{"id":26,"date":"2023-01-09T05:55:35","date_gmt":"2023-01-09T05:55:35","guid":{"rendered":"https:\/\/pkudsehcp-dev-001.azurewebsites.net\/en-us\/phenylketonuria\/?page_id=26"},"modified":"2024-07-15T07:36:26","modified_gmt":"2024-07-15T07:36:26","slug":"lifelong-burden","status":"publish","type":"page","link":"https:\/\/hcp.biomarin.com\/en-us\/pku\/lifelong-burden\/","title":{"rendered":"Lifelong burden"},"content":{"rendered":"<div id=\"acf-block-63f64f3300d1c\" class=\"hero hero-no-overlay\">\n            <div class=\"hero-background-image hero-background-image-desktop\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2023\/06\/new-pku-internal-hero-2.jpg?v=0.93);\"><\/div>\n\t    <div class=\"hero-background-image hero-background-image-mobile\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2023\/03\/hero-815x800.jpg);\"><\/div>\n    \t<div class=\"overlay\"><\/div>\n\t<div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t\t<div class=\"hero-content\">\n\t\t\t    \t\t\t\t\t\t\t\t\t\t\t\t    <h1>Phe control diminishes<br \/>\nwith age<sup>1,2<\/sup>\n<\/h1>\n\t\t\t\t\t\t\t\t\t\t\t\t\n                <p class=\"inline-buttons\">\n                                                        <\/p>\n\t\t\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n<\/div>\n\n\n<div id=\"acf-block-63f64f33028c7\" class=\"block split-content equal-bias bg-band bg-band-alternative block-zero-top block-zero-bottom\" data-muted-autoplay=\"false\">\n\t<div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t                                                                <div class=\"content-block first-block\">\n                                                                                                <h2>Compliance with blood Phe level recommendations declines with age<sup>1<\/sup><\/h2>\n<p>Guidelines recommend lifetime maintenance of Phe concentrations \u2264360 \u03bcmol\/L. However, Phe control typically drops off during adolescence, increasing the severity of neuropsychological symptoms and negatively impacting life trajectory <sup>2,3<\/sup><\/p>\n                                                                                                                                                                                                                                                                                                                    \n                                    \n                                                                                                                \n                                    \n                                    <figure>\n                                        <div class=\"image image-rounded\">\n                                                                                            <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2023\/02\/67percent_img2.svg\" alt=\"67% of adults with phenylketonuria (PKU) are above American College of Medical Genetics and Genomics target Phe ranges image\" \/>                                                                                    <\/div>\n                                                                            <\/figure>\n                                                                                    <\/div>\n                    \n                                            <div class=\"content-block second-block\">\n                                                                                                                                                                                                                                                                                    \n                                                                                                                \n                                                                                                                \n                                    \n                                    <figure>\n                                        <div class=\"image image-responsive image-rounded\">\n                                                                                            <div class=\"image-desktop\">\n                                                    <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2023\/03\/3.0_Bar-Chart_2.svg\" alt=\"Compliance with blood Phe levels bar graph image\" \/>                                                <\/div>\n                                                <div class=\"image-mobile\">\n                                                    <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2024\/06\/blood-Phe-level_stacked-key-1.png?v=0.93\" alt=\"\" \/>                                                <\/div>\n                                                                                    <\/div>\n                                                                                    <figcaption><p><small>Adapted from Jurecki et al. <em>Mol Genet Metab<\/em>. 2017.<\/small><\/p>\n<\/figcaption>\n                                                                            <\/figure>\n                                                                                    <\/div>\n                                                \t\t<\/div>\n\t<\/div>\n<\/div>\n\n<div id=\"acf-block-65ddfd5e412bb\" class=\"block split-content equal-bias bg-band bg-band-secondary content-align-top block-zero-top block-zero-bottom\" data-muted-autoplay=\"false\">\n\t<div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t                                                                <div class=\"content-block first-block\">\n                                                                                                <h2>Managing PKU can be challenging for your patients and their caregivers<\/h2>\n<p>Compliance with dietary management, regular monitoring of blood Phe levels, clinic appointments, and the mental and emotional stress of living with a chronic disorder can make maintaining blood Phe levels within the recommended range more difficult for patients over time. Helping patients and caregivers talk openly about their challenges living with PKU may keep them engaged.<sup>4-7<\/sup><\/p>\n                                                                                    <\/div>\n                    \n                                            <div class=\"content-block second-block\">\n                                                                                                <h5><strong>These example questions to ask your patients and their caregivers may help them talk openly about their challenges living with PKU.<\/strong><\/h5>\n                                                                                                                                                                                                                                                                                                                    \n                                    \n                                                                                                                \n                                    \n                                    <figure>\n                                        <div class=\"image image-rounded\">\n                                                                                            <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2024\/02\/chat_boxes_new-t2b-ow_small.svg\" alt=\"\" \/>                                                                                    <\/div>\n                                                                            <\/figure>\n                                                                                    <\/div>\n                                                \t\t<\/div>\n\t<\/div>\n<\/div>\n\n<div id=\"acf-block-63f64f3302917\" class=\"block split-content equal-bias bg-band bg-band-white block-zero-top block-zero-bottom\" data-muted-autoplay=\"false\">\n\t<div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t                                                                <div class=\"content-block first-block\">\n                                                                                                <h2>Young adults and adults with PKU can become trapped in a cycle of decline<sup>7<\/sup><\/h2>\n<ul>\n<li>Uncontrolled PKU inhibits daily functioning, contributing to poor management<sup>7,8<\/sup><\/li>\n<li>Patients with prolonged, elevated Phe levels often lack self-awareness of the severity of their symptoms<sup>7<\/sup><\/li>\n<li>At the same time, high blood Phe levels can lead to executive function impairments that make PKU management more challenging<sup>7<\/sup><\/li>\n<\/ul>\n                                                                                    <\/div>\n                    \n                                            <div class=\"content-block second-block\">\n                                                                                                                                                                                                                                                                                    \n                                    \n                                                                                                                \n                                    \n                                    <figure>\n                                        <div class=\"image image-rounded\">\n                                                                                            <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2024\/02\/3.0_Cycle-of-Decline-1900x1741.png\" alt=\"\" \/>                                                                                    <\/div>\n                                                                            <\/figure>\n                                                                                    <\/div>\n                                                \t\t<\/div>\n\t<\/div>\n<\/div>\n\n<div id=\"acf-block-65de008cd7c28\" class=\"block split-content equal-bias\" data-muted-autoplay=\"false\">\n\t<div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t                                                                <div class=\"content-block first-block\">\n                                                                                                                                                                                                                                                <div class=\"video-poster\">\n\t\t\t                            <a class=\"modal-video permitted\" href=\"https:\/\/player.vimeo.com\/video\/834483784?autoplay=1\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2024\/02\/vlcsnap-2024-02-29-13h22m03s761-1500x844.jpg);\">Play<\/a>\n\t\t                            <\/div>\n                                    \n                                                                                                                    \n                                                                                                                \n\t\t                                                                            <\/div>\n                    \n                                            <div class=\"content-block second-block\">\n                                                                                                <p><strong>Kelly: Loss of Phe control<\/strong><\/p>\n<p>Learn how a strong support system helped Kelly return to clinic<\/p>\n                                                                                                                                                                                                                                                                                                                                                                                                                                                                        <p><a class=\"button button-download\" href=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2024\/02\/Kelly-PKU-Video-Transcript.pdf?v=0.93\" target=\"_blank\">View transcript<\/a><\/p>\n                                                                                                                        <\/div>\n                                                \t\t<\/div>\n\t<\/div>\n<\/div>\n\n<div id=\"acf-block-63f64f3302937\" class=\"block wrapped-content block-tight-top block-tight-bottom\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-63f64f3302948\" class=\"block-wysiwyg\">\n            <h2>Learn more about PKU<\/h2>\n    <\/div>\n        <\/div>\n    <\/div>\n<\/div>\n\n<div id=\"acf-block-63f64f330295d\" class=\"block cards cards-2-col block-tight-top block-tight-bottom\">\n\t<div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t\t<div class=\"cards-title\">\n\t\t\t    \t\t\t\t\t\t\t<\/div>\n\t\t\t<div class=\"cards-wrapper\">\n\t\t\t    \n<div id=\"acf-block-63f64f3302978\" class=\"card card-product card-fixed-button card-centered\" data-muted-autoplay=\"false\">\n    \t    \n\n                    <img decoding=\"async\" class=\"card-image\" src=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2024\/02\/article_card2col_icon.svg\" alt=\"\" \/>        \n\t    <div class=\"card-content\">\n                            <h4>Summary of Expert Panel on PKU\n<\/h4>\n        \n                                                                                                                                                                                                                                                                                    \n                                                                                        <\/div>\n                                <div class=\"card-actions\">\n                            \n                            <p><a class=\"button button-download\" href=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2023\/05\/PKU-HCP-Publication-Summary-2.pdf?v=0.72\" target=\"_blank\">Download article<\/a><\/p>\n                                                                                    <\/div>\n<\/div>\n\n<div id=\"acf-block-63f64f3302996\" class=\"card card-product card-fixed-button card-centered\" data-muted-autoplay=\"false\">\n    \t    \n\n                    <img decoding=\"async\" class=\"card-image\" src=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/wp-content\/uploads\/sites\/2\/2024\/02\/question_card2col_icon.svg\" alt=\"\" \/>        \n\t    <div class=\"card-content\">\n                            <h4>Know your PKU management options\n<\/h4>\n        \n                                                                                                                                                                                                                                                                                    \n                                                                                        <\/div>\n                                <div class=\"card-actions\">\n                            \n                            <p><a class=\"button\" href=\"https:\/\/hcp.biomarin.com\/en-us\/pku\/pku-management-options\/\" target=\"_self\">Learn more<\/a><\/p>\n                                                                                    <\/div>\n<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n<\/div>\n\n<div id=\"acf-block-63f64f3302937\" class=\"block wrapped-content block-tight-top block-tight-bottom\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-63f64f3302948\" class=\"block-wysiwyg\">\n            <p><small><strong>References: 1.<\/strong> Jurecki ER, Cederbaum S, Kopesky J, et al. Adherence to clinic recommendations among patients with phenylketonuria in the United States. <em>Mol Genet Metab<\/em>. 2017;120(3):190-197. <strong>2.<\/strong> Brown CS, Lichter-Konecki U. Phenylketonuria (PKU): a problem solved? <em>Mol Genet Metab Rep<\/em>. 2016;6:8-12. <strong>3.<\/strong> Vockley J, Andersson HC, Antshel KM, et al. American College of Medical Genetics and Genomics Therapeutic Committee. Phenylalanine hydroxylase deficiency: diagnosis and management guidelines. <em>Genet Med<\/em>. 2014;16(2):188-200. <strong>4. <\/strong>Enns GM, Koch R, Brumm V, Blakely E, Suter R, Jurecki E. Suboptimal outcomes in patients with PKU treated early with diet alone: revisiting the evidence. <em>Mol Genet Metab<\/em>. 2010;101(2-3):99-109. <strong>5.<\/strong> van Wegberg AMJ, MacDonald A, Ahring K, et al. The complete European guidelines of phenylketonuria: diagnosis and treatment. <em>Orphanet J Rare Dis<\/em>. 2017;12(1):162. doi: 10.1186\/s13023-017-0685-22. <strong>6.<\/strong> Cazzorla C, Bensi G, Biasucci G, et al. Living with phenylketonuria in adulthood: the PKU ATTITUDE study. <em>Mol Gen Metab Rep<\/em>. 2018;16:39-45. <strong>7.<\/strong> Thomas J, Nguyen-Driver M, Bausell H, Breck J, Zambrano J, Birardi V. Strategies for successful long-term engagement of adults with phenylalanine hydroxylase deficiency returning to the clinic. <em>J Inborn Errors Metab Screen<\/em>. 2017;5:1-9. <strong>8.<\/strong> Bilder DA, Noel JK, Baker ER, et al. Systematic review and meta-analysis of neuropsychiatric symptoms and executive functioning in adults with phenylketonuria. <em>Dev Neuropsychol<\/em>. 2016;41(4):245-260.<\/small><\/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-26","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>Phe Control l Phe Level Decline in Young Adults<\/title>\n<meta name=\"description\" content=\"Uncontrolled PKU inhibits daily functioning, contributing to poor disease management and Phe control typically drops off during adolescence.\" \/>\n<meta name=\"robots\" content=\"index, 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