Home negative pressure ventilation: Report of 20 years of experience in patients with neuromuscular disease

doi:10.1016/0003-9993(85)90157-1

Archives of Physical Medicine and Rehabilitation

Volume 66, Issue 4, April 1985, Pages 239-242

https://doi.org/10.1016/0003-9993(85)90157-1 Get rights and content

Abstract

Twenty years of experience using negative pressure devices (NPD) at home to ventilate 40 patients with neuromuscular disease is presented. The purpose of the study was to determine the costs, complications, and clinical outcome of this form of respiratory support, and to ascertain the reasons for failure to institute effective negative pressure ventilation (NPV) in nine patients. Emerson tank respirators, used mainly to rest respiratory muscles at night, and intermittent postitive pressure breathing machines were used by 98% of patients at an average equipment cost of $2,700 annually. Patients in whom NPV was initiated on an elective rather than emergent basis saved an average of $12,000 during their initial hospitalization. Life table analysis shows a five-year survival of 76%, and a 10-year survival of 61%. Complications were minor and occurred at an average rate of less than one per year per patient at home on NPV. Failure to achieve satisfactory NPV in nine patients was associated with age (six patients were younger than 3 years of age), or severe thoracocervical scoliosis, which prevented proper fitting of the NPD. For reasons of safety, economy, and quality of life, NPV at home is the preferred treatment for patients having neuromuscular disease who need respiratory assistance.

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While recent pharmacologic advances in a number of neuromuscular diseases have revolutionized outcomes for a subset of patients, many more benefit from advances in management over the past several decades. The cumulative effect of improved airway clearance techniques, ventilation strategies, and nonrespiratory interventions has led to dramatic differences in the expected course of disease. While an understanding of a disease’s natural history provides historical context for the evolution of medical care, the current expected course provides a more useful point of reference for evaluating new therapies. Spinal muscular atrophy and Duchenne muscular dystrophy provide two examples of significant improvements in care leading to greatly improved outcomes, which has led to a paradigm shift toward a more hopeful outlook for neuromuscular disease.
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Sleep-related breathing disorders are common in Duchenne muscular dystrophy (DMD) with OSAS common in the first decade and hypoventilation developing in the second decade for reasons outlined previously.118 There is a long history of treating children with neuromuscular disorders including spinal muscular atrophy, myopathies, and dystrophies who develop chronic hypercapneic respiratory failure with a variety of different forms of noninvasive ventilation (NIV) or tracheostomy-positive pressure ventilation at home with improved survival and quality of life.80,119 The evidence supporting treatment of nocturnal hypoventilation before the development of diurnal respiratory failure is limited.
Sleep problems are common in many pediatric respiratory diseases including chronic lung disease of infancy, asthma, cystic fibrosis, sickle cell lung disease, and respiratory insufficiency due to neuromuscular disease and scoliosis. Given the adverse neurocognitive and physiologic effects associated with a deranged night's sleep, the goal of this article is to enhance the physician's ability to anticipate, recognize, and appropriately manage a variety of sleep-related problems including sleep-disordered breathing, gas exchange abnormalities, insomnias, and circadian rhythm disturbances in order to improve children's function and quality of life.
Non-invasive positive ventilation in the treatment of sleep-related breathing disorders
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Depending on the medical centers, the three main methods for delivering tidal volume were used: intermittent negative pressure ventilation [2–4], intermittent positive pressure ventilation (IPPV) by tracheostomy [4,5] or IPPV by mouthpiece [6,7]. These experiences have progressively encouraged a few clinicians to use therapeutic approaches such as these to chronically ventilate other types of respiratory insufficiency such as seen in Duchenne dystrophy, chest-wall deformities and even lung diseases [4,5,7–9]. Nevertheless, in the early 1980s, despite positive results, clinicians were reluctant to use these techniques, considering them too invasive (tracheostomy) or cumbersome or of limited efficacy to ventilate patients with impaired mechanics (negative pressure) [10].
This chapter addresses the use of long-term non-invasive positive pressure ventilation (NIPPV) (to the exclusion of continuous positive airway pressure) in the different clinical settings in which it is currently proposed: principally in diseases responsible for hypoventilation characterized by elevated PaCO₂. Nasal masks are predominantly used, followed by nasal pillow and facial masks. Mouthpieces are essentially indicated in case daytime ventilation is needed. Many clinicians currently prefer pressure-preset ventilator in assist mode as the first choice for the majority of the patients with the view of offering better synchronization. Nevertheless, assist-control mode with volume-preset ventilator is also efficient. The settings of the ventilator must insure adequate ventilation assessed by continuous nocturnal records of at least oxygen saturation of haemoglobin-measured by pulse oximetry. The main categories of relevant diseases include different types of neuromuscular disorders, chest-wall deformities and even lung diseases. Depending on the underlying diseases and on individual cases, two schematic situations may be individualized. Either intermittent positive pressure ventilation (IPPV) is continuously mandatory to avoid death in the case of complete or quasi-complete paralysis or is used every day for several hours, typically during sleep, producing enough improvement to allow free time during the daylight in spontaneous breathing while hypoventilation and related symptoms are improved. In case of complete or quasi-complete need of mechanical assistance, a tracheostomy may become an alternative to non-invasive access. In neuromuscular diseases, in kyphosis and in sequela of tuberculosis patients, NIPPV always significantly increases survival. Conversely, no data support a positive effect on survival in chronic obstructive pulmonary disease.
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2006, Kendig's Disorders of the Respiratory Tract in Children

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ArticleHome negative pressure ventilation: Report of 20 years of experience in patients with neuromuscular disease

Abstract

Am J Med

J Pediatr

Am J Med

Chest

Respiration Physiol

Application of abdominal pressure for artificial respiration

JAMA

Mechanical ventilation of patients with late stage Duchenne muscular dystrophy: management in the home

Arch Phys Med Rehabil

Long-term rehabilitation in advanced stage of childhood onset, rapidly progressive muscular dystrophy

Arch Phys Med Rehabil

Alveolar hypoventilation and hypersomnia in myotonic dystrophy

J Neurol Neurosurg Psych

Article
Home negative pressure ventilation: Report of 20 years of experience in patients with neuromuscular disease