And then after they completed the protocol we would survey the individuals about their experience. Half of them said they were more confident in their ability to safely compete, complete the exercises. Two-thirds said they would like to participate in the study again. Many of them wanted to improve on what they felt were some deficiencies in their performance and three quarters actually thought that the blood pressure monitoring was valuable in terms of feedback because we could tie that, as I mentioned, to their self-reported level of effort, so once they knew that that level of effort was safe in terms of blood pressure response it seemed to make them much more confident to engage in those exercises.
So the conclusions from this are that we found people with aortic disease can safely and effectively perform most moderate intensity exercises. We did notice that some patients became more hypertensive during exercises even as resting blood pressure was not significantly different than controls, pointing out that individualized measurement of blood pressure does provide value, especially for patients with thoracic aortic disease and regular training in terms of increased weekly moderate activity time also seems to blunt that rise in blood pressure with different exercises.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
We, we also looked at the aortic dissection subgroup. I mentioned that was a minority of patients but we wanted to make sure that was safe and effective for them. We did notice that they had that, they had a wider pulse pressure that is the difference between the systolic and diastolic pressure and we think that's related to increased stiffness after aortic dissection in this group.
However, there was no significant difference in the variability of exercise measurements between different repetitions. They weren't getting tired during exercise more frequently than healthy controls and they were able to finish the protocol with as many readings as the healthy controls and did not experience any complications.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
So our group has for the last two years studied the safety of exercise for our patients who come to clinic with thoracic aortic disease of all types and we started to do this systematically because our patients often ask us what is safe for them and we thought it would be great to get some, some data some objective data by which we could advise patients on their individual risk related to exercise.
So this is the protocol. We obtained some baseline information about each person including their weekly activity levels, how many minutes of exercise do they do, is it light, moderate or, or intense and then we also get, measure their blood pressure while they're doing a set of standardized exercise, the exercises. These are listed here and I and I think, I think the picture changes if you advance but I, I don't know if, yes okay, so bicep curls, leg lifts, these are six inch leg raises, hand grips, I think it's the next one, wall sits, there's a hand grip with a dynamometer, a wall sit, six, stationary bicycling and a treadmill.
These are what they do in our cardiac rehab facility supervised under by us and by the rehab facility team there and while they're doing this they wear the device that you see on the left, which is a space labs ambulatory blood pressure monitor, and we check their blood pressure in between exercise and during exercises while they're holding a steady state. We also asked them to rate the difficulty in each exercise for them using a 10-point scale. They point to it and tell us if it if it's very light, if it's moderate, or if it's very strenuous for them and so then we can correlate their blood pressure response to how they individually rate each exercise.
So we started, we published the data on the first approximately 50 subjects. We had 31 patients with some diagnosis of aortic disease. Most of these were aortic aneurysms. We had a few dissections, patients with dissections in the group that I'll tell you about and 14 age-matched healthy controls.
So what we found is almost everyone could do these exercises at a moderate intensity level and we were able to obtain blood pressure measurements on about 90 percent, so we did have some technical limitations but we actually got better at measuring exercise as we went along and most of these failed during cycling and leg raises where there's intense muscle contraction in movement and, and we have since come up with protocols that may make it easier for us to measure the pressure during these activities.
So in terms of the fee, of the safety we were really interested in knowing how many patients develop severe hypertension during exercises and this was relatively rare. You can see that six sub, six patients and no controls had systolic blood pressure events of more than 180, so this is at least one reading of more than 180 millimeters of mercury, which is quite hypertensive and most of these were during the isometric exercises, particularly wall sits and the hand grip and not during the dynamic exercises like bicycling. As you remember from, from Alan's talk, the dynamic, that typically we see lower and sustained responses to blood pressure during dynamic exercises and less predictable and larger blood pressure increases during isometric, so that was consistent.
And these are the, this corresponded to how they rated the exercises typically the, the highest rating on the scale of effort were in these leg raises and isometric exercises where you're bearing down and contracting so that also was expected.
So the, the good news was that overall there was no significant blood pressure difference in terms of their response to different exercises in our patients in orange versus the controls in blue here and this goes through all the exercises that I mentioned before: leg raise, hand grip, cycling, bicep curls, and wall sits and we tracked the systolic and diastolic pressure separately, so there wasn't as we initially thought there might be an exaggerated response because our patients may not exercise as regularly but overall we found that this was fairly good news.
We, we also found that patients who self-reported being less active actually became more hypertensive during exercise and we showed this by on the bottom plotting. The number of times they reported a systolic pressure during, we measured a systolic pressure greater than 180 millimeters of mercury during one of the exercises or more and the minutes of activity they reported per week on our questionnaire and you can see that of about, above about one hour per day of activity circled in red, we had very few patients who reported elevated blood pressures with any exercise and the ones that did again was mostly the isometric exercises like the wall sits and leg lifts not the, not the aerobic exercise, so there appeared to be a dose response between the amount of activity you do on a regular basis and your susceptibility to, to becoming hypertensive to a high degree during exercise.
And this, this is tracked here also so what we also found is that on the left for bicycling systolic blood pressure, so again an aerobic activity there was a linear relationship so as you beca, as you had more minutes of participated and more minutes of weekly activity the blood pressure response during your bicycling actually decreased. So the patient's, patients had that, had the most activity also had the smallest rise in their blood pressure.
This was not true, however, for isometric exercise so this is the wall sit, which tended to promote the largest rise in blood pressure and you can see that the dots are all over the place, so there was no consistent relationship between regular activity and isometric exercise. It's as if the body never completely adjusts to it in the same way that you can adjust to regular aerobic activities.
The one finding that we did, did show that was significantly different between patients with aortic disease and our healthy controls is that after their, after they stopped exercising their blood pressure would, would drop more abruptly within three minutes of stopping an exercise than control, so this is the average reading within three minutes of finishing an exercise in terms of and compared to their baseline blood pressure that is when they started
And you can see that in the orange the patients actually dropped below their baseline in terms of their systolic pressure within a few minutes after stopping exercise and also had a smaller had us, had a much more significant drop in their diastolic pressure and this does correlate anecdotally with patients telling us that they felt light-headed more often than controls although we didn't measure that directly in the, in the trial. We do hear that from patients from time to time that after they exercise they may feel light-headed, a little dizzy and that can also be related to the medications although we didn't find a direct relationship between medication use and the drop in the blood pressure in this particular set of individuals.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
2003年、当時大学生だったクリスティーン・ミセレンディーノは自身のブログである『But You Don't Look Sick』（『だけど君は病気に見えない』）に記事を投稿した。タイトルは「スプーン理論」。この記事の中でミセレンディーノは、全身性エリテマトーデス患者である自身の日常を、例えを用いて友人に理解してもらおうとする。インターネットの世界で生を受けたこの言葉は、今日では慢性疾患患者のコミュニティそして医療関係者の知るところとなっている。
患者らは現在、オンラインや The Marfan Foundation のソーシャルメディア上で自らの病気を公表することができるようになり、そのおかげで私は、患者目線で語られるマルファン症候群を知ることができるようになった。今日では、マルファンであることのメリット・デメリットを伝える動画にスマートフォンからアクセスできる。どちらの立場を取った動画であれ、これまでに公開された情報よりも有益な内容となっている。ブリガム・ヤング大学2年のアレグラ・スタートヴァンドは TikTok で、マルファン患者である自身に寄せられた「だけどあなたは病気に見えない」といった辛辣なコメントを公開している。
1968年、ジョーン・ディディオンは著書『The White Album』の中で、持病の慢性偏頭痛について記し、エッセイ『In Bed』では、今日、若いスプーニーらが作成し合うコンテンツに類似したテーマを取り上げている。これは「スプーニー」という言葉の誕生よりも遥かに昔のことだ。このエッセイの中で彼女は、偏頭痛の頻度と重篤度を認めることへの抵抗感、知り合いと医療従事者によるミスリードと拒絶、待ち焦がれた偏頭痛の受容について振り返っている。
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
Thank you so much, Alan. That was for that excellent introduction. As, as we mentioned there are, there's some growing body of evidence that exercise is actually not only safe but may be beneficial for, for individuals with aortopathies.
And this originated a few years ago in a study of mice with Marfan syndrome gene mutations, so these mice are very different from humans. I mean they don't, they don't have the same course of disease as humans do but they do share many characteristics including the development of aneurysms and acute aortic dissections of the thoracic aorta, so in this study they took they divided the mice into two groups: one was, was assigned to an exercise regimen where they were put on a treadmill for five months and the others were not allowed to exercise: they remained sedentary doing their normal daily activities and then they compared the mice that were sedentary to those who exercise at various levels based on the speed of the treadmill, which were up to 85 percent of their peak capacity.
So this slide shows the summary of what they found, so the key takeaway here is that the mice who exercised actually decreased the rate of aortic root growth compared to their sedentary littermates, meaning that what you can see here the dilation rate was actually lower here in the red in the mice that exercise than in the blue mice who were sedentary and this was really a revolutionary finding because people had might have expected that their that exercise could be safe but they had no anticipation of such a dramatic effect over a relatively a short period of time and importantly exercise did not lead to an increase in elastic tissue degeneration in the aortic wall when they looked at the pathology of the mice aortas, so this was another reassuring finding that promoted the safety of exercise.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
Finally moving on to the guidelines in competition again intense activities for high schools, college and professional athletes competitive sports when you have an underlying heritable thoracic aortic condition that's syndromic or non-syndromic really is very restrictive because of the levels of blood pressure that can be obtained in most types of competitive sports whereas non-competitive or recreational exercise what most of us do in life are much more rational and forgiving and recommend participating in this for 30 minutes five days a week in people who have underlying heritable disorders for fitness and health including aortic health.
And these guidelines have been codified, the Heart Association, European Society of Cardiology, the Marfan Foundation, Loeys-Dietz Foundation, now under the Marfan Foundation, all have had very nice recommendations including the 2022 aortic disease guidelines just published about exercise and even cardiac rehab in people after aortic dissections, avoiding collision, competition, strenuous activities, isometrics but favoring activities that involve the less isometric and more dynamic exercise at reasonable pace.
こちらのスライドにはガイドラインを載せています。米国心臓病学会、欧州心臓病学会、The Marfan Foundation、Loeys-Dietz Foundation の各団体が公表している内容はいずれも非常に素晴らしいものです。さらに、公開されたばかりの2022年度版の大動脈疾患ガイドラインでは、大動脈解離後の運動や心臓リハビリの他、衝突や競争、負荷のかかる運動、等尺性運動を避け、動的で無理のないペースでできる運動を推奨する、となっています。
I'm going to stop there and turn the mic over to my partner Dr. Siddharth Prakash, who's going to talk about some of the research and exercise in aortopathy.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
You can kind of get a sense about the blood pressure and heart response to exercise depending upon the level of exercise. The metabolic equivalent of task or MET is one way to measure that energy expenditure and there are tables and and different ways you can look it up to anticipate that. It's important because the higher the METs, the metabolic equivalence, which like basketball, soccer heavy farming, etc. much different than fishing, sitting, you know, a low level of table tennis, yoga, pilates, which are safe levels of physical activity. The higher the metabolic equivalent requirement in general the higher the blood pressure response.
So there are some rough guides to this but it can be variable and, and Dr. Prakash might talk about some blood pressure responses that we might expect with different physical activities. The heart rate response to exercise always is quite variable and depends upon conditioning, so if someone is in less a poorer condition they might have a resting heart rate of 90 or 100 so little physical activity could make that heart rate go up quite a bit whereas somebody on a high dose of the beta blocker might have a resting heart rate of 50 and can do quite a bit of exercise without much of a heart rate response, so if you think about that and we generally have more variation in recommendations about heart rate response. It's really more a matter of how do you feel when you're doing the physical activity and what level of exertion are you doing.
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
