Stroke Diagnosis, Increasing Numbers, New Research, as Life Moves on ...

It’s been a long year already, and a busy couple of months. As the world turns elsewhere and everywhere around us, our journey through it continues. There is no other way. I don’t know of any other way ... 

What the past two months have taught me (again) is that time waits for no one. It moves, and brings news and new developments, both bad and good, new findings, progress, and a few steps back. But regardless of how you label them, your march should continue onward to a new day, maybe in a new world, but hopefully with a renewed perspective. You live and you learn. And this is what these months have been for me: new lessons, more concentrated than usual, but not different than any other timeframe. 

On Valentine’s Day, my husband and I traveled to Charlotte, NC to assist The Family Heart Foundation with a screening drive. They are invited or organize on their own these events where people come to learn about why it is important to keep your LDL-C and your Lp(a) in a normal range and to educate how both these can be driven up by genetic factors. They offer free screenings of both tests, or you can take home information to order your free screening kit through their Cholesterol Connect (https://familyheart.org/cholesterol-connect) program. I really enjoyed not only giving back to a foundation that has done so much for people like me, but also chatting with people and seeing how their lights go off when you speak to them about the danger of high cholesterol and the heightened risks high cholesterol brings for heart attacks and strokes. I had a large number of individuals approach me with “heart disease (or stroke) runs in my family, and I wonder what my numbers are?” - I was glad that more and more people do make this connection. 

Charlotte, NC - February 14, 2026

A personal bonus gained from this event was that I, too, checked my levels, especially because my cardiologist doesn’t repeat the Lp(a) test, so I was curious to see if it is indeed relatively stable as it is believed. I have seen mine changing over time, and I never got a straight answer as to why that is. Some doctors say it’s because of other diseases, or conditions that might be present at the time of the test, and some others say that some of the medications I am taking for lowering LDL do have some effect on the Lp(a), as well. 

In May 2024, my Lp(a) was 102 nmol/ L; this time (February 2026) it came in at 44.63 nmol/L. The normal is up to 100 nmol/L. I used to tell people that my Lp(a) is borderline (slightly) elevated, but now it is perfectly normal, with this test. From what I read online both Repatha and Evkeeza (I take both) have been proven to lower Lp(a), although they are not specifically prescribed for this. I will follow up with my cardiologist, of course, to understand more, but I also know that he presents himself as an LDL specialist, so he might not be familiar with Lp(a)?! That is one thing that I find very confusing - the minute level of specialization of doctors nowadays is overwhelming.

But the cherry on top was when I read the news on March 13, 2026 that the American Heart Association (https://familyheart.org/2026-dyslipidemia-guidelines) released their new guidelines where they emphasize the importance of more often and earlier screenings of Lp(a), in addition to that of LDL-C. When I first heard about this blood test and how high LP(a) can pose an additional risk for heart disease (early 2000s), there was one lab in the whole state of North Carolina that performed the test; my doctor’s office had to send my blood out to get it tested elsewhere and it was not covered by insurance. A sign that progress is happening, despite the world’s best efforts. 

On a strictly personal health level, this time has also been a time of progressive discovery and struggles ... We took a trip to Nashville, TN for a few days for my husband’s birthday. We love to be active when we travel - we usually have a limited time to visit any place but we always want to get the most out of that short time. We usually park in a central place and walk all over just to get the feel for a destination (a park, a beach, a city etc). I am currently on four medications for my heart: Atenolol (for tachycardia and arrhythmia), Amlodipine (for high blood pressure), Isosorbide (to relax my arteries which are severely plaqued) and Furosemide (a diuretic, for heart failure). It took months, if not years of trial-and-error regimens to come up with this cocktail that finally works for me - in the sense that it gives me both the relief of symptoms, and also better pulse and blood pressure numbers. 

Out of all these drugs, I absolutely hate Furosemide (Lasix) because it interferes the most with my life. You are pretty much tied to a bathroom for at least 4 hours after you take it. When I travel, especially when I am on my feet in the middle of a city with no access to a bathroom for half a day, I tend to skip it. But the Nashville trip proved that I can no longer have this luxury. When I skip it now, I simply cannot breathe. The shortness of breath and the fatigue sets in with a few steps. Forget “walk a mile before the symptoms set in” - there is no time for that ... I muddled through this trip but now I am working on trying to figure out a schedule that I can keep while traveling because stopping the travels is out of the question, at least at this point in my life ... So, this was a lesson for sure. 

Right before this trip, I asked my cardiologist to run a Pro-BNP test which measures the severity of heart failure. During his care, the BNP had gone down to 110 during 2024, but it now back up to 629 pg/ml (it is normal up to 190 pg/ml). This very much tracks with how I feel - the fatigue and shortness of breath are signs of heart failure too. He didn’t change my drugs at this time but he did double the amount of Atenolol and Furosemide for a few months, then we’ll see what the tests show and how I feel. I do see improvement from the small change he did in the drugs. The addition of the Isosorbide later last year also has given me relief from my angina (chest pain) symptoms. 

Of course, I worry that there is a bigger underlying cause of all these worsening symptoms, but so far, we are planning to continue treating the symptoms, running the tests, and repeating the imaging of major arteries and of the heart, and we’ll go from there, should there be any new findings. 

My health investigations continued during this time, as my cardiologist felt like I should check my lungs just to make sure there is no underlying lung condition that would contribute to my shortness of breath. After 2 lung studies of various kinds and visits with two pulmonologists, the verdict was that my lungs are fine and whatever shortness of breath and fatigue I feel is from my heart and most specifically from the fluid build-up from heart disease. They suggested I continue treating the heart and I will see improvements in my symptoms, and this has proven to be mostly true. 

For years, also, I have felt increased dizziness and also for years I have tried with more doctors that I care to count to diagnose where this dizziness comes from. In my heart, because I know my body better than anyone, and because I know what FH does to the arteries, and also because I have seen so many people in my family struggling with this condition and watched them over the years navigating new symptoms and the aftermath of new events, I just knew the dizziness must come from some vascular problem. I always feel like my entire body is in a straight jacket (especially with effort) and I just know there is no “give” in my artery walls. I kept begging all the doctors to look at vasculature and see a relation between FH and what it can lead to cause dizziness. But doctors have their own agenda sometimes and they all shrugged and said: no, we need to look at your peripheral circulation (which is, quite amazingly, good), at your balance, and your ear, this is not from your heart or your severe stenosis in your carotids and abdominal arteries. I was not convinced.  

About 5 years after I started looking for answers to my dizziness, a kind ear-nose-and-throat doctor said simply - when I finally was sent to her to check my balance one more time and my ear canal, in a last effort to diagnose my dizziness (I cannot call it “vertigo” because times and again many doctors agreed that what I feel is not vertigo; the physical therapist that specialized in treating vertigo also said this is not it) - that she is shocked that with my history of vascular disease no one has done an MRI of my brain yet. But no one indeed has. So, she sent me to get one. This revealed that at some point in the recent past I had a stroke across both hemispheres of my cerebellum (the small brain). She concluded that this could very well have been 5 years ago when I first felt the symptoms and she referred me to a stroke-specialist neurologist which I am due to see in May. So far, the radiologist who read the scan, classified my stroke as a chronic cerebellar stroke, which means there is damage from a previous acute stroke but it is not “active” anymore. This means the scarring is still there but there is no active cause of it at this moment. Among the many symptoms I have had over the years (changes in speech, difficulty remembering words, poor coordination of extremities - which I assumed were a result from my open-heart surgery, or maybe some medications too, like statins, maybe others), dizziness is one of the main symptoms of a cerebellar stroke. And I have my dad’s sister as an example of this, too. 

I know a lot of people are shocked and fear for their lives when they are faced with a stroke diagnosis, but for me, it was really a sigh of relief. Since I was 8 and they diagnosed me with FH, I was told I would have both heart events and strokes early in life, I have seen my dad  and his dad killed by massive strokes, and many aunts and uncles either maimed or killed by strokes, so it was kind of expected that my time has, too, come - a lot later actually in life than I was originally told. I am sure that the fact that I started medications in my early 20s bought me 20+ years of somewhat a full life (whatever “full” means to me). 

So, reading the result of this MRI was somewhat of a relief: I now have a diagnosis that I can follow with a specialist and understand more about it, and what the next steps are. 

I remember that all the people touched by strokes in my family worked closely with neurologists, just like I have worked with cardiologists and lipidologists all my life, so this is an expected development of our disease, I would say - or, it is for me. As I always say: knowledge is power and knowing what to call something you feel puts you on the right path to managing it. 

Now, I am not sure what other special care the neurologist will suggest to prevent strokes, because from what I know the same guidelines for preventing heart attacks (which I have followed all my life) are also beneficial to preventing strokes. But I would like to monitor my brain closely, just like I do my heart, to understand the pace of disease progression and to try to ensure as much as possible a symptom-free or less-symptom-full life. The stroke, as sad as it sounds, was a welcome step in the right direction. It was freeing in a way. 

Back on the cholesterol-level front, my LDL-C keeps going up. Despite the combined therapy I am following (all maximum strengths of Lipitor, Zetia, Repatha, and Exkeeza), my numbers have gone up progressively, a little bit each month, from 58 mg/dl in June 2025 (when I was taking Praluent as my PCSK9, in addition to Lipitor, Zetia and Evkeeza)  to 100 mg/dl in March 2026 (I have taken Repatha since July 2025).

My latest numbers - March 2026

I noticed the increasing trend right after I switched to Repatha. The cardiologist also noticed and we tried to get it switched back to Praluent, but my health insurance prefers Repatha and we have applied twice (applied once and then appealed) and we have gotten denied twice. I spoke with the insurance to plead my cause but they said that unless the doctor personally calls and speaks with their medical advisor, there is little chance they will approve the switch back to Praluent. And it’s been taking a long time to get through to the doctor and his office to persuade them to get involved. The only switch in my medications was from Praluent (which I was taking from 2016 until 2025) to Repatha - and the switch also coincided with the increase in numbers ... 

I know people who swear by Repatha, and insist that they see better results on it than on Praluent. It is clearly not the same for me. After our attempt in 2025 to switch back to Praluent, the pharmacy on the doctor’s side canceled Repatha altogether from our system for me, so now we will need to reapprove it with Aetna and with the pharmacy which is a little ridiculous, but such are the bureaucratic ways of our medical system. Of course, there is no giving up and there is only one way forward - insist, push, convince, argue, and finally succeed to get on the right path with treatment. This has, unfortunately, been the battle I have waged for 28 years now, since I have come to this country. But all in all, I know nothing good comes without effort and I am incredibly grateful that I have had the opportunity to be here and have good jobs that pay for my health insurance so I can have access to care, albeit delayed and not without strife. 

I have encountered many defeats in my life as a patient but one thing I know for sure: I am not one to give up. There are infinite trips I still plan to take and infinite things I still want to be around for, and without my health nothing is possible. Not possible for me nor for anyone around me that I would still like to be here for. So, I try to learn every day what is new with my body, and what is new in the world, so I can adapt and move forward. Forward is the only way. 

Much health, everyone! A happy spring! 

 

With All Due Respect ...

Don’t get me wrong. I absolutely and whole-heartedly respect the medical profession. Simply because I am convinced I would not be here today if it had not been for some incredibly talented, intelligent and ultimately extremely gutsy doctors, nurses, and pharmacists that took the time to learn and educate me about a rare disease that was only known in a few elite circles of the medical literati. I want to make this clear, before I go on my rant below ... 

But there are many times, a lot more than I would like to count, a lot more than I would expect as a patient struggling with a complex disease that cause more disease every year,  where I do have no choice but to not trust my medical professionals and it’s not because of me. It’s either because of their ignorance, unpreparedness, or because of the insane, profit-driven medical world of today that simply does not allow them to allot the proper amount of time for me, or to stay on top of their research to know what treatment they should have me follow. 

I landed on this article (https://www.nbcnews.com/tech/misinformation/doctors-say-medical-misinformation-gotten-worse-survey-finds-rcna225804)  a few months ago and I knew I was going to have something to say about it, but it didn’t come outta me until after a recent visit to my cardiologist and another one to my new vascular surgeon. 

The article talks about a survey that a nonprofit organization ran amongst doctors that found out things like “doctors said they encountered patients influenced by misinformation or disinformation a moderate amount or a great deal of the time over the past year”, and “doctors encounter pseudoscience in their everyday practice of medicine, and it indicates how their jobs are changing in response to a new information environment in which distorted health claims spread easily online”. It continues to state that due to the patients’ misinformation, the doctors feel that “it’s frustrating. It’s demoralizing, it cuts to the core of what motivates most physicians, and that’s a desire to help people in the most fundamental of ways. And in a way, it’s a repudiation of all the different things that lead people to practice medicine.”

Yet another doctor not related to the survey admits that “it’s too much to expect a physician or nurse practitioner, for example, to address complex, deep-seated beliefs in an eight-to-twelve minute consult”, and later admits that “sadly, sometimes our colleagues fall for the falsehoods, too”. This is all I will quote from the article. I am sure you can read it for yourself and form an opinion. 

Bluntly, this is what I would have to say, and I only speak from experience: doctors should not at all be surprised or feel “demoralized” or “frustrated” that people turn to social media or Google for medical information, because they cannot address all the patients’ concerns in the extremely limited amount of time offered to them for a consult by a system that enforces quantity over quality in the pursuit of the bigger profit. 

I have had cardiologists that admitted that they see 23 patients a day. In case you are doing math, this means about 20 minutes per patient, if the doctor takes no bio or lunch breaks, no breaks for meetings, email answering, or additional calls for referrals and additional consultation, during an 8 hour work day. This simply is not possible, in other words. 

I have a rare disease with an extremely complex set of diagnoses: I have homozygous FH, coronary artery disease, tachycardia, arrhythmia, atherosclerosis, a mechanical aortic valve, heart failure, diastolic dysfunction, carotid stenosis, mesenteric and celiac ischemia, fatty liver and gall bladder tumefactive sludge, just to list the main ones. There is no time in the whole 8-hour day for a doctor to advise me on my symptoms, my course of treatment, and my complications from all these. Not to mention there is not one specialist I need, but several.

I have no choice but to go to Google and ask about my concerns, or even the interactions between all my medications that the various specialists throw at me. 

To complicate the landscape, doctors are so incredibly specialized nowadays that they cannot see (or refuse to see to save time) what the other specialists do. They often operate in a complete void.

Very often in my career as a patient, I have found (from my own research) that a doctor would prescribe something that would either hurt another part of my body already affected by disease, or conflict with another medication that another specialist prescribed for another affliction. None of my doctors, even the good ones, look outside their own boxes. 

But what is worse, the one thing that made me go “oh, please, docs, cry me a river” when I read this article was something more severe and something that has been bugging me all my adult life. The one thing that makes me personally turn to online information (which I agree is not always reliable but it at least gives you the illusion that you get some answers) is the fact that I cannot find the answers in my own doctors’ offices. 

So, the doctors can say all they want (at least those surveyed) that they are  frustrated and demoralized because people walk in with bad information, but the truth is - we, the patients, sometimes have no choice. Last week, I walked into my cardiologists office and after the recent tests that he did - a holter monitor that I wore for 2 weeks, and a referral to a vascular surgeon that yielded results from an abdominal CT that showed a worsening of my mesenteric and celiac arteries, in addition to a history of worsening blood pressure and pulse measurements, worsening shortness of breath and angina - his question to me was: “All right, what kind of imaging tests are you interested in pursuing next?”. 

If I had not had 42 years of experience with medical professionals, I would have fallen out of my chair. But this was a too-common question that I have unfortunately heard from multiple physicians. 

I am not an ageist in the least - I have had young medical professionals that are up on the newest technologies which I can appreciate, and I have had older ones that have a more wholistic approach that I love, and are into the business of actually touching the patient to find symptoms and listen to the body (the heart, just like the lungs tells you things when you just listen to it; but few docs do it anymore). This particular cardiologist was an older gentleman, which might incline someone to think that in addition to many years of medical education he had experience on his side, so he should know the next steps for the course of my treatments. He deferred it to me. 

So, where is a patient in my case supposed to turn for answers? How am I supposed to know what imaging tests would be the most appropriate for a correct and reliable diagnosis? The only place I have is somewhere online and who can verify the accuracy of those results?

I deferred it to him by saying “the educated, wise kid in this room is you, doc, so I defer it to you to know what test to do next that would give you the most accurate picture of what is going on so we can come up with the best plan for treatment.” 

He decided against more tests and instead he did what many of my doctors do - threw more pills at the problems ... I’ll try them and see where this will lead ... We'll reconvene in May and reassess the symptoms and go from there. Maybe. If he remembers what my complaints were to begin with and what tests he has done so far. 

It’s all trial and error. Unless I stumble upon a thing online that might sound pertinent and then I am sure I can pitch it to him and he’ll order it ... reliable, accurate or not ... Maybe?! 

Days after this appointment, I met with the new vascular surgeon who had ordered a peripheral artery study (which always comes out perfectly fine and she should have seen the results of the previous one in my chart, because my cardiologist in the same practice did an identical one just 2 years ago and it came out perfectly normal, but people don’t read anymore, so we’re forced to repeat tests 10,000 times if needed - more inconvenience for us, more time off from our lives and more money for them) and an abdominal ultrasound - which, to my uneducated brain is a lesser-accuracy test to an abdominal CT with contrast that the same cardiologist in the same practice ordered only 5 months before. The results for the ultrasound were almost identical to the CT done 5 months prior, and they showed a worsening in my celiac and mesenteric stenoses and an increased post-stenosis dilation than the year before.

When I went in to meet my new vascular surgeon and go over the results, she acknowledged my disease but she brushed it off with the (in my humble opinion) ignorant statement of “well, yes, you have disease, you have HoFH, so this is expected, but I look at you and you look perfectly healthy. So there is no immediate action.” 

I just about came unglued, as I always do when I hear this statement because HoFH as well as many heart conditions are invisible, but history shows us, not any less deadly than many other extremely acute afflictions. And I told her with all the honesty, tact and well-managed frustration that I could muster: “This is where you lose me, doc! This is where I have to tell you: I do believe my disease is not unimportant or un-worrisome, just because I manage to look good. This is not a professional assessment, with all due respect.” 

And again: I know what I saw in the test findings. I know that things like “stenosis” and “dilation” are bad, in the context of atherosclerosis, arterial disease, stenoses, HoFH. And yes, I know this because I Googled it! And not only that - I know this because I saw what it did to many people in my own family who also “looked good”. They “looked good” until they had a stroke that left them physically changed, impaired or dead. 

But where do I find the correct interpretation of results? Who can please connect the dots between my constantly worsening symptoms and the findings in my new tests, when the doctor simply says “yep, tests  - bad; you - look good. Go home and live your life”?! Where can I find objectivity and education when the doctors blow me off?! 

Naturally, I turn online because that is a convenient resource that at least pretends to know it all! But where can we, the patients, find the authority who can parse through the information for us, and educate us not only about a disease in general but about our particular case of the disease, so we can feel like we have a handle on it and can live healthy and successful lives? 

And one more thing: I have had doctors, doctors with a reputation that preceded them, that have pulled up Google in their office with me present to see “what else they need to do next” for me. 

I am guessing with the explosion of AI nowadays, things will only get dire. I very often use Google primarily (which has always been “powered by AI” but now it needs to be branded this way) and quite often I am mindful of the findings. Because oftentimes, I have no choice. 

So, what gives, I ask you?! We insult them if we go online and come in with false information that our limited knowledge and desperation cannot parse through, but they insult us by dismissing our afflictions or deferring us to make decisions when they should be the ones with the knowledge?! 

What gives?! 

The End of an Era: "Good Bye, Praluent. Hello, Repatha."

I have had a long, sinuous, adventuresome path with PCSK9 inhibitor drugs. 

A complete unknown when my FH was diagnosed 42 years ago, a dream or a vague promise for most of my adult life, they have become the drugs that probably extended my life, right along with my very invasive open-heart surgery 9 years ago. 

I remember around 2009 or so, the pharmacist that worked with my cardiologist at the time in Greensboro, NC, shared with me that there was this clinical trial on the horizon (not available yet) where they would test this new class of drugs, called PCSK9 inhibitors, and he thought that I would be a perfect candidate for signing up for it. But as a rule, I don’t want to participate in clinical trials. As a rule, I accept taking a treatment only when it’s been officially approved and only if there is no major impact on the quality of my life. 

Well, PCSK9 inhibitors were not approved, so I said no, however promising their clinical trials were at the time. Then, around 2011 or so, the same pharmacist went through some hoops to find my new information as I had moved to Utah and contacted me to share that they have a clinical trial in Salt Lake City that would allow me to participate. A couple of years or so later, he said they were seeing really good results with these new drugs for FH patients, and to please consider participating. He said he could contact the clinic in Utah on my behalf to give me a referral, but I politely declined again. 

At the time, my LDL levels were still dangerously high, going up and down between 250 and 300 mg/dl, although I was taking cholesterol-lowering drugs that were on the market at the time; but they were not enough. I still said no, I would not consider this but I was absolutely stunned that he remembered me and he went out of his way to find me and share this news with me. 

Fast-forward a couple of more years, and at the end of 2015 (the year when Praluent was finally approved), I was told that I needed pretty much emergency surgery to replace my severely stenotic aortic valve and to ultimately have several bypasses of blocked arteries. 

My surgery was performed in February 2016 and both the surgeon and the cardiologist sat me down very sternly and explained in great detail what intensive damage my very high cholesterol had done for the first 40 years of my life. They both encouraged me that if there is one thing I can do for myself, for a healthier life, and to ease the impact of cholesterol on my arteries, was to keep the cholesterol levels, particularly, the LDL, as low as possible through any therapy I can tolerate. 

My cardiologist at the time had been involved in the PCSK9 clinical trials in Utah, so he was very familiar with the drugs and with FH. He asked me to please consider these drugs as now they were approved and my LDL cholesterol was nowhere near normal. 

After seeing the results of my surgery and living through the really hard and lengthy recovery from it, as well as developing even more heart disease, I decided to start taking a PCSK9 inhibitor drug at least for 6 months or so to see if it truly would impact my levels so dramatically that it would be worth it in the long run. 

My cardiologist prescribed Praluent which I started taking in April 2016, about 2 months after my surgery. 

After the first month, my LDL dropped from 260 to 184. After 3 months, in July 2016, my LDL was 104. I was shocked! There were virtually no side effects. On the day of the injection and a couple of days after I had a runny nose like I was about to get a cold or like my allergies would act up. And then there was nothing else. I asked the cardiologist what made him choose Praluent over Repatha as both were available at the time. He quite simply said: “It was a coin toss! Either one would work. I just went with Praluent.” 

I have been happy with Praluent. Outside of the inconvenience of taking a painful injection every two weeks, worrying about keeping track of the schedule (easy to do with any calendar app), and ensuring I’d pack my injection pen when it would be due while I was traveling, it did wonders for my cholesterol levels - so all the challenges were small prices to pay to ensure my arteries would stay clean. 

My LDL target is 70 mg/dl. Praluent did not manage to lower my levels to lower than the low 100’s but it was better than walking about with 250-300 levels. So, I knew this would be a life-long drug for me. My artery disease, especially in my carotids, has stabilized. My carotid ultrasound used to be worse from year to year up until 2016. For the past 9 years, they have been mostly stable with no visible sign of worsening. 

In 2017, I was called upon to write an amicus brief to defend Regeneron’s lawsuit in court against the Repatha maker, Amgen, who was looking to push Praluent out of the market. I gave the perspective of the patient on Praluent and spoke about how important it was for people to still have access to Praluent, in addition to Repatha for various reasons. 

For me, I don’t like the fact that there is a chance of Repatha increasing your blood sugar levels. I don’t have diabetes, not even closely, but I do have a rich history of diabetes in my own family - virtually everyone with FH has eventually developed diabetes in my family. 

I have now been on Praluent for 9 years and I have managed it pretty well. In a way, I got very comfortable with it and it’s one of those instances of “you’re not afraid of what you know.” Even if there was not much thought, not more than “a coin toss”, in my doctor choosing it for me, it’s become my drug. What I am used to. What I know how it will affect me, for good or bad. That is a level of comfort that I struggle with letting go of. 

But in comes the year 2025 when my insurance company sent me a letter to notify me that starting with this year they will no longer pay for Praluent and I absolutely must switch to Repatha. I was very, very disappointed. I spoke to my cardiologist (I moved back to North Carolina so now I have a new cardiologist) and asked him if he could speak with the insurance company to persuade them to still continue covering my Praluent because I was afraid that my diabetes family history might catch up with me and I don’t want to risk adding another condition to my laundry list of issues. 

The doctor preemptively agreed to talk with them. He actually asked his nurse to call and see what she could find out. The nurse was not very empathetic about it. She called me and in no ambiguous terms said that “a family history of diabetes is not reason enough to not take Repatha and that only proving that elevated blood sugar while taking Repatha would be considered a reason to revise the insurance’s demand for switching to this drug.” It was not clear if this was her opinion, or something she was passing on from the insurance company. She encouraged me to try it and watch my sugar closely and we’ll react based on that, if necessary. 

I also spoke with my insurance company to ask them if I could please stay on Praluent, given my long-time record of it working fine for me, with virtually no side effects, and considering I have a history of diabetes in my family. They said those denying to pay for Praluent are actually not them, but my employer. They also said from what they had seen this year, my employer refused to pay for several other medications and from what they have seen from patients pushing back, they have not been too successful to make the employer eventually pay for a “non-approved” drug ... They said I was free to put in a complaint with the employer but they told me to be prepared to be told “no”. 

So, I conceded and accepted my fate ... Starting in August of this year, I will start taking Repatha and this last week was my last injection of Praluent. It’s like saying goodbye to an old and trusted friend. I have no idea what this new (to me) drug will do to me, but I know enough about how sensitive I am to changing drugs and how every drug is different, although it’s in “the same class” to be a little nervous about this switch. 

Of course, the recent lawsuit that found Amgen guilty of essentially bribing pharmacies to only prefer their product over Regeneron’s Praluent (https://www.fiercepharma.com/pharma/amgen-hook-pay-more-400m-after-regeneron-triumphs-cholesterol-drug-antitrust-suit) gives me additional pause. 

But what can one do? This is one of those cases, I feel, that what is good for the patient, or what the doctor recommends that might be good for the patient, does not always jive with what the money-making industries of pharmaceuticals and insurance companies are willing to make available for the patient. It’s one of the most frustrating parts about dealing with a disease that cannot be managed without medications. It’s adding the burden of unaffordability or muddling through preventable side effects to the burden of the disease itself. It never feels fair or compassionate, in any way. The “do no harm” is definitely overlooked in situations such as this. 

The (small) silver lining I have seen, from what I have read so far about Repatha, is that the drop in LDL levels seems to be higher than the drop with Praluent. But will it mean the same outcome to me? Only time (and trial) will tell. I will report back. 

A Giant Leap for Me ...

My First Evkeeza Infusion

It’s been a long time coming. I remember hearing about this new drug called Evinacumab (or Evkeeza) sometime in the fall of 2019 when attending The (then) FH Foundation’s yearly Summit (now, The Family Heart Foundation). One of the HoFH advocates at the event was participating in a clinical trial for it. I was terrified, but I think the world of people like her who are willing to put their body through anything, to navigate the unknown or the little known, in the darkness, it seems, to give us all better treatments. My words cannot be enough to express the gratitude for such people! 

In February 2021, Evkeeza was approved by the FDA for use in the USA for adults with HoFH, in addition to other cholesterol-lowering therapies. Two years later, the FDA approved it for children also. 

Around May 2021, I participated in a brainstorming session that Regeneron (the inventor of Evkeeza) organized with HoFH patients and medical professionals to test their marketing materials for Evkeeza on us. Amanda Seef-Charney, a Regeneron patient advocate, said at the meeting: “We must listen to and learn from individuals who so intimately know what it means to live with a rare disease. Only then can we begin to bring meaningful change ...”. This stayed with me. It’s so unusual when we have this almost invisible, rare disease to actually feel like you’re being heard. 

My own cardiologist did not hear me, when I asked whether I could be considered to be treated with this new drug in July 2021 (https://livingwithfh.blogspot.com/2021/07/who-knows-more-about-fh-you-or-your.html). He had no idea what I was talking about and the reason I went to him in the first place (starting with 2018) is because he advertised himself as a cardiologist specializing in lipid-lowering therapies. By November 2021, he started to look more closely into it and researching what we needed to do in order to get me approved. It was not until December 2023 that I actually got him to prescribe it and get it approved with my insurance. My first appointment was set for some time in February 2024, but the infusion center of his medical system had not heard of the drug and did not know how to order it. I knew I had to change course. 

So, I changed cardiologists and medical systems altogether. I started seeing my new cardiologist at the beginning of May 2024. Today, June 11, 2024, I finally got my first infusion of Evkeeza. It’s been such a long time coming (it'll be 3 years next month since I first asked my previous cardiologist if I can be on this) that it feels surreal to even believe I got it! 

The prior authorization was approved in less than two weeks, and the appointment was secured in two more weeks. As it turns out, this new (to me) medical system has an infusion center right here, in my small town - I usually have to travel at least 40 minutes one way to go anywhere from where I live. But only 12 minutes for the infusion center. It’s like Christmas. 

The appointment was early this morning (scheduled for 8AM). It was the first time in my life that  I stepped into an infusion center. Most people there were getting cancer treatments, but there were other folks with iron infusions and some other restorative medicines. The place had about 8 chairs that I could tell and they were all full. It felt packed and tight. No one was wearing a mask, including the staff, but my husband and I were. 

They weigh you just as soon as you get in because the infusion dosage is based on your weight. Then, because I am considered a woman of child-bearing age and with all my reproductive organs intact, I had to take a pregnancy test which is a urine test. They cannot give you the infusion if you are pregnant. They also drew blood for cholesterol levels which my doctor scheduled in advance. 

After all that and after the IV needle was in, they attached the IV bag. I took a picture of my first bag like it was gold! I hope it works! 

The IV lasted exactly one hour. After that, I waited for another 30 minutes to see if I had any reactions to it - mostly because this was my first time and we didn’t know. We scheduled three more appointments after this, as they like to schedule in advance to keep you on the one-month schedule that this drug is given by. 

During the IV, I felt dizzy a couple of times, but it was hard to tell what from. I do get dizzy occasionally, pretty much daily, and this felt no different than my usual dizzy spells. I also had this taste and smell of chlorine in my mouth and nose which was bizarre but it didn’t last too long. I got light-headed a few times, also and this was new, but not unmanageable. 

I came home right after it and I am taking the day off to just take it easy. So far, I have not felt anything unusual except for some light-headedness, or rather heavy-headedness as I feel like my head is too heavy and swimmy ... I also feel random tingling in my lips and palms, but it kind of comes and goes. No hives (they asked me to look for this), no swelling of the mouth or tongue. I also have a strange mild headache at the back of my head. This is unusual (my headaches are usually in my forehead and temples), and I think it’s what gives me the heavy-headedness, if you will. But, again, it’s totally manageable. The side-effects of a Covid shot are 1000 times worse than this! 

My husband came with me today because we didn't know what to expect from this drug. But I am sure that going forward, I will just go in by myself, barring any new developments in my overall health.

The infusion nurse said they will repeat the blood tests (for cholesterol levels) at every appointment. I asked if the liver function will also be monitored and she said she doesn’t believe so, but to call the doctor and ask that they add it. I know that it takes a full course of 6 infusions (6 months) before we see the full effect of Evkeeza, but the first rung on that ladder is now behind me after today. 

During the whole day, I have been hearing my late dad’s voice in my head. When I was 8 and they discovered that I had “a very severe form of familial hypercholesterolemia” (my total cholesterol was 734 mg/dl), I remember him saying: “one day, far, far away into the future, when you’ll probably be old or even after you’re gone, they might discover some kind of a miracle drug that might cure what you have. But it won’t be for a while. And it might be too late for you.”

When I was 8, they also told me I will not make it past my 25th birthday if I was lucky. I have been looking for this drug for only 3 years. I have been looking for an efficient treatment that will stop the progression of this disease for 41. 

Today is a huge milestone for me, in my journey, to add to the string of all the other milestones throughout my life: the first day that they discovered that I had familial hypercholesterolemia (when I was 8), the first day I took my first statin, or the day I learned without a shred of doubt that I have homozygous FH (I was 41), or the day of my open-heart surgery (I was 40). This is yet another milestone. What I hope for with all my heart is that this drug works. That it works so well that we’re done searching for more therapies. I hope that dad’s dream will be fulfilled and that, in fact, it’s not too late for me ...