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Ventilation CenterHow it WorksWho Can be Helped? Printer Friendly Page
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

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