Acute Effects of Mechanical Insufflation-Exsufflation on the Breathing Pattern in Stable Subjects With Duchenne Muscular Dystrophy

BACKGROUND: Duchenne muscular dystrophy (DMD) is characterized by progressive degeneration, wasting, and weakness of skeletal musculature, including respiratory muscles. Cough is also compromised with disease progression. Among cough-augmentation techniques, mechanical insufflation-exsufflation (MI-E) has demonstrated several clinical benefits in patients with chronic airway secretion obstruction and muscular weakness. In clinical practice, the use of MI-E in DMD patients is also suggested when they are stable with no airway infections. However, there is a paucity of studies that consider the effect of MI-E specifically on stable DMD patients who have adapted to the use of MI-E. METHODS: Twenty subjects with DMD with no active upper airway or lung infections, who used MI-E device regularly at home, were enrolled. They received a single MI-E treatment consisting of 5 cycles of 5 insufflations-exsufflations with their customary settings. Volume variations during quiet breathing, vital capacity, and cough before and after treatment were measured with optoelectronic plethysmography (OEP). RESULTS: A decrease in breathing frequency (P = .001) and the rapid shallow breathing index emerged (P = .007), while cough peak flow (Spirometer P = .86, OEP P = .58), vital capacity (Spirometer P = .78, OEP total chest wall P = .57), and end-expiratory volumes (Total chest wall P = .97, Ribcage P = .14, Abdomen P = .10) were not affected by the treatment. An increment of the chest wall volume variation during the expiratory cough phase was identified (P = .001), particularly due to an increase in abdominal expansion (P = .005). CONCLUSIONS: A single treatment of MI-E in subjects with stable DMD already adapted to the device can provide beneficial changes in breathing pattern through a significant decrease in breathing frequency and rapid shallow breathing. These findings suggest an improvement in short-term dyspnea, although there were no changes in lung-volume recruitment or unassisted cough peak flow.

[1]  S. J. Cala,et al.  Chest wall and lung volume estimation by optical reflectance motion analysis. , 1996, Journal of applied physiology.

[2]  D. Gozal,et al.  Respiratory muscle training in neuromuscular disease: long-term effects on strength and load perception. , 1999, Medicine and science in sports and exercise.

[3]  D. Trenchard.  Role of pulmonary stretch receptors during breathing in rabbits, cats and dogs. , 1977, Respiration physiology.

[4]  A. Pedotti,et al.  Compartmental Analysis of Breathing in the Supine and Prone Positions by Optoelectronic Plethysmography , 2004, Annals of Biomedical Engineering.

[5]  J. Nadel,et al.  Effect of differential vagal cold blockade on breathing pattern in conscious dogs. , 1973, Journal of applied physiology.

[6]  Stavros Tryfon,et al.  Hering-Breuer Reflex in Normal Adults and in Patients with Chronic Obstructive Pulmonary Disease and Interstitial Fibrosis , 2001, Respiration.

[7]  K. Bushby,et al.  Interventions for muscular dystrophy: molecular medicines entering the clinic , 2009, The Lancet.

[8]  J. Bach,et al.  A historical perspective on expiratory muscle aids and their impact on home care. , 2013, American journal of physical medicine & rehabilitation.

[9]  G Ferrigno,et al.  Three-dimensional optical analysis of chest wall motion. , 1994, Journal of applied physiology.

[10]  H. Coleridge,et al.  Reflexes Evoked from Tracheobronchial Tree and Lungs , 2011 .

[11]  J. Leiter,et al.  Entrainment of respiration in humans by periodic lung inflations. Effect of state and CO(2). , 1999, American journal of respiratory and critical care medicine.

[12]  R. Amin,et al.  Pulmonary management of the patient with muscular dystrophy. , 2005, Pediatric annals.

[13]  A. Grassino,et al.  Respiratory phase locking during mechanical ventilation in anesthetized human subjects. , 1986, The American journal of physiology.

[14]  A. Clément,et al.  Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases. , 2008, Chest.

[15]  A. Aliverti,et al.  Determinants of cough efficiency in Duchenne muscular dystrophy , 2014, Pediatric pulmonology.

[16]  A Pedotti,et al.  Abdominal volume contribution to tidal volume as an early indicator of respiratory impairment in Duchenne muscular dystrophy , 2009, European Respiratory Journal.

[17]  J. Guttmann,et al.  Short-term effects of positive end-expiratory pressure on breathing pattern: an interventional study in adult intensive care patients , 2005, Critical care.

[18]  J. Widdicombe,et al.  Lung reflexes in rabbits during pulmonary stretch receptor block by sulphur dioxide. , 1978, Respiration physiology.

[19]  A. Guz,et al.  The effect of lung inflation on breathing in man during wakefulness and sleep. , 1988, Respiration physiology.

[20]  R. Finkel,et al.  Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management , 2010, The Lancet Neurology.

[21]  C. M. Wiles,et al.  Respiratory involvement in primary muscle disorders: assessment and management. , 1993, The Quarterly journal of medicine.

[22]  R. Martin,et al.  The effect of a low continuous positive airway pressure on the reflex control of respiration in the preterm infant. , 1977, The Journal of pediatrics.

[23]  A. Pedotti,et al.  Low abdominal contribution to breathing as daytime predictor of nocturnal desaturation in adolescents and young adults with Duchenne Muscular Dystrophy. , 2012, Respiratory medicine.

[24]  J. Stocks,et al.  Influence of volume dependency and timing of airway occlusions on the Hering-Breuer reflex in infants. , 1998, Journal of applied physiology.

[25]  J. Benditt,et al.  The American College of Chest Physicians Consensus Statement on the Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy Undergoing Anesthesia or Sedation* , 2009, Pediatrics.

[26]  J. Stocks,et al.  Persistence of the Hering-Breuer reflex beyond the neonatal period. , 1991, Journal of applied physiology.

[27]  D. Pradon,et al.  Short-term effect of volume recruitment–derecruitment manoeuvre on chest-wall motion in Duchenne muscular dystrophy , 2017, Chronic respiratory disease.

[28]  U. Schara,et al.  Mechanical insufflation/exsufflation improves vital capacity in neuromuscular disorders , 2015, Chronic respiratory disease.

[29]  J. Bach,et al.  Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure. A different approach to weaning. , 1996, Chest.

[30]  J. Bach,et al.  Effects of Mechanical Insufflation‐Exsufflation on Respiratory Parameters for Patients with Chronic Airway Secretion Encumbrance. , 2004, Chest.

[31]  A. Guyton,et al.  Textbook of Medical Physiology , 1961 .

[32]  A Pedotti,et al.  Optoelectronic plethysmography in intensive care patients. , 2000, American journal of respiratory and critical care medicine.

[33]  J. Bach,et al.  Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. , 1997, Chest.

[34]  A. Pedotti,et al.  Effects of gender and posture on thoraco-abdominal kinematics during quiet breathing in healthy adults , 2010, Respiratory Physiology & Neurobiology.