Long-term ventilatory support in spinal muscular atrophy

Gilgoff, Irene S.; Kahlstrom, Emily J.; MacLaughlin, Eithne F.; Keens, Thomas G.

doi:10.1016/s0022-3476(89)80740-1

Cited by 66 publications

(38 citation statements)

References 12 publications

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“…Two case series252 267 report on positive experiences of children and young adults with DMD in the formulation of life plans, regardless of the decision made about long-term ventilator assistance. A case study of a boy with DMD by Penner and colleagues268 describes the ethics of disclosure and emotionally charged challenges surrounding advance care plans.…”

Section: Qol and Palliative Carementioning

confidence: 99%

British Thoracic Society guideline for respiratory management of children with neuromuscular weakness

Hull

Aniapravan

Chan

et al. 2012

Thorax

349

531

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Section: Qol and Palliative Carementioning

confidence: 99%

British Thoracic Society guideline for respiratory management of children with neuromuscular weakness

Hull

Aniapravan

Chan

et al. 2012

Thorax

349

531

View full text Add to dashboard Cite

“…Activities of daily living, such as shopping, cooking and cleaning, may be carried out with less difficulty than before. There are few studies on the effects of treatment of respiratory problems on education [17] but continued schooling is feasible despite the need for ventilatory support. Adults may be able to return to work [18] and long-term ventilatory support has been shown to reduce the number of days spent in hospital [4,[19][20][21] and improve psychosocial and mental function [19,22].…”

Section: Contraindicationsmentioning

confidence: 99%

Noninvasive ventilation for chest wall and neuromuscular disorders

Shneerson¹,

Simonds²

2002

Eur Respir J

164

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Neuromuscular and chest wall disorders are individually uncommon but together form an important group of conditions that can lead to chronic ventilatory failure. This is best recognised in scoliosis, kyphosis, following a thoracoplasty, in muscular dystrophies, such as Duchenne muscular dystrophy (DMD), and myotonic dystrophy, after poliomyelitis and with motor neurone disease (amyotrophic lateral sclerosis).If bulbar function is impaired, tracheostomy ventilation may be required, but in other situations, noninvasive ventilation is preferable. Positive pressure techniques using nasal and face masks are usually the first choice, but negative pressure ventilation is an alternative.There are no randomised-controlled trials regarding the indications for initiating noninvasive ventilation, but this is usually provided if there are symptoms due to nocturnal hypoventilation or right heart failure in the presence of a raised carbon dioxide tension in arterial blood (Pa,CO2) either at night or, more usually, in the daytime as well. There is no evidence that “prophylactic” ventilatory support is of benefit if this is provided before ventilatory failure has appeared. Careful selection of patients is required, especially in the presence of progressive neuromuscular disorders such as DMD and motor neurone disease.There are no randomised-controlled trials concerning the outcome of noninvasive ventilation in these conditions, but studies have shown an improved quality of life, physical activity and haemodynamics, normalisation of blood gases and slight improvement in other physiological measures, such as the vital capacity and maximal mouth pressures. Survival in chest wall disorders is ∼90% at 1 yr and 80% at 5 yrs, and similar figures have been obtained in nonprogressive neuromuscular conditions. If, however, the underlying disorder is deteriorating, particularly if it involves the bulbar muscles, it may limit survival despite the provision of adequate noninvasive ventilatory support.

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“…This is referred to in France as an abstentionist argument. 3,4,11,[13][14][15]17,18,21 However, HCPs, especially those who are not used to caring for these patients, very often do not have a clear understanding of the quality of life for children with neuromuscular conditions. Moreover, HCPs project their own views onto their understanding of the child's quality of life with SMA type 1; understandably considering such a life to be unbearable and imagining oneself as quadriplegic for the rest of one's life.…”

Section: Quality Of Lifementioning

confidence: 99%

Tracheotomy and children with spinal muscular atrophy type 1

et al. 2012

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Spinal muscular atrophy (SMA) type 1 is a genetic neuromuscular disease in children that leads to degeneration of spinal cord motor neurons. This sometimes results in severe muscular paralysis requiring mechanical ventilation to sustain the child's life. The onset of SMA type 1, the most severe form of the disease, is during the first year of life. These children become severely paralysed, but retain their intellectual capacity. Ethical concerns arise when mechanical ventilation becomes necessary for survival. When professionals assess the resulting life for the child and family, they sometimes fear it will result in unreasonably excessive care. The aim of this article is to present an analysis of ethical arguments that could support or oppose the provision of invasive ventilation in this population. This examination is particularly relevant as France is one of the few countries performing tracheotomies and mechanical ventilation for this condition.

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Long-term ventilatory support in spinal muscular atrophy

Cited by 66 publications

References 12 publications

British Thoracic Society guideline for respiratory management of children with neuromuscular weakness

British Thoracic Society guideline for respiratory management of children with neuromuscular weakness

Noninvasive ventilation for chest wall and neuromuscular disorders

Tracheotomy and children with spinal muscular atrophy type 1

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