{"id":99,"date":"2022-09-26T19:47:04","date_gmt":"2022-09-26T19:47:04","guid":{"rendered":"https:\/\/naglazymehcp-dev-001.azurewebsites.net\/en-us\/naglazyme\/?page_id=99"},"modified":"2023-06-05T15:27:03","modified_gmt":"2023-06-05T15:27:03","slug":"rate-of-progression","status":"publish","type":"page","link":"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/rate-of-progression\/","title":{"rendered":"Rate of progression"},"content":{"rendered":"<div id=\"acf-block-63f67b14be3ce\" class=\"block wrapped-content\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-63f67b14be5eb\" class=\"block-wysiwyg\">\n            <h1>Rate of Progression<\/h1>\n<h2>MPS VI disease progression varies<\/h2>\n<p>Age at symptom recognition, severity of symptoms, and rate of disease progression vary dramatically in MPS VI. Yet, most patients eventually experience significant declines in physical and functional well-being, ultimately become wheelchair-bound or bedridden, and die prematurely.<sup>1<\/sup> Physical limitations can also impact learning and development of motor skills.<sup>2<\/sup><\/p>\n<h2>Rapidly progressing MPS VI<sup>1-3<\/sup><\/h2>\n<p>Patients with rapidly progressing disease experience symptom recognition early on in life and severe debilitation. Death from infections, complications related to surgeries, and cardiopulmonary disease usually occur when they are teenagers or young adults. Rapidly progressing disease is very apparent at an early age.<sup>2<\/sup> Look for<sup>3<\/sup>:<\/p>\n<ul>\n<li>Macrocephaly<\/li>\n<li>Skeletal abnormalities<\/li>\n<li>Fixed flexion of fingers<\/li>\n<li>Hepatomegaly<\/li>\n<li>Umbilical hernia<\/li>\n<\/ul>\n    <\/div>\n\n<figure id=\"acf-block-63f67b14be61e\">\n    <div class=\"image image-align-left\">\n                    <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/wp-content\/uploads\/sites\/2\/2022\/10\/Rate-of-Progression-.jpg?v=0.33\" alt=\"Photographs showing the rate of progression\" \/>            <\/div>\n    <\/figure>\n\n<div id=\"acf-block-63f67b14be639\" class=\"block-wysiwyg\">\n            <h2>Slowly progressing MPS VI<\/h2>\n<p>Unlike rapidly progressing patients, slowly progressing patients often do not exhibit obvious symptoms. Differentiation into rapidly and slowly progressing disease is only apparent in extreme cases. The entire spectrum of the disease inhibits physical and functional well-being and results in a markedly shortened lifespan.<sup>2,4<\/sup><\/p>\n<ul>\n<li>Major causes of mortality include cardiac and pulmonary complications, and complications from surgery and general anesthesia<sup>2<\/sup><\/li>\n<\/ul>\n<p><a href=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/diagnosing-mps-vi\/\">Learn more about diagnosing MPS VI \u00bb<\/a><\/p>\n    <\/div>\n        <\/div>\n    <\/div>\n<\/div>\n\n<div id=\"acf-block-63f67b14be65c\" class=\"block references\">\n    <div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t    \t\t\t    <h4>References:\n<\/h4>\n\t\t\t\t\t\t                <ol>\n                                                                                                                        <li><span>Swiedler SJ, Beck M, Bajbouj M, <em>et al<\/em>. Threshold effect of urinary glycosaminoglycans and the walk test as indicators of disease progression in a survey of subjects with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). <em>Am J Med Genet A<\/em>. 2005;134A(2):144-150.\n<\/span><\/li>\n                                                                                                                                                <li><span>Akyol MU, Alden TD, Amartino H, <em>et al<\/em>. Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance. <em>Orphanet J Rare Dis<\/em>. 2019;14:118. \n<\/span><\/li>\n                                                                                                                                                <li><span>Wilcox WR. Lysosomal storage disorders: the need for better pediatric recognition and comprehensive care. J Pediatr. 2004;144(suppl 5):S3-S14.\n<\/span><\/li>\n                                                                                                                                                <li><span>Quartel A, Hendriksz CJ, Parini R, Graham S, Lin P, Harmatz P. Growth charts for individuals with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). <em>JIMD Rep<\/em>. 2015;18:1-11. doi:10.1007\/8904_2014_333.\n<\/span><\/li>\n                                                            <\/ol>\n\t\t\t\t\t<\/div>\n\t<\/div>\n<\/div>","protected":false},"excerpt":{"rendered":"","protected":false},"author":4,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"inline_featured_image":false,"footnotes":""},"class_list":["post-99","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.4 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Rate of progression - BioMarin Naglazyme HCP EN-US<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/rate-of-progression\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Rate of progression - 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