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Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members - PubMed

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class="collections-new" name="collections" value="new" data-ga-category="save_share" data-ga-action="collections" data-ga-label="collections_radio_new"> <label for="collections-action-panel-new">Create a new collection</label> </li> <li> <input type="radio" id="collections-action-panel-existing" class="collections-existing" name="collections" value="existing" checked="true" data-ga-category="save_share" data-ga-action="collections" data-ga-label="collections_radio_existing"> <label for="collections-action-panel-existing">Add to an existing collection</label> </li> </ul> </div> <div class="controls-wrapper"> <div class="action-panel-control-wrap new-collections-controls"> <label for="collections-action-panel-add-to-new" class="action-panel-label required-field-asterisk"> Name your collection: </label> <input type="text" name="add-to-new-collection" id="collections-action-panel-add-to-new" class="collections-action-add-to-new" pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/" maxlength="100" data-ga-category="save_share" data-ga-action="create_collection" data-ga-label="non_favorties_collection"> <div class="collections-new-name-too-long usa-input-error-message selection-validation-message"> Name must be less than 100 characters </div> </div> <div class="action-panel-control-wrap existing-collections-controls"> <label for="collections-action-panel-add-to-existing" class="action-panel-label"> Choose a collection: </label> <select id="collections-action-panel-add-to-existing" class="action-panel-selector collections-action-add-to-existing" data-ga-category="save_share" data-ga-action="select_collection" data-ga-label="($('#collections-action-add-to-existing').val() === 'Favorites') ? 'Favorites' : 'non_favorites_collection'"> </select> <div class="collections-retry-load-on-error usa-input-error-message selection-validation-message"> Unable to load your collection due to an error<br> <a href="#">Please try again</a> </div> </div> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Adding..." data-pinger-ignore data-ga-category="save_share" data-ga-action="collections" data-ga-label="add"> Add </button> <button class="action-panel-cancel" aria-label="Close 'Add to Collections' panel" ref="linksrc=close_collections_panel" aria-controls="collections-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="collections" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="bibliography-action-panel" class="bibliography-action-panel action-panel in-progress-dots-panel" aria-hidden="true" data-bibliography-open-panel-enabled="false" data-bibliography-open-panel-url-hash="#open-bibliography-panel"> <div class="inner-wrap"> <h3 class="action-panel-heading"> Add to My Bibliography </h3> <form id="bibliography-action-panel-form" class="bibliography-action-panel-form action-panel-content action-form action-panel-smaller-selectors" data-add-to-bibliography-max-amount="100" data-add-to-bibliography-batch-size="10" data-bibliography-delegates-url="/list-bibliography-delegates/" data-add-to-bibliography-url="/add-to-bibliography/" data-get-article-ids-by-search-url="/get-article-ids-by-search/" data-mybib-root-url="https://www.ncbi.nlm.nih.gov/myncbi/collections/mybibliography/"> <input type="hidden" name="csrfmiddlewaretoken" value="VFMgLFVLK0kFbwl0FdLDQmKdIHroTRdwzDIBgSGP1qvhYopukh16pVP2djnXq3uB"> <div class="action-panel-control-wrap bibliographies-controls"> <div class="choice-group"> <ul class="bibliographies-action-add radio-group-items"> <li> <input name="bibliography" id="my-bibliography" class="my-bibliography" type="radio" checked/> <label for="my-bibliography">My Bibliography</label> </li> </ul> </div> </div> <div class="bibliographies-retry-load-on-error usa-input-error-message selection-validation-message"> Unable to load your delegates due to an error<br> <a href="#">Please try again</a> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Adding..." data-pinger-ignore> Add </button> <button class="action-panel-cancel" aria-label="Close 'Add to bibliography' panel" ref="linksrc=close_bibliography_panel" aria-controls="bibliography-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="mybib" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="saved-search-action-panel" class="saved-search-action-panel action-panel " aria-hidden="true" data-saved-search-open-panel-enabled="false" data-saved-search-open-panel-url-hash="#open-saved-search-panel"> <div class="inner-wrap"> <h2 class="action-panel-heading"> Your saved search </h2> <form id="saved-search-action-panel-form" class="saved-search-action-panel-form action-panel-content action-form" data-create-saved-search-url="/create-saved-search/" data-try-search-terms-url="/try-search-term/" data-saved-search-root-url="https://www.ncbi.nlm.nih.gov/myncbi/searches/"> <input type="hidden" name="csrfmiddlewaretoken" value="VFMgLFVLK0kFbwl0FdLDQmKdIHroTRdwzDIBgSGP1qvhYopukh16pVP2djnXq3uB"> <div class="action-panel-control-wrap"> <label for="saved-search-name" class="action-panel-label saved-search-name-label required-field-asterisk"> Name of saved search: </label> <input maxlength="200" type="text" name="saved-search-name" id="saved-search-name" class="saved-search-name" value="" required pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/"> </div> <div class="action-panel-control-wrap"> <label for="saved-search-term" class="action-panel-label required-field-asterisk"> Search terms: </label> <textarea name="saved-search-term" id="saved-search-term" class="saved-search-term" required></textarea> </div> <div class="test-search-term-wrap"> <a href="#" class="try-search-term">Test search terms</a> </div> <div class="choice-group action-panel-extra-margin-top"> <span class="action-panel-label" id="fieldset-label"> Would you like email updates of new search results? </span> <fieldset id="saved-search-alert" aria-describedby="fieldset-label"> <legend class="usa-sr-only">Saved Search Alert Radio Buttons</legend> <ul class="radio-group-items"> <li> <input type="radio" id="saved-search-alert-yes" class="saved-search-alert-yes" name="saved-search-alert" value="yes" checked> <label for="saved-search-alert-yes" class="action-panel-label">Yes</label> </li> <li> <input aria-label="No radio input" type="radio" id="saved-search-alert-no" class="saved-search-alert-no" name="saved-search-alert" value="no"> <label for="saved-search-alert-no" class="action-panel-label">No</label> </li> </ul> </fieldset> </div> <div class="alert-schedule-wrap"> <div class="action-panel-control-wrap"> <label class="action-panel-label"> Email: </label> <span aria-label="Email address" id="saved-search-email" class="action-panel-label"><span class="action-panel-label-bold"></span> (<a class="myncbi-account-settings" href="https://www.ncbi.nlm.nih.gov/account/settings/">change</a>)</span> </div> <div class="action-panel-control-wrap action-panel-extra-margin-top"> <label for="saved-search-frequency" class="action-panel-label"> Frequency: </label> <select id="saved-search-frequency" class="no-border-panel-selector saved-search-frequency"> <option value="monthly">Monthly</option> <option value="weekly">Weekly</option> <option value="daily">Daily</option> </select> </div> <div class="action-panel-control-wrap saved-search-monthly-additional"> <label for="saved-search-monthly-on-day" class="action-panel-label"> Which day? </label> <select id="saved-search-monthly-on-day" class="no-border-panel-selector"> <option value="Sunday">The first Sunday</option> <option value="Monday">The first Monday</option> <option value="Tuesday">The first Tuesday</option> <option value="Wednesday">The first Wednesday</option> <option value="Thursday">The first Thursday</option> <option value="Friday">The first Friday</option> <option value="Saturday">The first Saturday</option> <option value="day">The first day</option> <option value="weekday">The first weekday</option> </select> </div> <div class="action-panel-control-wrap saved-search-weekly-additional"> <label for="saved-search-weekly-on-day" class="action-panel-label"> Which day? </label> <select id="saved-search-weekly-on-day" class="no-border-panel-selector saved-search-weekly-on-day"> <option value="Sunday">Sunday</option> <option value="Monday">Monday</option> <option value="Tuesday">Tuesday</option> <option value="Wednesday">Wednesday</option> <option value="Thursday">Thursday</option> <option value="Friday">Friday</option> <option value="Saturday">Saturday</option> </select> </div> <div class="action-panel-control-wrap"> <label for="saved-search-report" class="action-panel-label"> Report format: </label> <select id="saved-search-report" class="no-border-panel-selector saved-search-report"> <option value="DocSum">Summary</option> <option value="DocSumText">Summary (text)</option> <option value="Abstract">Abstract</option> <option value="AbstractText">Abstract (text)</option> <option value="MEDLINE">PubMed</option> </select> </div> <div class="action-panel-control-wrap"> <label for="saved-search-amount" class="action-panel-label"> Send at most: </label> <select id="saved-search-amount" class="no-border-panel-selector saved-search-amount"> <option value="1">1 item</option> <option value="5" selected>5 items</option> <option value="10">10 items</option> <option value="20">20 items</option> <option value="50">50 items</option> <option value="100">100 items</option> <option value="200">200 items</option> </select> </div> <div> <input type="checkbox" id="saved-search-send-if-no-result" class="saved-search-send-if-no-result" name="saved-search-send-if-no-result"> <label for="saved-search-send-if-no-result" class="action-panel-label smaller-checkbox"> Send even when there aren't any new results </label> </div> <div class="action-panel-control-wrap option-text-in-email-wrap"> <label for="saved-search-email-text" class="action-panel-label"> Optional text in email: </label> <textarea name="saved-search-email-text" id="saved-search-email-text" class="saved-search-email-text"></textarea> </div> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Saving..." data-ga-category="save_share" data-ga-action="alert" data-ga-label="save"> Save </button> <button class="action-panel-cancel" aria-label="Close 'Your saved search' panel" ref="linksrc=close_saved_search_panel" aria-controls="saved-search-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="alert" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="citation-manager-action-panel" class="citation-manager-action-panel action-panel" aria-hidden="true"> <div class="inner-wrap"> <h2 class="action-panel-heading"> Create a file for external citation management software </h2> <form id="citation-manager-action-panel-form" class="action-panel-content action-form" action="/results-export-ids/" data-by-search-action="/results-export-search-data/" data-by-ids-action="/results-export-ids/" method="post" data-by-search-method="post" data-by-ids-method="post"> <input type="hidden" name="csrfmiddlewaretoken" value="VFMgLFVLK0kFbwl0FdLDQmKdIHroTRdwzDIBgSGP1qvhYopukh16pVP2djnXq3uB"> <input name="results-format" type="hidden" value="pubmed"/> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Sending..." data-ga-category="save_share" data-ga-action="citation_manager" data-ga-label="save"> Create file </button> <button class="action-panel-cancel" aria-label="Close 'Send citations to citation manager' panel" ref="linksrc=close_citation_manager_panel" aria-controls="citation-manager-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="citation_manager" data-ga-label="cancel"> Cancel </button> </div> </form> </div> </div> <div id="rss-action-panel" class="rss-action-panel action-panel " aria-hidden="true"> <div class="inner-wrap"> <h2 class="action-panel-heading"> Your RSS Feed </h2> <form id="rss-action-panel-form" class="rss-action-panel-form action-panel-content action-form" data-create-rss-feed-url="/create-rss-feed-url/" data-search-form-term-value=""> <input type="hidden" name="csrfmiddlewaretoken" value="VFMgLFVLK0kFbwl0FdLDQmKdIHroTRdwzDIBgSGP1qvhYopukh16pVP2djnXq3uB"> <div class="action-panel-control-wrap"> <label for="rss-name" class="action-panel-label required-field-asterisk"> Name of RSS Feed: </label> <input maxlength="200" placeholder="Name" type="text" name="rss-name" id="rss-name" class="rss-name" value='' required pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/"> </div> <div class="rss-limit-wrap"> <div class="action-panel-control-wrap action-panel-extra-margin-top"> <label for="rss-limit" class="action-panel-label"> Number of items displayed: </label> <select id="rss-limit" class="no-border-panel-selector rss-limit"> <option value="5">5</option> <option value="10">10</option> <option value="15" selected="selected">15</option> <option value="20">20</option> <option value="50">50</option> <option value="100">100</option> </select> </div> </div> <div class="action-panel-actions"> <button class="action-panel-submit" type="submit" data-loading-label="Creating..." data-ga-category="save_share" data-ga-action="alert" data-ga-label="save"> Create RSS </button> <button class="action-panel-cancel" aria-label="Close 'Your RSS' panel" ref="linksrc=close_rss_panel" aria-controls="rss-action-panel" aria-expanded="false" data-ga-category="save_share" data-ga-action="alert" data-ga-label="cancel"> Cancel </button> </div> <div class="action-panel-control-wrap rss-link-copy-wrap"> <label for="rss-link" class="usa-sr-only">RSS Link</label> <input placeholder="Your RSS Feed Link" type="text" name="rss-link" id="rss-link" class="rss-link" title="RSS Link"> <button type="button" disabled class="rss-link-copy-button disabled" data-ga-category="save_share" data-ga-action="rss" data-ga-label="copy"> Copy </button> </div> </form> </div> </div> </div> </div> <div class="article-page" id="article-page" data-article-pmid="25203083"> <aside class="page-sidebar"> <div class="inner-wrap"> <div class="full-text-links"> <div class="full-view"> <h3 class="title"> Full text links </h3> <div class="full-text-links-list"> <a 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data-pinger-ignore> JAMA </button><div id="full-view-journal" class="journal-actions-dropdown dropdown dropdown-container" hidden aria-label="Dropdown menu for journal JAMA"><div class="title">Actions</div><div class="content"><ul class="journal-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22JAMA%22%5Bjour%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22JAMA%22%5Bjour%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_journal_name_link&amp;journal_abbrev=JAMA" data-ga-category="search" data-ga-action="journal_link" data-ga-label="JAMA"> Search in PubMed </a></li><li><a class="search-in-nlm-catalog-link dropdown-block-link" ref ="linksrc=search_in_nlm_catalog_link" href="https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22JAMA%22%5BTitle+Abbreviation%5D" data-ga-category="search_catalog" data-ga-action="journal_link" data-ga-label="JAMA"> Search in NLM Catalog </a></li><li><a class="add-to-search-link dropdown-block-link" ref ="linksrc=add_to_search_link" role="button" data-search-term='"JAMA"[jour]' data-ga-category="search" data-ga-action="add_to_search" data-ga-label='"JAMA"[jour]' href="#"> Add to Search </a></li></ul></div></div></div><span class="period">. </span><span class="cit">2014 Sep 10;312(10):1033-48.</span> </div> <span class="citation-doi"> doi: 10.1001/jama.2014.10517. </span> </div> <h1 class="heading-title"> Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members </h1> <div class="inline-authors"> <div class="authors"> <div class="authors-list"> <span class="authors-list-item "><a class="full-name" href="/?term=Yawn+BP&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Barbara P Yawn">Barbara P Yawn</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Olmsted Medical Center, Rochester, Minnesota." href="#full-view-affiliation-1" ref="linksrc=author_aff"> 1 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Buchanan+GR&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="George R Buchanan">George R Buchanan</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Texas Southwestern Medical Center, Dallas." href="#full-view-affiliation-2" ref="linksrc=author_aff"> 2 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Afenyi-Annan+AN&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Araba N Afenyi-Annan">Araba N Afenyi-Annan</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of North Carolina, Chapel Hill." href="#full-view-affiliation-3" ref="linksrc=author_aff"> 3 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Ballas+SK&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Samir K Ballas">Samir K Ballas</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Thomas Jefferson University, Cardeza Foundation, Philadelphia, Pennsylvania." href="#full-view-affiliation-4" ref="linksrc=author_aff"> 4 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Hassell+KL&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Kathryn L Hassell">Kathryn L Hassell</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Colorado, Denver." href="#full-view-affiliation-5" ref="linksrc=author_aff"> 5 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=James+AH&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Andra H James">Andra H James</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Virginia, Charlottesville." href="#full-view-affiliation-6" ref="linksrc=author_aff"> 6 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Jordan+L&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Lanetta Jordan">Lanetta Jordan</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Foundation for Sickle Cell Disease Research, University of Miami, Miller School of Medicine, Miami, Florida." href="#full-view-affiliation-7" ref="linksrc=author_aff"> 7 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Lanzkron+SM&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Sophie M Lanzkron">Sophie M Lanzkron</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Johns Hopkins School of Medicine, Baltimore, Maryland." href="#full-view-affiliation-8" ref="linksrc=author_aff"> 8 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Lottenberg+R&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Richard Lottenberg">Richard Lottenberg</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Florida, Gainesville." href="#full-view-affiliation-9" ref="linksrc=author_aff"> 9 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Savage+WJ&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="William J Savage">William J Savage</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Brigham and Women&#x27;s Hospital and Harvard Medical School, Boston, Massachusetts." href="#full-view-affiliation-10" ref="linksrc=author_aff"> 10 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Tanabe+PJ&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Paula J Tanabe">Paula J Tanabe</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Duke University, Schools of Nursing and Medicine, Durham, North Carolina." href="#full-view-affiliation-11" ref="linksrc=author_aff"> 11 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Ware+RE&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Russell E Ware">Russell E Ware</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Cincinnati Children&#x27;s Hospital Medical Center, Cincinatti, Ohio." href="#full-view-affiliation-12" ref="linksrc=author_aff"> 12 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Murad+MH&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="M Hassan Murad">M Hassan Murad</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Mayo Clinic College of Medicine, Rochester, Minnesota." href="#full-view-affiliation-13" ref="linksrc=author_aff"> 13 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Goldsmith+JC&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Jonathan C Goldsmith">Jonathan C Goldsmith</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland16Dr Goldsmith is now with the Rare Diseases Program, Office of New Drugs, US Food and Drug Administration, Bethesda, Maryland." href="#full-view-affiliation-14" ref="linksrc=author_aff"> 14 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Ortiz+E&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Eduardo Ortiz">Eduardo Ortiz</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland17Dr Ortiz is now a private senior consultant in Bethesda, Maryland." href="#full-view-affiliation-15" ref="linksrc=author_aff"> 15 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Fulwood+R&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Robinson Fulwood">Robinson Fulwood</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland18Dr Fulwood is now retired." href="#full-view-affiliation-16" ref="linksrc=author_aff"> 16 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Horton+A&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Ann Horton">Ann Horton</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="American Institutes for Research, Silver Spring, Maryland." href="#full-view-affiliation-17" ref="linksrc=author_aff"> 17 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=John-Sowah+J&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Joylene John-Sowah">Joylene John-Sowah</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland." href="#full-view-affiliation-18" ref="linksrc=author_aff"> 18 </a></sup></span> </div> </div> </div> <div class="short-article-details"> Affiliations <button class="more-details" id="toggle-authors" data-alt-text="Collapse" aria-controls="expanded-authors" aria-expanded="false" data-pinger-ignore data-fetch-url="/25203083/long-authors/" data-id-prefix=""> <span class="title"> Expand </span> </button> </div> <div class="extended-article-details" id="expanded-authors" aria-hidden="true"> <div class="expanded-authors" id="full-view-expanded-authors"> <div class="affiliations"> <h3 class="title"> Affiliations </h3> <ul class="item-list"> <li data-affiliation-id="full-view-affiliation-1" id="full-view-affiliation-1" ><sup class="key">1</sup> Olmsted Medical Center, Rochester, Minnesota.</li> <li data-affiliation-id="full-view-affiliation-2" id="full-view-affiliation-2" ><sup class="key">2</sup> University of Texas Southwestern Medical Center, Dallas.</li> <li data-affiliation-id="full-view-affiliation-3" id="full-view-affiliation-3" ><sup class="key">3</sup> University of North Carolina, Chapel Hill.</li> <li data-affiliation-id="full-view-affiliation-4" id="full-view-affiliation-4" ><sup class="key">4</sup> Thomas Jefferson University, Cardeza Foundation, Philadelphia, Pennsylvania.</li> <li data-affiliation-id="full-view-affiliation-5" id="full-view-affiliation-5" ><sup class="key">5</sup> University of Colorado, Denver.</li> <li data-affiliation-id="full-view-affiliation-6" id="full-view-affiliation-6" ><sup class="key">6</sup> University of Virginia, Charlottesville.</li> <li data-affiliation-id="full-view-affiliation-7" id="full-view-affiliation-7" ><sup class="key">7</sup> Foundation for Sickle Cell Disease Research, University of Miami, Miller School of Medicine, Miami, Florida.</li> <li data-affiliation-id="full-view-affiliation-8" id="full-view-affiliation-8" ><sup class="key">8</sup> Johns Hopkins School of Medicine, Baltimore, Maryland.</li> <li data-affiliation-id="full-view-affiliation-9" id="full-view-affiliation-9" ><sup class="key">9</sup> University of Florida, Gainesville.</li> <li data-affiliation-id="full-view-affiliation-10" id="full-view-affiliation-10" ><sup class="key">10</sup> Brigham and Women&#x27;s Hospital and Harvard Medical School, Boston, Massachusetts.</li> <li data-affiliation-id="full-view-affiliation-11" id="full-view-affiliation-11" ><sup class="key">11</sup> Duke University, Schools of Nursing and Medicine, Durham, North Carolina.</li> <li data-affiliation-id="full-view-affiliation-12" id="full-view-affiliation-12" ><sup class="key">12</sup> Cincinnati Children&#x27;s Hospital Medical Center, Cincinatti, Ohio.</li> <li data-affiliation-id="full-view-affiliation-13" id="full-view-affiliation-13" ><sup class="key">13</sup> Mayo Clinic College of Medicine, Rochester, Minnesota.</li> <li data-affiliation-id="full-view-affiliation-14" id="full-view-affiliation-14" ><sup class="key">14</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland16Dr Goldsmith is now with the Rare Diseases Program, Office of New Drugs, US Food and Drug Administration, Bethesda, Maryland.</li> <li data-affiliation-id="full-view-affiliation-15" id="full-view-affiliation-15" ><sup class="key">15</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland17Dr Ortiz is now a private senior consultant in Bethesda, Maryland.</li> <li data-affiliation-id="full-view-affiliation-16" id="full-view-affiliation-16" ><sup class="key">16</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland18Dr Fulwood is now retired.</li> <li data-affiliation-id="full-view-affiliation-17" id="full-view-affiliation-17" ><sup class="key">17</sup> American Institutes for Research, Silver Spring, Maryland.</li> <li data-affiliation-id="full-view-affiliation-18" id="full-view-affiliation-18" ><sup class="key">18</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland.</li> </ul> </div> </div> </div> <ul class="identifiers" id="full-view-identifiers"> <li> <span class="identifier pubmed"> <span class="id-label"> PMID: </span> <strong class="current-id" title="PubMed ID">25203083</strong> </span> </li> <li> <span class="identifier doi"> <span class="id-label"> DOI: </span> <a class="id-link" target="_blank" rel="noopener" ref="linksrc=article_id_link&amp;article_id=10.1001/jama.2014.10517&amp;id_type=DOI" href="https://doi.org/10.1001/jama.2014.10517" data-ga-category="full_text" data-ga-action="DOI" > 10.1001/jama.2014.10517 </a> </span> </li> </ul> <div class="in-clipboard-label " hidden> Item in Clipboard </div> </div> <div class="short-view" id="short-view-heading"> <div class="publication-type" >Review</div> <h1 class="heading-title"> Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members </h1> <div class="short-citation"> <span class="authors-list"> <span class="authors-list-item "><span class="full-name">Barbara P Yawn</span><span class="citation-separator">&nbsp;et al.</span></span> </span> <span class="citation-journal"> JAMA<span class="citation-separator">.</span> </span> <span class="date"> <time class="citation-year" datetime="2014">2014</time><span class="citation-separator">.</span> </span> </div> <div class="more-article-details-section with-display-settings"> <button class="more-article-details" id="toggle-article-details" data-alt-text="Hide details" ref="linksrc=toggle_article_details" aria-controls="full-article-details" aria-expanded="false" data-fetch-url="/25203083/long-authors/" data-id-prefix=""> <span 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="linksrc=search_in_nlm_catalog_link" href="https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22JAMA%22%5BTitle+Abbreviation%5D" data-ga-category="search_catalog" data-ga-action="journal_link" data-ga-label="JAMA"> Search in NLM Catalog </a></li><li><a class="add-to-search-link dropdown-block-link" ref ="linksrc=add_to_search_link" role="button" data-search-term='"JAMA"[jour]' data-ga-category="search" data-ga-action="add_to_search" data-ga-label='"JAMA"[jour]' href="#"> Add to Search </a></li></ul></div></div></div><span class="period">. </span><span class="cit">2014 Sep 10;312(10):1033-48.</span> </div> <span class="citation-doi"> doi: 10.1001/jama.2014.10517. </span> </div> <div class="expanded-authors" id="short-view-expanded-authors"> <div class="authors"> <h3 class="title"> Authors </h3> <div class="authors-list"> <span class="authors-list-item "><a class="full-name" href="/?term=Yawn+BP&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Barbara P Yawn">Barbara P Yawn</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Olmsted Medical Center, Rochester, Minnesota." href="#short-view-affiliation-1" ref="linksrc=author_aff"> 1 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Buchanan+GR&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="George R Buchanan">George R Buchanan</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Texas Southwestern Medical Center, Dallas." href="#short-view-affiliation-2" ref="linksrc=author_aff"> 2 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Afenyi-Annan+AN&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Araba N Afenyi-Annan">Araba N Afenyi-Annan</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of North Carolina, Chapel Hill." href="#short-view-affiliation-3" ref="linksrc=author_aff"> 3 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Ballas+SK&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Samir K Ballas">Samir K Ballas</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Thomas Jefferson University, Cardeza Foundation, Philadelphia, Pennsylvania." href="#short-view-affiliation-4" ref="linksrc=author_aff"> 4 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Hassell+KL&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Kathryn L Hassell">Kathryn L Hassell</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Colorado, Denver." href="#short-view-affiliation-5" ref="linksrc=author_aff"> 5 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=James+AH&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Andra H James">Andra H James</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Virginia, Charlottesville." href="#short-view-affiliation-6" ref="linksrc=author_aff"> 6 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Jordan+L&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Lanetta Jordan">Lanetta Jordan</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Foundation for Sickle Cell Disease Research, University of Miami, Miller School of Medicine, Miami, Florida." href="#short-view-affiliation-7" ref="linksrc=author_aff"> 7 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Lanzkron+SM&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Sophie M Lanzkron">Sophie M Lanzkron</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Johns Hopkins School of Medicine, Baltimore, Maryland." href="#short-view-affiliation-8" ref="linksrc=author_aff"> 8 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Lottenberg+R&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Richard Lottenberg">Richard Lottenberg</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="University of Florida, Gainesville." href="#short-view-affiliation-9" ref="linksrc=author_aff"> 9 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Savage+WJ&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="William J Savage">William J Savage</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Brigham and Women&#x27;s Hospital and Harvard Medical School, Boston, Massachusetts." href="#short-view-affiliation-10" ref="linksrc=author_aff"> 10 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Tanabe+PJ&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Paula J Tanabe">Paula J Tanabe</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Duke University, Schools of Nursing and Medicine, Durham, North Carolina." href="#short-view-affiliation-11" ref="linksrc=author_aff"> 11 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Ware+RE&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Russell E Ware">Russell E Ware</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Cincinnati Children&#x27;s Hospital Medical Center, Cincinatti, Ohio." href="#short-view-affiliation-12" ref="linksrc=author_aff"> 12 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Murad+MH&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="M Hassan Murad">M Hassan Murad</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Mayo Clinic College of Medicine, Rochester, Minnesota." href="#short-view-affiliation-13" ref="linksrc=author_aff"> 13 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Goldsmith+JC&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Jonathan C Goldsmith">Jonathan C Goldsmith</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland16Dr Goldsmith is now with the Rare Diseases Program, Office of New Drugs, US Food and Drug Administration, Bethesda, Maryland." href="#short-view-affiliation-14" ref="linksrc=author_aff"> 14 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Ortiz+E&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Eduardo Ortiz">Eduardo Ortiz</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland17Dr Ortiz is now a private senior consultant in Bethesda, Maryland." href="#short-view-affiliation-15" ref="linksrc=author_aff"> 15 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Fulwood+R&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Robinson Fulwood">Robinson Fulwood</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland18Dr Fulwood is now retired." href="#short-view-affiliation-16" ref="linksrc=author_aff"> 16 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=Horton+A&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Ann Horton">Ann Horton</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="American Institutes for Research, Silver Spring, Maryland." href="#short-view-affiliation-17" ref="linksrc=author_aff"> 17 </a></sup><span class="comma">,&nbsp;</span></span><span class="authors-list-item "><a class="full-name" href="/?term=John-Sowah+J&amp;cauthor_id=25203083" ref="linksrc=author_name_link" data-ga-category="search" data-ga-action="author_link" data-ga-label="Joylene John-Sowah">Joylene John-Sowah</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="National Heart, Lung, and Blood Institute, Bethesda, Maryland." href="#short-view-affiliation-18" ref="linksrc=author_aff"> 18 </a></sup></span> </div> </div> <div class="affiliations"> <h3 class="title"> Affiliations </h3> <ul class="item-list"> <li data-affiliation-id="short-view-affiliation-1" id="short-view-affiliation-1" ><sup class="key">1</sup> Olmsted Medical Center, Rochester, Minnesota.</li> <li data-affiliation-id="short-view-affiliation-2" id="short-view-affiliation-2" ><sup class="key">2</sup> University of Texas Southwestern Medical Center, Dallas.</li> <li data-affiliation-id="short-view-affiliation-3" id="short-view-affiliation-3" ><sup class="key">3</sup> University of North Carolina, Chapel Hill.</li> <li data-affiliation-id="short-view-affiliation-4" id="short-view-affiliation-4" ><sup class="key">4</sup> Thomas Jefferson University, Cardeza Foundation, Philadelphia, Pennsylvania.</li> <li data-affiliation-id="short-view-affiliation-5" id="short-view-affiliation-5" ><sup class="key">5</sup> University of Colorado, Denver.</li> <li data-affiliation-id="short-view-affiliation-6" id="short-view-affiliation-6" ><sup class="key">6</sup> University of Virginia, Charlottesville.</li> <li data-affiliation-id="short-view-affiliation-7" id="short-view-affiliation-7" ><sup class="key">7</sup> Foundation for Sickle Cell Disease Research, University of Miami, Miller School of Medicine, Miami, Florida.</li> <li data-affiliation-id="short-view-affiliation-8" id="short-view-affiliation-8" ><sup class="key">8</sup> Johns Hopkins School of Medicine, Baltimore, Maryland.</li> <li data-affiliation-id="short-view-affiliation-9" id="short-view-affiliation-9" ><sup class="key">9</sup> University of Florida, Gainesville.</li> <li data-affiliation-id="short-view-affiliation-10" id="short-view-affiliation-10" ><sup class="key">10</sup> Brigham and Women&#x27;s Hospital and Harvard Medical School, Boston, Massachusetts.</li> <li data-affiliation-id="short-view-affiliation-11" id="short-view-affiliation-11" ><sup class="key">11</sup> Duke University, Schools of Nursing and Medicine, Durham, North Carolina.</li> <li data-affiliation-id="short-view-affiliation-12" id="short-view-affiliation-12" ><sup class="key">12</sup> Cincinnati Children&#x27;s Hospital Medical Center, Cincinatti, Ohio.</li> <li data-affiliation-id="short-view-affiliation-13" id="short-view-affiliation-13" ><sup class="key">13</sup> Mayo Clinic College of Medicine, Rochester, Minnesota.</li> <li data-affiliation-id="short-view-affiliation-14" id="short-view-affiliation-14" ><sup class="key">14</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland16Dr Goldsmith is now with the Rare Diseases Program, Office of New Drugs, US Food and Drug Administration, Bethesda, Maryland.</li> <li data-affiliation-id="short-view-affiliation-15" id="short-view-affiliation-15" ><sup class="key">15</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland17Dr Ortiz is now a private senior consultant in Bethesda, Maryland.</li> <li data-affiliation-id="short-view-affiliation-16" id="short-view-affiliation-16" ><sup class="key">16</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland18Dr Fulwood is now retired.</li> <li data-affiliation-id="short-view-affiliation-17" id="short-view-affiliation-17" ><sup class="key">17</sup> American Institutes for Research, Silver Spring, Maryland.</li> <li data-affiliation-id="short-view-affiliation-18" id="short-view-affiliation-18" ><sup class="key">18</sup> National Heart, Lung, and Blood Institute, Bethesda, Maryland.</li> </ul> </div> </div> <ul class="identifiers" id="short-view-identifiers"> <li> <span class="identifier pubmed"> <span class="id-label"> PMID: </span> <strong class="current-id" title="PubMed ID">25203083</strong> </span> </li> <li> <span class="identifier doi"> <span class="id-label"> DOI: </span> <a class="id-link" target="_blank" rel="noopener" ref="linksrc=article_id_link&amp;article_id=10.1001/jama.2014.10517&amp;id_type=DOI" href="https://doi.org/10.1001/jama.2014.10517" data-ga-category="full_text" data-ga-action="DOI" > 10.1001/jama.2014.10517 </a> </span> </li> </ul> <div class="in-clipboard-label " hidden> Item in Clipboard </div> </div> </div> </header> <div class="actions-buttons inline"><div class="inner-wrap"><button class="full-text-links-button full-text-links-dialog-trigger" title="Open dialog with full text links" ref="linksrc=full_text_links"> Full text links </button><button class="citation-button citation-dialog-trigger" aria-label="Open dialog with citation text in different styles" data-ga-category="save_share" data-ga-action="cite" data-ga-label="open" data-all-citations-url="/25203083/citations/" data-citation-style="nlm" data-pubmed-format-link="/25203083/export/"><span class="button-label">Cite</span></button><button class="more-actions-button more-actions-dialog-trigger" title="Open dialog with more actions to take" ref="linksrc=more_actions_btn"></button><div class="display-options" data-format-key="format" data-default-format-value="abstract" data-current-format-value="abstract" role="region"><button class="trigger usa-button" title="Change format" data-ga-category="display_options" data-ga-action="display_options_dialog"><span class="button-label">Display options</span></button><div class="dropdown" hidden><div class="title"> Display options </div><div class="content"><div class="format-container"><span class="option-label"> Format </span><select name="format" data-initial-value="abstract" aria-label="Display format" id="inline-actions-display-format"><option value="abstract">Abstract</option><option value="pubmed">PubMed</option><option value="pmid">PMID</option></select></div></div></div></div></div></div> <div class="linked-articles" id="linked-correction-forward"> <h2 class="title"> Erratum in </h2> <ul class="item-list usa-unstyled-list"> <li class="raw-citation">JAMA. 2014 Nov 12;312(18):1932</li> <li class="raw-citation">JAMA. 2015 Feb 17;313(7):729</li> </ul> </div> <div class="abstract" id="abstract"> <h2 class="title"> Abstract </h2> <div class="abstract-content selected" id="eng-abstract"> <p> <strong class="sub-title"> Importance: </strong> Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused. </p> <p> <strong class="sub-title"> Objective: </strong> To support and expand the number of health professionals able and willing to provide care for persons with SCD. </p> <p> <strong class="sub-title"> Evidence review: </strong> Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1, 2014, addressed key questions developed by the expert panel members and methodologists. </p> <p> <strong class="sub-title"> Findings: </strong> Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (≥200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria in adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated. </p> <p> <strong class="sub-title"> Conclusions and relevance: </strong> Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. Many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals with SCD. </p> </div> </div> <p class="disclaimer" id="disclaimer"> <a href="/disclaimer/" target="_blank" ga_category="literature_resources" ga_action="disclaimer_link" ga_label="under_abstract">PubMed Disclaimer</a> </p> <div class="linked-articles" id="linked-commentary"> <h2 class="title"> Comment in </h2> <ul class="item-list usa-unstyled-list"> <li class="full-docsum"> <div class="docsum-content" style="display:inline;"> <a class="docsum-title" href="/25203081/" ref="article_id=25203081&amp;linksrc=comments_link" data-ga-category="comment_correction" data-ga-action="abstract_page_link" data-ga-label="linked-commentary"> The challenge of creating an evidence-based guideline for sickle cell disease. </a> <div class="docsum-citation full-citation"> <span class="docsum-authors full-authors">DeBaun MR.</span> <span class="docsum-authors short-authors">DeBaun MR.</span> <span class="docsum-journal-citation full-journal-citation">JAMA. 2014 Sep 10;312(10):1004-5. doi: 10.1001/jama.2014.11103.</span> <span class="docsum-journal-citation short-journal-citation">JAMA. 2014.</span> <span class="citation-part">PMID: <span class="docsum-pmid">25203081</span></span> <span class="no-abstract spaced-citation-item citation-part">No abstract available.</span> </div> </div> </li> <li class="full-docsum"> <div class="docsum-content" style="display:inline;"> <a class="docsum-title" href="/25562273/" ref="article_id=25562273&amp;linksrc=comments_link" data-ga-category="comment_correction" data-ga-action="abstract_page_link" data-ga-label="linked-commentary"> Management of patients with sickle cell disease. </a> <div class="docsum-citation full-citation"> <span class="docsum-authors full-authors">Pollack S.</span> <span class="docsum-authors short-authors">Pollack S.</span> <span class="docsum-journal-citation full-journal-citation">JAMA. 2015 Jan 6;313(1):90-1. doi: 10.1001/jama.2014.15895.</span> <span class="docsum-journal-citation short-journal-citation">JAMA. 2015.</span> <span class="citation-part">PMID: <span class="docsum-pmid">25562273</span></span> <span class="no-abstract spaced-citation-item citation-part">No abstract available.</span> </div> </div> </li> <li class="full-docsum"> <div class="docsum-content" style="display:inline;"> <a class="docsum-title" href="/25562274/" ref="article_id=25562274&amp;linksrc=comments_link" data-ga-category="comment_correction" data-ga-action="abstract_page_link" data-ga-label="linked-commentary"> Management of patients with sickle cell disease. </a> <div class="docsum-citation full-citation"> <span class="docsum-authors full-authors">Klings ES, Kato GJ, Gladwin MT.</span> <span class="docsum-authors short-authors">Klings ES, et al.</span> <span class="docsum-journal-citation full-journal-citation">JAMA. 2015 Jan 6;313(1):91. doi: 10.1001/jama.2014.15898.</span> <span class="docsum-journal-citation short-journal-citation">JAMA. 2015.</span> <span class="citation-part">PMID: <span class="docsum-pmid">25562274</span></span> <span class="free-resources spaced-citation-item citation-part">Free PMC article.</span> <span class="no-abstract spaced-citation-item citation-part">No abstract available.</span> </div> </div> </li> <li class="full-docsum"> <div class="docsum-content" style="display:inline;"> <a class="docsum-title" href="/25562275/" ref="article_id=25562275&amp;linksrc=comments_link" data-ga-category="comment_correction" data-ga-action="abstract_page_link" data-ga-label="linked-commentary"> Management of patients with sickle cell disease--reply. </a> <div class="docsum-citation full-citation"> <span class="docsum-authors full-authors">Yawn BP, Buchanan G, Hassell K.</span> <span class="docsum-authors short-authors">Yawn BP, et al.</span> <span class="docsum-journal-citation full-journal-citation">JAMA. 2015 Jan 6;313(1):91-2. doi: 10.1001/jama.2014.15909.</span> <span class="docsum-journal-citation short-journal-citation">JAMA. 2015.</span> <span class="citation-part">PMID: <span class="docsum-pmid">25562275</span></span> <span class="no-abstract spaced-citation-item citation-part">No abstract available.</span> </div> </div> </li> </ul> </div> <div class="similar-articles" id="similar"> <h2 class="title"> Similar articles </h2> <ul class="articles-list" id="similar-articles-list"> <li class="full-docsum"> <div class="docsum-content" style="display:inline;"> <a class="docsum-title" href="/30067867/" ref="article_id=30067867&amp;linksrc=similar_articles_link&amp;ordinalpos=1" data-ga-category="similar_article" data-ga-action="30067867" data-ga-label=""> Red blood cell transfusion to treat or prevent complications in sickle cell disease: an overview of Cochrane reviews. </a> <div class="docsum-citation full-citation"> <span class="docsum-authors 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Transfusion*"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=Blood+Transfusion" data-ga-category="search_mesh" data-ga-action="mesh_term_link" data-ga-label="Blood Transfusion*"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Blood Transfusion&quot;[MAJR]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Blood Transfusion&quot;[MAJR]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Child" tabindex="0" aria-controls="keyword-actions-mesh-terms-6" aria-expanded="false" data-pinger-ignore> Child </button><div id="keyword-actions-mesh-terms-6" class="keyword-actions-dropdown dropdown 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href="https://www.ncbi.nlm.nih.gov/mesh?term=Consensus+Development+Conferences+as+Topic" data-ga-category="search_mesh" data-ga-action="mesh_term_link" data-ga-label="Consensus Development Conferences as Topic"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Consensus Development Conferences as Topic&quot;[MeSH]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Consensus Development Conferences as Topic&quot;[MeSH]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Evidence-Based Medicine" tabindex="0" aria-controls="keyword-actions-mesh-terms-9" aria-expanded="false" data-pinger-ignore> Evidence-Based Medicine </button><div id="keyword-actions-mesh-terms-9" class="keyword-actions-dropdown 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ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Evidence-Based Medicine&quot;[MeSH]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Evidence-Based Medicine&quot;[MeSH]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Humans" tabindex="0" aria-controls="keyword-actions-mesh-terms-10" aria-expanded="false" data-pinger-ignore> Humans </button><div id="keyword-actions-mesh-terms-10" class="keyword-actions-dropdown dropdown dropdown-container" hidden aria-label="Dropdown menu for keyword Humans"><div class="title">Actions</div><div class="content"><ul class="keyword-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22Humans%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22Humans%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_keyword_link&amp;mesh=&quot;Humans&quot;[MeSH]" data-ga-category="search" data-ga-action="mesh_term" data-ga-label="Humans"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=Humans" data-ga-category="search_mesh" data-ga-action="mesh_term_link" data-ga-label="Humans"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Humans&quot;[MeSH]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Humans&quot;[MeSH]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link major" aria-label="Toggle dropdown menu for keyword Hydroxyurea / therapeutic use*" tabindex="0" aria-controls="keyword-actions-mesh-terms-major-11" aria-expanded="false" data-pinger-ignore> Hydroxyurea / therapeutic use* </button><div id="keyword-actions-mesh-terms-major-11" class="keyword-actions-dropdown dropdown dropdown-container" hidden aria-label="Dropdown menu for keyword Hydroxyurea / therapeutic use*"><div class="title">Actions</div><div class="content"><ul class="keyword-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22Hydroxyurea%2Ftherapeutic+use%22%5BMAJR%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22Hydroxyurea%2Ftherapeutic+use%22%5BMAJR%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_keyword_link&amp;mesh=&quot;Hydroxyurea/therapeutic use&quot;[MAJR]" data-ga-category="search" data-ga-action="mesh_term" data-ga-label="Hydroxyurea / therapeutic use*"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=Hydroxyurea" data-ga-category="search_mesh" data-ga-action="mesh_term_link" data-ga-label="Hydroxyurea / therapeutic use*"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Hydroxyurea/therapeutic use&quot;[MAJR]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Hydroxyurea/therapeutic use&quot;[MAJR]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Infant" tabindex="0" aria-controls="keyword-actions-mesh-terms-12" aria-expanded="false" data-pinger-ignore> Infant </button><div id="keyword-actions-mesh-terms-12" class="keyword-actions-dropdown dropdown dropdown-container" hidden aria-label="Dropdown menu for keyword Infant"><div class="title">Actions</div><div class="content"><ul class="keyword-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22Infant%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22Infant%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_keyword_link&amp;mesh=&quot;Infant&quot;[MeSH]" data-ga-category="search" data-ga-action="mesh_term" data-ga-label="Infant"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=Infant" data-ga-category="search_mesh" data-ga-action="mesh_term_link" data-ga-label="Infant"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Infant&quot;[MeSH]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Infant&quot;[MeSH]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Penicillins / therapeutic use" tabindex="0" aria-controls="keyword-actions-mesh-terms-13" aria-expanded="false" data-pinger-ignore> Penicillins / therapeutic use </button><div id="keyword-actions-mesh-terms-13" class="keyword-actions-dropdown dropdown dropdown-container" hidden aria-label="Dropdown menu for keyword Penicillins / therapeutic use"><div class="title">Actions</div><div class="content"><ul class="keyword-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22Penicillins%2Ftherapeutic+use%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22Penicillins%2Ftherapeutic+use%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_keyword_link&amp;mesh=&quot;Penicillins/therapeutic use&quot;[MeSH]" data-ga-category="search" data-ga-action="mesh_term" data-ga-label="Penicillins / therapeutic use"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=Penicillins" data-ga-category="search_mesh" data-ga-action="mesh_term_link" data-ga-label="Penicillins / therapeutic use"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Penicillins/therapeutic use&quot;[MeSH]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Penicillins/therapeutic use&quot;[MeSH]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Physical Therapy Modalities" tabindex="0" aria-controls="keyword-actions-mesh-terms-14" aria-expanded="false" data-pinger-ignore> Physical Therapy Modalities </button><div id="keyword-actions-mesh-terms-14" class="keyword-actions-dropdown dropdown dropdown-container" hidden aria-label="Dropdown menu for keyword Physical Therapy Modalities"><div class="title">Actions</div><div class="content"><ul class="keyword-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22Physical+Therapy+Modalities%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22Physical+Therapy+Modalities%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_keyword_link&amp;mesh=&quot;Physical Therapy Modalities&quot;[MeSH]" data-ga-category="search" data-ga-action="mesh_term" data-ga-label="Physical Therapy Modalities"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=Physical+Therapy+Modalities" data-ga-category="search_mesh" 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class="content"><ul class="keyword-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22Practice+Guidelines+as+Topic%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22Practice+Guidelines+as+Topic%22%5BMeSH%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_keyword_link&amp;mesh=&quot;Practice Guidelines as Topic&quot;[MeSH]" data-ga-category="search" data-ga-action="mesh_term" data-ga-label="Practice Guidelines as Topic"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=Practice+Guidelines+as+Topic" data-ga-category="search_mesh" data-ga-action="mesh_term_link" data-ga-label="Practice Guidelines as Topic"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Practice Guidelines as Topic&quot;[MeSH]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Practice Guidelines as Topic&quot;[MeSH]" href="#"> Add to Search </a></li></ul></div></div></div></li></ul> </div> <div id="substances" class="substances keywords-section"> <h2 class="title"> Substances </h2> <ul class="keywords-list"><li><div class="keyword-actions dropdown-block"><button class="keyword-actions-trigger trigger keyword-link" aria-label="Toggle dropdown menu for keyword Analgesics, Opioid" tabindex="0" aria-controls="keyword-actions-substances-0" aria-expanded="false" data-pinger-ignore> Analgesics, Opioid </button><div id="keyword-actions-substances-0" class="keyword-actions-dropdown dropdown dropdown-container" hidden aria-label="Dropdown menu for keyword Analgesics, Opioid"><div class="title">Actions</div><div class="content"><ul class="keyword-actions-links"><li><a class="search-in-pubmed-link dropdown-block-link" href="/?term=%22Analgesics%2C+Opioid%22%5Bnm%5D&amp;sort=date&amp;sort_order=desc" data-href="/?term=%22Analgesics%2C+Opioid%22%5Bnm%5D&amp;sort=date&amp;sort_order=desc" ref="linksrc=search_in_pubmed_keyword_link&amp;keyword=&quot;Analgesics, Opioid&quot;[nm]" data-ga-category="search" data-ga-action="substance" data-ga-label="Analgesics, Opioid"> Search in PubMed </a></li><li><a class="search-in-mesh-link dropdown-block-link" ref="linksrc=search_in_mesh_link" href="https://www.ncbi.nlm.nih.gov/mesh?term=%22Analgesics%2C+Opioid%22" data-ga-category="search_mesh" data-ga-action="substance_link" data-ga-label="Analgesics, Opioid"> Search in MeSH </a></li><li><a class="add-to-search-link dropdown-block-link" ref="linksrc=add_to_search_link" role="button" data-search-term="&quot;Analgesics, Opioid&quot;[nm]" data-ga-category="search" data-ga-action="add_to_search" data-ga-label="&quot;Analgesics, Opioid&quot;[nm]" href="#"> Add to Search </a></li></ul></div></div></div></li><li><div 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