Physical Medicine Interventions for
Skeletal and Cardiorespiratory Muscle Dysfunction
(Volume One: The Conditions and Their Medical and Surgical Management)
Table of Contents
Section 1 – Staging: Nomenclature, Historical, Medical, and Research Concerns
Myopathic Conditions with Respiratory Muscle Dysfunction
Neuropathic and Other Conditions with Respiratory Muscle Dysfunction
Classification and Management Staging
Medical Therapies and Clinical Trials
Lessons Gleaned From the History of Neuromuscular Disease Management
Nutrition and Neuromuscular Disease
Dysphagia and Dysarthria
Gastrointestinal Complications and Enteral Nutrition
Section 2 – The Ambulation Stage
9. Orthopedic Surgery and Prolongation of Brace-Free Ambulation
10. Physical Therapeutic Interventions
Section 3 – The Stage of Wheelchair Dependence
11. Scoliosis Management
12. Conservative Therapeutic Interventions and Assistive Equipment
Physical impairments and disabilities and respiratory muscle insufficiency result from neuromuscular diseases (NMDs), spinal cord injury, post-poliomyelitis, morbid obesity, central nervous system conditions like multiple sclerosis, stroke, and traumatic brain injury, some chronic medical and pulmonary conditions, as well as for people debilitated by prolonged stays in hospital critical care units. All of these patients can develop ventilatory pump failure. There are over three million Americans affected by NMD alone. Many become wheelchair dependent prematurely when not offered lower limb surgical interventions based on sound pathokinesiology and they die prematurely when not offered respiratory muscle aids. They also often unnecessarily suffer the placement of invasive airway tubes. Despite this, few people with NMDs or respiratory muscle dysfunction from other causes are offered definitive noninvasive management. This is largely because most countries have no physicians or clinics specialized in the holistic care of NMDs. Countries with multidisciplinary neuromuscular disease clinics, like the United States, have no single gatekeeper well-versed in all the relevant issues covered in these volumes. As a result, unnecessary, expensive invasive management options are incentivized rather than healthier and humane noninvasive alternatives. This typically results in suboptimal and disjointed rather than holistic care. Furthermore, noninvasive management experts are needed for both outpatient and critical care for both children and adults but there are only a handful of clinicians that fit the bill and they are virtually all noted in www.breatheNVS.com.
Diagnostic details and intricacies of NMDs and other neurological conditions are provided elsewhere. There are books dedicated to the comprehensive management of multiple sclerosis, spinal cord injury, surgical and medical treatments for obesity-hypoventilation syndrome, and chronic obstructive pulmonary disease. Yet, none of these other than those with chapters authored by Bach describe prolonging life by using continuous NVS and mechanical insufflation-exsufflation (MIE). Indeed, while medical texts are necessarily obsolete before they go to print since genetic characterizations and new medical therapies are being described all of the time, only since 2013 have there been mouthpiece NVS modes incorporated into the software of some portable ventilators despite its having been described as an alternative to tracheostomy mechanical ventilation in 1953. To this day no one other than Bach and the clinicians of have reported the extubation or decannulation of continuously ventilator dependent people. This is true despite the morbidity and mortality caused by invasive airway tubes which can permanently impair the ability to cough, swallow, speak, quality of life in general, and increase risk of cardiorespiratory complications, hospitalizations, institutionalizations, and premature death.
Physicians in any one field cannot keep abreast of all medical literature, yet optimal management requires knowledge of both pediatric and adult physical medicine and rehabilitation, orthopedics, respiratory medicine, otolaryngology, endocrinology, cardiology, gastroenterology, nutrition, physical, occupational, and respiratory therapy, augmentative communication, sexual counseling, pharmacology, and psychology. Routinely seeing specialists in all of these fields, none of whom may be specialized in NMD, is expensive and unnecessary for any clinician “gate-keeper” who understands the material in these volumes. Unfortunately, preventive care is disincentivized in the U.S. while routinely consulting specialists for invasive and otherwise unnecessary procedures isn’t.
Management of generalized muscle dysfunction deserves to be a medical specialty in its own right. While neurologists consider nerves, orthopedic surgeons treat bones, dermatologists skin, pulmonologists lungs, and cardiologists the heart, only physiatrists, doctors of physical medicine and rehabilitation, specialize in the diagnosis and treatment of muscle dysfunction and typically treat both children and adults. Further, there are no medical specialists who manage 90% or more of the medical and physical issues pertaining to NMD for all ages except in the NMD clinic of Rutgers University New Jersey Medical School. Elsewhere, upon reaching age of majority, patients are switched from one management team to another with quite possibly no members of either specialized in managing muscle dysfunction. The goal of this book is to resolve this problem by providing state-of-the-art information across all relevant medical specialties.
In the words of Sigmund Freud, “People who want to make a living from the treatment of nervous patients must clearly be able to do something to help them” (1909). Consuming over 20% of Gross Domestic Product (GDP), medical care in the United States is unsustainably expensive, yet rated only 37th by the World Health Organization (WHO). People with respiratory muscle dysfunction continue to suffer and die prematurely. Besides prolonging life more humanely by NVS and MIE, these volumes address functional and mobility issues, optimizing nutrition, the prevention of chest, back, and limb deformities, and the optimizing of independence. The crisis decision of whether or not to “go on a respirator” (undergo tracheotomy) can be avoided entirely. Patients will no longer need to die from preventable complications or undergo tracheotomies while awaiting FDA approval of potentially effective medical treatments.
This volume also features a novel concept, that of approaching NMD from a cell and organelle membrane perspective. Due to cellular imaging and the better understanding of cellular biochemistry and physiology over the past few years, the medical literature is catching up to what we have observed for over 20 years. Epigenetics is now emerging in the understanding that genetics is only the first layer of control. The stability and architecture of the cell and organelle membranes create a feedback loop to the genes via epigenetic and other mechanisms and as such are pivotal to the health and presentation (phenotype) of patients with NMDs. Both aberrant proteins and lipids need to be addressed. The mitochondrial and peroxisomal respiration, the endoplasmic reticulum, and all the organelles and cell membranes together drive the cell’s biochemistry. Most clinicians ignore the role of nutrition in membrane physiology. Indeed, most ignore the role of nutrition in general. We have a country in which about 70% of the population is overweight. However, when knowledge of nutrition is combined with that of cellular phospholipid metabolism a relevant clinical picture emerges. That picture which has emerged from clinical observations from over 20 years of designing dietary regimes for neurological/NMD/genetic diseases, is featured in Volume 1. As Edward Meryon pointed out in the 19th century (Chapter 6), what patients consume is paramount to their state of their health.
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