All people reach a physiological
peak at age 19. Muscle strength and endurance peak around
this time or soon afterwards. After this we lose 1 to
3% of our strength per year. People, such as those with
post-poliomyelitis or spinal cord injury or with neuromuscular
diseases such as Duchenne muscular dystrophy, spinal
muscular atrophy, amyotrophic lateral sclerosis or other
conditions, speak with less strength and have difficulties
at an earlier age than the rest of us. The difficulties
encountered from muscular weakness are in day to day
functioning and in breathing and coughing due to weakness
of inspiratory and expiratory (respiratory) muscles. Even
in "normal" elderly people in nursing care facilities
complications related to respiratory muscle weakness
are a leading cause of suffering and death ( see
article 65 of Dr. Bach's Bibliography)
People with generalized weakness can have difficulty
functioning that can be abetted with the use of assistive
equipment and training. These conditions can also
be a threat to survival when breathing, coughing,
throat, and heart muscles are very weakened. Fortunately,
today, much can be done to avoid suffering and death
from these causes. Typically, muscular conditions
as opposed to neurological conditions do not affect
the heart muscle. However, all people with myopathies
like Duchenne muscular dystrophy should be evaluated
for heart muscle weakness regularly. This is particularly
true today because we have new medications and medical
approaches to improving heart function (see
article 135 of Dr. Bach's Bibliography).
Typically people with weak breathing and coughing
muscles can function appropriately until a simple
cold causes the production of airway secretions that
the person is too weak to cough out. Bacteria multiply
in the secretions and the person develops pneumonia
and respiratory distress. He or she is taken to a
local hospital where oxygen is given and when breathing
is suppressed by the delivered oxygen, a tube is placed
through the nose or mouth into the lungs to evacuate
the secretions and provide breathing support. While
receiving support through the invasive tube, breathing
on one's own may be delayed or thought to be impossible. The
patient is then told that a tracheostomy tube is needed. This
is a tube that is passed through the neck and into
the airway. Typically, the hospitalization during
which a person undergoes tracheostomy following pneumonia
is 72 days. In reality, virtually no one who can speak
needs or should ever receive a tracheostomy tube and
those who have them should consider having them removed,
whether needing to use a ventilator or not. After
tube removal the great majority of ventilator users
can wean off of supported ventilation whereas this
rarely happens when one has a tracheostomy tube (see
article 76 of Dr. Bach's Bibliography).
Unfortunately, people are left to develop pneumonia
after pneumonia, have hospitalization after hospitalization,
and develop breathing difficulties and they are not
offered the respiratory muscle aids that can prevent
these problems. All that is typically done is that
patients are given oxygen that only makes the problem
worse and told that they must undergo tracheostomy
to survive. Yet, all people with experience using
tracheostomy tubes and noninvasive inspiratory and
expiratory muscle aids greatly prefer the latter (see
article 15 of Dr. Bach's Bibliography).
Patients helped by the Center include those with
the following disorders:
- Myopathies
- Muscular
dystrophies
Dystrophinopathies--Duchenne, Becker, limb-girdle,
Emery-Dreifuss, facioscapulohumeral, congenital, childhood
autosomal recessive, and myotonic dystrophy.
- Non-Duchenne
myopathies
congenital and metabolic myopathies like acid
maltase deficiency; inflammatory myopathies such as
polymyositis; diseases of the myoneural junction such
as myasthenia gravis; mixed connective tissue disease;
myopathies associated with systemic conditions such
as with cancer, cachexia/anorexia nervosa, and medications.
- Neurological disorders
spinal muscular atrophies; motor neuron diseases;
poliomyelitis; neuropathies; hereditary neuropathies;
any conditions with diaphragm paralysis; Guillain-Barr
Syndrome; multiple sclerosis; Friedreich's ataxia;
spinal cord lesions of any cause; sleep breathing
disorders; familial dysautonomia; Downs syndrome.
- Skeletal
and Connective Tissue Disorders
kyphoscoliosis; osteogenesis imperfecta; rigid
spine syndrome; spondyloepiphyseal dysplasia congenita.
- Restrictive
Lung Disease associated
with lung resection, tuberculosis, Milroy's disease
- Chronic Obstructive
Pulmonary Disease
|