“Heart Sisters”

Heart disease is not something to muck about with ... Sometimes, you live with it for a very long time, and sometimes you have no idea you have it until it’s too late ... The silent killer as they say ... 

I knew I would have heart disease one day because of my FH diagnosis at the age of 8, and because my doctors were savvy enough to know the potential damage that the cholesterol was causing my arteries would eventually end me in the operating room. If I was lucky. 

Because I knew the risk, I could watch my heart over time and see the progress of the disease before it was too late to do anything about it, and luckily, before it caused an event like a heart attack or cardiac stop ... 

I met many heart patients on my journey with FH and heart disease. I have met so many special women, especially, that have gone through so much more than me. I feel connected through some invisible thread with all of them. We are all different and all alike in some ways. And in many ways, we get each other at a deeper level. 

The one thing that you’ll learn when you’re an open-heart surgery patient is that no two journeys are alike with this disease and with surgery. We all have different thresholds of pain, we all have slightly different surgeries, and we all have different anatomys. Two heart-valve surgeries are never the same: mechanical valve vs bio valve; aortic valve vs mitral valve; one valve surgery, vs multiple valve surgeries; valve replacement vs valve repair; bypass surgery vs endarterectomy; stent implantation vs bypass surgery; aneurysm repair vs aorta transplant ... 

Surgeries can last 2-3 hours or 10-13 hours. You can come in and out of anesthesia or you can feel like you took the longest nap of your life and not remember a thing. Some people see “a bright light” while they are on the heart pump machine, others see nothing. Just peaceful sleeping ... Some feel like they left their bodies and are looking down on their surgeons from the ceiling, other patients think this is all imagination and too many horror movies watched... 

How we heal, how fast and how well, and what life-long side effects we are left with (because there will always be those) are all different from one person to another, too ... 

We are taught, as heart patients, to never look at others’ progress in recovery and wish we were them. To just focus on us, to be in tune with our own bodies, to listen to them, and to focus on getting our own bodies better ... 

And yet, despite so many differences and nuances because we are all different people, there are so many similarities between heart patients ... The scare, the dread of being opened up, the pain, whatever its level, the up and downs in the ICU, the nightmares, the confusion, the low pulse followed by tachycardia, the high blood pressure followed by deadly low numbers, the lung damage, the dreaded spirometer challenge (Gosh, I hated that thing!!), the experimenting with pain killers (one minute you want to OD on them they are so good for pain, the next you want nothing to do with them ‘cause they make you lethargic or make you throw up) - I have experienced all these and so have many of my heart patient friends ... 

I have been lucky to be close to my friend Kate, that I met through the website of a heart valve surgery support group during my surgery and the subsequent recovery, 9 years ago. We are of similar ages, and have known for a long time about our heart disease. We have been pen pals (do people even know what that means anymore?) ever since. We exchange notes and horror stories about doctors, frustrations with our recoveries, and complications from our continuous disease, but also successes and small victories (that are monumental to us). 

Kate is also my hero and my role model because she has had open-heart surgery now three times ... She just got out of her third one, to replace both her aortic and mitral valves. Although I have coronary artery disease and valve disease and she has multiple valve disease (this is the difference), our journeys have been very, very similar in a lot of striking ways (this is where we are alike). 

She just left the hospital today, after 12 days for this third surgery and she sent me a picture of her leaving the hospital. My heart jumped when I saw it. Because this was almost exactly the picture my husband took of me when I was leaving my hospital after my surgery 9 years ago. The resemblance simply made my heart skip a beat ... And all those similarities between Kate’s and my journey came rushing back ... 

2 August 2025 - 19 February 2016

All I could think about is: WOW! This is THE picture! This is the picture one takes after they survive the worst - hugging the heart pillow and smiling, high on the notion that we are still walking the earth with foreign materials in our chests forever and God knows what else ahead of us. Smiling because we’re alive. Because we cannot believe we’re breathing and on “the other side”. 

I am sure everyone will see something different in these pictures. But what I see is all the similarities that bring us closer together as time goes by. That heart pillow alone and how we're clutching to it - it is your survival security blanket; it helps you breathe without pain and the only thing that stabilizes your (still) very "crunchy" chest that feels like puzzle pieces not yet glued together.

Kate, too, like me, knew she had bad valves quite early in her life, in her late teens. And she stuck with it, too, and she watched it and she did something about it before it did something more awful to her  ... 

I also see in this picture both of us surviving despite the many shitty prognoses; surviving despite the inability to tell yourself one more time “it’s gonna hurt; it’s gonna be hell, but I gotta do it to live to see another sunset and get another kiss from my cat (or dog), to live to jump on another plane one day to see my earthly paradise (we all have one), far, far away, to make love one more time; gotta do it!” ... 

We’re both still weak in these pictures, we’re in the passenger seats, we relinquished the reins, we are being cared for (how lucky is that alone?), and we are weak-looking, but there is fire behind those eyes and there is resilience and there is grit. 

I am sure she’ll have stories to tell after her third visit in there, going through the deep, deep darkness of literally being put to death and then brought back again. This is how I feel when I look back on my surgery: they literally stop your heart that has no blood going through it for a while, and then they magically make it beat again. The next thing you know, you walk out of the hospital and you go home with a new lease on life ... Sounds simple, and trust me: both for Kate and I, this journey from when you go in till you get out felt like at least 10 years ... But that smile says it all ... 

When it comes to life’s challenges, open-heart surgeries are quite up there in kick-butt-ness, in my book. Every surgery has a risk, sure. Every paper cut can give you a lethal blood infection. But there are few things in life for which you have to die (like open-heart surgery) to get better... And we did it willingly. Thank goodness we knew ahead of time that we needed it ... And thank goodness that for heart disease there are ways to manage it. 

I can’t think of anything more humbling than being sick and coming back to life. I can’t think of a more character-building experience than going through what Kate has gone through, not once but three times ... She is not 50 yet ... 

The title of this blog belongs to Kate, hence the quotes - this is what she said when she saw these pictures: “Heart sisters foreva”. I could not agree more, and I could not feel more honored and proud to have her say that ... We may be different people, we may live 2000 miles apart, but we have this invisible bond that connects us across miles and time zones, and although every muscle hurts different for her and I, our hearts still beat to keep us alive, despite the different kinds of ordeals that they have endured, despite the different number of times they went silent. Our hearts are ultimately stitched up and broken just the same. And that will connect our lives and our stories forever ... 

Speedy recovery, my favorite warrior and welcome home! And I’ll leave you with the advice my own surgeon gave me 9 years ago, the day after my surgery: “It’s up and at ‘em from here out!” I have no doubt about it! 

The Heart Month

US president Johnson announced that February was Heart Health Awareness month in 1964 “to give heed to the nationwide problem of the heart and blood-vessel diseases, and to support the programs required to bring about its solution.” To this day, we still fight against the number one killer of all people - heart and vascular disease. 

February is a peculiar month for me. In addition to celebrating Heart Month along with everyone else, I multiple-times celebrate it as a heart-disease survivor and a member of a heart-diseased family. 

Every February 11 I celebrate the anniversary of my open-heart surgery which took place in 2016 (https://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html). This year, I even got to travel back to Utah where my surgery was done and meet again with my surgeon. It was such a gift! 

After 9 years, and with many ups and downs, my heart has continued to change (you have probably heard the adage “once a heart patient, always a heart patient”). But despite the changes, I continue to live a full life (which is adjusted for me), and I am counting my blessings. 

February 20th is the anniversary of this very blog. I started this writing adventure on this day in 2011 out of a complete desert of news and information about Familial Hypercholesterolemia. There was nothing out there to give me a map on how to navigate this condition and how to live with it successfully - because I was determined I was going to live with it at any cost The only semblance of a map was what I had learned from my family and the prospects were not very happy - everyone in my family with FH had had hard lives, marred by lonely sick years, disabling symptoms, many complications, long, lingering suffering, and early deaths. I was determined there has to be another way. 

I wanted to document every step in my journey - all the tests, all the ups and downs of the treatments, all the successful and failed attempts at any medication so that others who I knew were out there could have a “real-life experience” reference to know what to expect through their own journeys. I figured that if I could reach at least one person like me, who was looking for their “tribe”, my job and the job of this blog is done. I have not put any advertising and any thought into making this blog more than what it has been from day one, but the handful of people that I know I have reached with my stories are such a gift to me! I thank you all for reading, and for your feedback over the years! I know (you have told me) you find a place of belonging here, and for that I am grateful. 

A year after I launched this blog, The Family Heart Foundation (then, The FH Foundation) (https://familyheart.org/) was founded and that truly threw our visibility as FH individuals into the stratosphere and amplified our voices infinitely. 

February 28th is also Rare Disease Awareness Day. I have known I have “a rare form of FH” since the age of 8 when I was first diagnosed. Officially diagnosed with HoFH at the age of 40 after my open-heart surgery, I am now officially a “rare disease individual”. 

Along with finding out that I am now in this cool crowd of “rare disease people”, finding out that I have a rare disease also helped my family: certifying that I had HoFH meant that both of my parents have FH. Until then, because we never knew about mom’s family history and because her levels were considered borderline (because they were always much lower than mine and lower than my father’s), no one ever suspected her of FH. She was 62 at the time we found out my HoFH diagnosis and she had advanced cardiovascular disease already.

This year, I also celebrate the fact that mom survived her first (that we know of) heart attack a month ago almost to today. She went into the ER with difficulty breathing. Being a lung cancer patient, everyone suspected it was her lungs, but it was in fact a blockage in her aorta that was not sending enough oxygen to her body. She needed a stent right away because the blockage was significant but given her many co-morbidities and her weakened health they advised against it. They kept her in the hospital for a week and treated her blockage with medication, then sent her back home with 6 new drugs among which a statin. She is 71. To me, she is still very young. 

FH is not a joke. It is a silent killer and left untreated and ignored it won’t ignore you, I can promise you that. There will be a time when it will speak. And it will be loud, and often not pretty. 

Below is a picture of me and mom about 6 days or so after my surgery. She came to babysit me while I convalesced but in an odd twist of fate I had to babysit her, as she got sick with the first symptoms that eventually lead to diagnosing her lung cancer. 

This year, as she had her own heart issues, I wished to God that I could have been there for her (she lives in Romania), but technology is all we had to keep us connected. 

I am grateful for the genes she gave me. Good and bad. Bad, to force me to learn how to be a fighter and not take one day for granted, and good to be stubborn and strong enough to fight that fight and overcome. 

She and I are both moving forward - scarred but more aware. Weak but still fighting. 

Everyone should celebrate heart month because, as my sister reminded me the other week, “we all die of heart disease.” It is the only organ that decides when they call it. 

Stay healthy, friends and heart warriors, and always know your numbers (cholesterol, blood pressure, pulse, and oxygen) and stay informed. I am living proof that advancements in medicine are working miracles and not only improving but also prolonging healthy lives nowadays and allowing us to build up new memories. Let’s hope this continues! 

Happy Heart Month to all! 

Cath #4

Here we are again... A new cardiologist, a new medical system, another cath. This one was as much a test for the new team to familiarize themselves with my long-standing coronary artery disease, as much as my need to understand whether the new symptoms that I have been feeling and some of the bad blood test results that I have been receiving were because of new blockages in my coronary arteries. 

Since my first cath, when I was 30 (hard to believe that was almost 20 years ago), all the cardiologists I have seen have told me that my coronary artery disease (CAD) is so advanced and my cholesterol is so far from my target that the only reliable way to know for sure whether my arteries will need intervention, before a major event would happen, would be through a cath. They almost all recommended that I would have a cath every 5 years. 

The previous cardiologist that I had until earlier this year (for 6 years) was too nervous to do a cath at the 5 year mark because he said with the amount of artery disease that he was sure  I had it would not be prudent to have a cath, to not “dislodge something” (his words). 

But my cardiac symptoms have continued to change and not for the better. 

For a couple of years now, my shortness of breath has worsened. Even when I do light chores around the house, like carrying a laundry basket the 20 or so feet from my bedroom to the laundry room, I am breathless. My BNP (which, in layman’s terms, is a measure of the degree of heart failure your heart is in) has gone up to 700 pg/ml (normally, the target is under 100 pg/ml), but it has been fluctuating, too - anywhere from 200 to 700 pg/ml. 

My chest pressure is more intense when I exercise - not necessarily only in my chest, but my left arm, shoulder, shoulder blade and the left side of my neck all go numb and they feel like someone is stopping circulation to them or strangling me. It’s a scary feeling and it happens virtually every time I go for a brisk walk, climb even a small hill or walk in the heat. 

My blood pressure has worsened but I am controlling it better now with a drug that (finally!) works, after months of trial and error with many new and old drugs. 

Typically, even when they don’t recommend a cath every so often to determine CAD, they do recommend a cath when your symptoms or your heart blood tests are worsening. But even with all these changes, the previous cardiologist refused to do another cath. 

The new cardiologist agreed that it would probably be beneficial to do one. He skipped the stress test with contrast because he said we already know I have CAD. And whether the stress test comes back perfect, inconclusive, or modified, he would still follow it up with a cath. Somehow, through some kind of magic, he got the cath approved with the insurance (they typically want a less invasive and cheaper test before they approve the cath). 

After the procedure, right leg still flat, but ready for the IV to come out.

So, off to the cath lab we go. Everything went according to plan, and it was great to report that there is not much change in the status of my arteries since the last time they did the cath with my previous team, 6 years ago. 

The main findings were:

• the LAD (Left Anterior Descending, aka “the widow maker”) is still at 50% blocked, but that is open enough to not require any intervention at this time. All the segments of this main branch are listed with “no significant stenosis”. Since this artery irrigates pretty much the entire left side of your heart (which is “the pump”, responsible for sending blood to your entire body), this artery is the workhorse of the heart. So, it’s good news that, although it is diseased, it provides accurate flow to the heart. He made no special mention about the bypass graft for the LAD which was put in place 8 years ago when I had my open-heart surgery. The cath I had 6 years ago does not speak about the LAD graft/ bypass either, although it mentioned other grafts (I had four arteries bypassed) as either being open or completely occluded. 

• the Left Circumflex artery shows “no significant” stenosis in all its segments. 
• one of the Obtuse Marginal arteries (which irrigates the upper part of the heart and is one of the branches of the Left Circumflex artery is between 80-90% blocked. The bypass put in place when I had my surgery is completely (100%) closed. 
• there are two other arteries (the Left Posterior Descending and the Right Coronary) that are described as “small”, but without any mention of any disease. 

Their overall assessment is that, indeed, there is “existing advanced coronary artery disease” (the doctor even called it “aggressive”), but that at this time nothing requires an intervention and the disease seems to be stable from 6 years ago. 

This new interventional cardiologist (my regular cardiologist is not an interventional one, so he does not perform caths or any other procedures) was the first doctor that did not advise to keep getting caths every 5 years. He said to only get one when symptoms change. Which ... is relative, in my book. Like I said above - the symptoms have changed. However, the cath did not seem to reveal anything new. So, while I am thrilled that there is no new or more severe disease, the questions of the causes for the new shortness of breath, increased chest pressure, higher blood pressure and a higher level of BNP still remain. 

The cath lab doctors did recommend increasing one of my blood pressure medicines (Amlodipine) from 5 mg/ day to 7.5 mg/day, but I will wait to also chat with my regular cardiologist before I do that, as he prescribed a diuretic instead, for the same symptoms. So, I need to review with him my meds, my symptoms and my cath findings as he is more familiar with my larger heart condition than a doctor who saw me for 25 minutes in the cath lab. Making this decision to wait before making changes to my meds is something I have done often. I want the doctor with the most knowledge about me, about my personal case and history, about my extensive file of heart disease and hypercholesterolemia to be in charge of all the changes in my meds after we sit down and together explore the best approach and outcome. 

A word about the procedure itself: 

Everyone will tell you that a cath is not a walk in the park. It’s inconvenient, invasive, somewhat traumatic if you are intimidated by doctors and hospitals and needles; it’s kinda scary because it does come with risks, like any procedure and with side effects depending on the drugs they give you, or the treatment they might perform while you’re in the lab, or depending on how well the healing is going after you go home. But having done four caths, all at different labs and by different doctors, I am not scared of them anymore. 

You come to the hospital early (before daybreak, usually), you get into hospital clothes (all street clothes must go), you get your own bay in a long ward, complete with your own machines, a tv and a call button for the nurse. You get an IV put in, a couple of nurses and doctors come and take vitals from you and ask you about 10,000 questions. 

And then, they do some blood tests. Usually a complete metabolic panel (CMP) to look especially for infections or kidney problems, and I get an INR which shows whether your blood clotting number is normal. Four days before the cath I had to stop my blood thinner medication (Warfarin) so that the femoral artery that they access the heart through can heal after the procedure. They look for kidney problems because they use dye. You must have healthy kidneys so your body can flush it out after the test is done. Sometimes they give you saline through the IV after the test to speed out the elimination of the dye. But they didn’t do this this time. They just told me to drink a lot of water after I leave. 

After the tests and the interrogation are over, they wheel you in the cath lab which looks like an operating room that you see in the movies. They put you on this table and there is a huge TV next to you, where they will see your arteries. They usually give you sedation to relax you and so you won’t care about what is going on. But every time I have gotten a cath I have insisted on not getting sedation, because I want to be aware of what is going on and be able to speak with the doctors about the findings as they see them. This time was no different: I got no sedation and the nurse said he did not hear of anything like it. “What do you mean no sedation?” The recovery time is shorter if you get no sedation. 

They give you some local anesthesia at the top of your leg, in the groin area, and then they make a tiny slit in your femoral artery through which they insert a sheath through which they will insert catheters of various calibers that will go all the way to the arteries of your heart. You have a small X-ray-looking machine above your chest that I am assuming shows pictures of the catheter going through the arteries on the big TV screen. Once there, they inject dye and they look on the screen to follow the flow of the blood through the arteries. 

I had two doctors this time - one was the attending (he mostly supervised and shouted out the findings) and the other one was a fellow who actually performed the procedure. The attending spoke with me the whole time. 

Although you do get a local anesthesia, you do feel some sort of pressure while they maneuver the catheter inside the femoral artery. This cath was hands-down the most painful of all the ones I have gotten. But I still didn’t ask for any other tranquilizers or anesthesia. It was over in 30 minutes. 

After the procedure, you have to rest flat on your back for an hour, after a specialized nurse removes the sheath from your artery and applies massive pressure to stop the bleeding. After an hour of lying flat, they let you lift your head and recline, but you still cannot bend your leg for an hour. After that, they want you to walk around the long ward. After another hour of paperwork and advice on what to do home, you leave. I was at the hospital at 6.30 AM. We made it back home around 2 PM the same day. This was the first time when I did not leave the hospital in a wheel chair, but they told me that’s because I didn’t have any sedation, so the risk of being dizzy or unstable was very small. However, I do have artery disease, something could have been dislodged or cause a blockage or clot in the wrong vessel - but they didn’t seem too concerned with this. 

In the end, I am glad we did the cath. It put my mind at ease somewhat that no new blockages are formed and the heart still looks like it’s working normally and doing its job.This has been a life-long, constant, 24-7 job - to keep my heart clean and running. Just like the heart never rests, I never rest in babying it as best as I can. 

Between trying to keep my cholesterol as low as possible with whatever I can, following up with all the appointments, having a huge open-heart surgery at 40, where I had massive work done to clean out my sick arteries, replace my valve, replace my aorta, keeping my weight under control, learning as much as I can about FH and heart disease, learning to listen to all the symptoms, always fighting for the right care for me, switching doctors, traveling when needed, learning how to eat a clean diet, even when the whole world tells you that a diet won’t make a difference - all of these and more contribute to being here and living a full life, for me, at 49. This is so many more years beyond what my original prediction was when, at 8 years old when they diagnosed me with FH, the life expectancy that I was given was somewhere between 10 to 15 more years, at most. 

I am grateful for it all and most importantly for the thousands of people who constantly look for a cure. For the thousands of gutsy people who have FH and who participated in many clinical trials so we can get better drugs. And for some brilliant people that have been on my side. I have always felt like an experiment with this disease. And I am beyond the moon that the experiment has been successful thus far. I am also grateful that all my 4 caths have been planned procedures. I would hate to be wheeled into the cath lab in an emergency while having a heart attack. Things would look a lot different then, I am sure. And a lot of people out there find out what a cath is exactly like that. 

The Wins and Up-hill Journeys of 2023

Boy, it’s been an interesting year, to say the least. But then again: aren't they all?!

At the end of 2022, I lost my dad to several massive strokes (I spoke about this in my first blog this year - https://livingwithfh.blogspot.com/2023/01/new-year-new-thoughts.html) . Always the overachiever, he could not get just one stroke when his time came. Instead, he stroked in every area of his brain - small brain, large brain, and the brainstem, for safe measure ... All because of atherosclerosis due to untreated FH. 

This past year’s been largely spent in the shadow of this very, very dark and sad event that I started the year with. Dad was my beacon, the guiding force in my life ever since I can remember. Without him, I have felt like someone just turned the lights off all around me and I have been feeling around, trying to figure out how to live in complete darkness. 

It’s been a year of emotional un-health for all of us left behind, but mostly for my mom, for me and my sister. Our center, our patriarch is gone and the balance is off for all of us. The common belief in my family is that I am the strongest one. So, I have tried hard to be here for my sister and my mom. But even the stronger ones need strength - and I have surely felt this this year and have struggled to find it ... Trying to stay mentally afloat has been hard. Working and focusing on my physical health have been good distractors. 

My physical health has not been the best, but I’ll have to say it has not been the worst in my life, either. I am grateful for this. At the end of the day, truly, I am just simply grateful for another day. A friend of mine living with lupus has said to me something like this year “doesn’t every day feel like a victory?” - and she is so right. 

I am happy that I made it into the last days of 2023 - here I am adding another year to my life bouquet. Remember, I was told I was not going to make it past 18, or 25 at the most. Here I am stepping into my 49th year next year - God willing! This is definitely the best accomplishment of this year and of my life, really. The fact that I have stayed away from the ER and the ICU this year are the cherries on top! 

As I am looking back and drawing the line to close out this past year (something my dad always did), I think of all the good things, but also of the let-downs and disappointments about my health this year. Worth mentioning:

• We still have not figured out if my GI issues are caused by abdominal stenosis or not. We are still investigating this with both specialists (vascular surgeons as well as a GI doctor). More tests to come. I can only hope that it is not my stenosis because a whole year is a long lease if my gut is not getting proper blood-flow. 
• We have not figured out my dizzy spells. After years of my cardiologist refusing to do a scan of my brain blood vessels, I have finally gotten a vascular PA (that is right, not a surgeon, but a vascular PA working for a vascular surgeon) to admit that a head CT is past due for my advanced atherosclerosis, family history of strokes, and for my symptoms of dizziness that no other tests could elucidate. So, the test is coming up but it has not been done yet. I hope we find out something ...
• We have made almost no progress in improving my shortness of breath, despite achieving some good heart numbers this year ... (more about this below). I still get very winded when I do very light chores around the house. Climbing a hill or walking is much worse. 
• My cholesterol values started climbing, despite continuing the same combination of drugs that has been beneficial in the recent past. No idea why the numbers are up, but I wonder if stress has something to do with it ... The LDL cholesterol is 144 mg/dl (up from 101 mg/dl last December) and my total cholesterol is 210 mg/dl (up from 164 mg/dl last December). 
• Despite this trend, I am still waiting for my clinic to approve my Evkeeza treatment. Apparently, the insurance approved it but the bureaucracy of the clinic itself of trying to figure out how to manage a new drug as an infusion is crazy complicated. So, we are still waiting. We're also waiting for my liver to get better.
• To that point: my liver enzymes are also climbing. They have gotten as far as more than three times the upper limit (the AST and ALT values). They tell me they need to be much, much higher than these numbers for liver failure, but they are still concerned and trying to figure out what’s happening.
  
  We have been trying for months to stabilize them and they remain high as long as I am on a statin drug. I am planning to come back with a more detailed post about this, but I am currently “playing with drugs”, as I call it - trying to turn meds on and off and see what will keep the liver levels down and get the most benefit for lowering the cholesterol too. So, until I have the full picture and until we pin down what’s causing this and what drugs I will be on going forward, I will keep the confusion to myself for now.
  
  Right now, they have stopped the one statin that works best for me (Atorvastatin) and they are asking me to take a lower strength of a less potent statin (Pravastatin) to see what the liver is doing next. When I take no statin at all, the enzymes are normal.
  
  We have not checked the cholesterol levels during this trial-and-error period, but logic and experience tells me the levels are high, even higher than the ones listed above. The levels I wrote above were trending higher when I was on the full-strength of all four medications I normally took for cholesterol (Lipitor, Zetia, Praluent, and Nexletol).  Of course, the longer I go with higher cholesterol levels the worse it is for my vascular and heart health. But I’d rather not add liver damage to my laundry list of problems, so I am willing to try this new experiment to revamp the drug cocktail I am on.
  
  I wondered if stress has something to do with the liver functions too, till I saw the liver enzymes come to the middle of the normal range only by stopping the Atorvastatin for 10 days! 

Despite all these setbacks I have had some brighter spots this year too: 

• I finally made some progress on Evkeeza with my cardiologist. The jury is, of course, still out on this newer than new drug, but I am hopeful it will do something (good) to my numbers!
• Earlier this year I barely got the doctor to pursue setting me up with this (https://livingwithfh.blogspot.com/2023/08/access-to-newer-treatment-might-not-be.html), and now I am waiting for my liver numbers to stabilize before we introduce the new drug, but the clinic is finally ready to start. 
• I have managed for another year to keep my carotid stenosis at a lower than 50% amount of plaque build-up. After having had it as high as 65% in the past, having the readings come in as 50% or lower than 50% in some areas is an accomplishment. (The theory here is that Praluent has helped with stabilizing and stopping the progress of the plaque - just a theory from doctors). 
• I had a xanthoma successfully removed from under my eye (https://livingwithfh.blogspot.com/2023/12/xanthoma-removal-surgery.html) . This is the fourth fat deposit (most likely caused by high cholesterol) that I have had to remove in 48 years. 
• I have finally (after two years of trying different treatments) pinned down the right drug and the right dosage for my blood pressure and I have improved my BNP number (which shows the severity of your heart failure).
  
  What is even better than seeing lower numbers of the BNP (still not normal but lower), is that I feel better, especially when I walk. In normal weather, my angina and the cramps in my extremities are much less than they were before I started Amlodipine. My BNP has come down from 692 pg/mL (end of last year) to 323 pg/mL this past September (the normal values should be under 190 pg/mL). In weather that is too hot (above 80 F or too cold (low 40’s or lower), the angina and cramps are still pretty bad ... And the shortness of breath has not improved. The blood pressure numbers are more often “normal” with only weekly spikes (as opposed to daily). I take the little bit of good news where I can. 

All is not perfect. But all is not bad ... One foot in front of the other and I hope I can live another year to tell that year’s tale going forward. 

From all the good that’s in my life, I am most grateful that despite all the (un)health bumps,  I enjoy a full life, with good people, good food, and lots of travel and freedom to move about. Yes, being tied to a pill box, a blood pressure taker, a bi-weekly injection pen, an INR meter is not a completely care-free life, but I am never taking the freedom to move my body and drive and jump on a plane for granted. They are precious gifts! 

Going to New Orleans for Thanksgiving was one of the highlights of this year - here is me, in the French Quarter on Thanksgiving night: 

Other adventures included visiting three very different national parks for the first time (Saguaro National Park in Arizona, Indiana Dunes in Indiana and Jean Lafitte in Louisiana), two Frank Lloyd Wright homes (Taliesin West in Arizona and the Westcott House in Ohio), going to Europe and celebrating mom’s 70th birthday, spending a week with my nephews in the summer and taking them to their first baseball game and their first musical (Wicked), among other fun things. So looking forward to more adventures!  

I wish each and every one of you reading here much health and strength. They are so important to keep us going. Whatever makes you happy or makes you whole - hold on to that and you’ll stay afloat! 

Happy New Year! 

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.