References:

Wagner KR, Lechtzin N, Judge DP. Current treatment of adult Duchenne muscular dystrophy. Biochim Biophys Acta. 2007;1772(2):229-237.

Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med. 2004;170(4):456-465.

Johns Hopkins Medicine, health library. What you need to know about Duchenne muscular dystrophy. http://www.hopkinsmedicine.org/healthlibrary/conditions/adult/nervous_system_disorders/duchenne_muscular_dystrophy_22,duchennemusculardystrophy. Accessed August 27, 2018.

Mayer OH, Finkel RS, Rummey C, et al. Characterization of pulmonary function in Duchenne muscular dystrophy. Pediatr Pulmonol. 2015;50(5):487-494.

Muscular dystrophy association. Facts about Duchenne & Becker muscular dystrophies. Copyright 2001. Updated December 2009. https://www.mda.org/sites/default/files/publications/Facts_DMD-BMD_P-211_0.pdf. Accessed August 27, 2018.

US Department of Health and Human Services. National Institutes of Health. National Heart, Lung, and Blood Institute. Duchenne muscular dystrophy. February 2005. http://www.parentprojectmd.org/site/DocServer/filename?docID=126. Accessed September 8, 2017.

Kinnett K, Rodger S, Vroom E, Furlong P, Aartsma-Rus A, Bushby K. Imperatives for Duchenne MD: a simplified guide to comprehensive care for Duchenne muscular dystrophy. PLoS Curr. 2015;1-11.

Bushby K, Finkel R, Birnkrant DJ, et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol. 2010;9(1):77-93.

Lo Mauro A, Aliverti A. Physiology of respiratory disturbances in muscular dystrophies. Breathe. 2016;12(4):318-327.

PubMed Health. How the lungs work. Updated June 11, 2014. https://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0063039/. Accessed August 27, 2018.

Archer JE, Gardner AC, Roper HP, Chikermane AA, Tatman AJ. Duchenne muscular dystrophy: the management of scoliosis. J Spine Surg. 2016;2(3):185-194.

MDA.org. Duchenne muscular dystrophy (DMD). Signs and symptoms. https://www.mda.org/disease/duchenne-muscular-dystrophy/signs-and-symptoms. Accessed August 27, 2018.

Kang SW, Kang YS, Sohn HS, Park JH, Moon JH. Respiratory muscle strength and cough capacity in patients with Duchenne muscular dystrophy. Yonsei Med J. 2006;47(2):184-190.

Kravitz RM. Airway clearance in Duchenne muscular dystrophy. Pediatrics. 2009;123(suppl 4):S231-S235.

Katz SL. Assessment of sleep-disordered breathing in pediatric neuromuscular diseases. Pediatrics. 2009;123(suppl 4):S222-S225.

Mayer OH, Henricson EK, McDonald CM, Buyse GM, on behalf of the Cooperative International Neuromuscular Research Group (CINRG). Advances in pulmonary care in Duchenne muscular dystrophy. US Neurology. 2017;10(1):2-8.

National Institutes of Health. National Heart, Lung, and Blood Institute. What is respiratory failure? Updated December 19, 2011. https://www.nhlbi.nih.gov/health/health-topics/topics/rf. Accessed August 27, 2018.

National Institutes of Health. National Heart, Lung, and Blood Institute. What is atelectasis? Updated January 13, 2012. https://www.nhlbi.nih.gov/health/health-topics/topics/atl. Accessed August 27, 2018.

Wagner MH, Berry RB. Disturbed sleep in a patient with Duchenne muscular dystrophy. J Clin Sleep Med. 2008;4(2):173-175.

Bushby K, Finkel R, Birnkrant DJ, et al; for the DMD Care Considerations Working Group. Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9(2):177-189.

MDA.org. Who’s who on the health care team? December 31, 1998. https://www.mda.org/quest/article/whos-who-health-care-team. Accessed August 27, 2018.

Advanced, multidisciplinary, coordinated care. Stony Brook Children's. https://www.stonybrookchildrens.org/specialties-services/clinical-programs/duchenneMD. Accessed August 27, 2018.

Muscular dystrophy Canada. Duchenne muscular dystrophy: A guide for parents. http://muscle.ca/wp-content/uploads/2012/11/434E_Duchenne_MD_2007.pdf. Accessed August 27, 2018.

American Academy of Physical Medicine and Rehabilitation. Physical medicine and rehabilitation FAQs. http://www.aapmr.org/career-center/medical-students/physical-medicine-and-rehabilitation-faqs. Accessed August 27, 2018.

Parent Project MD.org. The diagnosis and management of Duchenne muscular dystrophy, a guide for families March 2010. http://www.parentprojectmd.org/site/DocServer/Family_Guide.pdf?docID=9321. Accessed September 8, 2017.

Sharma GD. Pulmonary function testing in neuromuscular disorders. Pediatrics. 2009;123(suppl 4):S219-S221.

The BTS COPD Consortium. Spirometry in practice, a practical guide to using spirometry in primary care. 2nd edition. April 2005. https://www.brit-thoracic.org.uk/document-library/delivery-of-respiratory-care/spirometry/spirometry-in-practice-a-practical-guide-(2005). Accessed August 27, 2018.

CDC.gov. NIOSH spirometry training guide, unit five: basic spirometric calculations. December 1, 2003. https://www.cdc.gov/niosh/docs/2004-154c/pdfs/2004-154c-ch5.pdf. Accessed September 8, 2017.

Johns Hopkins Medicine, health library. What is a spirometer? http://www.hopkinsmedicine.org/healthlibrary/conditions/adult/respiratory_disorders/spirometers_85,p01332. Accessed August 27, 2018.

WebMD. Lung function tests. http://www.webmd.com/lung/lung-function-tests#1. Accessed August 27, 2018.

Quora. What is spirometry? https://www.quora.com/What-is-spirometry. Accessed August 27, 2018.

Sachs MC, Enright PL, Hinckley Stukovsky KD, Jiang R, Barr RG; Multi-Ethnic Study of Atherosclerosis Lung Study. Performance of maximal inspiratory pressure tests and MIP reference equations for 4 race/ethnic groups. Respir Care. 2009;54(10):1321-1328.

Polat M, Sakinci O, Ersoy B, Sezer RG, Yilmaz H. Assessment of sleep-related breathing disorders in patients with Duchenne muscular dystrophy. J Clin Med Res. 2012;4(5):332-337.

Hoque R. Sleep-disordered breathing in Duchenne muscular dystrophy: an assessment of the literature. J Clin Sleep Med. 2016;12(6):905-911.

American Thoracic Society patient information series. Pulse oximetry. Updated March 2018. https://www.thoracic.org/patients/patient-resources/resources/pulse-oximetry.pdf. Accessed August 27, 2018.

Firestone Institute for Respiratory Health. Overnight oximetry—sleep study. January 17, 2013. https://fhs.mcmaster.ca/purr/documents/Oxygen-Overnightoximetry.pdf. Accessed August 27, 2018.

Penn State Hershey, Milton S. Hershey Medical Center. Anatomy and function of the respiratory system. June 29, 2012. http://pennstatehershey.adam.com/content.aspx?productId=28&pid=28&gid=000141. Accessed August 27, 2018.

Healthline. Acute upper respiratory infection. http://www.healthline.com/health/acute-upper-respiratory-infection?print=true. Accessed August 27, 2018.

Mayo clinic. Acute sinusitis. http://www.mayoclinic.org/diseases-conditions/acute-sinusitis/home/ovc-20199692. Accessed August 27, 2018.

Dasaraju PV, Liu C. Chapter 93, Infections of the respiratory system. Baron S, ed. In: Medical microbiology, 4th edition. https://www.ncbi.nlm.nih.gov/books/NBK8142/. Accessed August 27, 2018.

Normandin, B. All about pneumonia and how to treat it effectively. Healthline. May 31, 2017. http://www.healthline.com/health/pneumonia#overview1. Accessed August 27, 2018.

Centers for Disease Control and Prevention. Flu and children with neurologic conditions. Updated May 26, 2016. https://www.cdc.gov/flu/protect/neurologic-pediatric.htm. Accessed August 27, 2018.

Mayo Clinic. Bronchitis. April 11, 2017. http://www.mayoclinic.org/diseases-conditions/bronchitis/home/ovc-20315098. Accessed August 27, 2018.

Mayo clinic. Pneumonia. http://www.mayoclinic.org/diseases-conditions/pneumonia/home/ovc-20204676. Accessed August 27, 2018.

Polverino M, Polverino F, Fasolino M, Andò F, Alfieri A, De Blasio F. Anatomy and neuro-pathophysiology of the cough reflex arc. Multidiscip Respir Med. 2012;7(1):1-5.

Franca DC, Camargos PAM, Vieira BSPP, Pereira DAG, Parreira VF. Cough peak flow in preschoolers: success rate and test-retest reproducibility. Fisioter Pesqui. 2015;22(3):275-279.

University Hospital Southampton, NHS Foundation Trust. Patient information factsheet: Manual assisted cough. Updated May 2017. http://www.uhs.nhs.uk/Media/Controlleddocuments/Patientinformation/Respiratory/Manual-assisted-cough-patient-information.pdf. Accessed August 27, 2018.

McKim DA, Katz SL, Barrowman N, Ni A, LeBlanc C. Lung volume recruitment slows pulmonary function decline in Duchenne muscular dystrophy. Arch Phys Med Rehabil. 2012;93(7):1117-1122.

Toussaint M, Pernet K, Steens M, Haan J, Sheers N. Cough augmentation in subjects with Duchenne muscular dystrophy: comparison of air stacking via a resuscitator bag versus mechanical ventilation. Respir Care. 2016;61(1):61-67.

YourCare medical. Cough assist. https://www.yourcareonline.com/cough-assist. Accessed August 27, 2018.

Kang SW, Kang YS, Moon JH, Yoo TW. Assisted cough and pulmonary compliance in patients with Duchenne muscular dystrophy. Yonsei Med J. 2005;46(2):233-238.

Johns Hopkins Medicine, health library. What is BiPap? http://www.hopkinsmedicine.org/healthlibrary/test_procedures/neurological/bipap_135,314. Accessed August 27, 2018.

Alaska sleep clinic, Alaska sleep education center. What is BiPap therapy? The difference between BiPap and CPAP. January 17, 2018. http://www.alaskasleep.com/blog/what-is-bipap-therapy-machine-bilevel-positive-airway-pressure. Accessed August 27, 2018.

MDA.org. Not enough ZZZzzzs? February 29, 2008. https://www.mda.org/quest/article/not-enough-zzzzzzs. Accessed August 27, 2018.

Resmed. Invasive ventilation (IV). https://www.resmed.com/epn/en/hospital/indications-for-ventilation/treatments/iv--invasive-ventilation--.html. Accessed August 27, 2018.

DMD pathfinders. Ventilation and Duchenne. http://www.dmdpathfinders.org.uk/wp-content/uploads/2014/11/dmd-ventilation-faq-booklet-small.pdf. Accessed August 27, 2018.

LoMauro A, D’Angelo MG, Aliverti A. Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options. Ther Clin Risk Manag. 2015;11:1475-1488.

Salera S, Menni F, Moggio M, Guez S, Sciacco M, Esposito S. Nutritional challenges in Duchenne muscular dystrophy. Nutrients. 2017;9(6)1-10.

Birnkrant DJ, Bushby K, Bann CM, et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management. Lancet Neurol. 2018;17(3):251-267.

Parent Project muscular dystrophy. Care for the GI system. http://www.parentprojectmd.org/site/PageServer?pagename=Care_area_gi. Accessed August 27, 2018.

MDA.org. Duchenne muscular dystrophy (DMD). Medical management. https://www.mda.org/disease/duchenne-muscular-dystrophy/medical-management. Accessed August 27, 2018.

Havers F, Fry A, Peacock G, Finelli L. Influenza vaccination and treatment in children with neurologic disorders. Ther Adv Vaccines. 2014;2(4):95-105.

Centers for Disease Control and Prevention. People at high risk of developing flu-related complications. Updated January 23, 2018. https://www.cdc.gov/flu/about/disease/high_risk.htm. Accessed August 27, 2018.

Centers for Disease Control and Prevention. Flu and children with neurologic conditions. Updated May 26, 2016. https://www.cdc.gov/flu/protect/neurologic-pediatric.htm. Accessed August 27, 2018.

Savoy ML. Pneumococcal vaccine. Merck Manual, Professional Version. Updated July 2018. http://www.merckmanuals.com/home/infections/immunization/pneumococcal-vaccine. Accessed August 27, 2018.

Centers for Disease Control and Prevention. Duchenne/Becker treatment and care. Updated January 19, 2016. https://www.cdc.gov/ncbddd/musculardystrophy/treatments.html. Accessed September 8, 2017.

Birnkrant DJ, Panitch HB, Benditt JO, et al. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest. 2007;132(6):1977-1986.

Gurnaney H, Brown A, Litman RS. Malignant hyperthermia and muscular dystrophies. Anesth Analg. 2009;109(4):1043-1048.

Cleveland Clinic. 5 tips to help you breathe easier in hot weather. August 19, 2015. https://health.clevelandclinic.org/2015/08/5-tips-to-help-you-breathe-easier-in-hot-weather/. Accessed August 27, 2018.

Anderson, P. Mayo clinic.org. Preparing for safe travel to high altitude. http://www.mayo.edu/research/documents/preparing-for-safe-travelpdf/doc-10026905. Accessed August 3, 2017.

American Academy of Allergy Asthma & Immunology. Outdoor allergens. https://www.aaaai.org/conditions-and-treatments/library/at-a-glance/outdoor-allergens. Accessed August 3, 2017.

American Lung Association. Cold weather and your lungs. January 4, 2018. http://www.lung.org/about-us/media/top-stories/cold-weather-your-lungs.html. Accessed August 27, 2018.

ClinicalTrials.gov. Santhera, Duchenne muscular dystrophy. https://clinicaltrials.gov/ct2/results?cond=Duchenne+Muscular+ Dystrophy&term=santhera&cntry1=&state1=&Search=Search. Accessed August 27, 2018.

Reprinted from The Lancet, Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management, Vol 17, David J Birnkrant, Katharine Bushby, Carla M Bann, Susan D Apkon, Angela Blackwell, David Brumbaugh, Laura E Case, Paula R Clemens, Stasia Hadjiyannakis, Shree Pandya, Natalie Street, Jean Tomezsko, Kathryn R Wagner, Leanne M Ward, David R Weber Pages 251-267, Mar 1, 2018 with permission from Elsevier. http://www.elsevier.com