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Duchenne muscular dystrophy: continuous noninvasive ventilatory support prolongs survival

Descriptive

TypeOfResource
Text
TitleInfo
Title
Duchenne muscular dystrophy: continuous noninvasive ventilatory support prolongs survival
Name (type = personal)
NamePart (type = family)
Bach
NamePart (type = given)
John R.
Affiliation
Physical Medicine & Rehabilitation (PM&R), Rutgers University
Role
RoleTerm (authority = marcrt); (type = text)
author
Name (type = personal)
NamePart (type = family)
Martinez
NamePart (type = given)
Daniel
Role
RoleTerm (authority = marcrt); (type = text)
author
Affiliation
Hospital Clinico Universitario, Valencia (Spain)
Name (authority = RutgersOrg-Department); (type = corporate)
NamePart
Physical Medicine & Rehabilitation (PM&R)
Name (authority = RutgersOrg-School); (type = corporate)
NamePart
New Jersey Medical School (NJMS)
Genre (authority = RULIB-FS)
Article, Refereed
Genre (authority = NISO JAV)
Version of Record (VoR)
Note (type = peerReview)
Peer reviewed
OriginInfo
DateIssued (encoding = w3cdtf); (keyDate = yes); (qualifier = exact)
2011
Abstract (type = Abstract)
OBJECTIVE: To describe survival outcomes with noninvasive ventilation (NIV) for full ventilatory support, and a mechanically assisted cough and oximetry protocol in a series of patients with Duchenne muscular dystrophy.
METHODS: We monitored end-tidal carbon dioxide (PETCO2), SpO2, vital capacity, maximum insufflation capacity, and cough peak flow. Nocturnal NIV was initiated for symptomatic hypoventilation. An oximeter and mechanically assisted cough device were prescribed when the pa- tient’s maximum assisted cough peak flow fell below 300 L/min. Patients used up to continuous NIV and mechanically assisted cough to return SpO2 to > 95% during intercurrent respiratory infections or as otherwise needed. We recorded respiratory and cardiac hospitalizations and mortality, and quantified survival by duration of continuous NIV dependence (ie, unable to maintain oxygenation without the ventilator).
RESULTS: With advancing Duchenne muscular dystrophy, 101 nocturnal-only NIV users extended their NIV use throughout the daytime hours and required it continuously for 7.4 +- 6.1 years to 30.1 +- 6.1 years of age, with 56 patients still alive. Twenty-six of the 101 became continuously dependent without requiring hospitalization. Eight tracheostomized users were decannulated to NIV. Thirty-one consecutive unweanable intubated patients were extubated to NIV plus mechanically assisted cough. Of the 67 deaths (including 8 patients who died from heart failure before requiring ventilator use), 34 (52%) were probably cardiac, 14 (21%) were probably respiratory, and 19 (27%) were of unknown or other etiology.
CONCLUSIONS: Continuous NIV along with mechanically assisted cough and oximetry as needed can prolong life and obviate tracheotomy in patients with Duchenne muscular dystrophy. Unweanable patients can be decannulated and extubated to NIV plus mechanically assisted cough.
Language
LanguageTerm (authority = ISO 639-3:2007); (type = text)
English
PhysicalDescription
InternetMediaType
application/pdf
Extent
7 p.
Subject (authority = LCSH)
Topic
Duchenne muscular dystrophy
Subject (authority = local)
Topic
Mechanical ventilation
Subject (authority = LCSH)
Topic
Artificial respiration
Subject (authority = local)
Topic
Noninvasive ventilation
Subject (authority = LCSH)
Topic
Cough
Subject (authority = local)
Topic
Respiratory paralysis
Subject (authority = LCSH)
Topic
Respiratory therapy
Subject (authority = local)
Topic
Mechanical insufflation-exsufflation
Subject (authority = local)
Topic
Vital capacity
Subject (authority = local)
Topic
Cough flows
Extension
DescriptiveEvent
Type
Citation
DateTime (encoding = w3cdtf)
2011
AssociatedObject
Name
Respiratory Care
Type
Journal
Relationship
Has part
Detail
744-750
Identifier (type = volume and issue)
56(6)
Reference (type = url)
https://dx.doi.org/ 10.4187/respcare.00831
RelatedItem (type = host)
TitleInfo
Title
Bach, John R.
Identifier (type = local)
rucore30185400001
Location
PhysicalLocation (authority = marcorg); (displayLabel = Rutgers, The State University of New Jersey)
NjNbRU
Identifier (type = doi)
doi:10.7282/T3W09837
Genre (authority = ExL-Esploro)
Journal Article
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Rights

RightsDeclaration (AUTHORITY = FS); (ID = rulibRdec0004)
Copyright for scholarly resources published in RUcore is retained by the copyright holder. By virtue of its appearance in this open access medium, you are free to use this resource, with proper attribution, in educational and other non-commercial settings. Other uses, such as reproduction or republication, may require the permission of the copyright holder.
Copyright
Status
Copyright protected
Availability
Status
Open
Reason
Permission or license
RightsEvent
Type
Permission or license
AssociatedObject
Type
License
Name
Multiple author license v. 1
Detail
I hereby grant to Rutgers, The State University of New Jersey (Rutgers) the non-exclusive right to retain, reproduce, and distribute the deposited work (Work) in whole or in part, in and from its electronic format, without fee. This agreement does not represent a transfer of copyright to Rutgers.Rutgers may make and keep more than one copy of the Work for purposes of security, backup, preservation, and access and may migrate the Work to any medium or format for the purpose of preservation and access in the future. Rutgers will not make any alteration, other than as allowed by this agreement, to the Work.I represent and warrant to Rutgers that the Work is my original work. I also represent that the Work does not, to the best of my knowledge, infringe or violate any rights of others.I further represent and warrant that I have obtained all necessary rights to permit Rutgers to reproduce and distribute the Work and that any third-party owned content is clearly identified and acknowledged within the Work.By granting this license, I acknowledge that I have read and agreed to the terms of this agreement and all related RUcore and Rutgers policies.
RightsEvent
Type
Permission or license request response
DateTime (encoding = w3cdtf)
2016-06-21
AssociatedEntity
Role
Representative
Name
Ray Masferrer
Affiliation
Managing Editor, Respiratory Care
AssociatedObject
Type
Permission response
Name
Permission request response for Duchenne Muscular Dystrophy
Reference (type = digital)
http://dx.doi.org/doi:10.7282/T3B27XD3
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Technical

RULTechMD (ID = TECHNICAL1)
ContentModel
Document
CreatingApplication
Version
1.3
DateCreated (point = start); (encoding = w3cdtf); (qualifier = exact)
2011-05-18T10:52:25
DateCreated (point = start); (encoding = w3cdtf); (qualifier = exact)
2011-05-18T10:52:25
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