Abdominal Stenosis

As I mentioned in my previous post (https://livingwithfh.blogspot.com/2023/09/old-test-new-reaction.html), my cardiologist and the vascular surgeon ordered a CT scan with contrast of my abdominal aorta. This test was done in 2021 and it showed several areas of atherosclerosis of basically every branch of my abdominal aorta. 

Now, two years later, it shows the same areas of problems - some of the areas look stable (yay!), and some are slightly worse, or show new developments. For example, I have developed “collaterals”, which means additional (new) branches of the abdominal aorta that naturally grew to bypass the areas that are stenotic. So, the fact that the collaterals are there at all, to help with the proper irrigation of the gut area, is good, but the fact that they had to form at all says the original branches don’t provide proper circulation to the abdomen. 

In addition to the stenoses caused by the calcified plaque, because this is me, and I am not easy,  I also have a congenital defect of my celiac abdominal aorta called “median arcuate ligament compression“ (or MAL compression) which is rare condition in which the median arcuate ligament compresses the celiac artery which impairs blood flow to the liver, stomach and other organs. The median arcuate ligament  is the muscle and fibrous structure that wraps around the aorta at the diaphragmatic opening. So, this compression in addition to the atherosclerotic celiac artery causes a reduced flow to my abdomen. 

Here are the test results of the two scans, over the past two years: 

The December 2021 abdominal CT scan results show the following: 

• Small caliber abdominal aorta with extensive soft and calcified plaque

• Narrowing of the infrarenal abdominal aorta

• Two areas of moderate to severe stenosis in the proximal celiac artery and atherosclerotic plaque at the ostium and compression from the median arcuate ligament (MAL) with mild poststenotic dilatation. 

• Mild stenosis at the origin of the small mesenteric artery. 

The September 2023 abdominal CT scan results show the following:

 

• Stable: Severe calcified and noncalcified atherosclerosis of the entire abdominal aorta which is overall small in caliber, unchanged.

• Stable: There is mild to moderate relative narrowing of the infrarenal abdominal aorta at the inferior mesenteric artery, also unchanged.

• Slightly worse: Severe stenosis and angulation of the proximal celiac artery likely owing to a combination of atherosclerotic plaque and the median arcuate ligament (MAL) compression. (previously: “moderate to severe”)

• Slightly worse: Persistent moderate stenosis of the proximal superior mesenteric artery (previously: “mild”)

New (good and bad): 

• The presence of arterial collaterals between the celiac-superior mesenteric artery and to a lesser extent the superior - inferior mesenteric arteries territories suggest the presence of long-standing, hemodynamically significant stenosis in at least one of these vascular territories. 

In this scan, the white part to the left of my spine is the calcified aorta. The smaller vessels springing from it are my celiac (the one in the shape of a backwards L which shows the MAL compression) and the mesenteric arteries. 

Although the tests seem virtually unchanged, I have new symptoms that could be related to the various stenoses in my abdominal aorta. Some of these symptoms are a fairly consistent upper-abdominal pain, which is worse after I eat; the feeling of being “full” even when I first wake up in the morning and I have an empty stomach; occasional nausea with even mild exercise, softer and more often stools. 

When I went in to the vascular surgeon this past week to review these results, the surgeon got called into emergency surgery and was not able to make our appointment. I spoke, instead, to one of the PAs (not his own PA, who I knew and who knew my case, but a new one), and having just seen me for the first time, he said that the new symptoms worry him and he will need to chat with the doctor and see what, if anything, needs to be done next. But he also said that in his opinion, the results are not much different than the ones from two years ago, so the symptoms might not be related to the stenoses. 

I also mentioned that I have been examined by a gastro-enterologist earlier this year, to ensure none of these new symptoms are GI related and they are not, according to those tests. He said he will follow up with them and then he and the vascular surgeon will follow up with a plan, if there is anything to be done differently. At the very least, he suggested that I’d move the scans to every 6 months rather than every year to keep a closer eye on the abdominal aorta. 

Because the risk of too much radiation during a year’s time is there, he suggested I’d alternate between an ultrasound and a CT scan, every 6 months. Although I dread all these different tests (I have another one for my carotid arteries and another one for my heart - both yearly), I know that keeping a close eye on these conditions is really the best way to catch some huge change in time, before having an event. If my annual heart echos taught me anything before I had my open-heart surgery, they taught me this. 

 

Old Test. New Reaction.

One of the reasons that prompted me to start this blog was so that I can share with others the many different tests and interventions I was going through with FH. I sometimes felt like a human guinea pig - and I still do, 40 years later, with every test and every new drug they send my way. 

In addition to the quarterly lipid and liver panels that I have had done since I was 8, many other tests and investigations were added after FH had caused atherosclerosis and heart disease: yearly ultrasounds, CT scans and MRIs, every-five-year angiograms, yearly ABIs, which are like a radio-wave test (I think) checking for narrowing of your peripheral arteries. To say nothing of all the X-rays for every cough you can’t explain. 

I wanted folks that might be diagnosed with FH to have some expectation of what tests are needed or required and for what purpose. 

Every type of radiation ever invented, I believe, has gotten through this body at various intervals. I know not to wear my jewelry (or anything metallic) when I do an MRI and I know that the CT contrast solution will make you feel like you just wet yourself. 

When the technologist walks you to the CT/ MRI/ X-ray/ Ultrasound room and asks (they ask every-single-time) “have you ever had a CT/ MRI/ X-ray/ Ultrasound before”, I roll my internal eyes and always say “yes”. I know that I am not allergic to the dye in the CT exam and that I taste metal whenever they flush the IV lines with saline. I know I am allergic to the glue on the EKG stickies as well as the glue of medical tape, after they tape up the IV to my arm, or after they pull the IV line out or collect blood samples from me and apply pressure to send me home, covering the gauze with said tape. 

After all these years (40 years of FH and about half of those of heart disease), I pretty much know the drill at every test and I know my reactions, or lack thereof for every test. At least I thought I knew everything. Till today ... 

Today, I had to get a CT scan (with and without contrast) of my abdominal aorta. I knew the drill: they would put an IV in, make me take my bra off and my belt if I had one (I didn’t on purpose), make me lie on a table and put my body through a donut looking machine, move the table with me on it in and out of the donut a few times, tell me to hold my breath a couple of times, and at the half-way point inject the contrast solution in my veins through the IV. They asked me if I have had the test before, and I said yes. They asked if I had ever reacted in any way to the contrast solution and I told them no, but that I hate that it makes you feel like you lost control of your bladder. They said, well, yeah, they can’t do much about that (actually, they can do nothing at all about that unless they put you to sleep but they won’t do that). 

So, we go through the motions. The tech does the first part of the test (no contrast), and then she warns me that she will send the contrast in and she’ll do the test again. All I was expecting was the really warm feeling when the solution goes through my bloodstream and the crazy sensation I have told you about. I knew it should be about 2 minutes and then it’ll all feel normal again. 

Only this time, it was different. This time, that warm sensation did not last for two minutes. After about a minute it got worse. And was getting worse and worse as time went by. It was not just the warm feeling, but my head felt like it was about to blow up. My face was numb and felt paralyzed, and the ceiling of the room (all I could see when lying down) started spinning. The table felt rickety and shaky and my head was so hot I thought it would implode! I kept thinking “I am dying! Oh, Lord, this might be it! I am losing my consciousness and I am surely dying.” It lasted for what seemed like forever, although I am sure it was more like 10 minutes. The tech came in and I was not moving and I could not even answer her. I heard her voice but I didn’t know what she was asking. I vaguely heard her asking if I was OK, and I faintly said “I don’t think so”. 

After I explained to her what I was feeling, she took me to a different room and sent a nurse to come and take a look at me. My speech was slurred and faint, like in a whisper. 

The nurse evaluated my symptoms and concluded that what I was having is what they call a “physiological reaction” - not to be confused with an “allergic reaction”. She said with an allergic reaction, my tongue and throat would swell up, I would have hives and start sneezing and have an itchy face or body, but I was not having all of this. Instead, I was having something similar to a head-rush because the contrast was injected at a high speed. She said your body is kinda confused and trying to tell you “oh, no, what just happened?” - because it got too much dye too fast in a short amount of time. 

She said CT contrast is injected at various speeds and different tests in different areas of the body ask for different speeds. The abdominal CT tests require a higher speed and this is probably what happened. She said there is nothing they can do to prevent this other than just lower the speed. But she said sometimes this is not possible because lower speeds might not capture clear pictures. 

The nurse said for an allergic reaction, they can give you Benadryl, but for a physiological reaction, there is nothing but awareness and taking it easy after the reaction happens ... 

She said next time I am getting another CT like this to warn them that the higher speed will make me loopy but to expect that there is not much they can do about it. 

I was wearing a mask in the office and all I wanted was to be out and breathe some air without a mask. She offered to keep me in the office for a bit as I was still dizzy, even after 20 minutes or so from the test. But I walked out, holding on to walls and slowly making my way to my car. It was another 20 minutes in my car till I felt well enough to drive. 

Even after I got home (45 minutes later from waiting in the car for a while), I was still in a fog. I was still dizzy and unstable on my feet, and after having some lunch and feeling a bit stronger I felt completely drained for the rest of the day. 

I am posting this just as a personal account of what can happen over time - no two tests, I guess, are alike, even if on paper they are virtually the same test. The nurse said anything can happen with time. Your body changes, your body’s tolerance for things changes and the factors that influence your body at the time of the test are always changing. I might have been fine all these years getting CT scans like they’re a fun pastime in the photo booth at the fair, but my body is different now and it can react differently than ever before. You just never know. 

I don’t think I’ll ever discount any new “old” test that I am going to have from here on out - and will look at it with new eyes and know that anything can happen. 

And oh yeah, the tape they stuck on me during the IV and after they pulled it out did break me up all over, but at least this part I was expecting. 

Access to New(er) Treatment Might Not Be As Cut-and-dry As You Might Expect

Disclosure: I just want to say, as usual: I do not recommend any of the drugs mentioned in this blog post. These are solely my own experiences, research, and conclusion at this time. Mine, and no one else's. No drug companies or medical facilities contributed to or are to benefit from the opinions listed here.

Also, I would like to mention that I am aware of the privilege I have to have options and choices for treatments. Millions others (without exaggeration) are not as fortunate as this.

As you might remember, back in May (http://livingwithfh.blogspot.com/2023/05/treating-heart-disease-haggling-style.html), my LDL cholesterol was 125 mg/dl. This is hardly “acceptable” for someone with my load of risk factors (previous heart surgery, heart attack, family history of strokes and heart attacks, and almost 20 years of coronary artery disease). My target LDL is 70 mg/dl at this time - lower than a person with no risk factors. 

But with FH, especially with Homozygous FH, it’s what you have to settle for, most often. Maybe you cannot ever get to your target levels, but you can reduce the untreated levels massively - as it happens in my case and just call it your best effort. Last time my cholesterol levels were measured without any medication, my LDL was 475 mg/dl. Coming down to the current 125 is clearly an achievement not to be contested, I think. 

But could we do better than this?! I have heard of first-hand reports from HoFH individuals that have tried the new drug Evkeeza (https://evkeeza.com/s/) that they managed to reduce their LDL down to as low as 50 mg/dl. Evkeeza is in the class of drugs called “ANGPTL3 inhibitors” and it reduces the levels of all three major cholesterol-related fractions (LDL, HDL, and triglycerides). There is still no proof that it does reduce the advancement of atherosclerosis or cardio-vascular disease, but from what we know about high lipids, reducing their levels normally triggers the reduction of atherosclerosis. But this is just a hypothesis at this time in the history of FH drugs. 

The drug is approved only for Homozygous FH, at this time, but given my diagnosis (https://livingwithfh.blogspot.com/2017/08/the-long-journey-to-hofh.html) of HoFH, this seems to fit the bill for my case. 

As you might also remember from the post back in May (linked above), I usually have to haggle with my cardiologist for the right treatment.  Evkeeza is a relatively new drug. It was approved by the FDA in February of 2021. I asked my cardiologist about it in July 2021. At that time, he did not know anything about it. I asked again last year and he came back with the same answer and only evasively agreed to look into it. He suggested I might be eligible for Leqvio (Inclisiran - https://www.leqvio.com/) which is a PCSK9 inhibitor (similar to Praluent or Repatha) but given in a different dosage and with a different frequency. If I were on Leqvio, I would need to stop the Praluent that I am on now. If I were on Evkeeza, from what I know from my own research, I would be able to add it to my current drug regimen (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html). This way, I would receive the lowering benefits of all the other medications I am on, since they have different mechanisms of action. 

I tried asking my doctor again this May (2023) if I could qualify for Evkeeza. At that time, he begrudgingly said “well, I guess I can look into it, but I have no idea how hard it would be to get your approved, and I don’t even know if you would get approved.” 

I asked him to explain: I have a genetic test that shows clearly I am a Homozygous FH patient; I have lots of risk factors proven by a long history of atherosclerotic events, I have the family history, I have a worsening heart (according to the stress test that he did last year there is increased damage to the heart muscle, possible from completely closed capillaries - he speculates), and I am on four different therapies for lowering cholesterol and my LDL is still not at target. 

I asked him how he make it that I would not get approved? 

He said something like (or asked, rather):  “do you have a written test result that shows that a medical institution confirmed the HoFH diagnosis?”. It took all of my might not to scream when I heard this question and not to literally slap him. 

But I like to give people the benefit of the doubt and acknowledge that I am not their only patient and they have a busy schedule and no time for research, but still. The genetic test was one of the first documents I showed him back in 2018 when we first met. Plus, he had asked me for the “written genetic test result” several times when his office got me approved for Praluent and Nexletol (these are not medicines approved only for HoFH but the accurate diagnosis was needed to get the approval in both cases). Both times, his office confirmed that they would scan this test result into my records so it would always be on file. Either that didn’t happen, or he doesn’t read my files? It’s anyone’s guess. 

So, I sent him a new copy of my genetic test. This was in May. He said he would “look into it” and I never heard anything since (three months later). I asked for an update a couple of weeks ago and I finally got a note from him a week later: “Looking into it.” 

So, as of right now, I don’t know whether I qualify for Evkeeza (I can only guess with a strong file built by someone who understands my disease I might), nor if it’s even recommended for me. He never so much as said that it might not be good for me, for one reason or another. All I know about this drug is what I have found in my own research. 

But it irks me to no end that a treatment option might have been out there for me, for 2 years now, and I am not taking advantage of it. Especially with my heart condition still worsening ... 

In this disease, time is truly of the essence and the older we get (as we all are), the harder it will be to stave off the advancement of artery disease when LDL cholesterol levels are outside the limits. That we know. 

But switching to another doctor is not that easy - not with a complex disease as mine (and I am sure many of you can relate). There are things “set” with the doctor that takes care of both your heart and your lipids. 

To name a few of the things that would need to be restarted with another physician if I were to walk away from this one (I think you’re tired of me by now reading here how dissatisfied I am with him and how many times I thought of changing him out with another doctor): 

• He manages my yearly heart tests (echo and nuclear stress test, mainly but he is also on the vascular team that manages my carotids and abdominal aorta too). I would have to start fresh with another doctor to explain the need for all the tests, the history, etc ... 

• He approves my specialty-pharmacy drugs every year (Praluent and Nexletol) and his office does a flawless job with this. They have this down to a science (and I hope I am not jinxing myself for the future!)

• He manages my INR levels. Well, that is not fully true: I manage my own INR levels, but I cannot get measuring strips for my INR machine anywhere else unless I am enrolled in a “test at home” program that is setup through an INR clinic, like his practice has. 

• As an aside - I have met a lipidologist who would love to get Evkeeza approved for me, but he does not work with any insurance company, so I would have to pay for an office visit several times a year, and for the lab tests on my own and then ask for reimbursement. This is something that the current doctor (given that he is with a major healthcare system) does as a service right now. A minor inconvenience, I know, but I am seriously thinking at least of this switch to the lipidologist, if nothing else. I would still need the current cardiologist for all the heart-related issues. 

Our healthcare system is already complex and unyielding. Add the complexity of FH and heart disease to it and you’ve gotten yourself into quite the spiderweb. But some hair will have to come off when I pull that bandaid, eventually. Like I said: I am not getting any younger. I have less and less the desire to also get any sicker, too (although this might be unavoidable) - especially when there might be other options out there for better treatment. 

Maybe Evkeeza is not for me. Maybe there are some contraindications that I don’t know about from my own research. I am not a doctor, obviously, and I don’t know how to apply a drug to my unique disease profile. This is what I am looking at doctors to know and explain to me. But I need to know something - anything, about whether this is worth trying or not. Something more than “they’ll look into it.” They have been doing this for close to 2 years now (the first year they “never heard of it”). 

At this time, I feel like I have reached a crossroads but not sure what the direction will be from here ... 

Treating Heart Disease, Haggling-style

As readers of this blog probably already know, once you have cholesterol which is only somewhat managed but not fully, you start getting the additional baggage of heart disease and sometimes even strokes. So, as you well know, I manage two diseases - both high cholesterol and heart disease, hence the name of this blog. 

This entry is about how I have been trying to manage my heart failure condition in the past few months to a year now. My heart failure is only one of the several heart-related diagnoses that I have been saddled with. Some others are coronary artery disease (CAD), aortic stenosis, and atherosclerosis. 

I was diagnosed with a high BNP value in 2017 (for more information on that first diagnosis see this post from that year: ​​https://livingwithfh.blogspot.com/2017/05/cholesterol-and-heart-health-update.html). At that time, it was 285 pg/ml. But at that time, they did not call it heart failure yet. That didn’t happen until 2022. 

BNP shows the amount of damage in your heart and helps the cardiologist diagnose the level of your heart failure. My type  of heart failure is “Heart failure with preserved ejection fraction” which is a category all of its own. All other categories are different degrees of “heart failure with reduced ejection fraction”. My ejection fraction is good (unlike the heart failure with reduced ejection fraction), but the heart muscle is stiff which is what causes the high pressure, along with other symptoms, like shortness of breath. Outside of the BNP test required to measure the heart failure (a blood test which is not cheap - around $285 without insurance at my clinic), they can also measure the ejection fraction during your heart echocardiogram test which is something I get once a year. During this test, they can also see whether your heart is enlarged or not (more common with heart failure with reduced ejection fraction). Putting all these data points together, they can diagnose more precisely which type of heart failure (HF) you have. The treatments for any of the types vary slightly, so a correct diagnosis is preferred. 

Things have gotten worse for me since 2017 when they first measured an elevated BNP. It kept climbing all throughout last year, during which time my shortness of breath kept getting worse. For example, I could not go up one flight of stairs without feeling completely out of breath; nor could I walk across the living room with a half-full laundry basket en route to my laundry room without panting like a dog at the end of the 20 feet trek. Not ideal for a 47 year old who is not overweight. 

In November of last year, the BNP was the highest that it’s ever been, since we’ve measured it, at 692 pg/ml. Along with a worsening shortness of breath and higher BNP, my blood pressure has not been totally controlled, more often being around 160/ 50-60’s and more seldom measuring at around 120 / 50. Some days, it spiked to 180’s, too. 

During this time, the cardiologist continued to “play” with various drugs to try to manage my blood pressure, first. The belief is that if we control the pressure better, less damage is done to the heart muscle and the BNP will ultimately improve. 

I call it “playing” with the drugs because that’s exactly how it feels. When he decides to add drugs or change drugs for my blood pressure, the conversation goes something like this:

Doctor: Well, we could add the Spironolactone which is a diuretic and it’s a drug older than you are, or, we could add Farxiga which is a diabetes drug and newer. Which one do you want to try? It’s sixes to me! (this is all an almost exact quote)

Me: Well, what do they do? How are they different? (I am not sure that all patients ask this, but they should!).

Doctor: Well, one of them takes the water out of your system, the other takes the sugar out. Both have good results on improving heart function and ultimately BNP.

Me: Which one has fewer side effects? 

Doctor: They are both generally well tolerated, but the Spironolactone elevates your potassium, so we need to measure that periodically and ensure it stays low. No other side effects to worry about. (I have always struggled with a doctor telling me that a drug “is generally well tolerated”. I understand this is all they have sometimes, but I have found that the tolerance is always in the eyes of the beholder. And they never account for that.)

Me (dubious as I know from experience he leaves many side effects out): 

Well, I have not heard of either one, so let me research them and get back with you. 

And he lets me go home and we’ll talk about it at the next appointment, in three months... Then, I choose one (depending on what I find on the internet and what concerns I might have), and he works on the right dosage and how often to take it. And he puts me on it, we try it out for a while, I report any side effects, because there are always side effects (including ones the pamphlets never mention), and I decide whether the side effects are worth living with or we need to stop. 

The dialogue above happened sometime last year, when my BNP kept climbing up slowly till in November it got to 692 and my shortness of breath kept getting progressively worse. Before then, he tried many other drugs - I have been on Amlodipine since 2020; he also tried Losartan and Ramipril. But the blood pressure never really seemed to budge much. 

And then, he added Spironolactone. The blood pressure finally came down - my pattern was now reversed - more days of 120 / 50 and fewer days of 169 / 60. But the chest pressure was only marginally better, if noticeably better at all. And I started getting more palpitations and more panic-attack-type episodes than ever before ... But I stuck with it, because I wanted to see how the BNP would fare. 

He repeated the BNP in December and in February and both times, it was only slightly lower than when we started (581 and 598, respectively). So, finally, in February, I told him I am not sure I should continue with the Spironolactone. The side effects and the little benefit I was seeing did not justify everything else that was not conducive to a qualitatively good life.  I texted him (a luxury, I know, that I have with this particular cardiologist to be able just to text him!) and he said “Stop taking the Spironolactone.” That was that. Not replace it with something else, double up on the other things you’re taking (Amlodipine and Atenolol). Just stop! We have a follow-up appointment later this month, so I guess he figured he’ll make me guess on some new drugs again then! 

I stopped the Spironolactone but I started taking Amlodipine twice a day instead of once a day, basically doubling the daily dose at the very same time he told me to stop the other drug. The doubling was a judgment call I made, after remembering that he told me years ago that the dose of Amlodipine I was taking was a “child dose” (I am very sensitive to any amount of any drug, so he usually starts me on lower doses). So, I figured, if we double a child’s dose, we might get a better response for the blood pressure and by now I have gotten used to the side effects from Amlodipine. He was fine with that. 

Since February, when I made this change, the chest pressure has gotten so much better, the blood pressure continues to stay low, still with occasional spikes, but more rare, and the BNP dropped to 388 when we measured it in April this year - so, almost half of when it was the highest last year. Still too high (the normal is again, below 190 pg/ml), but I take any improvement. The trouble with doubling the Amlodipine, however, is more edema (swelling in the legs and feet) and more sensitive gums (more bleeding when brushing my teeth). You always have to know your body, notice what happens and try to weigh the bad against the good and try to figure out what is manageable for you. I am sure many of you out there would make totally different choices than me, when presented with the same alternatives as I have been. 

I am not sure what, if anything, he might suggest next. He has been bugging me about the Farxiga drug for several appointments now, but I am not sure about it. We will see ... 

On another note, (almost) no idea why this happened during this time, but my LDL cholesterol has come up since November. I have not changed anything about my diet, and outside of “playing” with the heart drugs, I have not introduced any other drugs. However, I lost my dad in November. My own grieving process and managing my mom’s depression from afar (she is in Romania and I am in the US) through her own grieving during this time has been excruciatingly hard. There are many proven studies that show that cholesterol rises with stress (has to do with a higher level of cortisol, the stress hormone, in your body - you can google this and there are virtually thousands of sources speaking about this). 

The latest change in my cholesterol levels

This is just a guess on my part. I have communicated to my cardiologist on several occasions that I am under a lot of stress lately, but when the cholesterol results came back earlier this month, I still got a note from him saying “don’t know why LDL is up. Will talk.” 

If I have learned anything during this journey, it is that the research I do on my own is just as effective, sometimes even more so, than the advice I get from my doctors. It is unfortunate and wrong, but it’s what it is. And I am not sure what broke what: the fact that the patients are more knowledgeable now than they were before caused the doctors to pay less attention and be less educated? Or the fact that the doctors are so much more super-specialized and not as thoroughly trained as before caused the internet to fill in the gaps because the demand from the patients is definitely there?! 

Like the cardio guy said to me: it’s sixes to me. I get my information from him, from other specialists and from the internet and try to make the best decisions. I figure the average of all those opinions is still better than doing nothing. 

Stay healthy, you all! And never stop fighting for your answers. 

It’s Been 7 Years!

There is something magical about number 7. It’s not my number (that would be 9), but it’s magical. I am not going to get into all the symbols of number 7 in all the cultures and throughout history. This is not about numerology. This is about the fact that 7 years ago today I got a new heart and a new lease on life. This is about reaching this milestone and being grateful.

7 years ago, in a hospital in the obscure (to those outside of the US) state of Utah, in a small town of barely over 100,000 people called Provo, I was getting my heart stopped, cut open, and I was having my aorta and aortic valve replaced with man-made parts. Then, I was having four bypass surgeries around four major coronary arteries that were between 90-99% blocked, and several endarterectomies to clean out the incredibly heavy amount of plaque that my 40 year-old body had accumulated due to this little known rare disease called Homozygous Familial Hypercholesterolemia. Are you awake yet?! 

I documented my surgical journey of 8 days in the hospital here (https://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html), for those who want all the details. Several years of cardiac rehab followed, all with ups and downs. This entry, is about the lessons I have learned in all the years that followed that day.

After that massive surgery, along with my body not feeling like my body anymore for a while,  several things transpired. I am guessing if you ever go through a similar journey, you might encounter these learnings, too, so I am sharing:

1. You might shamelessly, unapologetically fall in (platonic, respectful) love with your surgeon. My own surgeon reminded me that it was not only him, but the whole team (three surgeons and three cardiology surgical PAs) that gave me the miracle of a better-working heart, but I know he was the leader of the pack. The mastermind. The true artist. The guy with all the guts to reshape a heart so sick in just one, long (13+ hours) surgery, just because he made a commitment to himself that “he never wants to look at my heart again”. He made sure I never have to have that surgery more than once. How can you fall in love with the person who mended your broken heart? Literally?! I do not know how.

2. Even with a fixed heart, you can still have a heart attack - as my body proved just a week (7 days!) after he closed me up. This added insult to injury, let me tell you, but I was glad I was still in the hospital for it, with all the specialists around me. I survived that, too, although my heart is still reeling from the damage that attack did to my left ventricle.

3. You will re-learn your heart, its new antics, and your whole body after a shake-up like this! You will have new sensations in your chest, new symptoms of chest pain and dizziness, new types and intensities of tinnitus, and so much more! Nothing will feel the same as before the surgery. And it’ll take a couple of ER visits to learn that a heart palpitation might not always mean that you’re having another heart attack - sometimes, it’s just your blood pressure being low and asking for you to lay low for a spell! And the ER staff will give you a bag of salty chips to get the pressure back up and send you home. True story! 

4. You will learn that Coumadin will not kill you by making you bleed out! You’ll manage it, by fits and starts, and you will become begrudging roommates in this newly renovated body of yours. In the end, it’ll become like the rascal little brother that you sometimes love (it keeps you alive by preventing your new mechanical valve from clotting), but it annoys you sometimes when you can’t get a handle on what it decides to do one day (like make your INR twice as high as it’s supposed to be just because you exercised too much one week or your dad passed and you’re under too much stress). 

5. You will really learn the truth about what initially will feel like a cheesy cliche that “once a heart patient, you’re always be a heart patient.” This is, scout’s honor, the honest truth! There is no “cure” from heart disease, really. There is managing it, staying on top of it, but there is no “forget about it. This was in the past.” The disease will stay with you and it will morph into something else every year. Every month. Every day, even! There have been some big wins, for sure: my valve is very healthy and not leaking; my ejection fraction is healthy, around 50%. But there have been setbacks too: my smaller arteries which were too small for bypasses or endarterectomies have gotten sicker and possibly more clogged and continued to damage my heart muscle. My heart muscle, especially my left ventricle, is now stiff and my heart is in the beginning stages of heart failure (with preserved ejection fraction, which is the more rare and harder to treat version, so I have learned in the past year). The annual tests, and the quarterly cardiology visits continue and will continue for the foreseeable future. 7 years later. Indeed, I will stay a heart patient as long as I live. Some old symptoms are here, but changed. And new symptoms have popped up. But this is you now. And this is your reality. And this is OK. Have gun, will travel! You have the tools, the knowledge and the experience, and most importantly: your heart is still ticking - you learn to trust and move on. Being a patient is far better (sometimes), than being dead! And having a disease you can manage is a blessing! You will learn this to be true. I sure, we all want to be fully healthy. But if you were to have a disease, better have one you can manage.

6. You will learn how to be happy and feel safe in this body. Broken, scarred and winded, you will learn what it wants and move on. Take that trip you always wanted (I always wanted to go on a cruise. I went on two during these past 7 years), climb that mountain that you think it’s too much (I remember how I felt like I was going to die before I reached the top of Ensign Peak in Salk Lake City, but it felt like I conquered the world when reaching the top and taking in the view of the Salt Lake and the valley), fly across the world again, even alone. It’ll be scary, but you’ll do it. I (along with millions like me) had the unique misfortune to hit the Covid years during these last 7 years and that “stole” a couple of years of my life. But I learned how to travel locally and how to be careful and still found some joy in the temptations that were close-by. The world is beautiful even outside your front door. And I am glad to see another day to enjoy it.

7. You will learn how to advocate for yourself even more than before. There will be a time when you might have to change doctors - because your original “dream team” that first diagnosed you, babied you before and after your surgery won’t be available - because you’ll move or they’ll retire. The new people won’t listen to you, will assume you don’t know much about medicine or about what really happened during that surgery - but you’ll have to learn that your voice carries and you can always scream louder. You will make them listen, or fire them. And they will, eventually, keep you alive. But you will know that the force is in your lungs. 

8. You will learn to be humble. First off, you will learn to love your body again and thank it for carrying you through another day. I used to refer to my heart after surgery as "oh, my stupid heart", and I don't do that anymore! That was insulting to it. It was demeaning. Now, I cherish it and thank it for every beat, every single second. I bow to it with humility! It does such hard work and it's been through hell. You will learn the miracle of good days, of the days when you don’t feel like an elephant is taking a seat on your sternum, and you will feel grateful to the miracle of medicine that’s helping you through. 

9. You will absolutely become some kind of a germaphobe! No doubt about it! Bacteria and viruses will scare the crap out of you, especially when you read about folks getting sick with endocarditis and having the valve replacement surgery redone because no antibiotics worked to cure the heart infection! It will scare you witless, I guarantee it! 

10. You will learn abbreviations and acronyms that you never thought you’d learn in your life: OM, LAD, CVD, CAD, AHA, EF, HFpEF or HFrEF. No dictionary needed here!

If there is just ONE thing that I could share about how you make it through the hell of open-heart-surgery and heart disease and back is this: don't think too much of the future. Not necessarily in deep, fine details, anyway. Give yourself bite-size milestones to reach every day: today, you might focus on breathing better; tomorrow - on eating more properly; the following day on walking a few feet further. Whatever it is. Focus and do it with all your might, energy and heart. Keep moving forward through your everyday milestones and one day, you'll look back and be amazed at what you've been through, how much you've accomplished, and how strong you are as a result. Telling yourself "I want to climb Mount Everest 6 months after my surgery!" when you still have tubes going through your ribs and you can't take one breath without screaming in pain is nothing but demoralizing. Tell yourself you will kill that tube beast soon; focus on learning how to breathe with it; allow you body to heal and get strong by paying attention to it every day, and Everest will wait for you at the end of that journey. I promise it!

I think the one thing most dear to me that I cherish today is that I am still here. Today, while I am thinking of all the hardships I have overcome with gratitude, I am definitely not thinking about the ones that still lie ahead of me. If I do the work of today, I know I'll be as prepared as I can be for tomorrow's challenges. I have to trust that. Today, I am mostly taking a minute to be grateful. Today, I am thanking my team, my family (my husband is my Guardian Angel, my nurse, my psychologist and so much more!), God, and my body for being here for me through it all. To paraphrase Anne Lamott, "life is such a show-off".

Again, like I said above: I am humble. I know there are forces bigger than me at play. I am merely a raft on the angry ocean, being pulled away, and thrown ashore, but still intact. Damaged, and full of weeds, but still afloat. I’ll cheer to this and hope for at least 7 more "magical" years! 

A journey in time: from the day after the surgery, going home after the heart attack, happy in cardiac rehab, with my amazing surgeon, Dr. John Mitchell, at the top of Ensign Peak a year later and today - 7 years later! (you may click the picture for a larger view)