{"id":493041,"date":"2022-09-14T16:51:39","date_gmt":"2022-09-14T13:51:39","guid":{"rendered":"https:\/\/en.buradabiliyorum.com\/going-the-distance-jonathans-story\/"},"modified":"2022-09-14T16:51:39","modified_gmt":"2022-09-14T13:51:39","slug":"going-the-distance-jonathans-story","status":"publish","type":"post","link":"https:\/\/buradabiliyorum.com\/en\/going-the-distance-jonathans-story\/","title":{"rendered":"#Going the distance: Jonathan\u2019s story"},"content":{"rendered":"<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_84 counter-hierarchy ez-toc-counter ez-toc-custom ez-toc-container-direction\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Table of Contents<\/p>\n<label for=\"ez-toc-cssicon-toggle-item-6a2e92d8ce1de\" class=\"ez-toc-cssicon-toggle-label\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #dd3333;color:#dd3333\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewBox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #dd3333;color:#dd3333\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewBox=\"0 0 24 24\" version=\"1.2\" baseProfile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/label><input type=\"checkbox\"  id=\"ez-toc-cssicon-toggle-item-6a2e92d8ce1de\" checked aria-label=\"Toggle\" \/><nav><ul class='ez-toc-list ez-toc-list-level-1 ' ><li class='ez-toc-page-1 ez-toc-heading-level-1'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/buradabiliyorum.com\/en\/going-the-distance-jonathans-story\/#%E2%80%9CGoing_the_distance_Jonathans_story%E2%80%9D\" >&#8220;Going the distance: Jonathan\u2019s story&#8221;<\/a><ul class='ez-toc-list-level-2' ><li class='ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/buradabiliyorum.com\/en\/going-the-distance-jonathans-story\/#Challenged_by_a_mysterious_opponent\" >Challenged by a mysterious opponent<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/buradabiliyorum.com\/en\/going-the-distance-jonathans-story\/#On_the_ropes\" >On the ropes<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/buradabiliyorum.com\/en\/going-the-distance-jonathans-story\/#A_proper_diagnosis_at_last\" >A proper diagnosis, at last<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/buradabiliyorum.com\/en\/going-the-distance-jonathans-story\/#A_healthy_dose_of_vigilance\" >A healthy dose of vigilance<\/a><\/li><\/ul><\/li><\/ul><\/nav><\/div>\n<h1><span class=\"ez-toc-section\" id=\"%E2%80%9CGoing_the_distance_Jonathans_story%E2%80%9D\"><\/span>&#8220;Going the distance: Jonathan\u2019s story&#8221;<span class=\"ez-toc-section-end\"><\/span><\/h1>\n<div>\n<p style=\"text-align: center; margin-bottom: 0px;\"><strong>Created by<\/strong><\/p>\n<p style=\"text-align: center; margin-top: 0px;\"><a rel=\"nofollow noopener\" target=\"_blank\" href=\"https:\/\/sr.studiostack.com\/c\/link?l=983980&amp;s=983978\"><img decoding=\"async\" data-sizes=\"auto\" style=\"display: inline-block; width: 230px;\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/MitoCanada-Logo-09_22.png\" alt=\"\u201cMito Canada logo\"\/><\/a><\/p>\n<p>Jonathan Fleisch is 70 years old, and he\u2019s not ready to slow down. He\u2019s still bouncing back from a recent surgery and, when he talks about his recovery, it\u2019s clear that he\u2019s mostly just frustrated that he can\u2019t spend as much time on his bicycle as he would like to.<\/p>\n<p><span style=\"font-weight: 400;\">But though he\u2019s limiting his rides to 35 kilometres this summer\u2014rather than the 80-plus-kilometre circuits he\u2019s used to\u2014Jonathan is unfailingly practical about not overexerting himself and creative about supplementing his fitness regimen in other ways. He is, after all, no stranger to working within the constraints of his body. <\/span><\/p>\n<p><span style=\"font-weight: 400;\">He has lived all his life with carnitine palmitoyltransferase II (CPT II) deficiency, a rare metabolic condition that is one of six types of long-chain fatty acid oxidation disorders, or LC-FAOD. Untreated, CPT II can cause severe and painful muscle damage and, in severe cases, death.<\/span><\/p>\n<div class=\"longform-fwimg-container\"><img decoding=\"async\" data-sizes=\"auto\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Jonathan-SUB-2.jpg\" alt=\"\"\/><\/div>\n<p><span style=\"font-weight: 400;\">\u201cCPT II is the mitochondrial enzyme that allows for the long-chain fatty acids to enter the inside of the mitochondria,\u201d explains Dr. Mark Tarnopolsky, Director of the Neuromuscular and Neurometabolic Clinic at the McMaster University Medical Centre. \u201cWithout this enzyme, there\u2019s less fat that can be burned for energy in times of need, such as fasting, illness and exercise. The muscles and heart can run out of energy under these stresses and there can be damage to the tissue, resulting in a leak of cellular components into the blood. This is called rhabdomyolysis, and it can cause muscle pain and, sometimes, lasting damage to the kidneys.\u201d<\/span><\/p>\n<p><span style=\"font-weight: 400;\">If Jonathan<\/span><span style=\"font-weight: 400;\">\u00a0isn\u2019t careful, his <\/span><span style=\"font-weight: 400;\">muscle fibres will break down, releasing toxic metabolites that will damage vital organs<\/span><span style=\"font-weight: 400;\">. But, with his CPT II deficiency, as with his surgery recovery, Jonathan<\/span><span style=\"font-weight: 400;\">\u2019s<\/span><span style=\"font-weight: 400;\"> brand of vigilance involves developing a thorough understanding of where exactly his limits are so that he can operate safely within them.<\/span><\/p>\n<h2><span class=\"ez-toc-section\" id=\"Challenged_by_a_mysterious_opponent\"><\/span><b>Challenged by a mysterious opponent<\/b><span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><span style=\"font-weight: 400;\">Though he has lived in Canada for nearly half his life, the \u201cJo-burg\u201d accent of Jonathan\u2019s<\/span><span style=\"font-weight: 400;\">\u00a0youth still comes through clearly. Growing up <\/span><span style=\"font-weight: 400;\">in South Africa in the 1950s, CPT II deficiency was not part of Jonathan\u2019s lexicon. He had certainly never once heard the word rhabdomyolysis, so the symptoms and complications of his illness went unexplained for years.<\/span><\/p>\n<div class=\"longform-fwimg-container\"><img decoding=\"async\" data-sizes=\"auto\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Jonathan-SUB-3.jpg\" alt=\"\"\/><\/div>\n<p><span style=\"font-weight: 400;\">\u201cI would get stiff and have pain in my muscles that progressively got worse and worse until I did something to mitigate it,\u201d says Jonathan. \u201cI have a sister who has the same issue, but we didn\u2019t have any idea as to the cause. At one stage we were taken to a doctor and he intimated that it most probably was psychosomatic. But that didn\u2019t change anything for us. We carried on as we had been.\u201d<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Having always been an active child, Jonathan found his enduring love of sport when he was enrolled in a boxing class with a friend at just eight years old, even if his early days in the ring were inauspicious. \u201cI was a shorty, and my friend was a very long guy with long arms, so I would always get punched in the nose,\u201d Jonathan recalls, laughing. \u201cI gave up on that very quickly and went into judo, where my reach didn\u2019t matter as much. I loved it. Pretty soon judo was all I did. It\u2019s not an endurance sport, so the stiffness that I would get when I exercised for a long time didn\u2019t hamper me much.\u201d\u00a0<\/span><\/p>\n<div class=\"longform-fwimg-container\"><img decoding=\"async\" data-sizes=\"auto\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Jonathan-SUB-4.jpg\" alt=\"\"\/><\/div>\n<h2><span class=\"ez-toc-section\" id=\"On_the_ropes\"><\/span><b>On the ropes<\/b><span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><span style=\"font-weight: 400;\">Jonathan went on to win a judo championship at the national level\u2014he was unstoppable.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Though he still suffered stiffness and pain following extended exercise, he had found that it was easily alleviated by his self-prescribed remedy of \u201ctwo buns and a Coke.\u201d Whatever was causing his discomfort, Jonathan wasn\u2019t inclined to worry about it.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Then, he became extremely ill. What seemed to be a routine case of the flu aggravated his underlying condition. Illness and stress are common episode triggers for people with CPT II deficiency. It quickly became clear that this was no longer something Jonathan could ignore.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">\u201cI was 20 years old and all of a sudden I was in excruciating pain,\u201d Jonathan recalls. \u201cThey put me straight into the intensive care ward. I was delirious and I was experiencing kidney failure. I was in the hospital for three months, and they had me on dialysis eight times over that period. I was put on a very specific diet. No condiments, no salt, only very bland foods. The only time I was allowed to eat whatever I wanted was when I was actually connected to the dialysis machine. I remember how unbelievably tasty food became during those hours.\u201d<\/span><\/p>\n<div class=\"longform-fwimg-container\"><img decoding=\"async\" data-sizes=\"auto\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Jonathan-SUB-5.jpg\" alt=\"\"\/><\/div>\n<p><span style=\"font-weight: 400;\">Jonathan\u00a0was released from the hospital 30 pounds lighter than he had entered, and still without an accurate diagnosis. He hadn\u2019t had the weight to spare on his slim athletic build but, within a year, he had gained it all back and was on the judo mat once again. Nothing slowed him down.\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400;\">\u201cI only know one thing,\u201d Jonathan says. \u201cAs you push forward, you don\u2019t go halfway. I\u2019m a no-pain-no-gain sort of guy. I\u2019ve always just pushed forward.\u201d<\/span><\/p>\n<p><span style=\"font-weight: 400;\">And so, without looking back at his hospitalization,\u00a0Jonathan <span data-redactor-style-cache=\"background-color: rgb(255, 203, 0);\">married his wife, Gwen<\/span>, welcomed two children and immigrated with his young family to Canada. There, he met Dr. Tarnopolsky<\/span> <span style=\"font-weight: 400;\">and finally received an accurate diagnosis of CPT II deficiency<\/span><span style=\"font-weight: 400;\">.<\/span><\/p>\n<h2><span class=\"ez-toc-section\" id=\"A_proper_diagnosis_at_last\"><\/span><b>A proper diagnosis, at last<\/b><span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><span style=\"font-weight: 400;\">\u201cJonathan was very typical of CPT II deficiency patients in the symptoms he presented, and his initial misdiagnoses are also a typical experience,\u201d says Dr. Tarnopolsky. \u201cThere are many things that can cause rhabdomyolysis and thus it can take time to get a diagnosis. Since finally receiving the correct diagnosis, Jonathan has done quite well. Unfortunately, we do also see much more severe and life-threatening cases in some children and some adults.\u201d<\/span><\/p>\n<div id=\"attachment_1240269\" style=\"width: 1885px\" class=\"wp-caption aligncenter\"><img loading=\"lazy\" decoding=\"async\" data-sizes=\"auto\" class=\"wp-image-1240269 size-full lazyload\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Dr-Tarnopolsky.jpg\" alt=\"Dr. Mark Tarnopolsky\" width=\"1875\" height=\"2500\"\/><\/p>\n<p class=\"wp-caption-text\">Dr. Mark Tarnopolsky<\/p>\n<\/div>\n<p><span style=\"font-weight: 400;\">Jonathan has become the most active member of his own care team, shaping his diet and fitness regimen meticulously, armed now with the full knowledge required to do so. This is a disease that, Dr. Tarnopolsky explains, requires constant management, much like other chronic conditions like diabetes. CPT II deficiency is not curable, but with the right <a href=\"https:\/\/buradabiliyorum.com\/en\/category\/download-scripts-themes-apps\/\" data-internallinksmanager029f6b8e52c=\"9\" title=\"Download Scripts &amp; Themes &amp; Apps\" target=\"_blank\" rel=\"noopener\">app<\/a>roach, it\u2019s possible to live a healthy life not limited by the condition.\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Ongoing research is also providing medical innovations to help manage the disease, although Jonathan prefers to go purely the route of lifestyle-as-treatment. He continues to exercise religiously, spending time on the treadmill or stationary bike when the weather keeps him indoors. When he\u2019s out on his bicycle, he knows what food to pack and when to consume it to keep his enzyme levels in check.\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400;\">In recent years, Jonathan has gotten involved with <a rel=\"nofollow noopener\" target=\"_blank\" href=\"https:\/\/sr.studiostack.com\/c\/link?l=988889&amp;s=983978\">MitoCanada<\/a>, the national foundation supporting those living with mitochondrial disease, and he appreciates that there\u2019s an active community to encourage research and provide support for Canadians living with all forms of mitochondrial dysfunction.<\/span><\/p>\n<div class=\"longform-fwimg-container\"><img decoding=\"async\" data-sizes=\"auto\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Jonathan-SUB-6.jpg\" alt=\"\"\/><\/div>\n<h2><span class=\"ez-toc-section\" id=\"A_healthy_dose_of_vigilance\"><\/span><b>A healthy dose of vigilance<\/b><span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><span style=\"font-weight: 400;\">Jonathan thinks about his condition every day, because a constant awareness of his health is now ingrained in him. Everything Jonathan does is intentional, directed at building his strength, respecting his limits, maximizing his joy, and always pushing forward. For him, this means never losing track, even for a moment, of what his body is telling him, because he knows that not very much has to slip before he risks another episode like the one that hospitalized him in his native South Africa all those years ago.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">But, he emphasizes, there\u2019s a world of difference between adapting his lifestyle to his health and letting his health dictate his life. As Dr. Tarnopolsky says, the biggest driver of good outcomes for CPT II deficiency patients is how well they adapt to managing their condition. And Jonathan is nothing if not adaptable. Managing his changing health is a lot of work, but Jonathan puts that work in precisely so that he can stay in the driver\u2019s seat. And an outside observer might never see just how rigorously his course has been charted.<\/span><\/p>\n<div class=\"longform-fwimg-container\"><img decoding=\"async\" data-sizes=\"auto\" src=\"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Jonathan-SUB-7.jpg\" alt=\"\"\/><\/div>\n<p><span style=\"font-weight: 400;\">\u201cI would say that, to this day, 98 per cent of the people I know have no idea that I have this condition, and that\u2019s how it has always been for me,\u201d says Jonathan. \u201cI cope with my condition as I cope with anything else. It affects my life, but it doesn\u2019t run my life. More than anything, it\u2019s a motivation to keep me going. That\u2019s what I hope other people will learn from my story. This condition is an opportunity to motivate yourself if you don\u2019t let it take over your life. CPT II deficiency is not a life sentence.\u201d<\/span><\/p>\n<p><i><span style=\"font-weight: 400;\">Living with a long-chain fatty acid oxidation disorder (LC-FAOD) comes with unique challenges. Staying connected to updated information will help focus your efforts on the best possible care. To learn more about CPT II, or another type of LC-FAOD, visit <\/span><\/i><a rel=\"nofollow noopener\" target=\"_blank\" href=\"https:\/\/sr.studiostack.com\/c\/link?l=983984&amp;s=983978\"><i><span style=\"font-weight: 400;\">FAODinFocus.ca<\/span><\/i><\/a><i><span style=\"font-weight: 400;\">.<\/span><\/i><\/p>\n<p><strong>MitoCanada is dedicated to creating a world where all lives are powered by healthy mitochondria. Present in almost every cell in our bodies, mitochondria are mighty, energy-producing structures that generate the energy we need to survive.<\/strong><\/p>\n<p><strong>When mitochondria are dysfunctional, the impact on the body can be devastating. If you or someone you know is living with, is at risk of developing, or believes they may have mitochondrial disease, you can learn more at <a rel=\"nofollow noopener\" target=\"_blank\" href=\"https:\/\/sr.studiostack.com\/c\/link?l=983982&amp;s=983978\">MitoCanada.org<\/a>.<\/strong><\/p>\n<p><i><span style=\"font-weight: 400;\">This article was sponsored by Ultragenyx Pharmaceutical Inc., a supporter of MitoCanada. The authors had full editorial control over the content of the article. The views expressed in this article are not necessarily those of Ultragenyx; Ultragenyx does not take a position or express an opinion as to the treatment-related or other advice provided by this article.<\/span><\/i><\/p><\/div>\n<p><script async defer crossorigin=\"anonymous\" src=\"https:\/\/connect.facebook.net\/en_US\/sdk.js\"><\/script><\/p>\n<blockquote><p><strong><span style=\"color: #ff6600;\">If you liked the article, do not forget to share it with your friends. Follow us on\u00a0<span style=\"color: #ff0000;\"><a style=\"color: #ff0000;\" href=\"https:\/\/news.google.com\/publications\/CAAqBwgKMLG0nwswvr63Aw\" target=\"_blank\" rel=\"nofollow noopener noreferrer\">Google News<\/a><\/span>\u00a0too, click on the star and choose us from your favorites.<\/span><\/strong><\/p><\/blockquote>\n<blockquote>\n<p style=\"text-align: center;\">For forums sites go to <span style=\"color: #ff9900;\"><a style=\"color: #ff9900;\" href=\"https:\/\/forum.buradabiliyorum.com\/\" target=\"_blank\" rel=\"noopener\">Forum.BuradaBiliyorum.Com<\/a><\/span><\/strong><\/p>\n<\/blockquote>\n<blockquote>\n<p style=\"text-align: center;\"><strong>If you want to read more <a href=\"https:\/\/buradabiliyorum.com\/en\/category\/news\/\" data-internallinksmanager029f6b8e52c=\"2\" title=\"News\" target=\"_blank\" rel=\"noopener\">News<\/a> articles, you can visit our <span style=\"color: #ff9900;\"><a style=\"color: #ff9900;\" href=\"https:\/\/en.buradabiliyorum.com\/general\/\" target=\"_blank\" rel=\"noopener\">General category.<\/a><\/span><\/strong><\/p>\n<\/blockquote>\n<p><span style=\"color: black;\"><a style=\"color: #ff9900;\" href=\"https:\/\/www.macleans.ca\/longforms\/life-with-carnitine-palmitoyltransferase\/\" target=\"_blank\" rel=\"noopener\">Source<\/a><\/span><\/p>\n","protected":false},"excerpt":{"rendered":"<p>&#8220;Going the distance: Jonathan\u2019s story&#8221; Created by Jonathan Fleisch is 70 years old, and he\u2019s not ready to slow down. He\u2019s still bouncing back from a recent surgery and, when he talks about his recovery, it\u2019s clear that he\u2019s mostly just frustrated that he can\u2019t spend as much time on his bicycle as he would&#8230;<\/p>\n","protected":false},"author":1,"featured_media":493042,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"fifu_image_url":"https:\/\/www.macleans.ca\/wp-content\/uploads\/2022\/09\/Jonathan-SUB-1-766x431.jpg","fifu_image_alt":"","footnotes":""},"categories":[1],"tags":[133266],"class_list":["post-493041","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-general","tag-mitocanada0922"],"_links":{"self":[{"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/posts\/493041","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/comments?post=493041"}],"version-history":[{"count":0,"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/posts\/493041\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/media\/493042"}],"wp:attachment":[{"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/media?parent=493041"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/categories?post=493041"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/buradabiliyorum.com\/en\/wp-json\/wp\/v2\/tags?post=493041"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}