing parents the exercises and in making
sure that any mobility aids are comfortable.
At the time of diagnosis or shortly after, boys with Duchenne muscular
dystrophy have a tightening or shortening of the tendon Achilles (heel
cord). One of the first tasks of the physiotherapist is to show parents
how to stretch the heel cords. It is best if this can be done by the
parents at home several times per day. These daily exercises will
be maintained over years and it can be difficult to make sure they
are always completed. Parents should not feel guilty if occasionally
they have to miss a stretching exercise. Sometimes a night splint
is also prescribed. This is a polypropylene (plastic) splint that
fits over the foot and behind the ankle and calf. It keeps the foot
at 90 degrees to the leg and as a result the heel cord is kept stretched
rather than shortened during the night. Some boys find the night splints
are too uncomfortable and sometimes their use has to be discontinued.
It is important for parents not to blame their child or to feel guilty
themselves if the night splints are not tolerated.
As muscle weakness increases and various tendons become tighter the
physiotherapist will increase his or her involvement with stretching
exercises and sometimes the fitting of splints or braces to maintain
the ability to walk. At the appropriate time the physiotherapist will
be very involved in obtaining the correct wheelchair and in helping
to solve many seating and other problems that arise. Keeping the patient
sitting upright as much as possible to minimize the development of
scoliosis (curvature of the spine) is important. Hydrotherapy |
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Surgical Support |
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Sometimes boys with Duchenne muscular dystrophy require operations
to correct contractures (joints out of alignment) and scoliosis (curvature
of the spine). They are not used in every case and are only offered
after careful assessment of all medical considerations. Orthopedic
surgeons do these operations.Because weakness does not equally affect
all muscles that move a joint, some joints can be pulled out of alignment.
If this persists for long enough it will not be possible to get the
joint back into its correct position. This is called a contracture.
The ankles, knees and hips are places where contractures occur early.
In the later stages joints in the arms are also affected. Toe
walking (walking on "tip toes" or the ball of the foot)
is due to tightening of the Achilles tendon at the back of the ankle.
This tightening (equine deformity) prevents the heel striking the
ground properly. Particularly in the wheelchair stage, other muscles
around the ankle also become tight. If measures are not taken to
minimize this tightening, the front of the foot may not only point
down but the whole foot may start to turn under so that the outer
side of the foot rests on the foot plate of the wheelchair (equino-varus
deformity). As mentioned previously, the use of physiotherapy and
plastic splints can help slow down the development of these conditions.
If toe walking proves to be a problem or if in the wheelchair phase
the deformity causes problems or is cosmetically unacceptable, an
operation can be performed to correct the malalignment. This operation
is called elongation of the tendon-Achilles (ETA). Under some circumstances
a tendon may be transferred from the back to the front of the ankle
at the time that the ETA is being performed, to help pull the front
of the foot up. This is called a tibialis posterior transfer operation.
The feet need to be in a plaster cast for about 3 weeks after an
ETA, but for longer after the transfer operation. Hence the procedure
is usually reserved for patients who cannot walk. |
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Treating Scoliosis |
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A major problem during the wheelchair phase may be the development
of scoliosis (curvature of the spine). The muscles on either side
of the spine help to keep the bones of the spine in the correct position.
If the muscles are weakened, no matter what the cause, the bones will
drift out of alignment, usually sideways but also with some rotation.
Parents may notice that there is more of a "hump" on one
side of the back of the chest than the other. This is due to a rotation
of the bones of the spine and results in the ribs being more prominent
on one side. If parents notice any evidence of scoliosis they should
report it to the physiotherapist or doctor. Although scoliosis does
not always develop, once a significant spinal curvature has occurred
it is likely to get worse over the years and will require treatment.
If untreated, spinal curvature can cause discomfort, reduce breathing
capacity; interfere with the use of the arms and cause difficulty
for caregivers during activities such as dressing. Sitting in a wheelchair
can become more uncomfortable. There can also be significant psychological
effects on self-image. Attempts to stop increasing curvature by
various body jackets and bracing devices have not been successful.
It is now felt that once this problem has developed the only reliable
way to prevent deterioration is to operate to straighten the spine
and fuse the bones together by bone grafts. The spine is kept straight
with various rods and wires attached to the bones while waiting
for them to fuse solidly together. The rods do not need to be removed.
It is usually performed at 13 or 14 years of age. Although the operation
is a major one, most boys have tolerated it well and have benefited
physically and psychologically from having it done.
Tightening of muscles and fibrous tissue at the front of the hips
and around the knees occurs as mobility decreases. This tightening
increases in severity once the wheelchair stage is reached. Similar
tightening develops in the arms at a later stage than in the legs.
Operations are not needed in the arms or hands but sometimes they
are required at the hips. Occasionally operations other than those
mentioned above are necessary. It should be remembered that not
all boys with Duchenne muscular dystrophy require all, Sometimes
even any, of these operations. |
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Respiratory Care |
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Both physiotherapy and hydrotherapy contribute significantly to
good respiratory function. Boys with Duchenne muscular dystrophy are
no more susceptible to infection than others and in the early years
there is no need for anything other than "routine" treatment
of coughs and colds. Later on, however, particularly in the wheelchair
stage, the chest muscles will be weaker than usual and it will be
more difficult to cough up secretions that accumulate in the breathing
passages. This leads to what is called collapse and consolidation
of the lungs. Therefore, should a head cold or an upper chest infection
develop early use of antibiotics and particularly chest physiotherapy
is usually recommended to help prevent more serious complications.
Sometimes hospitalization is required for more intensive therapy than
can be given at home.As the chest muscles become weaker the lungs
are unable to take in as much oxygen and get rid of as much carbon
dioxide from the blood as usual. Sometimes a build up of carbon dioxide
in the blood during the night causes daytime drowsiness, headache
and lethargy, particularly in the mornings. The life span of people
with Duchenne muscular dystrophy is determined largely by the strength
of the chest muscles needed for breathing and coughing. In recent
years, advances in the ability to provide mechanical assistance
for breathing have meant that options for relieving some of the
symptoms of breathing failure in the late stages of the disease
have increased. The options range from using a mask over the nose
at night to full mechanical ventilation during the day via a tracheostomy
tube (a tube in the windpipe). Whether any of these techniques are
used depends very much on the personal views of the affected person
and his family. A detailed discussion of the pros and cons and the
problems involved in assisted ventilation is beyond the scope of
this booklet. |
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The Heart |
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The heart is a specialized muscle and while it is not as severely
affected as muscles in the arms and legs, nevertheless some abnormalities
do occur. Fortunately these do not usually cause a serious problem.
However, sometimes in the late teenage years or afterward there can
be problems if the heart muscle becomes weak and is not effectively
pumping blood to other parts of the body. Increasing lethargy or tiredness,
swelling of the ankles or shortness of breath, particularly at night,
may be signs of a heart abnormality and medical advice should be sought.
Treatment with medications can usually relieve these symptoms.
If there are symptoms and signs of a heart failure these can usually
be relieved by drugs that are commonly used to treat older people
with heart failure from other causes. Tablets to decrease body fluid
(diuretics) and to stimulate heart muscle (usually dioxin) are used.
Occasionally other drugs are used to treat abnormalities in heart
rhythm. |
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B. General Care |
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Bones |
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When bones are not subjected to the normal stresses of everyday
living (such as occurs during walking, running and jumping), they
lose calcium and become soft or brittle. Soft bones fracture more
easily than normal bones. This is not often a problem until well into
the wheelchair phase, when accidents such as falling from the chair
or during transfers to or from the chair may cause a fracture. Sometimes
it is not very obvious that a fracture has occurred, until an X-ray
is taken because of persisting pain or increasing swelling. If
most of the weight of the body is put onto one buttock for long
periods while sitting in a wheelchair there may be discomfort in
that area or even sciatica (pain down the back of the leg) if there
is pressure on the sciatic nerve. |
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Bowel Management |
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Good diet and a regular bowel routine are essential and these habits
can be established early in a child’s life. It is important
that the person with muscular dystrophy avoids becoming constipated,
particularly as his ability to move around decreases. Constipation
may lead to abdominal discomfort and pain and may also result in what
appears to be diarrhea. This paradoxically occurs when liquid bowel
material flows around the accumulated hard fecal material. If
constipation does become a problem regular enemas or bowel washouts
may be required. If a boy attends a school for the physically disabled
it is usually possible to arrange for these treatments to be given
at school. Usually, an oral medication is also started. When the
bowel is emptied the enemas can be stopped and control maintained
with the oral medication. |
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Sleeping |
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During sleep most people toss and turn without realizing that they
are doing it. When weakness is moderate to severe it is not possible
for boys with Duchenne muscular dystrophy to turn or move about much
in order to make themselves comfortable. Parents often have to help
the boys move or turn so that they can get back to sleep. This is
difficult for parents, as they too need to have good sleep. Medical
attendants or therapists will be able to offer advice about various
mattresses that can sometimes help. It has been the experience
of many parents that "water beds" significantly assist
the comfort levels of children affected by MD. In many instances
the number of night turns has been reduced for 6-8 times per night
down to 1 - 2 per night. It should be noted that "bladder"
type waterbeds have been found to be generally not suitable. The
preferred type being thy "waveless" baffled bladder, offering
the highest degree of comfort and stability during (un) dressing. |
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Dietary Advice |
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As weakness increases and mobility decreases, particularly when
a person is limited to a wheelchair, energy requirements that are
less and less food are required. Therefore it is important to monitor
food intake. The problems caused by excessive weight gain cannot be
over emphasized. Not only is obesity bad for the health of the child,
it also increases difficulties for caregivers who are required to
assist with everyday activities such as dressing, bathing, toileting.
Dietitians are able to advise on the level of food intake required
and on the appropriate balance of the various components of the
diet. Good dietary advice plays an important role for the older
person with Duchenne muscular dystrophy but the basis for good care
lies in the appropriate attitude of parents to diet in the early
stages of the disease. |
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C. Positive Approaches |
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If people can face these feelings with courage, they can reduce
their stress and anxiety and move towards coping positively and creatively.Seeing
yourself coping, in your own mind's eye, is an essential key to this
process. For example, a mental picture of your son enjoying himself
at school will help you find ways of making that possible and your
eagerness will be passed on to your child so that he too will be excited
about going to school. |
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Barriers to a Positive Approach |
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There can be understandable barriers to a positive approach. Often
parents blame themselves for their child's illness. They may feel
they have been punished for something they have done. A genetic disease
is no one's fault. By some unknown failure in the biological process
an error occurs. However, it is not uncommon for mothers to feel guilty,
blaming themselves, although incorrectly, for causing the disease.
Fathers too can have difficulty accepting that they have a disabled
son who will not be able to do things that they would like them
to do. |
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Sharing Feelings with Your Spouse |
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Couples can overcome these painful feelings by recognizing them
and sharing them with each other. Fathers may find this difficult
because of society expectations that they should be strong and not
show their emotions. They need time and perhaps the right situation
to express their feelings. Every person has their own way of resolving
problems. |
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Significance of a Father's Role |
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Normally children become more independent as they grow older. However
for boys with Duchenne muscular dystrophy, just as this independence
begins to develop, the progression of the disease makes them physically
dependent again. A father has an important part to play at this time
and can make a wonderful contribution to a boy's life. Although it
is not always possible to give lots of time, given the need to earn
a living, any time available is valuable and constructive. Other male
figures close to the family that can also provide support and guidance. |
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Every Family Member Counts |
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Parents must remember that each member of the family has needs that
must be recognized. Although the child affected with Duchenne muscular
dystrophy has needs that are great and obvious, brothers, sisters
and spouses can become upset if all of the attention is focused on
one child. Try to strike a balance in meeting all of the families'
needs. |
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Role of Outside Activities |
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Another opportunity for gaining independence and confidence, particularly
in coping with a variety of people and situations is for your child
to participate in activities outside the home. Not only does participation
develop independence and a positive self-image but also the activities
themselves provide a wonderful source of pleasure and enjoyment. Scouting
activities and electric wheelchair sports, such as hockey, balloon
soccer, and blow darts, are specifically for boys with Duchenne muscular
dystrophy. There is also a wide range of community programs available. |
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Counseling |
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Given the demands and challenges of situations that they meet, at
times a family may find it useful to seek professional counseling
and assistance. This should be thought of as a positive and constructive
action rather than a failure. |
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D. Ways to Minimize the Harsh
Effects |
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Few ways to minimize the affect of DMD to a patient before &
after its effect
The available ways (remedies) show only hope and sustain and not the
treatment (cure) till date in factual. But a patient's family (parents)
with knowledge about DMD, skill to take care properly and attitude
to serve him heartily means more. |
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Some of the tips to reduce the
long-term effects and side effects of DMD |
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- A DMD patient should be in touched with following specialists
and follow their medical suggestions to minimize the effects of
disease, like General Physicians, Chest Specialist, Cardiologist,
Orthopedist, Nutritionist, Neurologist, Physiotherapist, Occupational
Therapists, Psychologist and other professional specialist.
- Use of AFO & KAFO for convenience on standing and walking.
- The contractured parts of joints in legs, knees and heaps should
be surgically operated to make expandable and comfortable.
- Wearing of Night Splint shoes made of plastic while sleeping
a long time in night to prevent the leg contractures and banding.
- Use of wheelchair when there is real problems on standing &
walking.
- Surgical operation of the spinal curvature (deformation) to
re-position it if is bent. The children can breathe better and
often need less assisted respiration afterwards.
- Use of belt on heap as per the suggestions of Doctors.
- Use of Tracheostomy Ventilation (breathing through an opening
in the widepipe) for breathing disorders.
- Regular and proper physical exercises only with the suggestions
of professional experts and physiotherapists are most common and
effective aid to delay muscle deterioration and joint contracture
for several years.
- Regular tests of chest, heart echo, pathological test of urine
and stool on six monthly average schedule to know about the accurate
position of the patient.
- Use of steroids (deflazacort & prednisone) on strict recommendations
of concerned Doctors to activate & strengthen the muscle to
some extent.
- Consumption of secondary food items likes a high protein, Calcium,
Vitamin-D, low fat, low sugar and low salt etc on the recommendation
of nutrition specialist.
- A DMD suffering child should be treated like a normal child
(not as disabled/handicapped) and should be provided with proper
education according to his will. It helps to boost up him morally
and feels himself normal as others. Computer program always comes
first as a possible profession and help to further activities
forever, and other like drawing, painting, music, writing etc.
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