{"id":31,"date":"2024-11-18T12:14:43","date_gmt":"2024-11-18T12:14:43","guid":{"rendered":"https:\/\/www.naglazyme.com\/en-us\/?page_id=31"},"modified":"2026-06-03T09:53:25","modified_gmt":"2026-06-03T09:53:25","slug":"rapidly-and-slowly-progressing-mps-vi","status":"publish","type":"page","link":"https:\/\/www.naglazyme.com\/en-us\/rapidly-and-slowly-progressing-mps-vi\/","title":{"rendered":"Rapidly and slowly progressing MPS VI"},"content":{"rendered":"<div id=\"acf-block-673c63dd0f192\" class=\"block wrapped-content\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-673c63dd0f312\" class=\"block-wysiwyg\">\n            <h1>Rapidly and slowly progressing MPS VI<\/h1>\n<h2>Rapidly progressing MPS VI<\/h2>\n<p>Some people with MPS VI show signs early and clearly. Babies with this rapidly progressing MPS VI may not grow well. Other symptoms or medical issues may quickly emerge, including<sup>1-5<\/sup>:<\/p>\n<ul>\n<li>Coarse facial features<\/li>\n<li>Enlarged tongue<\/li>\n<li>Vision problems<\/li>\n<li>Breathing problems<\/li>\n<li>Disease of the heart valves<\/li>\n<li>Stiff joints<\/li>\n<\/ul>\n<p>With these obvious symptoms, patients with rapidly progressing MPS VI are often diagnosed early.<\/p>\n    <\/div>\n\n<figure id=\"acf-block-673c63dd0f52f\">\n    <div class=\"image image-rounded image-align-left\">\n                    <img decoding=\"async\" class=\"\" src=\"https:\/\/www.naglazyme.com\/en-us\/wp-content\/uploads\/sites\/2\/2025\/12\/NAG_ProgressingPatient.png?v=0.37\" alt=\"\" \/>            <\/div>\n    <\/figure>\n\n<div id=\"acf-block-673c63dd0f570\" class=\"block-wysiwyg\">\n            <p>Symptoms may be recognized early in life for rapidly progressing MPS VI or later in adolescence for slowly progressing MPS VI. All patients with MPS VI eventually experience significant disability and may experience a shortened life span.<sup>2<\/sup><\/p>\n    <\/div>\n        <\/div>\n    <\/div>\n<\/div>\n\n<div id=\"acf-block-673c6477f1297\" class=\"block wrapped-content\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-673c6477f150b\" class=\"block-wysiwyg\">\n            <h2>Slowly progressing MPS VI<\/h2>\n<p>Other people with MPS VI may have symptoms that are slower to develop. At first, some of their symptoms, like constant ear infections, may just seem like common childhood problems. On the other hand, a problem that is unusual in children, like stiff joints, may be seen as something that affects only the bones. For these \u201cslowly progressing\u201d patients, it can take the doctor a while to see that different symptoms, affecting many parts of the body, are all really due to MPS VI.<sup>5,6<\/sup><\/p>\n<h2>Whether rapidly or slowly progressing, MPS VI requires treatment<\/h2>\n<p>There is no hard line between rapidly and slowly progressing MPS VI. There are degrees in between. Symptoms can be very different from patient to patient. Even though the symptoms are not initially clear in people with slowly progressing MPS VI, GAGs are still building up in their cells. It is still causing problems in their organs. Over time, people with slowly progressing MPS VI will notice more and more symptoms and the condition will progress. The symptoms of MPS VI need both symptom-based care and treatment with enzyme replacement therapy (ERT). All MPS VI patients are candidates for ERT treatment with NAGLAZYME<sup>\u00ae<\/sup> (galsulfase). NAGLAZYME provides the enzyme the body needs to break down and get rid of GAGs.<sup>2,6,7<\/sup><\/p>\n    <\/div>\n        <\/div>\n    <\/div>\n<\/div>\n\n<div id=\"acf-block-691f590b85a3e\" 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, et al. 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):144-151.\n<\/span><\/li>\n                                                                                                                                                <li><span>Akyol MU, Alden TD, Amartino H, et al. 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. <em>J Pediatr.<\/em> 2004;144(suppl 5):S3-S14.\n<\/span><\/li>\n                                                                                                                                                <li><span>Clarke L, Ellaway C, Foster H. Understanding the early presentation of mucopolysaccharidosis disorders: results of a systematic literature review and physician survey. <em>J Inborn Errors Metab Screen<\/em>. 2018;6:1-12.\n<\/span><\/li>\n                                                                                                                                                <li><span>National MPS Society. Maroteaux-Lamy syndrome. A guide to understanding MPS VI [booklet]. Accessed March 24, 2026. <a href=\"https:\/\/mpssociety.org\/wp-content\/uploads\/2025\/03\/MPS_VI_Final_Booklet.pdf\" target=\"_blank\" rel=\"noopener\">https:\/\/mpssociety.org\/wp-content\/uploads\/2025\/03\/MPS_VI_Final_Booklet.pdf<\/a>\n<\/span><\/li>\n                                                                                                                                                <li><span>D\u2019Avanzo F, Zanetti A, De Filippis C, Tomanin R. Mucopolysaccharidosis type VI: an updated overview of the disease. <em>Int J Mol Sci<\/em>. 2021;22(24):13456.\n<\/span><\/li>\n                                                                                                                                                <li><span>NAGLAZYME [package insert]. Novato, CA: BioMarin Pharmaceutical Inc; 2024.\n<\/span><\/li>\n                                                            <\/ol>\n\t\t\t\t\t<\/div>\n\t<\/div>\n<\/div>\n\n<div id=\"next\" class=\"block wrapped-content\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-673c5f22ea475\" class=\"block-wysiwyg\">\n            <p style=\"text-align: right;font-size: 1.1rem\"><a style=\"text-decoration: none\" href=\"https:\/\/www.naglazyme.com\/en-us\/how-is-mps-vi-diagnosed\/\"><strong>NEXT PAGE &gt;<\/strong><\/a><\/p>\n    <\/div>\n        <\/div>\n    <\/div>\n<\/div>","protected":false},"excerpt":{"rendered":"","protected":false},"author":3,"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-31","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.7 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Rapidly and Slowly Progressing MPS VI | Patient<\/title>\n<meta name=\"description\" content=\"MPS VI affects people in very different ways. 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