title: Is there a role for palliative care in progressive pediatric neuromuscular diseases? The answer is "Yes!
abstract: The consequences of prolongation of survival can be oversimplified, for example, by equating technologically prolonged survival with indefinitely prolonged high quality of life. When this oversimplified view is embraced, the prognosis of ultimately fatal diseases like DMD may be viewed with unrealistic optimism and palliative care may seem irrelevant or misguided. However, we have shown that the sequelae of prolonged survival are complex. For example, NPPV does not protect prolonged survivors of progressive NMDs from potentially debilitating medical complications that can cause elevated burden of disease, high burden of care, and the potential for impaired quality of life. Also, the sequelae of prolonged survival can negatively affect a wide variety of stakeholders, including patients and their families, medical professionals, and society. It is our view that, when the implications of prolonged survival are examined carefully, their complexity is revealed, and the potential for palliative care to provide support and to relieve suffering in prolonged survivors of progressive NMDs becomes apparent. Thus, we advocate development of an integrative care model for patients with progressive NMDs, blending technological therapies with adoption of palliative strategies as patients approach end of life.
authors.affiliation: Department of Pediatrics, MetroHealth Medical Center, Case Western Reserve University, Cleveland, Ohio, USA.
authors.forename: David J
authors.initials: DJ
authors.lastname: Birnkrant
authors.forename: Garey H
authors.initials: GH
authors.lastname: Noritz
mesh_terms: D002648:Child; D018450:Disease Progression; D006301:Health Services Needs and Demand; D006801:Humans; D008020:Life Support Care / Q000458:organization & administration*; D008136:Longevity; D018447:Medical Futility; D017146:Models, Organizational; D009468:Neuromuscular Diseases / Q000150:complications / Q000517:prevention & control* / Q000523:psychology; D009937:Organizational Objectives; D010166:Palliative Care / Q000458:organization & administration* / Q000523:psychology; D010372:Pediatrics / Q000458:organization & administration*; D011379:Prognosis; D011788:Quality of Life; D012944:Social Support; D013315:Stress, Psychological / Q000209:etiology / Q000517:prevention & control; D016312:Treatment Refusal