Reviewed by Nicholas S. Hill, MD, Tufts-New England Medical Center, Boston, Massachusetts
If you have a neuromuscular condition such as post-polio syndrome, ALS, or Duchenne muscular dystrophy, you may not realize that your breathing muscles are weak and can become weaker. You may have difficulty breathing in deeply enough to fully expand your lungs or coughing strongly enough to clear mucus from your lungs.
It is essential to have periodic measurement of your respiratory muscle function and strength, and your oxygen and carbon dioxide levels to determine whether you may need to use a breathing machine (bilevel device or ventilator) during the night and/or need help with coughing. If you are a polio survivor, measurements of forced vital capacity (the maximum amount of air one can exhale) should be taken BOTH when you are sitting upright and lying down (in the supine position).
Even after you begin using assisted ventilation (a bilevel device or ventilator) it is critical that you periodically have your breathing monitored to prevent respiratory complications in the future and to improve your quality of life and survival. The recommendations below were written for individuals with Duchenne muscular dystrophy, but they are also appropriate for people with ALS and post-polio syndrome.
Routine Evaluation of Respiratory Function
Reference: Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patient with Duchenne muscular dystrophy. Am J Respir Crit Care Med. 2004;170:456¬-65. Official Journal of the American Thoracic Society. ©American Thoracic Society. Reprinted with permission.
Journal Articles: Breathing and Sleep Problems in Neuromuscular Conditions
Simonds AK. Recent advances in respiratory care for neuromuscular disease. Chest 2006; 130:1879-1886
Bach JR. Bilevel pressure vs. volume ventilators for amyotrophic lateral sclerosis. Chest 2006; 130: 1949
Bourke SC, Tomlinson M, Williams T, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurology 2006; 5:140-147
Lechtzin N, Wiener CM, Clawson L, et al. Use of noninvasive ventilation in patients with amyo-trophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2004; 5(1): 9-15
Servera E, Sancho J. Appropriate management of respiratory problems is of utmost importance in the treatment of patients with amyotrophic lateral sclerosis. Chest 2005; 127:1879-1882
Chatwin M, Ross E, Hart N, et al. Cough augmentation with mechanical insufflation/
exsufflation in patients with neuromuscular weakness. Eur Respir J, 2003; 21:502-508
Lechtzin N, Shade D, Clawson L, Wiener CM. Supramaximal inflation improves lung compliance in subjects with amyotrophic lateral sclerosis. Chest 2006; 129:1322-1329
Duchenne muscular dystrophy
Finder JD, Birnkrant D, Farber C, et al. Respiratory care of the patient with Duchenne muscular dystrophy; ATS consensus statement. Am J Respir Crit Care Med 2004; 170:456-465
Kohler M, Clarenbach CF, Böni L, et al. Quality of life, physical disability, and respiratory impairment in Duchenne muscular dystrophy. Am J Respir Crit Care Med 2005; 172:1032-1036
Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J 2006; 28:549-555
Late effects of polio (includes post-polio syndrome)
Bach J. Management of post-polio respiratory sequelae. Ann NY Acad Sci 1995; 25, 753: 96-102.
Bach J, Alba, A. Pulmonary dysfunction and sleep disordered breathing as post-polio sequelae: evaluation and management. Orthopedics 1991; 14:1329-1337
Gay, PC, Edmonds, LC (1995). Severe hypercapnia after low-flow oxygen therapy in patients with neuromuscular disease and diaphragmatic dysfunction. Mayo Clin Proc 1995; 70:327-330
Hsu, A , Staats, B. Postpolio sequelae and sleep-related disordered breathing. Mayo Clin Proc 1998; 73:216-224
Soliman M, Higgins S, El-Kabir DR, Davidson A, Williams, A, Howard, R. Non-invasive assessment of respiratory muscle strength in patients with previous poliomyelitis. Respir Med 2005; 99: 217-22
Prepared by International Ventilator Users Network (IVUN) www.ventusers.org (2010)
Tagged as: breathing , evaluation , hypoventilation , underventilation