Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing.

Department of Pulmonary Medicine, Hospital Clinico Universitario de San Juan, San Juan de Alicante, Spain.
American Journal of Physical Medicine & Rehabilitation (Impact Factor: 2.2). 06/2002; 81(6):411-5.
Source: PubMed


To quantitate prolongation of survival for patients with Duchenne muscular dystrophy with the use of noninvasive intermittent positive-pressure ventilation (IPPV) with and without access to a protocol involving mechanically assisted coughing.
In this retrospective review of all patients with Duchenne muscular dystrophy visiting a neuromuscular disease clinic, patients were trained to use mouth piece and nasal IPPV and mechanically assisted coughing to maintain oxyhemoglobin saturation >94% (protocol). Survival was considered prolonged when noninvasive IPPV was required full time.
Ninety-one of 125 patients used noninvasive IPPV part time for 1.9 +/- 1.3 yr, and 51 went on to require it full time for 6.3 +/- 4.6 yr. Of the 31 noninvasive IPPV users who died without access to the protocol, 20 died from respiratory causes and seven died from cardiac causes. None of the 34 full-time noninvasive IPPV users with access to the protocol underwent tracheotomy or died from respiratory complications during a period of 5.4 +/- 4.0 yr, whereas three died from heart failure. Five patients with no breathing tolerance were extubated or decannulated to continuous noninvasive IPPV.
Noninvasive respiratory aids can prolong survival and permit extubation or decannulation of patients with Duchenne muscular dystrophy with no breathing tolerance.

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    • "With natural disease progression, physical and functional capacity of individuals with MD decrease [3], [4]. It is known that the distal upper limb becomes the only way of interacting with technology resources such as computers, joysticks to control motorized wheelchairs and joysticks to play videogames [8]. "
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    ABSTRACT: Computer technologies have been applied in several professional practice areas, including Occupational Therapy. These constitute a new treatment modality. The goal of this paper is to present the results of the use of an Augmented Reality tool applied to an upper limb rehabilitation program in Occupational Therapy sessions. For this study we selected Duchenne Muscular Dystrophy patients with physical and functional limited abilities.
    Information Systems and Technologies (CISTI), 2013 8th Iberian Conference on; 01/2013
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    • "Use of low-span nocturnal-only BiPAP has been reported to prolong the life of ALS patients by a few months [10-12]. However, one center has reported an eight-year survival in patients with ALS through the use of full-setting, continuous non-invasive ventilation (NIV) as needed [13] and has also reported similar benefits for patients with Duchenne muscular dystrophy [14,15]. The diagnosis of ALS was made on the basis of clinical and electromyographic findings, as defined by revised El Escorial criteria for diagnosing ALS [16]. "
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    ABSTRACT: Little has been written about the use of non-invasive ventilation in the home care of amyotrophic lateral sclerosis-motor neuron disease patients with end-stage respiratory muscle failure. Nocturnal use of non-invasive ventilation has been reported to improve daytime blood gases but continuous non-invasive ventilation dependence has not been studied in this regard. There continues to be great variation by country, economics, physician interest and experience, local concepts of palliation, hospice requirements, and resources available for home care. We report a case series of home-based amyotrophic lateral sclerosis-motor neuron disease patients who refused tracheostomy and advanced non-invasive ventilation to full-setting, while maintaining normal alveolar ventilation and oxygenation in the course of the disease. Since this topic has been presented in only one center in the United States and nowhere else, it is appropriate to demonstrate that this can be done in other countries as well. We present here the cases of three Caucasian patients (a 51-year-old Caucasian man, a 45-year-old Caucasian woman and a 57-year-old Caucasian woman) with amyotrophic lateral sclerosis who developed continuous non-invasive ventilation dependence for 15 to 27 months without major complications and were able to maintain normal CO2 and pulse oxyhemoglobin saturation despite a non-measurable vital capacity. All patients were wheelchair-dependent and receiving riluzole 50 mg twice a day. Patient one developed mild-to-moderate bulbar-innervated muscle weakness. He refused tracheostomy but accepted percutaneous gastrostomy. Patient two had two lung infections, acute bronchitis and pneumonia, which were treated with antibiotics and cough assistance at home. Patient three had three chest infections (bronchitis and pneumonias) and asthmatic episodes treated with antibiotics, bronchodilators and cough assistance at home. All patients had normal speech while receiving positive pressure; they died suddenly and with normal oxygen saturation. Although warned that prognosis was poor as vital capacity diminished, our patients survived without invasive airway tubes and despite non-measurable vital capacity. No patient opted for tracheostomy. Our patients demonstrate the feasibility of resorting to full-setting non-invasive management to prolong survival, optimizing wellness and management at home, and the chance to die peacefully.
    Journal of Medical Case Reports 01/2012; 6(1):42. DOI:10.1186/1752-1947-6-42
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    • "Studies comparing the results of noninvasive versus invasive ventilatory assist have reported patients that received non-invasive IPPV showed fewer respiratory complications, lower admission rates, and shorter duration in their hospital stay when admitted.29,30 Another study comparing these two methods has reported that people generally favor the non-invasive method and this method is superior in aspects of convenience, safety, general comfort, speech, preservation of swallowing function, sleeping, and appearance.29,31 Research on the pathophysiology of applying noninvasive ventilator has determined the use of non-invasive ventilator can increase the maximum inspiratory pressure in patients with chronic alveolar hypoventilation as well as temporally increase VC.34,35 "
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    ABSTRACT: In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.
    Yonsei Medical Journal 07/2006; 47(3):307-14. DOI:10.3349/ymj.2006.47.3.307 · 1.29 Impact Factor
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