Ventilation for neuromuscular disease and restrictive lung disease

Neuromuscular disease

Non-invasive ventilation has been shown to improve daytime arterial blood gases and the symptoms associated with respiratory failure for NMD patients:1

  • Less feeling of weakness
  • Improved swallowing
  • Reduced dyspnea
  • Less feeling of fatigue and sleepiness
  • Fewer morning headaches
  • Better concentration
  • Improvement in mood

In amyotrophic lateral sclerosis (ALS) patients, NIV improves quality of life and sleep-related symptoms. In patients without severe bulbar dysfunction, NIV prolongs survival by 7 months.2

In Duchenne muscular dystrophy (DMD) patients:

  • A retrospective review of 100 consecutive patients born between 1970 and 1990 found that NIV improved survival. Spinal surgery did not increase forced vital capacity, but in combination with nocturnal ventilation, further improved median survival.3
  • Significant reduction in hospital in-patient days.4

Restrictive lung disease

Treating a patient with respiratory failure associated with restrictive lung disease can help alleviate some of the symptoms of poor respiration, including:5

  • Dyspnea on exertion
  • Peripheral edema
  • Orthopnea
  • Morning headaches
  • Fatigue
  • Poor sleep quality
  • Loss of appetite

Indications for NIV for the treatment of respiratory failure include symptoms or signs of cor pulmonale, and one of the following:

  • Daytime arterial PaCO2 ≥ 45 mm Hg
  • Nocturnal SaO2 < 88% for five consecutive minutes
  • Progressive neuromuscular disease (NMD) with PImax <60 cm H2O or FVC<50% of predicted value

Non-invasive ventilation (NIV) is an effective treatment option for hypercapnic respiratory failure in patients with restrictive lung disease.5


  • 01

    Bourke SC et al. Noninvasive ventilation in ALS: Indications and effect on quality of life. Neurology. 2003; 61:171–177.

  • 02

    Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006 Feb;5(2):140-7

  • 03

    Eagle M, Bourke J, Bullock R, et al. Managing Duchenne muscular dystrophy—the additive effect of spinal surgery and home nocturnal ventilation in improving survival. Neuromuscular Disorders. 2007; 17:470–5

  • 04

    Leger P, Bedicam JM, Cornette A et al. Nasal intermittent positive pressure ventilation. Long term follow up in patients with severe chronic respiratory insufficiency. Chest. 1994; 105:100–105.

  • 05

    Shneerson JM, Simonds AK. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J. 2002; 20:480-487

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