After 6 Years

It’s hard to believe that it’s been 6 years since my open-heart surgery. In some ways, that seems like a blink of an eye. A 6 year old would barely be even fit to start real school yet! But in the measurement of the time that open-heart surgeries recoveries are measured by, 6 years seems like a lifetime. 

February 11: 2016 (right before they rolled me into the OR) - 2022

I hated having such a huge surgery in February - my least favorite month. The month that, historically, has gotten people sick and killed among my loved  ones. A cruel, cold, dark (albeit short) month that’s not always been kind to me. But it had to be in February, because at the end of January, the month before, they found two arteries blocked in my heart (the LAD was at 99%, another one - an OM - was at 90%). The aortic valve was failing, in severe stenosis, and the aortic arch had an aneurysm. The cardiologist wanted the surgery done even sooner, if he could get me and the surgeon aligned on a date. 

I documented my surgery day, step by step, here: https://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html.

And the day before the surgery (the “pre-op” day), here:

https://livingwithfh.blogspot.com/2016/02/the-day-before-open-heart-surgery.html

If you ever have time to read it all, you will see that what was said the day before, what they prepared me for, mostly came true, but there were a few surprises. When I came out of the surgery, everyone who came into my room, ICU nurses, several surgeons who assisted me during the actual surgery (12+ hours), all the three PAs, breathing and physical therapists, and even my rock, my husband - all of them reminded me that I “had just been hit by a Mack truck.” You cannot possibly be very well hit by a Mack truck and expect every single thing to go “according to plan”. You cannot be hit by a Mack truck and plan for it, step by step, then hold God and the world accountable for every one of those steps. You are bound to be surprised, and thrown off the rails, and, oh, yeah, even shocked. 

The day of the surgery was a pretty peaceful day for me, consciously. Yes, my body went through hell; yes, it got killed, drained from all the blood, frozen for more than half an hour, and was brought back to life. But, mercifully, I don’t remember any of it. Sometimes I hear about people remembering bright lights and seeing their lives flash before their eyes, or floating above their bodies in the OR, but I experienced none of this. 

As peaceful as that day was, the days in the hospital that followed were intense! Just full of emotions, but more than anything full of new sensations, of discovering new parts of my body I didn’t know I had or parts that I didn’t know could hurt - my skin hurt. I could not wear ribbed tees or feel the creases in my hospital bed sheet because my skin felt like it was completely burnt ... The two days after the surgery, it took all I had to just stand up, held up by the nurses, beside my bed. Forget walking and balancing on my own. That didn’t come till it was almost time to go home, more than a week later ... I don’t think I ever walked during the hospital stay without holding on to the back of a wheel chair. And the breathing! Oh, my word! I could not breathe! I could not speak, I’d whisper. They wanted me to blow into this plastic tube and felt like that was my last breath, there it went into that stupid tube. At the end of that effort they’d all look disappointed because I never could blow strong enough for them! It was horrible. My lungs took such a beating! Even after three months of recovery, when I went back to work finally, my coworkers were saying that they could never believe I could speak so quietly. I don’t have a big voice to begin with but after the surgery, it was nothing but a whisper. 

Once I got home, the life “after OHS”, the life on my own, without hospital care and around-the-clock medical staff explaining every new symptom, started. I was terrified! Again, I had to relearn my body! I had to learn how to sleep again because no position felt comfortable. No bed or chair. I had to learn how to cough and laugh and sneeze again so as not to let my chest come loose ... I had to build an appetite again although nothing sounded good. I had lost 10% of my body weight in that surgery and I didn’t have that much to lose to begin with: I was 95 lbs at my lightest. 

I went through ER scares when I became too dizzy and light-headed, or when I cut my finger chopping veggies and was scared that the Coumadin might make me bleed forever ... I went through years (not even sure that’s even over yet!) of being scared that my INR will be too high (I’ll bleed to death) or too low (my mechanical valve would clot and I’d die of an embolism or something). Although I manage it better than ever before now, it is still a challenge to keep it tightly in my range, especially when seasons change or when I travel and add unusual, unfamiliar stress to my daily life. 

I went to physical therapy twice, first to build strength in my upper body, right after surgery, and almost two years later to build stamina to be able to walk for a mile at a time without my chest hurting too much and without feeling like I was going to faint from light-headedness. 

I stopped traveling the year of the surgery because I was afraid of getting an infection on airplanes. That was when my immunity was still fighting to heal my heart. I stopped traveling again for the past two years because being a cardiac patient with very high cholesterol for the rest of my life puts me in the “high risk” group for people who might get more severe or possibly lethal Covid. 

I do get out some and I meet with friends and even saw family across the country and in Canada last year. But I limit who I see and if I can’t meet people mostly outside I put it off for later. I am still nervous to fly. Maybe this coming summer ... We’ll see ... 

As much as I hate putting my life on hold, going through that surgery to rebuild my heart, with all its complexities, and then going through years of recovery to function somewhat normally again and being rewarded with a good life, all things considered, seems like the most precious gift. I don’t want to throw myself willingly at risks that are not absolutely necessary for survival, to endanger this beautiful gift. So, I’d rather wait out the pandemic, or not expose myself to other sources of more sickness or things that could damage my heart (or valve) again. In the whole scheme of things, it’s a small sacrifice.

I’ll have to say this though: my journey has been more lucky, more blessed than I have ever hoped for. So much more blessed than others’ paths - although there is no comparing journeys here. Everyone’s path is different and everyone feels pain and blessings at different temperatures. However, I can say, I am lucky to not have had too many complications in these past 6 years of renewed life. 

I wish I can tell you that the minute you walk out of that hospital after your heart surgery you’ll never look back and you’ll be as good as new with not a worry in the world about your health. But I’d be lying.  You will never be the same person you were before. In some ways you’ll be worse and in some ways you’ll be better. In some other ways, you’ll be much, much better. In almost all the ways, you’ll just be different. A different “you” that you’ll need to get reacquainted with. You’ll maybe be more hopeful, more appreciative of your health, with more respect for what your body can do and for its ability to miraculously heal, even after being hit by that Mack truck! 

I can tell you, though, this, for myself: I have zero regrets for anything related to this surgery. There has been no question in my mind that I needed this surgery. This was not elective: this was a surgery to save my life. My aortic valve had only a 0.49 cm opening, it was almost closed. My arteries were stenotic. My chest pain was constant. My ability to walk and exercise was very much reduced. I am very grateful that for my condition there was a course of action. There are so many afflictions out there that have no cure. So many diseases that come with a stopwatch that is set to expire in months, some in weeks or days. This was not the case with my heart disease: there was something they could do. I am grateful for that. 

I am also grateful for my team of doctors in Provo, Utah, at the Utah Valley Regional Medical Center. My cardiologist and my surgeon are my heroes, my true soldiers who fought as hard as me to get me back to “normal”. But the other members of the team, the PAs, the other surgeons, the medical staff, the technicians, and then the cardiologist I have had in North Carolina, after I moved, not to mention my family, my husband first and foremost (who tied my shoes and put on my socks after I got out of the hospital and when I could not bend over; who clipped my nails because the neuropathy in my hands could not work the clippers), my sister who is my biggest cheerleader - they all made me who I am today, when it comes to my heart and overall health. 

I have noticed that I can walk further and faster than ever before and that my stamina has improved little by little every year. I can now walk even in cold temperatures (30s-40s) or hot ones (80s) and can go longer than before. I walked a mile and a quarter last night in low-50s temperatures (with wind in my face that made it feel like the 40s) without even breaking a sweat. I used to be out of breath walking from my car to my office in 40F temperature before my surgery. Inclines are still a challenge to this day. I live in NC now and boy, there is not one street that’s not on some sort of a hill. Day to day, the progress you notice might be imperceptible, but when you look back at all these years, and compare what you could do then and what you can now, it’s pretty amazing ... 

A month ago my INR was 3.1 (too high since my range stops at 2.5). This past weekend it was 1.7 (within range but a little too close to my bottom of 1.5 for comfort). But I have learned how to live with this ever-swinging pendulum. I have bought a CoaguChek machine that measures your INR and I check it myself as many times a week as I want to just to know how to adjust my food accordingly. I usually check it once a week if no changes in diet, exercise, or stress level have occurred. I take it with me when I travel because it’s harder to plan for your food when you’re not home. This brings me a lot of peace of mind.

I have never, not once, regretted my choice of valve. It took sleepless days of reading just about everything there was to read on the internet about all the possible valve options that I had and when I found the On-X mechanical valve and learned about the benefits and came to peace about living on Coumadin (if you ever do!), I never looked back. I love knowing that at least if everything goes according to plan and I avoid infections and clots, my valve will outlive me and I won’t have to have my chest cracked open again. At least not for the valve, anyway. 

I got all sorts of other “work” done with my OHS: I had a quadruple by-pass, as well as endarterectomy of all the four arteries that they bypassed, and a new, grafted, ascending aorta. I also had an aneurism repair and additional endarterectomy of my aortic arch ... I get a yearly echo for my heart and both the current cardiologist and the surgeon read it and so far it all checks out, thank goodness. My ejection fraction is lower than before the surgery, but at 55% is still pretty good, considering. I still have high blood pressure, but we try to keep it under control with medication and exercise. The coronary artery damage that I might still have in my heart is believed to be in my very small vessels (which cannot be properly diagnosed with an echo; an angiogram is required for that and I am not due for a new one for another 3 years or so, barring any new symptoms, like acute chest pain, dizziness, collapse, etc). As long as I keep active, the belief is that I’ll help my body create new blood vessels that would build a natural bypass for any vessel that might clog in the future ... So far, every year I have been able to exercise more and more ... I am not a marathon runner, far from it, but my stamina has improved so much!

I look back on every anniversary and thank God, life, and the universe for the inspiration they all had to keep me in the light that fateful (in a good way!) day of February 11, 2016. Every day on this day, it’s my “other” birthday and I am grateful when I add another one to my heart age. I think my sister once said: “you are almost 47 in human years, but your heart is only 6.” That’s right! My heart, the engine of my body, is barely even old enough to go to school! Oh, the possibilities! 

Maybe having my Heart Day during Heart Month (February) was not a coincidence after all. I have made peace with that. 

Happy Heart Month to all the heart warriors out there! Embrace your body, embrace your heart and keep fighting the good fight. It’s always a good one when you keep that engine running! 

Much health to everyone! 

If you’d like to see my journey in pictures from my heart surgery day to today, you can do so here: https://wanderworldpics.shutterfly.com/22602. 

Beware of Unknown Tests and Bogus Charges

The “real” story is long and convoluted, as all medical stories are. But I will distill it to a simple recount: they did one test (MRI); they charged me for three.

I have several MRI/ ultrasound/ CT-scan/ doppler appointments a year to check for various things in various areas of my body: I get a heart echocardiogram (or ultrasound) once a year; a carotid ultrasound, a leg doppler and a stress test (sometimes a nuclear stress test) every two years, and now, more recently, an abdominal MRI (sometimes, ultrasound) every year. They are keeping an eye on various areas in my body that show arterial stenosis due to the built-up of plaque.

This new (and soon to be former – see: http://livingwithfh.blogspot.com/2021/04/educating-doctors-visit-to-my-vascular.html) vascular specialist doctor ordered an abdominal MRI with and without contrast. The day of the test, I checked with the technician that was supposed to do my actual test what exactly the test was – like I said this was a convoluted story and there was some iffiness from the doctor’s office about what test was actually ordered from the radiology department, but I knew that the one thing I was there for, the one thing the doctor and I agreed upon to watch, was my abdominal aorta. So, I was expecting an abdominal aorta MRI.

The radiology technician confirmed he was about to do an abdominal MRI with and without contrast. I was good.

And then, the results came a couple of days later in MyChart: the radiology department had apparently done the following tests that same day:

·       CARDIAC MRI HEART MORPHOLOGY AND FUNCTION W/ WO CONTRAST

·       CARDIAC MRA CHEST WITH AND WITHOUT CONTRAST

·       CARDIAC MRI ABDOMEN ANGIOGRAM WITH AND WITHOUT CONTRAST

I opened each of these tests in MyChart to look for the results of each one. They had copied and pasted the exact same results from clearly an abdominal MRI test: there was no mention of the heart, no mention of the chest arteries, no mention of the heart morphology – there were just mentions of the abdominal aorta, the renal aorta, the mesenteric, and iliac aortas. Also, I noticed that one of the tests said “cardiac MRI angiogram” and what I had was not an angiogram … And each of the three tests had identical – letter for letter – results. Obviously, they were not the same test (why the different names?), but they had the same findings. I was just a little bit upset, you can say.

I panicked, because I know that when the doctor says they did a test and it shows up in your MyChart Test Results section, the next place you’ll see them will be in your bill. I have done these tests enough to know they are never lower than a couple of thousands of dollars (usually more) each.

I approached the mix-up with the doctor who ordered the tests, and he insisted that all three of them had been done. I asked him to show me the results of all of them. He could not – he had the same results three times over, as did I, on my end, in MyChart. He insisted that the tests were done (although I told him time and again that I verified with the radiology tech about what test I was receiving and he did not say I was getting three tests), and that the radiology doctor sent the wrong results. The doctor contacted the radiology doctor after I left his office.

I was almost in tears, because by then I had received the bill which showed I owed roughly $12,000 – about $4000 for each of the three tests. I am lucky to have insurance, but I had not met my deductible and even after that, I was still responsible for a chunk of the cost. It also felt unfair that even the insurance should have to pay for tests I did not get nor that were needed at that time.

A couple of hours after I left the doctor’s office the doctor himself called me to apologize: he talked to the radiology doctor and they had made a mistake: they in fact confirmed what I said all along, that they had performed one test and that was the abdominal MRI without the angiogram part of it and that the other two should be taken out of my account.

A couple of days later, the head of the billing department called me with an apology to assure me that I will only be responsible for one test, an abdominal MRI, and that the other two will be taken out of my bill. I told them the bill was already sent to the insurance who paid their share and they assured me they will refund the money to the insurance and that the insurance overpaid (how many times does this happen: hospitals reimbursing insurance companies?!).

In the end, the final bill was for around $4000 for just one of the three tests, but I wonder how many times this slips and people are overcharged for what they did not get done. Especially for chronic patients, like us, who have a number of tests done every year. I know people who are not savvy enough to read their results online and see what they describe; they just trust what the doctor says and move on. They would have received the bill, shaken their heads that the darn medical system is way over-bloated (which still is) and would have paid.

Some lessons this has taught (or reminded) me (of):

• Always pay attention to the tests they recommend and try to understand what they are for and whether they are needed.
• Question multiple tests – are they for the same thing? Can one test cover the scope of all the multiple tests?
• Ask the doctor to explain whether you need an MRI or would an ultrasound (typically cheaper, but not as accurate as an MRI) be good enough? For areas that are larger (think a whole organ versus one small artery), ultrasounds are usually enough.
• Always read your results and try to make sense of them even if it is just to ask your doctor additional questions about your treatment plan.
• Look at your bill twice: does each charge verify and match your real experience. If not, call the doctor’s office first; remember that the insurance only bills for what they receive from the medical institution. Start with the doctors/ clinic/ hospital to understand why they told you one thing and sent the insurance something else.

In short, as always: stay alert and be your own advocate. Always.

Educating Doctors – A Visit to My Vascular “Specialist”

I find that for the most part, doctors are more and more knowledgeable and “educated” about Familial Hypercholesterolemia (FH). They seem to understand how it works, what to prescribe, what tests to perform, why keeping the cholesterol numbers down is important and what to expect the aftermath to be if they can’t keep those numbers low enough.

I am also pleasantly surprised when a doctor understands that age has nothing to do with this disease. I have lots of respect for doctors who understand that in cases of more severe FH (like the homozygous kind) you must be as aggressive as possible with what the medical world has to offer (drug therapy and interventions) as early in the life of the patient as possible to get ahead of the disease and ensure the patient does have a life ahead of them.

I love and have huge respect for doctors who have the courage to act before a so-called symptom kills you. Cardiovascular disease is one of the main complications from FH and with this disease a symptom can often be fatal. I have had the luck to work with doctors who had the courage to act before a symptom was too late for me … I am here today because of such doctors.

But then there is this other crowd of doctors who are not covered in the “for the most part” crowd. Doctors who are so disconnected from the patient in front of them and so stuck in the letter-of-the-book (not sure what else; I want to give them the benefit of the doubt that they have read a book and not just simply call them ignorant) that they got taught in probably an old medical school course book from 1970’s when the research was done all on a 60+ year old male population, or something. Such is my current (till now) vascular specialist.

A while back, my cardiologist decided to refer me to one of his colleagues (who happens to be a former resident of his) that specializes in vascular health to manage my vascular disease, while he can focus on my heart health and my cholesterol management. I didn’t oppose him because I felt like I did need better management of my else-where vascular disease that does not necessarily live in the heart and he already has his hands full with my heart and cholesterol. I have seen this new specialist three times. So far, he is vague at best when speaking about my disease and how he plans to “manage” it.

On my latest appointment last week, we had spent about 45 minutes talking about the various stenoses that I have in the abdominal arteries:

• the main aortic trunk is more than 50% blocked (if I really do the math it is about 75% blocked based on what the normal lumen’s area is considered for me: the narrowest dimension of the aorta measures 0.6 x 0.4 cm when it’s supposed to be 1.2 x 1.2 cm); the transcript of the latest MRA notes “severe atherosclerosis of the abdominal aorta”;
• there is significant stenosis at the origin of the celiac trunk of more than 75%;
• there is mild proximal stenosis in the superior mesenteric of 50%;
• there is mild stenosis at the ostium of the right renal artery (~50%).

The entire time we’re seeing each other, as he also has said in previous appointments, he says “you have lots of disease.” He acknowledges this, which is somewhat reassuring. But then there is the part where I say “what do we about this?” And he comes back with “nothing. We watch it.” He confesses that "they (meaning specialists like him) are not trained to manage stenosis, really, but only aneurysms," and I don't have an aneurysm yet. I am a little taken aback and nervous because it seems to me like waiting for an aneurysm to happen might be too late. I want to be ahead of this. His uncertainty about how to deal with my (what seems to me) advanced stenoses is not very comforting. 

He explains that his biggest worry is not the celiac, mesenteric, or renal arteries, but it is the aorta. I am “happy” to hear this because my heart surgeon who also saw these results agrees that that’s very tight. Now, I had an endarterectomy of my aortic arch when I had my open-heart surgery, and the ascending aorta was so severely diseased that they replaced it with a Bentall graft. Based on my history, I ask him if we don’t need to be more aggressive about addressing at least the aorta if nothing else. He says no, because “there is not as much action through the aorta in your abdomen as it is in your heart”. So, we wait, because the disease in your heart aorta does not compare with the one in the abdominal aorta, so the threshold should not compare either. I am circumspect, but …  

He also offers that even with a bigger blockage he will be reluctant to have me see a vascular surgeon because I am “too young to have this kind of intervention.” He continues that “we really need to wait for more symptoms before we act. If you stop eating because of pain and you lose weight, then we can think about intervention.”

At this point, I sorta lose it. For full disclosure, I do not seek surgeries for the sake of surgeries. But in the past surgeries seemed to be the only option to save my life. And these findings scare me more than another bypass or graft. All I keep thinking about is my surgeon describing the amount of disease in my ascending aorta that he had never seen before. I was 40 when I had my heart surgery.  So, I remind him that age has absolutely nothing to do with the amount of disease in my arteries. I remind him that I have FH. That I have Homozygous FH and for the first half of my life my cholesterol was 600 mg/dl! I also remind him that my cholesterol now, although normal by healthy people’s standards – finally, just in the past few months!) is by far not “normal” for someone with a history of FH, heart attack and cardiovascular disease. So the disease in my arteries will likely continue. I also remind him that I am aging which will make the arteries even more stiff. So, shouldn’t we be a little more mindful about my particular situation and forget about what some book says about when such a surgery would be appropriate?

He back-tracks a little and he nods “yeah, that’s all true, but we also have to consider the risks of surgery. You realize that they would have to sever that aorta and by pass it with another blood vessel?” And I just chuckle: “You obviously don’t remember or don’t understand what I had done to my heart five years ago, if you think this scares me.” I also tell him that I do not want to “wait till I can’t eat anymore and start losing weight”. That seems more than irresponsible to me.

And then we talk about blood pressure. Usually, this is something I manage with my cardiologist and he has been trying to stabilize it for over a year with no success. But after doing some reading on my own and after talking with my surgeon, there are indications that stenosis particularly in the renal arteries can cause hard-to-manage blood pressure. So, I run this by him. He says, and this is an exact quote: “Well, your vascular disease has nothing to do with your blood pressure. You have just a regular, garden-variety blood pressure which is probably OK.”

I am almost speechless, but I find my voice, just barely to ask him to explain this “garden-variety blood pressure” of which I have not heard before. And he says “well, just a blood pressure that normally comes with age. You and I are the same age, so we normally get a bit of an elevated blood pressure as we age. That’s all. Easily managed with drugs.”

I remind him that mine is not easily managed with drugs. I also say, defeated, but wanted to give him one last “education” if I could, if not for me, for those people who will likely and unfortunately come after me: “So, we just got done talking about my history of severe FH, heart disease, massive surgery for 99% blockages in all main branches of the heart, and a trashed aorta; we got done talking about my abdominal stenoses and how my aortic trunk is so tight that, in your words, ‘scares you’, and you don’t think any of these findings have absolutely anything to do with my blood pressure? You think what I have is a ‘garden-variety’ blood pressure?!”

He pauses as he realizes the corner he painted himself into and says: “Well, no. That’s probably true. Those are some good points. All the stenoses probably have something to do with it, yes. But let’s leave it to the cardiologist for now.” Which is all fine and dandy, but ... is he considering these points in outlining his plan or is he not? He seems to me like he is not sure ... 

I was shaking so badly, I didn’t feel like I had the strength to argue anymore. We said our good-byes, I made the next appointment and he agreed to move my “watching tests” to 6 months rather than space them out to a year.

I am reconsidering ever going back, truly. I didn’t feel for one second that he actually saw me. I never felt like he really understood the risk of FH, the status of the vascular disease I have or whether he had a plan for it all. It would feel irresponsible of me to place my life in his hands. So, I probably won’t. Not anymore.

I wrote this blog mostly to share that you will need to be alert to ensure you are indeed getting the best care. I am probably a little bit too far on the end of educating my doctors rather than looking at them as the absolute authority for truth when it comes to this disease, but that is mostly because I have spent my life in this body and I have lived with this disease, I have seen it act in people in my family – most of the time watching doctors shrugging and not knowing where to find the answers. If you have just been diagnosed, you probably don’t have this perspective, so what I can tell you is: stay alert. Read, inform yourself, and yes, educate your doctor if you need to. It’s all part of your care.

I also wrote this blog for those doctors, medical professionals, medical journal writers who have told me in the past that “no, doctors don’t speak like this!” when I would quote doctors with some off-the-cuff statement that would render me more lost than when I first came to their offices. And I am telling you – yes, they do speak like this. To be in front of a clearly demonstrated cardiac and cardiovascular patient and to diagnose them with “garden-variety blood pressure” takes guts. And some doctors obviously go there …

What Being Cholesterol and FH Aware Means to Me

Those of you who follow this blog should already know my story (http://livingwithfh.blogspot.com/2011/03/my-story.html). Just in case you don’t, here’s a short summary: 

My parents knew that my dad’s side of the family had FH long before I was born. Dad had it, and his father had it, as well as most of his uncles and aunts on his father’s side. When I was 8, my pediatrician recommended that my parents would check my cholesterol level as well, because my liver was enlarged. My LDL was 525 mg/dl and my total cholesterol was 734 mg/dl. 

My mom fainted when she found these numbers, as she was the lab tech that actually read them for the first time. They didn’t have much hope that I would survive my teens. No one had ever seen such levels in a small child in 1983 Romania and with no medication or procedures on the market, they had little hope that I’d make it very long. Even in Communist Romania, with virtually no access to cholesterol-lowering drugs, they did know one important thing about FH: that it leads to premature heart disease or strokes at an early age. My grandfather had his first stroke at 48 and then, two years later, another massive one which left him bedridden. Even at my fragile age of 8, I had grown up to see my grandfather decay fast and I understood so much. 

My parents kept giving him as an example to me, of what will, for sure, happen to me, only much, much earlier in life because my cholesterol levels were so much higher than his. Even at that age, I learned what cholesterol meant (a white, fat substance that clings on to your blood vessels and organs and prevents them from working right), and what one can do about it: extremely severe diet (at the time, my parents would try anything so I was on a no-meat, no-fat, no-oil diet for about two years before they gave up on that because it was not really working for my numbers), and medication (in the beginning they gave me a white powder-drug called Cholestyramine which is a binder drug, but that didn’t help much either). I think I was 9 or 10 when I could tell you very eloquently what atherosclerosis,  dyslipidemia, and familial hypercholesterolemia meant. Google them, please! 

I went from doctor to doctor, from specialist to specialist till I was about 14 or 15. Everyone shrugged. No one knew what else to do. When I was in my late teens (19 and in college), I started to have heart symptoms: mainly tachycardia and arrhythmia so they put me on a beta blocker. They also did my first echo at that time and they saw modifications then, although I am not remembering exactly what kind. All they told me was “this is perfectly normal given your disease, but it’s serious.” 

I moved to the US when I was 23 and my first priority was to get on a treatment. Since then, I have tried every statin there is, and added much more to my regimen of drugs. Even with everything I have been throwing at this disease (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html), my LDL cholesterol has only recently (this month) been within normal ranges but  still not at my ideal target (below 70 mg/dl). 

Even with much access to medication and specialists in the United States, my heart problems have increased over the years: my aortic valve and my aorta replaced, my aortic arch repaired for an aneurysm and four by-pass grafts of the major arteries of my heart. I also had a heart attack following my surgery. All this before my 41st birthday. It was then when I had access to genetic testing and finally my “very severe case of FH” had its own name: Homozygous FH, which is the rarer and more severe form of FH. This meant that my mom also has the disease. Because her levels had always been in the upper 200’s and because she doesn’t have much knowledge about her family history she never suspected it when she compared her levels with dad’s and mine. In her late 60’s now, she has just been diagnosed with a slew of heart problems, among which a stenotic aortic valve and severe atherosclerosis of most major arteries of her heart. 

In addition to my heart problem, I have moderate to severe stenosis in my carotid arteries and at least one instance of severe stenosis in one branch of my abdominal aorta. For now, we are keeping a close watch on these areas, with yearly exams and visits to a vascular specialist. 

I would not be sitting here writing this for you all today if it were not for the fact that I have been aware early in my life about what cholesterol means and what FH can lead to. I have taken every treatment that seemed to match my body and my needs and have made choices in my life that allowed me to stay as healthy as I can. Sometimes those choices went against the popular expectations, like choosing to not have children because I simply didn’t want to pass this legacy on, and I didn’t know if I was going to be around for them. 

However, I have had a full life otherwise.  I am proud of my family and their support, and proud of my marriage, my travels and my work. I would have had a different story, or not been here to tell you a story at all if it were not for the fact that very early on, I knew what this means and how to manage it and I started doing that as soon as it became possible. By then, I knew exactly what specialists I needed to see and what questions to ask. Awareness is key, with FH. 

September is cholesterol awareness month and September 24th this year is FH awareness day. If you know a little bit about your family history and you know you have people in your family either with high cholesterol (especially when they lead an otherwise healthy life) or with heart disease or stroke, urge your doctor to check your cholesterol and know what the numbers are and what they mean (http://livingwithfh.blogspot.com/2016/07/my-current-cholesterol-numbers.html) . Do this especially if you have children or think you might have them one day. Knowing early will allow you to start treatment early and slow the progression of atherosclerosis. Your life is worth so much more! 

Stay healthy and stay aware. 

The “Good” and the Not so Good about Living with a High-risk Condition during the COVID19 Pandemic

Summary of a presentation I gave to Romanian medical students about how to handle the pandemic in the US, and how I personally handle it as a high-risk, heart disease patient

A couple of weeks ago, a friend of mine who teaches English at the Medical College in my hometown of Iasi, Romania asked me to have an online “class” with her first year students. These were students in the Dental and Pharmacy schools of the Medical College she teaches at. The topic was wide open, but I chose to speak about how I am faring through the COVID19 pandemic with a high-risk disease, like FH and heart disease.

Here are some notes on what I covered. I also speak a little about the questions they asked me at the end. Some of them surprised me with their compassion and insightfulness. After all, these are first-year students – so, the majority of them are no older than 19-20.

The COVID19 Pandemic and My Disease

You all know by now that one of the high-risk groups for COVID19 are heart patients. In addition to this, I also spoke in my presentation about how some of the early symptoms of COVID19 are also symptoms I manage daily because of my conditions (FH, heart disease, autoimmune disease).

To compare: some of the COVID19 symptoms and warnings are:

·       High-risk conditions:

o   Heart disease

o   Compromised immune system diseases

·       Symptoms/ complications:

o   Shortness of breath/ chest pain

o   Persistent cough

o   Strokes (30-40 year old patients)

Mirroring some of these, my relevant symptoms and possible complications:

·       Heart disease

·       High risk for strokes (because of FH and mechanical valve)

·       Moderate autoimmune disease (increased inflammation)

·       Persistent cough (side effect of blood pressure medication)

·       Shortness of breath

·       Chest pain (angina)

·       Increased vulnerability with (any) infection (because of mechanical aortic valve)

Some of the concerns I have here are: should I get infected, and should I have any of the early symptoms of the disease, how would I be able to tell the difference between some of my symptoms and the viral infection? Just something we have to think about every day.

Why the Quarantine is Hard for Me

As I mentioned before, I already have some symptoms that might make COVID19 hard to spot:

·       Consistent cough from medication.

·       Daily chest pressure and shortness of breath from angina.

I have had some appointments that have been canceled or might be canceled:

·       My yearly physical was canceled in March and the directive was “call us back in June”.

·       The dentist’s office was closed for 8 weeks (at the time of the presentation I didn’t know when it might open again). Any tooth trouble could be sign of an infection which should be addressed immediately for heart patients, as you know.

·       Canceling or postponing my yearly appointments for my cardiologist, vascular specialist, and all the yearly tests that keep an eye on the state of my heart and arteries. These are appointments that I must keep for my peace of mind, if nothing else. Advanced heart disease can worsen in months, so these are important check points for me to ensure another year (or so) of cardiac health.

·       One of the warnings for COVID19 is not to go to the ER or Urgent Care, unless they screen you first. But in the case of a heart attack or stroke, I would need to be in there immediately. Not sure how fast this will happen under the quarantine precautions of every medical office, and I hope I will never need to find out. Living with the fear of how it will work or what I will be exposed to once there is unsettling, for sure.

I have had some difficulty finding drugs and supplies:

·       I have a severe Vitamin D deficiency which is an added risk for cardiac health. I take a pretty high daily dose of vitamin D that must be vegan, because I can’t tolerate the animal vitamin D. I can’t find the vegan, high-dose Vitamin D in a store. Online, I have found that most everyone is now out of stock for Vitamin D. I have found one supplier that still had it. It took me twice as long as normal to receive it in the mail and it cost me three times what I normally pay for it.  

·       Everyone is completely out of strips for my INR machine which I use weekly. Some suppliers (like Amazon) still ship them but only to individuals who have an “Amazon medical account” (which I had never heard of before). I also found out that Roche, the manufacturer of the strips and of the machine only ships to medical offices, as well, and not to individuals. I am working with my INR clinic to be a registered “home tester” with Roche so they can send me the strips directly. This requires a bit of setup and lots of red tape – I have been trying to get this set up for three weeks and I am still not officially registered with the home tester providers, and I have still not received any strips.

Why the Quarantine Is Easy for Me

As weird as this might sound, there are some things that this quarantine requires that have come somewhat easy to me. Some of this is due to the fact that I live, every day, with some of the measures now required from everyone in place. 

For example:

·       As I mentioned before, I must avoid infection at all cost, because of my mechanical valve. For this, I have a very strict routine for disinfection and cleanliness to protect me from bacteria and viruses.

·       I already had most of the needed supplies:

o   Disinfectant, and antibacterial soap.

o   Masks and gloves.

So, when all these supplies were out of stores, I already had them in the house. I still have most of it, even two months and almost two weeks later.

In addition, I take PCSK9 and statin drugs (for cholesterol) and they are known to help with decreasing inflammation and boost the immune system.

Some personal reasons that definitely helped with the mental survival during the isolation are:

·       I have worked from home every day with remote offices in three different countries since 2017. So, it took virtually not adjusting to entire days spent on Zoom and being remote from my teams.

·       Another personal benefit is that I am Romanian, and we cook. A lot. So, trying to figure out a home-made meal three times a day has not been that hard.

How the Presentation Was Received

I was really impressed by how the students receive my presentation. Since I was crossing borders and I was addressing students from another country, you can imagine some of their curiosity was aroused by the foreign-ness of it all. Some of their questions were around how we do the pandemic here, versus what they were used to in Romania.

Some of them asked why I think the US has such a high number of cases. My personal opinion is that, outside the fact that we do have a large population, people just don’t listen. I am shocked when I go to the grocery store and people don’t respect the distance, they don’t wear masks and pretty much do everything they can to get others infected – like jumping behind the cash register when the cashier is supposed to be isolated, or sneezing and coughing without covering their mouth, or rubbing their nose, then touching boxes of products and then putting them back on the shelves. Just mindless carelessness.

Another question which I thought was very thoughtful and insightful for 19-20 year olds was how do I think people will be able to cope with this pandemic emotionally. Honestly, I struggle with the answer to this. Right now, I think we (my husband and I, my sister and her family and my parents and mother in law – all quarantined in two different states and three different countries) are doing OK, emotionally. For one reason or another, we can keep busy and stay connected with one another and things are pretty much settled into the new routine. But I do see people struggling all the time with the isolation, with the psychosis of not getting the disease, especially those of us who are at a higher risk of complications. Honestly, I don’t know how people will cope emotionally. What we can do, I think, is reach out and ask, and check and offer a sympathetic ear, and an open facebook/ facetime/ Skype/ Zoom/ phone line …

Unrelated to COVID19, one young gentleman asked a very specific question about the cost of the PCSK9 drugs: these drugs are, for the most part, nowhere near being mainstream in Romania. It was a surprise that a first year medical student knows about them at all. His question was very specific, too: “ I know these are very expensive. How can you afford them? Do you buy them out of your own pocket? Or is your insurance or pharmacy paying for them?” This gave me hope that maybe these drugs, which are life-savers for so many FH patients, might become more familiar in Romania, too, and that one huge hurdle that prevents so many people from getting them (cost) is already familiar to the medical world there.

I hope everyone is doing well, from a physical as well as an emotional perspective. Hang in there, protect yourselves, and others around you. And I hope we can all meet on the other side of this soon. Much health!

A New Member of my Health-care Team – a Vascular Specialist

You will learn fast that when it comes to FH you very often don't take just one medication. You take several. You don't follow up with just one test. You follow up with multiple tests (blood tests, imaging, etc). Very often, you don't see one specialist, but you see several, as a routine. This is a story about all that. 

Following up on my January appointment with my cardiologist (http://livingwithfh.blogspot.com/2020/01/the-en-garde-life-of-fh-patient.html), I got an MRI of my abdominal aorta in February. The list of findings was long, but the most important ones are the following:

1. There is a significant stenosis at the origin of the celiac trunk (>75%).
2. There is moderate proximal stenosis in the superior mesenteric (~50-75%)
3. The right renal artery has an acute take off. There is moderate stenosis (eccentric in shape) at the ostium of the right renal artery (~50%).

These are the most important concerning areas that this latest MRI revealed.

I will also have to add that for the first time in … I am not sure how long of a time, the MRI tech chased me down the hallway after the test was over and after the nurse took out my IV to ask me about my history. He, of course, did not say anything about the findings, as they never do, but he had a cheat-sheet with hand-written notes in his hand and he wanted to understand my history, my background, and why I had such an involved surgery four years ago (https://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html). I wondered when he did that what he found or whether what he found was unexpectedly worse than what he was hoping for… Maybe this is why no techs ever talk with you when you have these tests – because if they do, you start wondering and worrying. I wondered, but not worried. With my history, you take pretty much each day as it comes and try not to expect too much.

A couple of days later, I got an email from my cardiologist saying something to the effect that “it all looks good. Abdominal aorta unchanged from the ultrasound we did in December.” I pulled my December results and the findings then were quite different than what this MRI showed. Then, they noted the following:

Mild to moderate calcified plaque was identified in the proximal to distal segment of aorta. Elevated velocities were recorded in proximal to distal segment of aorta,
suggesting a greater than 50% stenosis by velocity criteria. The
bilateral common iliac arteries were visualized (…)  with mild to
moderate calcified plaque identified. Elevated velocities with biphasic
waveforms were recorded in proximal segment of common iliac arteries
bilaterally, suggesting a greater than 50% stenosis by velocity criteria.
(…) Elevated velocities were recorded in the proximal segment of celiac axis artery and superior mesenteric artery, suggesting a greater than 50% stenosis by velocity criteria. Decreased diameters in caliber were identified throughout.

As you can see, the findings were quite different. So, I questioned him. He insisted that the findings are “the same, but the tests are different, so we can’t compare apples-to-apples: one was an ultrasound, one was an MRI, so the measurements are different but the diagnoses is the same: I have disease, but there is nothing they want to do now because it’s not near 90%, so I need to just wait it out.” (pretty much an actual quote from the cardiologist).

I did not feel comfortable about his answer. I do like the work he is doing for my heart, but I felt like he was taking my aorta findings quite lightly. I pulled out my operative report and started to highlight all the references the surgeon had made to my “porcelainized aorta” and the “severe stenoses” he had found in multiple arteries. The aortic valve was so calcified only one leaflet (out of three) was functional. And I wanted to sit down with a vascular specialist to understand exactly what kind of a risk my arteries are facing. From my annual heart echo, I have a pretty good understanding of my heart, I think, but I feel like I need a “watch” plan for my arteries just the same, in addition to my heart.

When I had my surgeries, the three surgeons that were in the OR all told me the state of my arteries was one of the worst they had ever seen and my own surgeon repeatedly told me that I need to monitor them closely, not only in my heart but everywhere else in my body.

So, I pushed for more answers. I asked the cardiologist for a referral to a vascular specialist and apologized if he thought I didn’t trust him – I do, but I need someone more specialized in arteries the way he is in the heart. He didn’t take it the wrong way (I hope) and he made an appointment himself with one of the interventional cardiologists and vascular specialists in the same clinic. I met with this new doctor last week and it was quite a different conversation than I had with the cardiologist.

He does manage hearts, but his specialty is all “the other” arteries, outside of the coronary ones. He agrees that I should be equally concerned about my arteries as I am with my heart. He explained that the difference between the ultrasound and the MRI is that the tests are indeed different and the level of detail is higher on the MRI.

He would like to monitor me, and get on a yearly monitoring plan, probably a CT – Angiogram scan every year to all the arteries between my jaw and my knees. Although he agreed that the most accurate test would be an MRI (and with the least amount of radiation), he would need to order three MRIs to capture the length of the arteries he wants monitored, whereas with a CT-A, he can order one test. The MRI, although the most accurate, would triple the cost. 

The main area of concern is my abdominal aorta and especially my celiac trunk, but also the mesenteric and renal arteries, as well as the subclavian arteries which come up as stenotic in the carotid ultrasounds.

Now, I add one more test to my yearly plan: in addition to a heart echo, we will add a CT-Angiogram scan which will probably also replace the yearly carotid ultrasound that I normally do.

The vascular specialist will work together with the cardiologist to follow the development of my disease closely, both for my heart and for my other areas of my arteries. 

I’ll have to say, I feel better about having a plan. The cardiologist was maybe going to repeat the tests, but the thing with him is: he keeps doing a different test every time – an ultrasound, an MRI, a CT scan. I felt like we need some consistency, to build a baseline, and to see a clear progression from where we started, over time. This is what I did for the heart and this is how I knew when things were bad enough in the heart that I needed to have surgery. The vascular specialist agrees. He also “promised” me that I will probably have more surgeries in my lifetime to correct some of these areas as the disease in them looks pretty advanced for a person my age. So, watching this closely is that much more important, so we know when we need to act, hopefully before some major event should happen.

I guess, the moral of this story is like always: if you have doubts, if you need more answers, ask, explore, push and get them. There is no shame and no guilt in wanting to be in the know about your own body. It is your life, after all!

Happy health, you all! And I wish you all answered questions, and answered prayers!

Long Time, No Write …

How in the blue blazes has it come to be December already?? I cannot believe that my last blog was in July! We were eating peaches and getting our skin scorched in the sun and now, we’re watching the Christmas tree glittering in front of the fireplace. What a crazy idea, the passing of time?!

But passes it does, and it’s not asking us any for permission either …

And I don't even have a good reason for my long absence other than ... life. Life happens, you know, and lucky we are that and when it does! 

It’s been a busy year, personally, and for that I am grateful. It has also been a healthy year (or should I say, as I always do, “healthy for me”), and for that I am doubly grateful. We have travelled a little here and there, even went on a cruise in November. I only traveled for work one more time since I wrote here last. Travel is still one of the things I enjoy the most, and one of my more challenging things. I have the most trouble finding things to eat when I am away from home, because everything has to be vegan but not very green. The world still thinks that vegan means “a plate full of greens”, so it’s a bit challenging. I also have trouble sleeping and really getting comfortable. When I travel for work it’s always at very high altitude and I can hardly breathe and I tire even easier there. But I am so much in awe that I can still do it and live to tell the tale.

In October, my husband and I were lucky enough to be able to go to the FH Summit in Atlanta, GA. This was our first time although this has been happening every year for seven years now. The FH Foundation surely knows how to throw an educational event! We have learned so many new things, we have seen how much more people know about FH and how much more educated people (including doctors) are about this disease now. This Foundation has truly been doing a labor of love! We have learned about how doctors screen better nowadays for it, what therapies are out there in research or even approved but maybe not as well-known. I was the most pleasantly surprised at how incredibly approachable the doctors were at this conference, and how willing they were to learn from us, the patients, about what it means to live with this condition. If you or someone you know has FH, I would recommend attending, if you can, at least once. It will open your eyes and your hearts!

Besides the travel which is always draining for my heart especially, we have had a challenging year emotionally, too. My mother has still been battling lung cancer; my dad just had a stroke (more about this in a future post, I promise!), our last living kitty died while we were away on our cruise and my husband left a job and just started a new one which was a bit of a roller coaster, as well. Through and for it all, we are beyond grateful that we’re (almost) all still here and alive and managing our health the best we can. ‘Cause if you don’t have health how in the world can you do all the other commitments life throws at you?

Lately, I have had new symptoms of dizziness and light-headedness, low blood pressures and high, too. My INR nurse has noticed that around the holidays and when I travel I have a hard time keeping my INR in range (I still fight with this ONE number!). I mean, I have accepted that my cholesterol will always be high, but the INR cannot make up its mind! One day it’s 1.4, a week later it’s 2.9! Go figure! But this is what “normal” is for me now.

I wish I had more time for naps, as I feel drained almost always, but not really sure how to make time for that. The simple act of living for people like us means constant effort and awareness. I was telling my husband (as if he didn’t know quite well!) that there is not one thing that I put in my mouth that I don’t question: “oh, could it have butter? What about eggs? Could they have boiled this in chicken broth? Or since we’re in the South, could they have fried this in bacon?” I love eating at Oriental places because they never sneak in cheese into anything and they always steam their rice! At least that! There is no hour in the day that doesn’t pass without asking “oh, did I take my morning drugs? Is it time for Coumadin yet?” Or any week that I don’t think “is this Wednesday time for my bi-weekly Praluent shot?”

But we all, no matter who we are and what we’re up against, have a cross to bear one way or another.  We all have our own journeys. I am sure thousands of you have kids you worry about, parents you want to see living to an olden, ripe age. Jobs you hate and which stress you and yours out every hour of every day, houses you want to get out of or dying to get into … All I have this sick ol’ heart and my cholesterol. And this is OK, in the scheme of things, I guess. This … is manageable.

I promise to return much, much sooner with more updates about recent blood tests and heart and aortic tests, and also about what unfortunately happens as a complication of FH (will update you on my dad’s stroke). I promise I won’t be “on vacation” for months again.

Happy December to all till then! And never forget: make time for yourselves before you help others.