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8 - Cystic Fibrosis in Pregnancy
- from Section 3 - Pulmonary Conditions Not Specific to Pregnancy
- Edited by Stephen E. Lapinsky, Lauren A. Plante
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- Respiratory Disease in Pregnancy
- Published online:
- 14 April 2020
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- 09 April 2020, pp 73-81
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Summary
Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that results in a multisystem disease. CF most commonly affects the respiratory, hepatobiliary, digestive and reproductive systems, though the majority of morbidity and mortality results from progressive obstructive lung disease and chronic pulmonary infection. In recent years, the survival of patients with CF has increased significantly with a recently reported median survival of up to 51.8 years of age.
Contributors
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- By Mitchell Aboulafia, Frederick Adams, Marilyn McCord Adams, Robert M. Adams, Laird Addis, James W. Allard, David Allison, William P. Alston, Karl Ameriks, C. Anthony Anderson, David Leech Anderson, Lanier Anderson, Roger Ariew, David Armstrong, Denis G. Arnold, E. J. Ashworth, Margaret Atherton, Robin Attfield, Bruce Aune, Edward Wilson Averill, Jody Azzouni, Kent Bach, Andrew Bailey, Lynne Rudder Baker, Thomas R. Baldwin, Jon Barwise, George Bealer, William Bechtel, Lawrence C. Becker, Mark A. Bedau, Ernst Behler, José A. Benardete, Ermanno Bencivenga, Jan Berg, Michael Bergmann, Robert L. Bernasconi, Sven Bernecker, Bernard Berofsky, Rod Bertolet, Charles J. Beyer, Christian Beyer, Joseph Bien, Joseph Bien, Peg Birmingham, Ivan Boh, James Bohman, Daniel Bonevac, Laurence BonJour, William J. Bouwsma, Raymond D. Bradley, Myles Brand, Richard B. Brandt, Michael E. Bratman, Stephen E. Braude, Daniel Breazeale, Angela Breitenbach, Jason Bridges, David O. Brink, Gordon G. Brittan, Justin Broackes, Dan W. Brock, Aaron Bronfman, Jeffrey E. Brower, Bartosz Brozek, Anthony Brueckner, Jeffrey Bub, Lara Buchak, Otavio Bueno, Ann E. Bumpus, Robert W. Burch, John Burgess, Arthur W. Burks, Panayot Butchvarov, Robert E. Butts, Marina Bykova, Patrick Byrne, David Carr, Noël Carroll, Edward S. Casey, Victor Caston, Victor Caston, Albert Casullo, Robert L. Causey, Alan K. L. Chan, Ruth Chang, Deen K. Chatterjee, Andrew Chignell, Roderick M. Chisholm, Kelly J. Clark, E. J. Coffman, Robin Collins, Brian P. Copenhaver, John Corcoran, John Cottingham, Roger Crisp, Frederick J. Crosson, Antonio S. Cua, Phillip D. Cummins, Martin Curd, Adam Cureton, Andrew Cutrofello, Stephen Darwall, Paul Sheldon Davies, Wayne A. Davis, Timothy Joseph Day, Claudio de Almeida, Mario De Caro, Mario De Caro, John Deigh, C. F. Delaney, Daniel C. Dennett, Michael R. DePaul, Michael Detlefsen, Daniel Trent Devereux, Philip E. Devine, John M. Dillon, Martin C. Dillon, Robert DiSalle, Mary Domski, Alan Donagan, Paul Draper, Fred Dretske, Mircea Dumitru, Wilhelm Dupré, Gerald Dworkin, John Earman, Ellery Eells, Catherine Z. Elgin, Berent Enç, Ronald P. Endicott, Edward Erwin, John Etchemendy, C. Stephen Evans, Susan L. Feagin, Solomon Feferman, Richard Feldman, Arthur Fine, Maurice A. Finocchiaro, William FitzPatrick, Richard E. Flathman, Gvozden Flego, Richard Foley, Graeme Forbes, Rainer Forst, Malcolm R. Forster, Daniel Fouke, Patrick Francken, Samuel Freeman, Elizabeth Fricker, Miranda Fricker, Michael Friedman, Michael Fuerstein, Richard A. Fumerton, Alan Gabbey, Pieranna Garavaso, Daniel Garber, Jorge L. A. Garcia, Robert K. Garcia, Don Garrett, Philip Gasper, Gerald Gaus, Berys Gaut, Bernard Gert, Roger F. Gibson, Cody Gilmore, Carl Ginet, Alan H. Goldman, Alvin I. Goldman, Alfonso Gömez-Lobo, Lenn E. Goodman, Robert M. Gordon, Stefan Gosepath, Jorge J. E. Gracia, Daniel W. Graham, George A. Graham, Peter J. Graham, Richard E. Grandy, I. Grattan-Guinness, John Greco, Philip T. Grier, Nicholas Griffin, Nicholas Griffin, David A. Griffiths, Paul J. Griffiths, Stephen R. Grimm, Charles L. Griswold, Charles B. Guignon, Pete A. Y. Gunter, Dimitri Gutas, Gary Gutting, Paul Guyer, Kwame Gyekye, Oscar A. Haac, Raul Hakli, Raul Hakli, Michael Hallett, Edward C. Halper, Jean Hampton, R. James Hankinson, K. R. Hanley, Russell Hardin, Robert M. Harnish, William Harper, David Harrah, Kevin Hart, Ali Hasan, William Hasker, John Haugeland, Roger Hausheer, William Heald, Peter Heath, Richard Heck, John F. Heil, Vincent F. Hendricks, Stephen Hetherington, Francis Heylighen, Kathleen Marie Higgins, Risto Hilpinen, Harold T. Hodes, Joshua Hoffman, Alan Holland, Robert L. Holmes, Richard Holton, Brad W. Hooker, Terence E. Horgan, Tamara Horowitz, Paul Horwich, Vittorio Hösle, Paul Hoβfeld, Daniel Howard-Snyder, Frances Howard-Snyder, Anne Hudson, Deal W. Hudson, Carl A. Huffman, David L. Hull, Patricia Huntington, Thomas Hurka, Paul Hurley, Rosalind Hursthouse, Guillermo Hurtado, Ronald E. Hustwit, Sarah Hutton, Jonathan Jenkins Ichikawa, Harry A. Ide, David Ingram, Philip J. Ivanhoe, Alfred L. Ivry, Frank Jackson, Dale Jacquette, Joseph Jedwab, Richard Jeffrey, David Alan Johnson, Edward Johnson, Mark D. Jordan, Richard Joyce, Hwa Yol Jung, Robert Hillary Kane, Tomis Kapitan, Jacquelyn Ann K. Kegley, James A. Keller, Ralph Kennedy, Sergei Khoruzhii, Jaegwon Kim, Yersu Kim, Nathan L. King, Patricia Kitcher, Peter D. Klein, E. D. Klemke, Virginia Klenk, George L. Kline, Christian Klotz, Simo Knuuttila, Joseph J. Kockelmans, Konstantin Kolenda, Sebastian Tomasz Kołodziejczyk, Isaac Kramnick, Richard Kraut, Fred Kroon, Manfred Kuehn, Steven T. Kuhn, Henry E. Kyburg, John Lachs, Jennifer Lackey, Stephen E. Lahey, Andrea Lavazza, Thomas H. Leahey, Joo Heung Lee, Keith Lehrer, Dorothy Leland, Noah M. Lemos, Ernest LePore, Sarah-Jane Leslie, Isaac Levi, Andrew Levine, Alan E. Lewis, Daniel E. Little, Shu-hsien Liu, Shu-hsien Liu, Alan K. L. Chan, Brian Loar, Lawrence B. Lombard, John Longeway, Dominic McIver Lopes, Michael J. Loux, E. J. Lowe, Steven Luper, Eugene C. Luschei, William G. Lycan, David Lyons, David Macarthur, Danielle Macbeth, Scott MacDonald, Jacob L. Mackey, Louis H. Mackey, Penelope Mackie, Edward H. Madden, Penelope Maddy, G. B. Madison, Bernd Magnus, Pekka Mäkelä, Rudolf A. Makkreel, David Manley, William E. Mann (W.E.M.), Vladimir Marchenkov, Peter Markie, Jean-Pierre Marquis, Ausonio Marras, Mike W. Martin, A. P. Martinich, William L. McBride, David McCabe, Storrs McCall, Hugh J. McCann, Robert N. McCauley, John J. McDermott, Sarah McGrath, Ralph McInerny, Daniel J. McKaughan, Thomas McKay, Michael McKinsey, Brian P. McLaughlin, Ernan McMullin, Anthonie Meijers, Jack W. Meiland, William Jason Melanson, Alfred R. Mele, Joseph R. Mendola, Christopher Menzel, Michael J. Meyer, Christian B. Miller, David W. Miller, Peter Millican, Robert N. Minor, Phillip Mitsis, James A. Montmarquet, Michael S. Moore, Tim Moore, Benjamin Morison, Donald R. Morrison, Stephen J. Morse, Paul K. Moser, Alexander P. D. Mourelatos, Ian Mueller, James Bernard Murphy, Mark C. Murphy, Steven Nadler, Jan Narveson, Alan Nelson, Jerome Neu, Samuel Newlands, Kai Nielsen, Ilkka Niiniluoto, Carlos G. Noreña, Calvin G. Normore, David Fate Norton, Nikolaj Nottelmann, Donald Nute, David S. Oderberg, Steve Odin, Michael O’Rourke, Willard G. Oxtoby, Heinz Paetzold, George S. Pappas, Anthony J. Parel, Lydia Patton, R. P. Peerenboom, Francis Jeffry Pelletier, Adriaan T. Peperzak, Derk Pereboom, Jaroslav Peregrin, Glen Pettigrove, Philip Pettit, Edmund L. Pincoffs, Andrew Pinsent, Robert B. Pippin, Alvin Plantinga, Louis P. Pojman, Richard H. Popkin, John F. Post, Carl J. Posy, William J. Prior, Richard Purtill, Michael Quante, Philip L. Quinn, Philip L. Quinn, Elizabeth S. Radcliffe, Diana Raffman, Gerard Raulet, Stephen L. Read, Andrews Reath, Andrew Reisner, Nicholas Rescher, Henry S. Richardson, Robert C. Richardson, Thomas Ricketts, Wayne D. Riggs, Mark Roberts, Robert C. Roberts, Luke Robinson, Alexander Rosenberg, Gary Rosenkranz, Bernice Glatzer Rosenthal, Adina L. Roskies, William L. Rowe, T. M. Rudavsky, Michael Ruse, Bruce Russell, Lilly-Marlene Russow, Dan Ryder, R. M. Sainsbury, Joseph Salerno, Nathan Salmon, Wesley C. Salmon, Constantine Sandis, David H. Sanford, Marco Santambrogio, David Sapire, Ruth A. Saunders, Geoffrey Sayre-McCord, Charles Sayward, James P. Scanlan, Richard Schacht, Tamar Schapiro, Frederick F. Schmitt, Jerome B. Schneewind, Calvin O. Schrag, Alan D. Schrift, George F. Schumm, Jean-Loup Seban, David N. Sedley, Kenneth Seeskin, Krister Segerberg, Charlene Haddock Seigfried, Dennis M. Senchuk, James F. Sennett, William Lad Sessions, Stewart Shapiro, Tommie Shelby, Donald W. Sherburne, Christopher Shields, Roger A. Shiner, Sydney Shoemaker, Robert K. Shope, Kwong-loi Shun, Wilfried Sieg, A. John Simmons, Robert L. Simon, Marcus G. Singer, Georgette Sinkler, Walter Sinnott-Armstrong, Matti T. Sintonen, Lawrence Sklar, Brian Skyrms, Robert C. Sleigh, Michael Anthony Slote, Hans Sluga, Barry Smith, Michael Smith, Robin Smith, Robert Sokolowski, Robert C. Solomon, Marta Soniewicka, Philip Soper, Ernest Sosa, Nicholas Southwood, Paul Vincent Spade, T. L. S. Sprigge, Eric O. Springsted, George J. Stack, Rebecca Stangl, Jason Stanley, Florian Steinberger, Sören Stenlund, Christopher Stephens, James P. Sterba, Josef Stern, Matthias Steup, M. A. Stewart, Leopold Stubenberg, Edith Dudley Sulla, Frederick Suppe, Jere Paul Surber, David George Sussman, Sigrún Svavarsdóttir, Zeno G. Swijtink, Richard Swinburne, Charles C. Taliaferro, Robert B. Talisse, John Tasioulas, Paul Teller, Larry S. Temkin, Mark Textor, H. S. Thayer, Peter Thielke, Alan Thomas, Amie L. Thomasson, Katherine Thomson-Jones, Joshua C. Thurow, Vzalerie Tiberius, Terrence N. Tice, Paul Tidman, Mark C. Timmons, William Tolhurst, James E. Tomberlin, Rosemarie Tong, Lawrence Torcello, Kelly Trogdon, J. D. Trout, Robert E. Tully, Raimo Tuomela, John Turri, Martin M. Tweedale, Thomas Uebel, Jennifer Uleman, James Van Cleve, Harry van der Linden, Peter van Inwagen, Bryan W. Van Norden, René van Woudenberg, Donald Phillip Verene, Samantha Vice, Thomas Vinci, Donald Wayne Viney, Barbara Von Eckardt, Peter B. M. Vranas, Steven J. Wagner, William J. Wainwright, Paul E. Walker, Robert E. Wall, Craig Walton, Douglas Walton, Eric Watkins, Richard A. Watson, Michael V. Wedin, Rudolph H. Weingartner, Paul Weirich, Paul J. Weithman, Carl Wellman, Howard Wettstein, Samuel C. Wheeler, Stephen A. White, Jennifer Whiting, Edward R. Wierenga, Michael Williams, Fred Wilson, W. Kent Wilson, Kenneth P. Winkler, John F. Wippel, Jan Woleński, Allan B. Wolter, Nicholas P. Wolterstorff, Rega Wood, W. Jay Wood, Paul Woodruff, Alison Wylie, Gideon Yaffe, Takashi Yagisawa, Yutaka Yamamoto, Keith E. Yandell, Xiaomei Yang, Dean Zimmerman, Günter Zoller, Catherine Zuckert, Michael Zuckert, Jack A. Zupko (J.A.Z.)
- Edited by Robert Audi, University of Notre Dame, Indiana
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- The Cambridge Dictionary of Philosophy
- Published online:
- 05 August 2015
- Print publication:
- 27 April 2015, pp ix-xxx
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Infection Prevention and Control Guideline for Cystic Fibrosis: 2013 Update
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- Lisa Saiman, Jane D. Siegel, John J. LiPuma, Rebekah F. Brown, Elizabeth A. Bryson, Mary Jo Chambers, Veronica S. Downer, Jill Fliege, Leslie A. Hazle, Manu Jain, Bruce C. Marshall, Catherine O’Malley, Suzanne R. Pattee, Gail Potter-Bynoe, Siobhan Reid, Karen A. Robinson, Kathryn A. Sabadosa, H. Joel Schmidt, Elizabeth Tullis, Jennifer Webber, David J. Weber
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- Journal:
- Infection Control & Hospital Epidemiology / Volume 35 / Issue S1 / August 2014
- Published online by Cambridge University Press:
- 10 May 2016, pp. s1-s67
- Print publication:
- August 2014
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The 2013 Infection Prevention and Control (IP&C) Guideline for Cystic Fibrosis (CF) was commissioned by the CF Foundation as an update of the 2003 Infection Control Guideline for CF. During the past decade, new knowledge and new challenges provided the following rationale to develop updated IP&C strategies for this unique population:
1. The need to integrate relevant recommendations from evidence-based guidelines published since 2003 into IP&C practices for CF. These included guidelines from the Centers for Disease Control and Prevention (CDC)/Healthcare Infection Control Practices Advisory Committee (HICPAC), the World Health Organization (WHO), and key professional societies, including the Infectious Diseases Society of America (IDSA) and the Society for Healthcare Epidemiology of America (SHEA). During the past decade, new evidence has led to a renewed emphasis on source containment of potential pathogens and the role played by the contaminated healthcare environment in the transmission of infectious agents. Furthermore, an increased understanding of the importance of the application of implementation science, monitoring adherence, and feedback principles has been shown to increase the effectiveness of IP&C guideline recommendations.
2. Experience with emerging pathogens in the non-CF population has expanded our understanding of droplet transmission of respiratory pathogens and can inform IP&C strategies for CF. These pathogens include severe acute respiratory syndrome coronavirus and the 2009 influenza A H1N1. Lessons learned about preventing transmission of methicillin-resistant Staphylococcus aureus (MRSA) and multidrug-resistant gram-negative pathogens in non-CF patient populations also can inform IP&C strategies for CF.
Analysis of prescribing practices in an acute psychiatric ward
- John Tully, Elizabeth Park, Allison Dunne, Ivan T Murray, Colm McDonald, Brian P Hallahan
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- Irish Journal of Psychological Medicine / Volume 27 / Issue 2 / June 2010
- Published online by Cambridge University Press:
- 13 June 2014, pp. 82-85
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- June 2010
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Objectives: Prescription writing is a basic clinical skill for all doctors; however, errors in prescriptions are frequent and can result in significant harm to patients both from sub-therapeutic dosages and from over prescribing. This study examines the prescribing practices of ‘as required’ medications in an acute psychiatric inpatient unit attached to a university teaching hospital.
Method: A clinical audit was conducted to examine both prescribing practices of ‘as required’ medication and recording of ‘as required’ medication in nursing notes. A series of educational interventions were undertaken in an attempt to improve prescribing practices in relation to ‘as required’ medication and two further audits were completed to assess any change in practice and whether such changes were sustained.
Results: Psycho-education led to an improvement in a number of prescribing practices, including increased generic prescribing, improved documentation of indications for medication administration and increased writing of medication dosages in acceptable abbreviations. These benefits were maintained four months after the education interventions. Rates of documenting the frequency of medication correctly and recording the administration of medication in nursing notes remained low.
Conclusion: Our audit cycle clearly demonstrates that education can ameliorate prescribing practices and these improvements were maintained four months after education was delivered. However some aspects of prescribing practice remained deficient, and more focused educational interventions are required in these areas.
COMMISSION 41 WORKING GROUP on ASTRONOMY AND WORLD HERITAGE
- Clive Ruggles, Gudrun Wolfschmidt, Ennio Badolati, Alan Batten, Juan Belmonte, Ragbir Bhathal, Peter Brosche, Suzanne Dbarbat, David DeVorkin, Hilmar W. Duerbeck, Priscilla Epifania, Roger Ferlet, Jos Funes, Ian S. Glass, Elizabeth Griffin, Alexander Gurshtein, John Hearnshaw, George Helou, Bambang Hidayat, Thomas Hockey, Jarita Holbrook, Manuela Incerti, S. O. Kepler, Rajesh Kochhar, Edwin C. Krupp, Kurt Locher, Penka Maglova-Stoeva, Areg Mickaelian, Bjorn R. Pettersen, Mara Cristina Pineda de Caras, Gennadiy Pinigin, Luciana Pompeia, Zhanna Pozhalova, Shi Yun-li, Irakli Simonia, Francoise Le Guet Tully, Richard Wainscoat
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- Journal:
- Proceedings of the International Astronomical Union / Volume 6 / Issue T27B / December 2010
- Published online by Cambridge University Press:
- 14 May 2010, pp. 267-269
- Print publication:
- December 2010
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What follows is a short report on the Business Meeting of the Astronomy and World Heritage Working Group held on Thursday August 6, 2009. This was the first formal Business Meeting of the Working Group since its formation following the signing of the Memorandum of Understanding between the IAU and UNESCO on Astronomy and World Heritage in October 2008.
Aripiprazole in the treatment of the psychosis prodrome: An open-label pilot study
- Scott W. Woods, Elizabeth M. Tully, Barbara C. Walsh, Keith A. Hawkins, Jennifer L. Callahan, Shuki J. Cohen, Daniel H. Mathalon, Tandy J. Miller, Thomas H. McGlashan
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- Journal:
- The British Journal of Psychiatry / Volume 191 / Issue S51 / December 2007
- Published online by Cambridge University Press:
- 02 January 2018, pp. s96-s101
- Print publication:
- December 2007
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Background
Research studies for the treatment of the putative prodromal phase of psychotic disorders have begun to appear
AimsTo obtain preliminary evidence of the short-term efficacy and safety of aripiprazole treatment in people with the psychosis prodrome
MethodFifteen participants meeting prodrome criteria (mean age 17.1 years, s.d.=5.5) enrolled in an open-label, single-site trial with fixed-flexible dosing of aripiprazole (5–30 mg/day) for 8 weeks
ResultsIn the mixed-effects repeated-measures analysis, improvement from baseline on the Scale of Prodromal Symptoms total score was statistically significant by the first week. No participant converted to psychosis and 13 completed treatment. Neuropsychological measures showed no consistent improvement; mean weight gain was 1.2 kg. Akathisia emerged in 8 participants, but the mean Barnes Akathisia Scale score fell to baseline levels by the final visit. Adverse events were otherwise minimal
ConclusionsAripiprazole shows a promising efficacy and safety profile for the psychosis prodrome. Placebo-controlled studies are indicated
Evaluation of the ambulatory and home care record: Agreement between self-reports and administrative data
- Denise N. Guerriere, Wendy J. Ungar, Mary Corey, Ruth Croxford, Jennifer E. Tranmer, Elizabeth Tullis, Peter C. Coyte
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- Journal:
- International Journal of Technology Assessment in Health Care / Volume 22 / Issue 2 / April 2006
- Published online by Cambridge University Press:
- 28 March 2006, pp. 203-210
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Objectives: Although measuring the utilization of ambulatory and home-based healthcare resources is an essential component of economic analyses, very little methodological attention has been devoted to the development and evaluation of resource costing tools. This study evaluated a newly developed tool, the Ambulatory and Home Care Record (AHCR), which comprehensively evaluates costs incurred by the health system and care recipients and their unpaid caregivers.
Methods: The level of agreement between self-reports from 110 cystic fibrosis care recipients and administrative data was assessed for four categories of health services: home-based visits with healthcare professionals, ambulatory visits with healthcare professionals, laboratory and diagnostic tests, and prescription medications.
Results: Agreement between care recipients' reports on the AHCR and administrative data ranged from moderate (kappa=0.41; 95 percent confidence interval, 0.16–0.61) for physician specialist visits to perfect (kappa=1.0) for physiotherapy visits.
Conclusions: By evaluating and standardizing a resource and costing tool, such as the AHCR, economic evaluations may be improved and comparisons of the resource implications for different services and for diverse populations are possible.
How Should an Unconscious Person with a Suspected Neck Injury be Positioned?
- Barry D. Gunn, Norman Eizenberg, Morry Silberstein, Joan M. McMeeken, Elizabeth A. Tully, Barry C. Stillman, Douglas J. Brown, Geoffrey A. Gutteridge
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- Journal:
- Prehospital and Disaster Medicine / Volume 10 / Issue 4 / December 1995
- Published online by Cambridge University Press:
- 28 June 2012, pp. 239-244
- Print publication:
- December 1995
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Introduction:
Awareness of the risk of spinal-cord damage in moving an unconscious person with a suspected neck injury into the “lateral recovery position,” coupled with the even greater risk of inadequate airway management if the person is not moved, has resulted in a suggested modification to the lateral recovery position for use in this circumstance.
Hypothesis:It is proposed that the modification to the lateral recovery position reduces movement of the neck. In this modification, one of the patient's arms is raised above the head (in full abduction) to support the head and neck. The position is called the “HAINES modified recovery position.” HAINES is an acronym for High Arm IN Endangered Spine.
Methods:Neck movements in two healthy volunteers were measured by the use of video-image analysis and radiographic studies when the volunteers were rolled from the supine position to both the lateral recovery position and the HAINES modified recovery position.
Results:For both subjects, the total degree of lateral flexion of the cervical spine in the HAINES modified recovery position was less than half of that measured during use of the lateral recovery position (while an open airway was maintained in each).
Conclusion:An unconscious person with a suspected neck injury should be positioned in the HAINES modified recovery position. There is less neck movement (and less degree of lateral angulation) than when the lateral recovery position is used, and, therefore, HAINES use carries less risk of spinal-cord damage.