Never Give Up - Back to Praluent

I am contemplating launching a new label for some of the blogs, and I should call that new category “never give up”. As I mentioned many times before, always, and I mean always, be prepared to advocate for yourself. I can never stress this enough. 

If there is just one message I can get across to those who are just now learning to navigate FH and its convoluted journey through medicine, therapies and the right specialists, is this: it will take time and patience, and more than anything, it will take effort, to learn and become knowledgeable. And when you get there, put that to good use to advocate for yourself. Never rely on anyone else, no matter how promising their commitment seems to be. 

I know it may seem self-serving, egotistical and self-centered to do so, but at the end of the day, this is about you, your life, your health, your family, your support system. You don’t want to give up because of doctors who may or may not know enough about your condition, because of bureaucracy, or because of neglect from so many people involved in the process. This is your battle. It can get lonely, but you’ll learn in the process that all you have to do is know your condition, know the facts, and speak up. And I assure you that there will be people who will, eventually, listen and help. 

I also need to preface this blog by reminding everyone that I do not endorse any medications over any others. The names of the medications I am referring to in my blogs are what works for me. I know many people who have the same condition (HoFH) and are using a different therapy combination. Just like there are no two bodies alike, there are no two conditions identical, nor two treatments identical.

So, please read this with this in mind. The names are given here just to exemplify my own journey. They do not endorse any of the medicines I am referring to, nor their manufacturers. 

To remind you, close to a year ago (https://livingwithfh.blogspot.com/2025/07/the-end-of-era-good-bye-praluent-hello.html), in the middle of the year, with no changes to my insurance whatsoever, my insurance company decided to stop paying for my PCSK9 inhibitor injection drug (Praluent) and replaced it with Repatha, which they said, they will fully cover. I had been on Praluent since 2016 (9 years) without any issues. My cholesterol dropped by about 40% which was the biggest drop by one medication alone. I was impressed. 

It was like saying “goodbye” to a friend when I had to stop the Praluent and order the new drug. I wanted to cry. Plus, as many of us who are on drugs for the long haul with a life-long condition know, you always wonder what will be the side effects with the new drug? Will I be able to tolerate it? There is always some undesired effect from a drug that you have to either make a decision you can live with or you simply cannot take it. So, I was nervous. I also am extremely incredulous of people to promise you that “two medications are identical, so you can switch to the other one easily.” I personally have not found this to be true at all. There is always a compromise you’re making.

I have tolerated Repatha well but there were some differences that I could notice, especially at the injection site. But tolerable. After three and even six months on Repatha, however, my LDL numbers were higher than my levels while on Praluent. With Praluent, my LDL dipped for the first time in my life under 70 mg/dl which was the target for LDL levels for people with increased risk. Since then, the target has moved to 55 mg/dl for people like me, with very high risk of cardiovascular disease. My levels have never been as low as all that, even with Praluent (and all the other therapies combined). But it was in the 70s range, or lower than 70, 7 times while on Praluent, while it was in the 70s only twice , and never below 70 mg/dl with Repatha. 

So, after 5 months of Repatha I spoke to my cardiologist to see whether we can convince the insurance to let me switch to the Praluent again since the numbers were visibly better. He agreed. He only can request such a change. No amount of advocating to the insurance coming from me would ever convince them. They needed to hear my specialist doctor (not a PCP) reach out and explain the medical need for this. So, he contacted them in November last year with what he thought was a compelling message about switching back to Praluent. 

And his request was denied. I received a letter from my insurance in the mail in November, never explaining why, but just communicating the decision. I called the doctor again in December (because like you already know about me, I don’t quit that easily) and I asked again: can we please appeal this decision. He said, sure, we’ll appeal. (Again, this request came from me, he would have stopped at the denial). And his office did appeal. 

In December, I again, received a letter from the insurance saying “your appeal was denied”. I crossed my arms and pouted for a while. The letter did say I have the right to a second appeal for 6 months since the denial of this one, but I told myself I’ll try again after The Holidays. 

I had a call with my nurse advocate from my insurance earlier this year and I gave her my LDL-C levels and asked her: “do you think I have a case to keep pushing for a switch? My numbers are better on Praluent, my doctor said the goal for all these drugs is to keep the LDL-C levels as low as possible, why can they not see this and just approve the switch? Repatha is obviously not that good for me. And there is an alternative that they have paid for years.” She agreed that looking at the numbers, I do have a case to push for another appeal, but that she encourages me not the appeal alone. To get the cardiologist to call (not write to) the insurance and speak with their medical advisor and explain the medical necessity for such a switch. 

Since January I have been trying to email my cardiologist (several times) to make him understand that he would need to call and speak with someone or else we’ll keep getting denied. I got silence. Weeks went by, my appeal clock was ticking (I was only allowed to appeal until early June), and nothing, no answer from the doctor. 

Well, as my luck would have it, his office messed up some other drugs (somewhat unrelated to the PCSK9 inhibitors) which prompted me to escalate those problems to his clinical pharmacist (since they were drug related) who is the person who actually gets the prior-authorization approvals for all my specialty drugs in the first place (and I thought he would take my requests for appeals to her, but ... maybe not?). She was not aware that I was trying to get my PCSK9 inhibitor switched. The pharmacist and I talked for a while, we looked over my levels (again) in late March and she promised she would appeal a second time and she also agreed that there is a clear case here for better therapy, so we should continue trying. 

And on May 8, I got an email from her and from my insurance nurse advocate to tell me that the insurance did approve the second appeal and they are allowing me to take Praluent for another year. The prescription expires in May 2027, but at that time, we would have to go through the same process again to get it pre-authorized and approved. Next time, I will skip the cardiologist altogether and I will go directly to the pharmacist. 

So, now I am back to my old trusty drug and hoping it’ll perform as well as in the past. 

First Praluent injection in almost a year. High hopes.

Of course, with all this disruption and rollercoaster drug-switchin’ my levels are still higher than they should be, but it takes up to three months for Praluent to work at its full efficiency and I have only taken one injection so far. 

It took, all in all, 7 months to get this switched. Several years ago, when I was advocating to take additional therapy with a newly-approved ANGPTL3 inhibitor infusion, it took me 3 years and a change of medical systems and cardiologists to finally benefit from that therapy. But we did it! (the new doctor and me). 

As they often say “time is plaque” when it comes to “bad” cholesterol and these delays and waits are one of the most burdensome roadblocks in our healthcare and well-being. But I cannot afford to give up. Not ever. Not while I know there is a solution. Time is of the utmost essence here. 

I hope this entry helps, not to scare you, but to give you an example of how this battle is real but how success is also possible. Just stay informed, alert, and state your case. Always. 

The best of luck in your journeys and much, much health! 

Latest numbers (click the picture for a larger view)

Repatha, Evkeeza - New Experiences and Numbers

As I mentioned in my July post (https://livingwithfh.blogspot.com/2025/07/the-end-of-era-good-bye-praluent-hello.html), my insurance decided to stop paying for Praluent and instead force me to switch to Repatha, the other PCSK9 inhibitor drug on the market. 

I tried to dissuade them to switch me, quoting concern for developing pre-diabetes (a known side-effect of Repatha) but I got turned down. Not sure by whom, honestly. The nurse at my doctor's office said originally that no, if they require me to be on Repatha, there is no going back to Praluent. Then, when I pushed with "but I have a very large, well-documented family history of diabetes and the doctor promised that he would support my case for sticking with Praluent", she shut me down almost immediately with "Well, we asked, but unless we try it and prove your sugar is going up, they won't budge." Doubtful. When did they ask? In the literally 20 seconds between her firm rebuttal and her mention of asking? But sometimes you choose to not fight every battle and give it a shot. 

I had other concerns too, that Repatha might not be as effective for me as Praluent was, or that it might have other side-effects Praluent never had. I also wondered if Repatha might, in any way, interfere with Evkeeza, my infusion drug for HoFH. 

I still don't know if it does or not. But since July when I switched to Repatha, I noticed a couple of new things. First, Repatha is not as painful to inject as Praluent was. Maybe the needle is not as large, or maybe the amount of the drug is not as much - I don't know. But it doesn't hurt as it goes in. However, it hurts a lot worse than Praluent after it's in. The injection spot hurts for a couple of days after I take it and my entire leg hurts for a few hours, too. Sometimes till the next day.

I am not sure if this is Repatha or not, but since I switched to it, my LDL numbers went up first, and now, after about 3 months they are just starting to come back slowly. But is that Evkeeza or Repatha's doing?  

I was told that I'll see the full effect of Evkeeza at about a year since the start of the infusion. After a year and a month, my LDL was the lowest it has ever been in my life, at 58 mg/dl. Just think about this, for a patient with HoFH that comes from an LDL of close to 600 mg/dl. But then, right after I added Repatha, it starting going slowly up. 

After I started Repatha, I also noticed a change in the side effects I feel after the infusion. I have always felt tired the day of the infusion and the next day. For about 24 hours, I feel like I have mild symptoms from taking a vaccine or something. I was used to this. But after starting Repatha (my pharmacist thinks it might be related), I also started feeling very hot and tingly while the Evkeeza solution goes in my vein. And about 48 hours after I take the infusion, my sense of taste is altered. A lot of things taste rotten or putrid. It's like some people report from having Covid. Even my favorite foods, like fish and potato chips taste bitter-rotten of sorts.

I mentioned this to the pharmacist who works with my cardiologist and ordered these prior-authorized drugs for me, and she said there are certain things they can try: they can give me benadryl before the infusion or they can slow down the infusion. 

I chose to skip adding yet another drug to my limitless cocktail, so they increased the time. They used to time it for an hour, and now it's timed for an hour and a half. The hot and tingly feeling is much reduced, if at all present. The taste alteration is still there after the infusion but not as long-lasting nor as strong. 

I still cannot quite tell whether the change in symptoms to begin with was from any or all of these drug changes or just a coincidence? Nor if the adjustment made any difference or that, too, might have been just a fluke. 

The truth still remains that I am still very scared of what Evkeeza might do, apart from truly keeping my LDL levels low. The drug definitely seems incredibly potent for LDL cholesterol, even as stubborn as mine has been historically. But it just got approved for kids as young as 1 year olds (look it up, please!), so here's  hoping that they have enough strong, pertinent research to make such a groundbreaking decision (some countries don't even approve statins for kids under 18 and we feel like we know everything there is to know about them) . I am also scared of Repatha too, because it is relatively new (only 10 years old) and it's new to me. So, I am sure there is a lot more room to learn here.

For now, I am enjoying the lower numbers, hopeful that they will protect my arteries for a little while longer. I am also happy so far that my sugar has not increased, yet. Still watching it like a hawk, though, and asking for a test for it every chance I get. 

My levels as of October 2025
Click the picture to see a larger view

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. 

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

A Mixed Bag: Some Good Things, Some Bad, and a Whole Bunch of Guessing, as Usual

Today was an odd appointment with my cardiologist, to say the least. It was my 3-month appointment (this is routine for me), where we were supposed to discuss the recent tests that he had ordered (a heart echo, a carotid ultrasound, recent blood work, and the results of my neurological tests) and, as always, assess if there are any changes needed in medication.

Right off the bat, he admitted that he didn’t review my tests before he walked in the room. He said he did see them when they were done (in September), but he had not reviewed them this morning before he walked in the room (intern in tow) to see me. So, he needed a minute. (My appointment was at 8:40 AM and he was already an hour late, so I guess: busy morning!)

My cholesterol went up slightly, as you can see below, but he said he will consider it a “lab error”. Well, which one was the error: the last one that showed it the lowest I have ever had it? Or this time, which is more in line with everything else we’ve done for the past year? No answer.

My AST (a liver enzyme) is elevated but only slightly (43 U/L and it’s normal between 15-41 U/L). But I have had it as low as 26, so … there is some reason for concern there. He said to repeat it in 3 months before our next appointment. We repeat the same tests before every appointment: a lipid panel, a liver and renal panel, a uric acid (because of the Nexletol/ bempedoic acid which elevates the uric acid and because in my 20’s I used to have gout attacks frequently).  The AST is part of the liver panel. He asked me if I want to do an extra measurement at 6 weeks but he said “he didn’t care; it was up to me”. OK, then … let’s just do them all at the same time which is in 3 months. (I love when he says “he doesn’t care” or “to him it’s six of this or half a dozen of the other”. Sounds so reassuring!)

My heart echo write-up mentioned for the first time “diastolic disfunction”. I asked him about this and he explained that what this means is when the heart fills up with blood, it increases in volume but it should not increase in pressure. In my case, there is some pressure that is measurable, but that it is “mild”. He said this is “normal” and “almost expected” in my case, having had a heart attack, open-heart surgery, and coronary vascular disease for many years. He said he is not extra concerned about it, as long as my aortic valve is clear (which it is) and my ejection fraction is good, which at 55% it is.

The narrowing of all my carotid arteries is increased compared to the measurements of two years ago, but the percentage is all the same – between 50-69%. This seems like a huge range to me, but that’s where they place my numbers.

For those more curious, here are my measurements for both the right (first) and the left (second) carotid arteries:

MEASUREMENTS – Right/ Left
------------------ -------------- --------------

Central Carotid Artery
CCA Proximal 249/ 19 cm/sec - 216/ 23 cm/sec
CCA Mid 168/ 21 cm/sec - 230/ 23 cm/sec
CCA Distal 141/ 19 cm/sec - 199/ 24 cm/sec

Internal Carotid Artery
ICA Proximal 136/ 24 cm/sec - 191/ 22 cm/sec
ICA Mid 189/ 36 cm/sec - 134/ 21 cm/sec
ICA Distal 160/ 30 cm/sec - 157/ 22 cm/sec

CCA/ICA Ratios 1.340 - 0.960

External Carotid Artery
ECA 550 - 260
Vertebral 93/ 16 cm/sec - 115/ 15 cm/sec
Subclavian 305 - 327

He said that the worst narrowing is in my External Carotid which is of least concern, because it’s the one that vascularizes the face which gets blood supplies from a “million other places” (his words), so there is no concern for no blood supply there.

I have an appointment with a vascular surgeon and he asked me to follow up with him for a second opinion on the carotid findings.

If it were not for me to mention the neurological test that he ordered to diagnose peripheral neuropathy, he would not have discussed it. I told him that the test showed that I did not have peripheral neuropathy. He was glad about that. He had suspected there was something neurologically wrong because my dizzy spells. Well, not sure what worked, but my dizzy spells are very mild now and very infrequent, and my muscle spasms and cramps are also much better, too. The dizziness definitely does not last for a whole day anymore. I started taking CoQ10 (my decision) which I guess must have made my muscle cramps less frequent, but I don’t think that it had anything to do with fixing the dizzy spells. In addition, my primary doctor diagnosed me with possibly anemia (low red cell count) and a B12 deficiency, so I started taking B12 vitamin supplements at about the same time as the CoQ10 – about 2-3 months ago. He agreed that this deficiency and the anemia could have caused the dizziness for sure. So, we’ll just continue with this treatment and the regular doctor is planning to check the B12 levels again at our 6 month follow-up.

We also talked about the heart symptoms: how’s the blood pressure, how’s the chest pain, how is the shortness of breath? How do I get along with the newest drug he put me on to treat all these (Amlodipine). I told him that the chest pain and shortness of breath are stationary, but I have more stamina when I walk (I can go further and on steeper inclines through the shortness of breath and the angina because I feel like my heart is getting enough blood supply). My neck still cramps, but after a longer walk. The blood pressure is medium-high (in the yellow-orange range on the machine) a lot more often than mostly high (red range), like it was before the Amlodipine. My gums are still very sensitive because of the Amlodipine but I am working with the dentist to use softer brushes, better paste to not irritate them too much.

After the physical consult, he said he thinks “I have more fluid than what he would like for me to have” and to back off the salt. This is the first time in my “heart-patient career” that anyone has said anything about salt, because typically my fluid is under control. He said my legs look fine but that my chest shows signs of too much fluid. He gave no reason as to why all of a sudden my fluid retention is higher, and no recommendation on what to stop or start doing (other than salt intake) to help with this.

As for the FH treatment, he said he would like to try the “twice a year siRNA PCSK9 inhibitor which might come out in the US sometimes next year” – his guess -  (he was referring to Inclisiran - https://www.novartis.com/news/media-releases/novartis-receives-eu-approval-leqvio-inclisiran-first-class-sirna-lower-cholesterol-two-doses-year) to replace the twice-weekly Praluent injections that I take now. I have asked him again (http://livingwithfh.blogspot.com/2021/07/who-knows-more-about-fh-you-or-your.html) about adding Evkeeza to the current treatment and he said “that would be another option as well”, but he made no recommendations about it. About this, I am puzzled: my LDL is nowhere near the “target” number of 70 mg/dl or lower, but he did not recommend adding anything else to my current drug regimen.

So, a mix of findings and if I were to summarize, I would say:

-          Heart function is stationary (no idea what the coronary arteries are doing because we would need a cath angiogram for that)

-          Arteries are showing advancing disease

-          Cholesterol (LDL) is still elevated, not at ideal levels for my disease and my history

-          Liver function a bit modified

-          Quality of life/ symptoms (dizziness, muscle cramps, chest pain and shortness of breath) somewhat improved.

I walk gently towards The Holidays with kind of a mixed bag and lots of unanswered questions. But … it’s better than six years ago when I was walking in with “you must have open-heart surgery in one to three months at the longest.” So, I’ll take it.

Why Are Cholesterol Numbers So Hard to Understand?

Although my cardiologist will tell you that I know more about cholesterol than he does, this is truly not a rhetorical question. I am really looking for the answer to it. 

After 36 years of watching these numbers, I am as puzzled by my cholesterol numbers as ever. The good news is – I am still at the better end of where the numbers need to be. But the bad news is, although I have seen some normal numbers while on my current regimen of drugs, the current numbers are not normal anymore, and they have not been this year.

If you have read the previous entry (https://livingwithfh.blogspot.com/2019/05/fh-is-pain-in-butt-literally.html), you know that my numbers were very low at some point last year (around September) and then they started inching back up. My cardiologist has exhausted all the guesses he has had so far (I think!) as to why this happened, since I have not made one change in my lifestyle nor medication.

We have exhausted guessing about the quality of the drugs maybe going down, or the fact that my body might be resistant to Praluent all of a sudden (maybe to the other drugs, too?!) – we still don’t have an answer to this last theory. As of late, he was trying to test the idea that maybe I was not injecting my Praluent properly, so for the past three shots (this is six weeks), he had me come into the clinic and let his nurse inject it “the correct way”. Which I did. Her correct day was not much different than mine: we both inject in the thigh, alternating thighs every two weeks. The numbers, as you can see below, have dropped a little, but not enough to really consider them being in a new range.

Sigh …

The guessing continues, and at this point this is where we are: guessing, or wondering, still.

The next step is to meet with him next week and try to understand what the immediate future plans hold. Some things we talked about were:

• Wait it out and do nothing. Continue with the current regimen and just keep the numbers as low as possible; they are still some of the lowest ever, so this is good.
•  Another option is to switch to Repatha. Maybe I respond better to it than to Praluent?!
  Maybe we have given Praluent a fighting chance and this is the best it can do for me.

At this point, this last option makes me angry and deflated, because this means a renewed battle with the insurance company to get Repatha approved, when I thought I was pretty much in the “safe zone” with the Praluent, since they have been approving it for three years. Sigh, again … Also, I am not sure what this would mean for my bottom line: for now, Praluent is clearly their preferred drug, because I have a $0 copay for it. Not sure how much Repatha would be.

I will wait for the appointment with the cardiologist next week to see where we go from here. But so far, so not so very good, I guess, you can say. I keep reading to see if others experience this “plateau” stage at all, and whether there is anything in the current research about how Praluent works (or stops working or changes the way it works) in the long run. So far, researching and staying tuned is about all I can do.

Much health, everyone! And many answered questions to all!

“FH Is a Pain in the Butt. Literally.”

The title needed quotation marks because it was suggested by my husband after my latest appointment with my cardiologist. I hope after reading this post you will concur with its appropriateness.

As we all know, managing FH is a journey of discovery. You have the condition, and then you have the complications. You have some treatment and then you have some side effects that also need to be managed. Some drugs work, some don’t, some drugs work for a while and then stop. And then they need other drugs to help them up and then their little helpers stop working … After 20+ years of managing this disease with drugs I am still puzzled about what does work and why. And about what makes drugs (or other things, like diet, my body and its hormones and its natural disposition) stop working with me to keep the numbers low. I am always learning and always starting over, it seems.

I know that a lot of people who are just now being diagnosed want to know the magic trick or cocktail they need to be on that will ensure they can manage the numbers and prevent events. But the truth is, as I have found it: there is no silver bullet. It’s what works for you, and that answer can take a while to figure out. Just make sure you get good doctors that understand the disease and why and how it should be managed differently than regular “high cholesterol” and try what is available out there (there are lots of options now!), and learn your body along the way and know what works for you. That would be my advice.

And with that, yes, I am myself still looking for that magic trick that would make my numbers normal – which has happened just once since I was first diagnosed 36 years ago. As you can see below, somehow, and we don’t know why, my total cholesterol and my LDL (which is more important) were within normal ranges once in September 2018. But since they, they have been going back up. Since January of this year, we have been trying to figure out why this is. In the words of my cardiologist, “I wanna know what happened to those 60 mgs of LDL! I wanna give them back!” – but we have not managed to find a reason for this.

We know that the numbers dipped to normal levels only after we added the Praluent. So, naturally, we think the Praluent might also be responsible for the numbers going back up, somehow. We went as far as contacting the Praluent manufacturer, but we have not gotten very far with them. In the beginning they said there is no known evidence that a patient could build resistance to the drug, but the second time (more recently) when we contacted them, they said there is a very small margin for resistance (I cannot remember if they said there is 0.3 or a 0.03% chance of building resistance – is what their research shows. They said “it is not zero, but it is very small”.) The course of action now is to go to the clinic and have the nurse inject the Praluent instead of me doing it on my own, just to ensure that I am administering it correctly. They also asked me where and how I inject the drug, and I told them: I have been doing it the same way, in the side of my thigh, alternating legs every 2 weeks about the same time of the day for 3 years now. Now, the manufacturer rep said it needs to be injected in the top (not side) of the thigh, so we will try that for a while and see if the numbers change. Again, a word of caution for folks out there: the cardiologist and the manufacturer both told me again that the drugs work best when injected in a muscle and not in a fat tissue; so, not in your stomach, but in your thigh or upper arm, where there is more muscle. At least, this is the advice they gave me.

I am not aware of any other changes that could trigger these numbers to go the other direction for me, and the cardiologist did not suspect anything else to be the culprit. So, we will see. Maybe it’s time to try Repatha instead?! Not sure …

In addition to this journey, there is the parallel journey of making sure that my heart and my arteries stay healthy, or whatever “healthy” means to me. So, to ensure that, the cardiologist ordered for the first time ever a complete body scan (with contrast) of all my arteries. I say “all”, but I mean only the arteries in the trunk of my body, if you will: from my neck to my pelvis – so, no brain, and no legs.

His idea was that if the damage in the arteries in my heart was so severe three years ago when I had my surgery, then he is wondering about the status of the rest of my arteries in my body – is there an aneurism anywhere else like I had in my aortic arch? Are there any blockages that need to be opened up like the four that needed bypassing in my heart?! What exactly is going on?!

The test showed, as you would suspect, atherosclerotic disease of various severities pretty much in every artery scanned. Very few had mild disease, but most of them had moderate, or moderate-to-severe disease. The worst (moderate to severe) are my thoracic and my abdominal branches of my aorta, where “the infrarenal abdominal aorta in particular is severely narrowed down to a minimum cross sectional diameter of 0.6 x 0.6 cm just above the takeoff of the inferior mesenteric artery.” I am told that this diameter would be equal to about a 70% blockage. The severity seems to match the one in my carotids, which is between 65-75%. Just like the carotids, the course of action here is to do nothing right now, and just to repeat with an ultrasound every year and ensure that the blockage does not become bigger. If it gets closer to 90% then surgery for a graft or a stent might be recommended.

The doctor said that if this blockage would cause symptoms, because of where it is located (right after the aorta leaves the kidney area and before it splits to go to my legs), I would notice a sharp pain in my buttocks. I don’t notice that, but I do have pain in my calves when I exercise – he said he doesn’t think that pain is related to this, because my buttocks would also hurt in this case. Hence, the title of this blog.

So, as of right now, these are the things I need to watch pretty much every year for as long as I live:

• Cholesterol numbers (every 3-6 months, typically, but we’re doing them more often now because we’re trying to figure out why they have gone back up)
• Liver enzymes (every 3-6 months)
• INR (weekly) for the health of my mechanical aortic valve
• Heart echo (yearly)
• Carotid ultrasound (every 2 years)
• Thoracic and abdominal aorta ultrasound (every 1-2 years – this is new and the cardiologist will confer with a vascular surgeon before he sets a definite schedule).

Of course, at any given time when a new symptom comes up, he orders other tests, as well.

Some might say that FH and all the complications that come from it is a full time job. I prefer to look at it as a life style: it is part of our bodies just as much as the color of our eyes. We, people who have it, learn to incorporate all this knowledge and all the tests in our daily routine. We need to know that along with vacation once a year, we need to make time for a couple or three echos and 3-5 blood tests. Plus, daily medicine and a healthier than average lifestyle. Knowledge is power and knowledge and taking advantage of the medical developments out there will give us a healthier and fuller life. None of us have control over the length of our lives, but I at least try to have some control over the quality of what I have left.

I am grateful that we have the tools that allow us to know and manage this disease. We can be mobile, aware, and do pretty much everything we want to do in and with our lives. There are millions of patients out there suffering from so many other afflictions that do not get this lucky.

Onward, all! It’s the only way …