Noninvasive ventilation during gastrostomy tube placement in patients with severe duchenne muscular dystrophy: case reports and review of the literature
PedPal Lit; Adult Follow-Up StudiesGastrostomy Humans Laryngeal Masks Male Muscular Dystrophy; Duchenne/therapy Positive-Pressure Respiration/methods Retrospective Studies Severity of Illness Index
Individuals with Duchenne muscular dystrophy may benefit from gastrostomy tube feeding due to progressive dysphagia and malnutrition. However, due to their severely impaired pulmonary function, these individuals are at risk of severe complications when they are sedated or undergo anesthesia for the procedure. We previously described a technique of noninvasive positive pressure ventilation to provide respiratory support during gastrostomy tube placement in such patients, but this technique had risks and limitations. In this case report, we examine two alternative techniques we used to provide respiratory support successfully to patients with severe muscular dystrophy and malnutrition who underwent percutaneous endoscopic gastrostomy tube placement. We then review the literature and discuss the potential benefits, risks, and limitations of the above techniques and of other options for gastrostomy placement in people with severe muscular dystrophy.
2006
Birnkrant DJ; Ferguson RD; Martin JE; Gordon GJ
Pediatric Pulmonology
2006
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.20356" target="_blank" rel="noreferrer">10.1002/ppul.20356</a>
Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management
Female; Humans; Male; Diagnosis; Differential; Duchenne/diagnosis/genetics/therapy; Glucocorticoids/administration & dosage/adverse effects/therapeutic use; Muscular Dystrophy
Duchenne muscular dystrophy (DMD) is a severe, progressive disease that affects 1 in 3600-6000 live male births. Although guidelines are available for various aspects of DMD, comprehensive clinical care recommendations do not exist. The US Centers for Disease Control and Prevention selected 84 clinicians to develop care recommendations using the RAND Corporation-University of California Los Angeles Appropriateness Method. The DMD Care Considerations Working Group evaluated assessments and interventions used in the management of diagnostics, gastroenterology and nutrition, rehabilitation, and neuromuscular, psychosocial, cardiovascular, respiratory, orthopaedic, and surgical aspects of DMD. These recommendations, presented in two parts, are intended for the wide range of practitioners who care for individuals with DMD. They provide a framework for recognising the multisystem primary manifestations and secondary complications of DMD and for providing coordinated multidisciplinary care. In part 1 of this Review, we describe the methods used to generate the recommendations, and the overall perspective on care, pharmacological treatment, and psychosocial management.
2010
Bushby K; Finkel R; Birnkrant DJ; Case LE; Clemens PR; Cripe L; Kaul A; Kinnett K; McDonald C; Pandya S; Poysky J; Shapiro F; Tomezsko J; Constantin C; DMD Care Considerations Working Group
Lancet Neurology
2010
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1016/S1474-4422(09)70271-6" target="_blank" rel="noreferrer">10.1016/S1474-4422(09)70271-6</a>