The Educated Patient’s Post

Transcript

Yeah. You know, I spent a lot of time on this, almost 20 years now, taking care of patients with obstructive hypertrophic cardiomyopathy. And the challenge is that initially most people felt that it was a very rare disease. So if people came with symptoms, classic cardiac symptoms such as shortness of breath or dizziness or chest pain, most cardiologists and patients would would not think to that this would be a heart muscle problem like hypertrophic cardiomyopathy because we felt it was so rare. And because of that, most of the imaging missed it. All the time. And they were being billed as all kinds of other diseases that were more common. They're really not the real problem. So hypertrophic cardiomyopathy is basically a problem where the muscle squeezes quite well and so oftentimes it can be mistaken as a very healthy normal functioning heart. But the truth is a heart has to be able to relax first before it squeezes, almost like cocking your arm back before throwing a ball. You can't really throw it if your arms not cocked back. So the same thing with the heart. And then we started realizing when the heart gets too thick, it gets very stiff and that stiffness impacts how it is able to essentially **** back and allow itself. To push blood forward. And then in some patients, if the thickness gets even even larger, it can cause obstruction. In other words, the blood has to come into the heart, then it has to go out of the heart through a channel called the outflow tract. And if that area is is blocked because the muscle is so thick, then the blood can't get out. And so once that happens, people get a lot of symptoms. And the challenge of hypertrophic cardiomyopathy is symptoms are the same as almost all heart disease because any heart disease impacts how the heart functions. And so you'll get only a few symptoms. With all of these things, and you have to take a lot of time to figure out that this is the real problem in these patients. So the first thing is awareness. And then patients, I agree with Barb that they have to be their own advocate, but a lot of patients can't because they they know something's wrong. But then if doctors say that everything checks out, everything looks OK, then they say I guess I'm overweight or I guess I'm getting older and they live with it for some time. And that's a problem because they're living with sometimes pretty profound heart failure or heart dysfunction symptoms. The one of the challenges in diagnosis is that for many years we felt that hypertrophic cardiomyopathy is something that happens to kids or young adults only. Because everything you see in the newspapers are in the media would be these young athletes who collapse and they're found to have hypertrophic cardiomyopathy or an arrhythmic problem from that disease. And so that gives a false sense that as you get older, it can't possibly be this problem because we feel this problem is mostly related to sudden cardiac arrest or passing out. But later on, the vast majority of patients actually have heart failure symptoms. They get more short of breath. They get dizzy, They do get some chest pain, but mostly it's really fatigue and shortness of breath. And those, as you know, are very vague symptoms. People, some people can, you know, exercise quite a bit, and then they still say they're short of breath and other people can't do anything but because they're vague symptoms. People oftentimes look for other things, but the most common misconception is that it can affect people in middle to late life. And the vast majority however, are in middle to late adult life. The the average age where you're diagnosed with hypertrophic cardiomyopathy is probably in your 40s. And so by then people are often saying, well, I probably don't have a genetic heart problem because it would have shown up long ago. And that is exactly the opposite of reality for this disease. Yeah. So the treatment options have evolved. Really. The evolution includes several medications that were used not specifically for hypertrophic cardiomyopathy, but were used in a variety of heart conditions. And these would include beta blockers, for example, very common medications that help the heart by relaxing it and and trying to prevent some of the obstruction. So we use that medication for a long time, that class of medications for a long time with this disease. And it did help some symptoms, but some patients did not. Have any benefit to the medication or they continue to have an alarming symptoms of significant shortness of breath or even passing out. So for those patients, there were other surgical options. So you can either shave the muscle down that's too thick or you could do something called ablation, which is basically killing that piece of the muscle and shrinking it down to normal thickness of the blood can get past it. All of these procedures were to eliminate the obstruction, meaning that the part that's that's affecting the flow of blood out of the heart. If you can shrink that part down, then you have more room for blood flow. And they do work. But as you can imagine, they have their own potential risks. Any invasive procedure has risks. So over the past few years we've been fortunate to have medications that really target the actual problem and hypertrophic cardiomyopathy, which is this issue with the heart muscle on a cellular microscopic basis really contracting too much. And these medications have recently been approved and utilized as in Barb. And what we've found is that these medications seem to improve the obstruction dramatically and improve the symptoms dramatically in these patients. So this is the the current landscape now is to is to start with some of the older medications, but then potentially move on to Maverick Hampton as an indication to improve symptoms and functional status these people. I think I was interested to hear what Barber said too, because I think one of the things that I guess I take for granted a little bit is that patients do need a diagnosis. And it it the first most important thing is making them feel comfortable that they know what's going on. They can have a handle on it, and they can expect to be improving in those symptoms. So success is individualized to the patient. So I look at my patients. Try to figure out, well, what's affecting them. Is it tiredness? Is it dizziness? Is it passing out? Is it shortness of breath? And I try to figure out what's going on with their heart that is causing that. Because hypertrophic cardiomyopathy can have a variety of different features to it depending on what, what part of the heart is thickened. And I try to figure out, well, how much of this is due to the obstruction? How much of this is due to bowel problems or other problems. And I listened to my patients because at the end of the day, success is measured by the them feeling that those symptoms have gone away. More substantially improved so that they can get their life back. And that is the point. The point is getting your life back. But also more than that, many of the patients with hypertrophic cardiomyopathy, they actually get improvement even even more than they expected because they've been living with it for a long time. So what they consider getting their life back may be actually not good enough because they might feel even younger because the disease has really taken away some years in terms of their symptoms. So success for me is that patients don't have fatigue, they get their energy back, they don't get short of breath. They can do all the activities they want to do during the day. You know, some people don't do as much, some people do a lot. So for me, it depends on what they're doing. But I want them to be able to do everything that they want to do. And I often have to ask my patients that they're able to do everything you want to do, which means including going to the mall, getting groceries, carrying her back up the stairs, that kind of stuff. It's important to me that that the treatments meet their lifestyle. Meaning meets where they're at that what they are able to do, they can do all of that. And it's not good enough for me to just to say that someone has gotten better, but rather that they're, that they're where they want to be to have a, you know, to have a happy life and, and a good quality of life. So the symptoms should, but with these medications and all the treatments, the goal is to get them to have no shortness of breath to never pass out or be at risk of passing out because passing out is very dangerous if it happens at the wrong period of time. Certainly no chest pain. And being able to function, at least you know, generally speaking, climbing two flights of stairs without shortness of breath allows you to do almost everything. So I usually ask those kinds of quests. Yeah, I would just say that the first thing is that hypertrophic cardiomyopathy, even though awareness is there, can be hidden. And even if someone says that it doesn't look like you have it, maybe get a second opinion because it can be very subtle and there are specialists who will look a little bit deeper. And those same specialists will also probably perhaps be able to get the final diagnosis if it's still elusive. The other thing is that no treatments are without risks. And the beta blockers, the invasive therapies and even the cardiac myosin inhibitors like Mavic, Campton, there are risks. And so they do need to be monitored. In this country, there's a monitoring form called the REMS program where patients need to be checked by ultrasound before and after regular intervals to make sure there's no heart failure or any potential complications from the medication. You want patients getting better, you don't want any risks. And so during that time they should be advised about any they get sick or they have any irregular rhythms or they have any new medications, those should be alerted to us. So like every disease, there's a partnership with doctors. And so find the doctor that you feel comfortable with. Has the expertise and, you know, work together as a team to get you where you need to be.