Working on a Title….


In the process of constructing this post……………Ventilation is the complex motor act of moving air in and out of the lungs and body. Ventilation depends on skeletal muscle.

We all know about neuromuscular disease, especially when the progression is visible and occurs in front of our eyes. Loosing the ability to stand, walk, to swallow…are amongst the more visible aspects of neuromuscular damage. Damage to the neuromuscular system which is involved with breathing, especially acquired damage, preventing normal rate or depth or pattern of ventilation may be much harder to detect and, we are learning, this kind of injury may be unknown to the person who has sustained it. I do not know why this is, but it seems to be due to the nature of the interaction between the autonomic nervous system and the hidden injuries affecting skeletal muscles of the body. Not breathing is not an option. Not maintaining “good enough” chemical homeostasis is not an option. pH is key to correct chemical reactions necessary for function of the human body. Even if the respiratory muscle fibres and/or their nerve connections are damaged, life and hence cell metabolism continually produces endogenous carbon dioxide and the respiratory muscle system must still continue to operate in order to maintain chemical homeostasis. Endogenous carbon dioxide is needed but not in excess. And the excess MUST be exhaled ! And this involves the neuromuscular skeletal ventilatory system.

Paula and I [and Dr Kraepelin] have discovered that acquired, one time hidden, non progressive and invisible and unfelt damage affecting parts of the ventilatory system, making it more difficult to breathe normally [even with healthy lungs] , can be disclosed by carefully and mindfully counting the respiratory rate. We all discovered that people are not aware of their baseline respiratory rates at rest. They are not aware of whether their baseline breathing rate/depth is normal or abnormal. This is most likely because the autonomic nervous system will direct all relevant players [including muscle, thermoregulation, circulation of blood, nutrients, etc.] in keeping PCO2 and pH ratio’s “good enough” for chemical interactions to take place normally. The discomfort and distress that occurs when chemical homeostasis becomes disturbed seems to be felt internally and is difficult to describe. The patient might not mention shortness of breath and may not look like they are short of breath because the skeletal respiratory muscles [neck, torso] may not be working normally. Thus the patient might have visible consequences [not being able to speak more than 3-4 words at a time, change in the voice-sound different , weaker, strained, and might slouch when sitting or standing, etc..] without linking this to their difficulty breathing. And so, in a person with a non progressive neuromuscular hidden and unknown injury, the doctor may not become aware that carbon dioxide levels are rising and may be sublethal, but toxic and causing immense distress and cognitive dysfunction.

[To be continued, not yet constructed in a seemingness manner…..still jumping around, all over the place….]

Ventilation serves four main purposes: (1) exchange of oxygen (O2) and (2) carbon dioxide (CO2), (3) control of blood pH, and (4) oral communication [30]. In other words, ventilation is necessary to sustain life, and the central nervous system’s (CNS) control of ventilation is closely regulated, accordingly.

Ventilation refers to the movement of air into and out of the pulmonary system, which includes the nose, mouth, trachea, and lungs. The terms breathing and respiration are also often used synonymously to refer to this process of ventilation; though to be clear, respiration is actually the process of gas exchange at the cellular level rather than the movement of air within the system.

The total volume of air transported into and out of the lungs in 1 min is referred to as the minute ventilation (VE) and is simply the product of the average volume of each breath (tidal volume; VT) and the frequency of breathing (fB) [159]. Stickford, A.S.L., Stickford, J.L. Ventilation and Locomotion in Humans: Mechanisms, Implications, and Perturbations to the Coupling of These Two RhythmsSpringer Science Reviews 2, 95–118 (2014).

Ventilation is the complex motor act of moving air in and out of the lungs and body.

Neuromuscular diseases (NMDs) encompass a diverse group of diseases which may affect the upper motor neurons, lower motor neurons, peripheral nerves, the neuromuscular junction, and muscles. Although this definition may include a large variety of disorders, the term “neuromuscular” is reserved for inherited or acquired diseases predominately manifesting with motor dysfunction. From the pulmonologist’s perspective all NMDs present with a common trait: impairment of ventilatory function. This impairment is caused by either compromised airway patency and protective reflexes and/or by reduced respiratory pump efficiency. These pathophysiological events result in sleep-disordered breathing, followed by diurnal hypoventilation at later disease stages. In addition, patients with NMDs are prone to developing various other respiratory complications such as infections, atelectasis, and aspiration syndromes [1, 2]. The introduction of ventilatory support in the early stages of symptomatic sleep hypoventilation, especially in the form of noninvasive ventilation (NIV), as well as various other advances in respiratory care have contributed significantly to the improvement of survival and the quality of life of patients with NMDs [3–6]. NMDs can be hereditary or acquired, slowly or rapidly progressive [7, 8] Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review Review Article , Athanasios Voulgaris,1,2 Maria Antoniadou,1,3 Michalis Agrafiotis,1,3 and Paschalis Steiropoulos1,2 | Open Access Volume 2019 |Article ID 2734054 |


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