9 Years

When I went into my open-heart surgery, 9 years ago today, I never thought I would live to see 9 more years. My heart was in bad shape. The surgery ended up being even more involved than the plan outlined it to begin with. But more than that, I never thought I would live a full life after the good surgeon took apart my heart and stitched it back together. 

I knew I had more life in me, and I knew this surgery was not going to be the end of it. But I never imagined that 9 years later I would look back and say that I have lived a full life, either. 

But here I am. A life beyond any expectations.

A full job, all the trips I wanted to take. Buried one beloved pet. Buried one parent. Taking care of the second one from across the world. Watched my nephews turn into teenagers and young adults. Moved across the country. Zoomed over the ocean a few times, zoomed across the country for work, sometimes on my own. Medical bracelet on my right wrist, and tons of notes in my wallet that tell the world that I have a heart and a clotting condition (because of my mechanical aortic valve and because I must take Warfarin to prevent my blood from clotting and blocking the valve). Praying that whoever needs to know this information will find it. And off I go. Always cautious, always with all the risks in the back of my mind, always prepared for what would happen if I can no longer speak for myself, but I am not about to lock myself in a bubble, either. Time is always of the essence and time is short, for all of us. 

As I said 9 years ago, I say this again today: once you’re a heart patient, you are always a heart patient, but you learn to live with your limitations because there is no other way. Your limitations become your new boundaries, but you learn how to fit your dreams within those boundaries and you move on. 

There have been scares over the years. In the first couple of years, I went to urgent care when I cut my fingers in the kitchen and learned how to deal with the bleeding. I went to ER every time my blood pressure was off and I was dizzy, light-headed, or my pulse would race.

When I travel, my INR (coagulation marker) tends to go very high. I am supposed to keep it at 2.5 (for the upper limit), but it sometimes wanders off to 3.8. I am terrified I would bleed internally so I limit my physical activity for fear of bumping my head or something and I eat more greens to try to lower my INR.

I fell several times really hard. One time, I got a large open wound (as big as one whole thigh) in the muck of a dirty beach. I thought for sure I'd get some flesh-eating bacteria in my blood and the valve will have to be replaced, but it didn't happen (I drowned my wounds in hand sanitizer and alcohol which I keep in my car).

Covid just about paralyzed me with fear! With all the statistics of it damaging your heart, your lungs, and reputation of being harder on cardiac patients, I thought if I get it, it would surely kill me or at the very least land me in the ICU. But I have had it twice now (not for lack of protection as I am one of the most careful people I know - I still wear a mask in crowded places), and I have been very lucky to come out on the other end unharmed.

My valve is fine. But my heart is weakening because of my underlying cholesterol issues which continue to damage my coronary arteries. My heart muscle has thickened and I now have what they call “heart failure”. I am one of the lucky ones, though, because my ejection fraction (my "pump") is still high and strong - this is the more rare heart failure.

I learned to keep an eye on the “heart failure indicator” (called BNP, which is measured by a simple blood test) and with the proper medicine and the proper cardiologist I keep that number under control, as much as I can.

I still have neuropathy on my left hand, but there is nothing that can be done about that. I type fine with both of my hands, and sometimes I am spotty on the letters meant to be typed with my left hand, but I know to keep an eye on those and go back and fix them right away. It’s second nature now. I have some shortness of breath and the chest pains come and go, especially with exercise, extreme heat, or extreme cold. 

My blood pressure wanders upward sometimes, but most days it’s normal (staying on top of the meds is key). When I go to some place with an altitude higher than 2000 ft, my pulse races, but when I am at sea level, it’s a lazy 55-60 beats per minute. I have some dizzy spells, tinnitus, and something which I call “pixelated vision” at times (all these started happening right after my surgery and they are still here), where the image in front of me breaks up in what I can only describe as pixels. But again, all these have become old friends and we have learned to coexist. 

As always, today, I read some excerpts from my surgery report and some of my husband’s emails to my relatives and friends while I was in surgery for 12+ hours and hanging between life and death. 

More than anything, today I feel humbled. I feel lucky beyond bounds and humbled. I am not sure why God wanted me to live through this surgery and much, much more beyond. But I am looking forward every day to find out.  

Much health to everyone and as a heart warrior friend once said: “keep ticking!”

The day after my surgery, and today. 9 years is a long time.

 

 

Here We Go (Higher) Again!

Happy New Year, everyone! 

I wish and sincerely hope that you are all having a good year so far and that 2025 will bring you everything you set out to achieve - healthwise, and otherwise. 

To be honest, they always tell us that “less is more”, don’t they? I have always struggled with this saying and I have always believed it’s such a pile of nonsense. But for the case I am about to talk about here, less is indeed more. Unfortunately. Less drug means more cholesterol. 

My journey with Evkeeza has been great so far, at least from a standpoint of what my cholesterol levels have been - levels so close to my target of 70 mg/dl and even lower. In November, my LDL level was 67 mg/dl which is lower than my target. This is stuff which dreams and fantasies have been made of, for me. I never in 42 years did I dream that I would come anywhere close to my target (my “natural” LDL would be somewhere around 475 mg/dl). But there we are. Or rather, there we were in November. 

In December, at my last cardiology appointment, the doctor decided to reduce some of the other medication I have been on for years (https://livingwithfh.blogspot.com/2024/12/the-importance-of-advocacy.html) on the account that Evkeeza does more than it’s been expected to do. So, since November, I have stopped taking Ezetimibe and I have cut the Atorvastatin in half (40 mg, instead of the 80mg that I have taken for 20+ years). 

But at my January Evkeeza appointment, the numbers didn’t look so good anymore, as you can tell below. The LDL more than doubled (since November), to 142 mg/dl. 

Click on the picture for a higher view

(insert your losing move jingle here)

I typically get my infusions on Fridays, because I am usually tired the following day and I want to have the weekend to get some rest afterwards. So, Friday evening when the numbers were posted on MyChart, was followed by two non-working days where I could not reach my doctor. 

I made the decision on my own to double my Atorvastatin back up that Friday night. And I did. First thing on Monday, I emailed my cardiologist and told him that given the latest numbers, I would like to go back to my “usual” 80 mg of statin. He replied that he too was very disappointed to see the numbers creep up and that his advice is too, to double the Lipitor back up. 

So, here we g(r)o(w) again back to “the maximum tolerated (and allowed) statin dosage. 

It’s a guessing and a trial-and-error kinda game - managing this disease. I wish I can tell you that after 42 years of managing HoFH I got a handle on it and I have it under control, but I don’t. Dosage adjustments, trying new medication, trying combining new and old meds all the time, getting tested and then going back to square one, or making 2 steps in the right direction and one step back - it is all part of the lifestyle. 

The target is always: keep an eye on the numbers and adjust what you can regularly. That is all. I have not gotten discouraged because I am used to meds not working for me. My goal is to try the most of what I can try and tolerate and I have access to and just keep at it. The rest is up to whatever my body decides to do. I do my part. The rest is left to science and luck. 

For most of my life, I was not anywhere near “the target”. So, not hitting that is not proof of failure to me. Even with a small dip in the numbers, I am happy that it happened and I am always moving forward. I have learned that there are always things to try and always options. And when I hit a wall and there are none, all I have had to do was to wait for a few months or years and something else will pop up. And if I am really lucky, I might still be around to take advantage of it. 

I am just happy and have been lucky that there is research out there to always seek more ways for us to control this. 

Looking forward, hopefully, to the next blood test which will be sometime in February and which might show a more hopeful story. 

I wish you smooth journeys, full of silver linings out there! And make it a great 2025! 

The Importance of Advocacy

It’s been a roller-coaster of a year, so no wonder I have not visited this site since August. But I do hope to fix that in the coming months (I hope I didn’t just jinx myself). 

I tell you what: it pays off to be stubborn!

I wanted to write a word about the importance of advocacy and most importantly, the importance of advocacy for yourself. I have written quite a bit about this. In fact, if you do a simple search for “advocate” or “advocacy” on this blog, you’ll be able to find quite a bit on this subject.

There is no one in the world that knows your body and you better than yourself. You should always advocate for you. What I have found out after 41 years of being an FH patient is that doctors will fall into roughly three categories:

1. They will have no clue what to do with you. They will shrug and tell you, “well, this is genetic, so there is not much we can do to fix this. It is what it is. Go about your life as you normally would.”

2. They will have a god complex and declare that they know exactly what to do about it and they will apply all treatments “by the book”. Or so they say: they will start with changing your lifestyle, your diet, your exercise routine, then they will give you the drugs they have available, start with the least “scary” ones perhaps (like a bile sequestrant or a Zetia - both that trap the cholesterol you get from your bad diet into your GI tract), then with statins, if you’re lucky, and maybe they will add some other drugs when those won’t work. They will expect each drug to work according to its literature and with what they learned in school. They will expect each drug to have exactly the side effects listed in the paperwork that comes with them. If you bring on your own side effects, and if your numbers don’t match the predictions on the prospect, they will ask you if you’re taking them according to the right schedule, or if you know how, in fact, to inject your own drug (in the case of injections), etc ... They will just assume you’re doing something wrong, instead of having the wisdom to know that you are unique and each drug should work uniquely to every individual, instead of knowing that any drug is not a “one size fits all” experience.
   I call this category of doctors,  “doctors that treat me by the book, and never look at me for the unique individual that I am with a unique make-up and history". They think they know, but what they know is a template. This is the most dangerous category of doctors, I think, and they can do the most damage. 

3. Finally, there will be the doctors who both know what they are doing and listen to you as an individual. They look at your unique case and treat you according to your type of elevated lipids, according to your body type, family history, and medical history and sensitivity to drugs and other things. This category is rare and you’re lucky to find them. 

In all these cases, however, the specialist remains the same: you! Although they can be  the guide, you should be the specialist and you should work as a team of equals. You know how your body responds to new medicine. You know whether your body takes lower doses or is slower to respond to a medicine. You know how much you can take of what. And you should feel empowered to guide them, as well. 

Here’s my case. I was diagnosed with FH when I was 8. I have seen all the specialists you can see that (typically say that they) can manage this disease (endocrinologists, lipidologists, cardiologists). Since 1983, I have been on almost all medications that have been available on the market since then till now. No matter what the drug paperwork says, I can tell you with mathematical accuracy that: 

• Niacin absolutely makes me nuts. The flushing is so intense, I literally feel like I am about to die burning in flames. And no, there are no secrets or tricks for me that work to actually make it possible for me to stay on it. Not for me. 
• Bile-acid sequestrants make me gag and give me terrible stomach acid. I do believe this has caused and even chronicisized my GERD, although I stopped taking this type of medicine in my teenage years.  
• Zetia gives me gas so painful I want to punch something. When I first started taking it, I cried myself to sleep at night, it was so intense. I got used to it over time, but it took years. 
• Nexletol upsets my liver enzymes and elevates my uric acid. The elevated liver enzymes are only casually mentioned in the literature and for a relatively small percentage of patients. They also say they will come down over time. I took Nexletol just fine for a couple of years and only after that, they started climbing.
• Outside of the cold-like symptoms that Praluent gave me in the beginning, it is virtually side-effect-free now, after 8 years and it did the most good for me until recently when a yet newer drug worked even better. But not before I had to fight for it with two cardiologists. 

This year, as you might remember, I changed my cardiologist (who also manages my cholesterol). The old one took over two years to figure out how to give me access to Evkeeza, a new infusion drug especially approved for Homozygous FH patients. In the end, he failed, and although I had a long history of seeing him, I left and found a new doctor. 

The new cardiologist describes himself as not only a lipid, but also an FH specialist. He has lots of patients on LDL Apheresis at the only LDL Apheresis center in my state (I believe). When I walked into his office my total Cholesterol was 307 mg/dl and my LDL was 238 mg/dl. At the time, I was taking Lipitor, Zetia, and Praluent. I came to him because I had wanted to try Evkeeza (https://www.evkeeza.com/s/) since 2021. 

The cardiologist told me he would prescribe Evkeeza for me but that will not be enough. He insisted that I need to wrap my head around apheresis (which I have refused to do even before my open-heart surgery and before getting a mechanical aortic valve which are making it more hard now to get a port) and I will absolutely need to only take Evkeeza to supplement a weekly apheresis treatment if I want to see my LDL anywhere near 70 mg/dl (which is my target). He said with Evkeeza alone I could maybe get as low as 110, but never around 100, much less lower than that! 

He told me that approving Evkeeza would be lengthy, because of the prior authorization process, but in the meantime, he could schedule me for apheresis right away. I think I spent an hour in his office trying to explain to him that I will not, under any circumstance, do apheresis - it’s a matter of personal choice and I have said “no” to it for close to 30 years. He insisted that he will be the first doctor who will crack this stubbornness and will get me to do it. I left on a promise to think about it, but only after he would help me to do the minimum of 6 months of Evkeeza treatments and proved that it won’t bring my numbers low enough. 

His office was better than he gave them credit for, and they approved my Evkeeza almost right away. I started the infusion almost a month to the date from my first appointment with him. The drop in numbers was immediate. After just one month of treatment, my LDL dropped to 100 mg/dl. (remember when he insisted that even after a full treatment of 6 months it might just get closer to 110, but definitely never lower?). After 6 months, I am at 75 mg/dl. And this is with no Ezetimibe (which he discontinued because he now believes Evkeeza works better than expected for me) and with half the strength of Lipitor that I was taking before (I was taking 80 mg when I saw him first, and I am taking 40mg now). 

At my last appointment he said: “You convinced me. I am not ashamed to admit that you were right and I was wrong. You proved me wrong. And I thank you for it. No more talk of apheresis. You proved that this new drug works.” 

I, of course, have no merit in this, other than I just wanted to try a new treatment. I know that drugs work for me. I have seen them working at various efficiency levels. I have met a lot of folks on Evkeeza who describe LDL levels of 50 mg/dl. Even people with HoFH with higher levels than mine describe unbelievable results. I didn’t know if it would work this well, but I knew I wanted to try it. I am glad I didn’t let my guard down and I didn’t sign up for apheresis like the doctor so strongly pushed for it.

There is nothing wrong with apheresis per se, of course, but it is just not my personal choice. There is a certain trauma and invasiveness that I associate with apheresis and I would rather avoid it at all cost, if possible. There is a certain commitment to it, and I travel too often and too much to commit being tied to a hospital bed for at least half a day every week. Now that I am very concerned with clotting and infection (because of my mechanical valve and my blood thinners), I do not want a port implanted anywhere in my body, unless there are literally no other options. And I truly wanted to try something less invasive (if you can call a once a month infusion “less invasive”) that could actually work better for a longer period of time than a week. I just knew my limits and what I can and cannot commit to. And I knew my body. Praluent worked so well for me, from the beginning, that I knew another “new” drug might too. 

The mechanism of each of the drugs I was taking at the time is completely different, but I have just seen that newer drugs do work better for me. So, I wanted so badly to try it. And I am glad I pushed and held my ground. 

And of course, I am lucky and grateful that I found a doctor who would actually listen to me. He did dig his heels a bit in the beginning because of his many years (probably as many as my whole life) of what he perceived to be a tried-and-true treatment that he is very familiar with and with which he has seen results, but my perseverance won out. It’s a two way street - and I am glad we met in the middle. 

My new cardiologist has a favorite phrase when we discuss treatments and numbers and drugs. He often congratulates me for my knowledge of FH and treatment options (most of my knowledge comes from self-teaching since the internet is such an exhaustive source of information now), but also from following and advocating for the Family Heart Foundation (https://familyheart.org/) - their webinars and knowledge sharing of the newest findings in treatments is invaluable) and then he says “we’re on the same page”. 

I am glad that about this treatment we finally managed to meet  ...  on the same page. 

My most recent numbers, after 6 months of Evkeeza

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. 

“Help. Thanks. Wow”

This past week I have had a “Help. Thanks. Wow” moment, to paraphrase one of my favorite writers, Anne Lamott. You might remember that I have fought and fought with a former cardiologist to get the Evkeeza treatment. I did that for more than two years, after which I had to admit that he was not right for me and we had to part ways. 

Since May of this year when I started with a new cardiologist who thought it was “criminal” (his word, not mine) to keep me away from a very effective treatment that would easily be approved for me when my LDL cholesterol was hanging out at 238 mg/dl (a continent away from my 70 mg/dl target), things have moved pretty fast: I have been approved for Evkeeza and I have already gotten two infusions - in June and July. 

This past week, it was already time for my second infusion. It went with a bit of drama, because it is me, and because I am never easy. The nurse asked me if I had any reaction after the first infusion. I told her that it was not a huge one but that I did have a massive headache that lasted for more than 24 hours. She was all worried that because of how this drug works it might cause a stroke (because of the already existing plaque in my arteries). 

Because the “massive headache” is not one of the “normal” side effects listed in the drug’s prospect papers, she needed someone to verify that it would be OK to continue with the treatment. So, she had to call my pharmacist, her pharmacist, my cardiologist, and I called my Regeneron contact - to ensure it’s safe for me to get the second infusion. After waiting for about two hours, we got all the clearance from everyone and she did the second infusion. 

Getting the second infusion, after the "drama" was over

I will need to remember that the protocol is always the same for each infusion: they will do a pregnancy test before every one of them, then they will draw blood ordered by the doctor. Because my liver has been super sensitive when I have added drugs to my regimen in the past, I always want to keep an eye on my ALT and AST values when I introduce anything new. The doctor had requested only a direct LDL cholesterol level and a lipid panel and I had to do some convincing that she always needs to order a liver panel, too. She eventually found a way to order a CMP (Complete Metabolic Panel) which looks at kidneys, liver, and some other things. 

They will do the lipid and LDL cholesterol every time, before each infusion, to see if the medication works. But she said I will need to talk to the doctor (which I did twice but it didn’t “take”, apparently) to also add a liver panel. We’ll try again. 

Later the same day, I got my results in MyChart and I just about fell off the chair I was sitting on when I saw them: my calculated LDL is now 100 mg/ dl (or my direct LDL is 96 mg/dl) from 226 mg/dl a month ago. 

The lowest value I have ever had was 107 mg/dl about three years ago and that was while I was also taking Nexletol, and it was more of a fluke because it immediately jumped back up to around 120 mg/dl . But I had to stop the Nexletol, because it was causing elevated liver values. After I stopped the Nexletol, the LDL went up to anywhere between 220-280 mg/dl (taking the outlier months out here, when it was even higher, in the 300 range). 

Please click the picture for a larger view

Given that this is just one infusion so far, I do hope that this is just the beginning of an ever-descending trajectory. I cannot explain the feeling of happiness and gratitude that I feel to see that I am moving closer to a “normal” range for something that has been deemed abnormal my entire life! 

I am grateful that I was able to switch doctors, and that this doctor understood the urgency of getting on this treatment and he worked so incredibly fast to get me to it. It’s a great thing when everything lines up. I am also grateful to Regeneron and to my insurance who are helping me pay for this. Although I don’t know how much the “shelf” price is for Evkeeza, I am positive I would not be able to afford it otherwise ... 

I am still in shock, but so elated. 

What I can tell you is this: never ever settle. Never accept “no” for an answer and always look for the right doctor, the right team who will partner with you to give you the best care! 

And just ... “WOW”! 

Much health, everyone!