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Recent Advances in Respiratory Care For Neuromuscular Disease Research Paper

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Galen (Greek physician, 131-201 AD) was the first to realize that respiration depends on mechanical factors provided by the thoracic wall (Benditt, Pp.829-837). The neuromuscular control of the respiratory system includes the central nervous system (higher respiratory centers and the spinal cord). It includes also the peripheral motor nerves, the respiratory muscles, and the feedback mechanisms (Benditt, Pp.829-837). This explains why neuromuscular diseases affecting the respiratory muscles are major contributors in the disease state and even mortality whether this may be in the short or long-standing scenery (Benditt, Pp.829-837). The aim of this essay is to review the recent advances in respiratory care for patients with neuromuscular disorders.

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Respiratory function tests include measurements like vital capacity (VC) and total lung capacity (TLC). These measurements are of limited value in assessing the degree of respiratory muscle weakness in neuromuscular disease. Evaluation of respiratory muscle weakness, in these diseases, by maximal static inspiratory and expiratory pressures and maximal sniff maneuvers provide a better assessment.

Chest x-ray, although provides visualization of the diaphragm, yet gives little information, if any, about its function. Diaphragm fluoroscopy provides a better real-time assessment of the diaphragm than x-ray, however, the disadvantages of this test are it exposes the patient to ionizing radiation and it has poor specificity and sensitivity. Recently, instead of assessing the diaphragm movement, respiratory clinicians use ultrasound as an alternative to assess the diaphragm in the juxtaposition to the rib cage. This provides better visualization of the pleura and diaphragm. Measuring the diaphragm thickness at end-expiration, and comparing it with the change at end inspiration should enable clinicians to assess diaphragm strength, and shortening respectively (DePalo and McCool, Pp.201-209).

The neuromuscular disease represents nearly 5% of cases of assisted mechanical ventilation (Tobin, Pp.1986-1996). The aim of mechanical ventilation is to assist the weak respiratory muscles in producing the mechanical events needed for respiration, thus preventing hypoxemia and respiratory acidosis. There are three main modes of mechanical ventilation. A- assist control ventilation; where the tidal volume the patient needs is preadjusted and the ventilator starts to create this volume when respiratory muscles efforts begin or autonomously if no such effort occurs within a preadjusted period of time.

B- Alternating (intermittent) mandatory ventilation, where the respiratory clinician adjusts the number of breathing actions of a fixed volume for the ventilator to deliver. Between these controlled breaths, the patient can breathe voluntarily. C- Pressure support ventilation, where the respiratory clinician adjusts the ventilator on pressure mode rather than a volume mode as in the previous two types (Tobin, Pp.1986-1996). Ali and others (Pp. 1403-1407), assumed the initial ventilation modes for patients with neuromuscular disorders to be intermittent (alternating) mandatory ventilation or assist control ventilation, with lower tidal volume and higher positive end-expiratory pressure to ensure lung protection during mechanical ventilation.

The second objective for mechanical ventilation in neuromuscular disease is the inability to clear chest secretions (the patient may be drawn in his or her secretions) as in cases of Guillain-Barre syndrome. In these patients, the indication for endotracheal intubation is when there is a decrease of vital capacity to 15 ml/kg or less, and there is a need for clearing chest secretion. Early tracheotomy, if the patient appears to need an endotracheal tube for > three weeks, has many advantages.

Giving the patient less sedation to tolerate the endotracheal tube, shorter time of mechanical ventilation with easier weaning, and lower hospital-acquired infection rates are the most important advantages (Ali and others, Pp.1403-1407). The use of non-invasive mechanical ventilation has reduced the rate of tracheotomies; the main indication for the procedure remains clearance of secretion. Although airway clearance is an important objective in the management of neuromuscular disease patients with respiratory problems, yet, there is no standard measure for cough assessment. In addition, there are individual differences among patients about the possibility of aspiration.

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Thus, patient relatives can learn techniques like manually assisted cough enhancement, other techniques like using electromagnetic stimulators are suitable while the patient is in the ICU (Hill, Pp.1065-1071). Cough assistance devices (mechanical in-insufflator) known as cough machine is an example. The indications to use this equipment are in patients with neuromuscular disease who are unable to expectorate with the help of chest percussion or postural drainage and are unable to clear secretions because of decrease peak expiratory volume. In these conditions, it can substitute tracheotomy or other invasive procedures intended to clear secretions.

It acts by creating a gradually increasing positive pressure in the upper airway through a mouthpiece or a facemask. Then it rapidly changes to negative pressure resulting in a high expiratory flow rate from the lungs.

Simonds (Pp. 1879-1886), summarized the indications of non-invasive mechanical ventilation in patients with neuromuscular diseases as follows:

Clinical problems of the respiratory system: Non-invasive ventilation (NIV) is used as a preventive measure to respiratory failure, to change chest wall/lung growth characteristics, to rest muscles of respiration, management of nocturnal hypoventilation, and management of hypercapnic ventilatory failure.
During concurrent medical conditions: Where NIV is a line in the management of concurrent chest infection, during pregnancy or as a preoperative measure before gastric tube placement.
NIV is one of the palliative treatments in terminal cases.

Patients with neuromuscular disease may remain stable on NIV for a variable period ranging from few months as in amyotrophic lateral sclerosis to few years as in Duchene muscular dystrophy. NIV is administered twice a day via a facemask or a mouthpiece depending on the condition of buccal muscles. Problems with NIV arise when bulbar palsy develops, when maintenance of arterial gas tension becomes difficult, failure of clinical overall improvement, or developing unsorted interface (facemask or mouthpiece) problems. In any of the previous conditions, the deliverance of mechanical ventilation through a tracheotomy tube is the solution (Simonds, Pp.1879-1886).

In some congenital neuromuscular diseases as type 1 spinal muscular atrophy and Duchenne muscular dystrophy, the chest has the shape of a small bell. This occurs because of the relative strength of the diaphragm in relation to the very weak intercostals respiratory muscles resulting in reduced chest wall expansion with possible pulmonary complications. Simonds (Pp.1879-1886) assumed that NIV should have a beneficial effect on chest wall expansion, compliance (area-pressure relationship), and hyperinflation despite the lack of controlled trials to support this suggestion.

The American Thoracic Society (ATS) statement report in 2004 (ATS consensus, Pp.456-465) recommended that all patients with neuromuscular diseases including Duchenne muscular dystrophy should undergo routine evaluation of respiratory function and respiratory muscle strength. Forced vital capacity (FVC) below 1 L is still the best negative indicator of the case progression. Patients with neuromuscular diseases should be evaluated for other respiratory disorders like obstructive sleep apnea (OSA), gastroesophageal reflux, and upper airway aspiration. Less frequently, blood count, serum bicarbonate level, and chest x-ray are required.

The underlying principle of respiratory muscle training is to increase muscle strength, however, opinions vary from considerable to minimal improvement. In addition, as recently described, a protective nitric oxide release mechanism may be deficient in these patients, which may result in muscle damage because of muscle training (ATS consensus, Pp.456-465).

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CPAP appears to be of limited value in patients with neuromuscular disease as it counteracts only the collapsibility of the upper airway dilator muscle, not the respiratory muscles. Instead, nocturnal NIV is ideal for nocturnal hypoventilation. The attending physician should consider daytime NIV (intermittent positive pressure ventilation) when PCO2 exceeds 50 mm Hg or hemoglobin saturation is persistently 92% or less (ATS consensus, Pp.456-465).

Mechanical ventilation does not prevent neuromuscular disease progression; however, it affects the quality of life of those patients. Therefore, it should always be an option offered by the physician who should always explain the objective, value, and other details connected to its use. Patient education serves the purpose of the patient and family take a more active part in management. Educational schemes should be suitable to the disease and the present state of illness; and should start when the diagnosis is confirmed.

Home care for a patient with neuromuscular diseases is an interlinking collection of services delivered in a medically uncontrolled environment as patients and their families are a central part of the caregiving team. The continuing unrelieved patient condition, cost, and complexity of the process are problems that the patient, family, and physician have to face with this type of care. Although many disparities of respiratory complications of the neuromuscular disease are emphasized in the literature, yet, many knowledge defects need to be uncovered especially in the fields of pathophysiological and clinical management.

Works Cited

Ali, M I, Fernandez-Perez, E R and Pendem, S. “Mechanical Ventilation With Guillain-Barre Syndrome.” Respir Care vol 51(12) 2006. p. 1403-1407.

ATS Consensus Statement. “Respiratory Care of the Patient with Duchenne Muscular Dystrophy.” Am J Respir Crit Care Med vol 170 2004. p. 456-465.

Benditt, J O. “The Neuromuscular Respiratory System: Physiology, Pathophysiology, and a Respiratory Care Approach to Patients.” Respir Care vol 51(8) 2006. p. 829-837.

DePalo, V A. and McCool, F. D. “Respiratory Muscle Evaluation of the Patient with Neuromuscular Disease.” Seminars in Respiratory and Critical Care Medicine vol 23(3) 2002. p. 201-209.

Hill, N S. “Neuromuscular Disease in Respiratory and Critical Care Medicine.” Respir Care vol 51(9) 2006. p. 1065-1071.

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Simonds, A K. “Recent Advances in Respiratory Care for Neuromuscular Disease.” CHEST vol 130 2006. p. 1879-1886.

Tobin, M J. “Advances in Mechanical Ventilation.” N Engl J Med vol 344(26) 2001. p. 1986-1996.

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