Article

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: 1.73). 06/2002; 81(6):411-5.
Source: PubMed

ABSTRACT 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.

2 Bookmarks
 · 
125 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Mechanically assisted coughing is the combination of mechanical insufflation-exsufflation to the airways in conjunction with an exsufflation-timed abdominal thrust. It has permitted in-home long-term survival of respirator-dependent users of continuous noninvasive intermittent positive pressure ventilatory support in the United States since shortly after it became available in 1952. By contrast, the first European patients to benefit from continuous noninvasive intermittent positive pressure ventilatory support were not described until institutionalized continuous noninvasive intermittent positive pressure ventilatory support users with Duchenne muscular dystrophy were described in Belgium in 2006, 2 yrs after mechanically assisted coughing was approved for sale by the European Union. Domiciliary management with continuous noninvasive intermittent positive pressure ventilatory support and mechanically assisted coughing has subsequently been described in Europe. This work describes the historical development of mechanically assisted coughing in the framework of aiding the respiratory muscles to prevent respiratory failure and avoid invasive airway intubation. Some current mechanical in-exsufflators include oscillation as a surrogate for physiologic mucociliary transport along with their assisted coughing function. Noninvasive management prolongs life while promoting cost containment and preserving quality-of-life for patients with respiratory muscle impairment.
    American journal of physical medicine & rehabilitation / Association of Academic Physiatrists 10/2013; 92(10):930-41. · 1.56 Impact Factor
  • Tanaffos 01/2013; 12(1):70-3.
  • Source
    [Show abstract] [Hide abstract]
    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

Full-text (2 Sources)

Download
273 Downloads
Available from
Jun 1, 2014