Yearly Carotid Ultrasound and New Numbers

Part of the FH and heart-disease journey is to familiarize yourself with a life peppered by doctors’ appointments. And yes, that is not a typo: there will be many doctors, many specialists, and many tests during a “normal” year of your life. 

Most of the time, I see these appointments as routine. I still chuckle when a coworker takes a whole day off for a stress test and is incredibly fearful when their doctor orders an EKG or a heart echo. There is no comparison in medical journeys, and I am going to be the first one to admit that.

But I only speak for me, now; and to me, these are “the easy” tests. In fact, I have met more doctors that agree that stress tests are a waste of time and money and they are seldom accurate or reliable. So, if they allow me the privilege to choose my test (which most of my doctors do, for whatever reason), I usually skip the stress test, and go for something more invasive even but hopefully more accurate. If, that is, my insurance also affords me this luxury. 

You will find sometimes that the insurance demands a lesser (even non-conclusive) test to be performed if it’s cheaper rather than approve a more costly but a more accurate test. No, the business of medical care, especially in this country, but we are not alone, is not a fair game. 

So, this month, it was time for my yearly carotid ultrasound. Ultrasounds are not invasive, by the way, and still believed to be the first in line for basic imaging.

The results of this test used to be a little worse every year back when my LDL cholesterol was hanging out around 250+ mg/dl. But since I started taking Praluent (in 2016) which brought my LDL down to 150 mg/dl (twice the target, but still much lower), the carotid ultrasound results have been pretty stable, or stationary, showing a buildup of plaque hovering around 50%. They have been so stable in fact, that some doctors argue that I don’t have to do this test every year anymore, that my plaque seems stable and with this amount, there are no interventions recommended. So, they say, we should move the test to every 2 or 3 years. 

So far, I have not been convinced that we should do that. I have seen cardiovascular disease go downhill in less than a year with FH, so I don’t trust my body that it will ever - regardless of how well the drugs perform - stabilize in such a way where I won’t have to watch what the disease is doing to me ... So, I insist we do the tests. 

Also, the impetus of my insistence on this particular test has been my symptoms. No amount of medical science and no amount of doctor “smarts” can convince me of something my own body flags as “not right”. 

For years, I have had numbness and tingling in both my arms, all the way down to my fingernails. It is worse with exercise and it is worse in my left arm. With exercise, my shoulder, and left arm, as well as the left side of my neck become numb, almost like a huge claw squeezes every bit of blood drop out of them! 

I bring these symptoms up with every cardiology (and vascular specialist) appointment and they take notes, but offer little in return. Others venture a guess of “well, that could be neurological”. And I did have neurological studies done to both my arms (I would not recommend them to my worst enemy) and although there were some findings (ulnar nerve neuropathy in my left arm and carpal tunnel in my right), the symptoms from these afflictions are different than what I feel when I exercise. The level and the place of the numbness is different, and the feeling of the “huge claw” only comes on with effort. 

Over the years, although my carotids have been more or less stable, some of my other arteries have started to see signs of more advanced atherosclerosis. Usually, they give me a percentage of the stenosis or plaque. This year, they spelled it in no ambiguous terms: “subclavian stenosis”. Not once, but several times in the test findings they emailed me. Some branches of the carotid artery (like the right external carotid) also appear stenotic, but the subclavian is pretty much stenotic, with no doubt. No other approximations or guesses of what the percentage might be. 

In full disclosure, these are the findings from the carotid ultrasound:

1. Left subclavian artery flow appears stenotic.
2. Right CCA demonstrates significant plaque.
3. Right ECA appears stenotic.
4. Right ICA stenosis less than 50%.
5. Right subclavian artery appears stenotic.
6. The Left ICA stenosis less than 50%.
7. Flow in the right subclavian artery appears stenotic.
8. Flow in the left subclavian artery appears stenotic.

**PSV is 125-180 cm/s & ICA/CCA ratio >2.0 is also consistent with 50-69% stenosis.

**Comments right side: PST noted throughout the CCA.

My doctor is yet to reply to all this. I will wait for another week or so and then reach out for more details from him, although I am not too hopeful he’ll recommend any course of action. I have been told time and again that without close to a 100% stenosis or an aneurism, there is not much they would want to do, regardless of the symptoms (which they are more than happy to just medicate, much to my dismay!), because there are too many risks involved in performing a bypass on the arteries or even more in adding stents. 

But this is why I insist on repeating the test every year: what if, from one year to the next, we go from "stable" to an aneurysm, or even a tear? What if, like this year, we go from “50% to stenotic”. 

In lieu of a doctor’s feedback, I, of course, turn to the internet. And this is what the Cleveland Clinic says about the symptoms for subclavian stenosis:

• Muscle cramps when you use the affected arm.
• Arm pain or tiredness when you use the affected arm.
• Tingling or numbness (paresthesia) in the affected arm.
• Dizziness
• Fainting
• Vertigo 

I have experienced all of them for years, except for the last 2. My dizziness occurs daily now. And most if not all of these are not related to ulnar or carpal tunnel neuropathy.

As I understand (and as I have lived) it, stenoses (many of them everywhere where there is an artery) are a byproduct of FH. Just the normal collateral damage that years of high cholesterol has done to your blood vessels. 

The little bit of a good news this month has been the continuing dropping LDL numbers (thanks to the new Evkeeza infusion which seems to be going well). 

I have to literally pinch myself every time I get the new values after my infusion treatment that shows my LDL in the two-digit range. As you can see, I come from a (“natural”) LDL of 520! I still cannot believe the LDL of 65 mg/dl is mine! I wonder every month if they got my blood mixed up with someone else’s. 

I cannot thank medical research enough for the advancements they have made during my lifetime. This disease that was nothing but a death sentence to me, when diagnosed at 8, has become something I can manage now. This is why it is so paramount that we encourage medical research going forward. It gives not only hope, but literal life to people!

The hope is that I am not adding more damage to my arteries by adding more cholesterol to what has already accumulated there for the past 45+ years. But there is plenty of damage done already and with an aging body and inevitable hormonal changes, I must still continue to stay vigilant and repeat these yearly routine tests, just to understand what is still going on and to have a chance to plan, if faced with an ultimatum. And as I have known several times in my life - ultimatums do happen ...

In this scope, regardless of doctors’ orders, I will continue to fight to know more and not less; to stay on top of every change and progression, such as it will be. 

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 ... 

COVID19 and Heart Valve Disease

If you're a heart patient like me, I am sure you have been paying attention to this year's tumultuous rumble about how we are considered "high risk" individuals when it comes to COVID19. But what does this exactly mean? Are we more at risk to get it? Are we more vulnerable for complications if we do get it? How does COVID19 really affect the heart? Does it affect its entirety? The valves? The heart tissue? The pump function?

If you have just been diagnosed with heart valve disease, or any other type of heart affliction that requires surgery, should you go ahead and proceed with the surgery now? Wait till the virus passes? Wait till there is a cure or a vaccine? 

If you do have a prosthetic valve, will it get infected faster and more severely from this virus, like your surgeon told you when you got it that bacteria might get it infected? 

If you want answers to all of these questions and a lot more insight, I do encourage you to read this free e-book (or watch the webinar that produced it), made available by Adam Pick, the founder of the Heart Valve Surgery website and community. I have found information here about heart disease in times of COVID, and also answers to questions I had even before this virus appeared in the world, all the way back before my surgery which was more than four years ago. 

Happy reading! You will not be disappointed. 

Click the picture to access e-book or a link to the recorded webinar

Between a Rock and a Hard Place with the Praluent Refill Authorization

… and why I am fighting for this still.

I have been sitting on this post for a while, not wanting to write it. There is so much negativity in the world today that my heart constantly aches. And I felt like this blog post will be negative, and I really don't want to add to that at this point in time, in our world, today.

But I felt, on the other hand, that I needed to continue with the saga of my trying to control my numbers and my advancing heart disease, and since a new chapter has been developing, I knew I needed to relate it. As usual, I hope this helps folks trying to stay healthy, out there.

A brief recap is needed: in December I was posting about how I was trying to refill my Praluent prescription and how the refill was denied twice, by the same insurance company that approved it 6 months before (see post from December here: http://livingwithfh.blogspot.com/2016/12/the-struggles-to-stay-on-praluent-and.html).

The doctor's (cardiologist) office faxed them all the information they requested, and they denied the refill twice. I received the denial letters on both occasions. What struck me as complete 'bogus' reasoning (for lack of a better word) was that in the first letter, they mention that I was denied the refill because I was not meeting 2 conditions, based on the information sent in by the cardiologist. After the nurse faxed them even more information, they sent the second letter of denial and this time, there were 5 (FIVE) conditions that they were looking for and I did not meet. So, it seems like for no reason, every time you give them what they want, they keep moving the finish line, raising the bar even higher. It definitely does not feel fair, but I am sure insurance companies are not in the business of fairness. But I digress.

I examined the list of the 5 conditions to see if I meet them. I am not going to reproduce them here, but if you want them, leave me a comment and I can share them with you.

They are written very much in 'lawyer talk' with 'either / or' and 'both or...' caveats, so they are a bit challenging to decipher, but I meet all five of them. This made me wonder if the nurse filled out some paperwork with mistakes in it, where she might have missed some of the conditions that I am sure I qualify for, unequivocally.

On my last week's appointment with the cardiologist, I asked the nurse if she can share with me what she sent to the insurance. She was not happy to share, but then again, this is not a happy nurse, usually. But she gave me a copy of what she sent, begrudgingly. She added that 'none of her patients ever gets approved for refills and from what she knows, about three quarters of all patients get denied for refills. They are approved for the first 6 months, and then get denied for the refills.” I asked the cardiologist, after she said all that, and gave me the papers, and he confirmed he statement. My cardiologist is also the head of the research program for Praluent and Repatha, for our state, so he works closely with patients just like me, as part of his daily practice.

When looking at the papers that the nurse filed, I could see she missed to check several boxes of conditions that I definitely have recorded in their files. The following things were missed and unchecked on the form she faxed to the insurance company:

• The diagnosis of Atherosclerothic Cardiovascular Disease (ASCVD);
• The ASCVD symptom of stable or acute angina;
• The ASCVD symptom of coronary or other revascularization;
• They checked that this medication (meaning Praluent) is being used in combination with another PCSK9 drug, which is incorrect;
• They checked that there is no presence of tendinous xanthomas in me, or any relative, which is incorrect.

However, they have checked many other boxes which still meet the 5 conditions as stated by the second letter I received:

• They sent my confirmed diagnosis as HeFH (heterozygous familial hypercholesterolemia);
• For the symptoms of ASCVD, they checked acute coronary symptoms and history of myocardial infraction;
• They mentioned that I have been on high dose of statins coupled with zetia for more than 12 months (since 2011);
• They showed that my LDL is higher than 100 mg/dl on statins (the value they used is 184 mg/dl and that was on statins, zetia and one month of 75 mg of Praluent) in addition to the ASCVD;

All I am thinking is that all the things that were missed could have proven to be even more convincing to the insurance company that I am really in need of this drug. However, I know this sounds wimpish and weak, but I cannot get my nurse to re-fax the complete information to the insurance. I have never found her to be extremely helpful, but on top of that, she is also so sick of dealing with the insurance companies and so sick of them continuously denying the applications.

I talked with the cardiologist and he agreed that several things were not checked, and he would ask her to fax it again. With me present, he asked her and she refused. She said “everything is on the paper and everyone gets denied. She (meaning me) is not the only one.” End of story. She also said she included the ICD codes which testify ALL of my diagnoses, but I looked up the codes on the fax sent to the insurance and two of them are for “mixed hyperlipidemia” and one of them I cannot find (I29.10). None of them say coronary heart disease, revascularization, myocardial infraction, AVR, or HeFH.

So, right now, I am pretty much stuck in between an incomplete application filed by my cardiologist's office and an insurance who refuses to renew the drug, even though they qualified me as a patient who needs the drug 6 months before.

What I know for sure is that I cannot have the cardiologist's office appeal this yet again. At least I don't know how to make them do it. But, I still have 90 days left from the last denial letter when I can appeal on my own, and I am getting the papers ready to be sent in today. Fingers crossed.

I am not sure if I could get an approval on my own, but the insurance papers say that I can appeal myself, and add more proof. I will use the papers already signed by the doctor, and add information that is missing in the paper which I can obtain online, from their web portal. Hopefully, this will add to the case.

And now the reason why I am fighting for this so much. During my lifetime, I have seen my total cholesterol levels in the 700's and 500's. For most of my adult life, my numbers have been (on lots of maximum dose of many drugs) in the 300's or, at best and rarely, upper 200's. After six months of Praluent, my numbers are finally in the normal range. 

My numbers, since April 2016, which was the last test before I started Praluent.
(click to enlarge picture)

When I saw those results, I was worried that something else might happen to me. I was worried that these new drugs are so powerful that they are going to render me dead if I quit them – they really did reverse my FH, judging by the numbers, and with virtually no side effects. I was elated at seeing the new values, but also scared, as if something powerful took hold of me. I felt like Bradley Cooper in Limitless. The drug is definitely working, but what happens when I stop taking it?!

There is also a belief that with improving the numbers, the cardiovascular disease will be reversed, too. Praluent (and Repatha) are too new to know by how much it would be reversed, but the studies are being done now, so we'll hopefully know soon.

All I am thinking, every day, is how my heart surgeon described the damage he had found in my heart arteries, and in my aorta and aortic valve, all because of cholesterol build-up. My major goal in life has always been to keep those numbers down, but I could never bring them so low that they would not affect my heart. And after an aortic valve replacement, an aortic graft, an aortic arch repair and a quadruple bypass, I know what all that build-up does to your heart. I was so incredibly lucky to have survived this!

Now, that I know there is something out there to bring those numbers in the normal range and possibly prevent further damage to my heart, carotids, brain, I don't want to leave anything un-turned to ensure I get access to it.

I feel like there are still things I need to explore, and it all starts with the first step. That step, for me, is to appeal once again and add more evidence to my case so that the insurance takes yet another look at my file.

And because when it rains it usually pours, this news came out just a few weeks ago: https://www.bloomberg.com/news/articles/2017-01-05/amgen-wins-ban-on-sanofi-s-sales-of-praluent-cholesterol-drug . Right now, Sanofi is appealing this decision, so there is a delay in this ban being implemented yet. For right now, there is still Praluent on the market and my cardiologist is supplying me with samples. But even if they are continuing to sell, being dependent on one doctor's sample supply is not a viable solution for treatment. I will continue to rebut their decision to not revew this and I will continue to update everyone on how it's going.

I know this fight is not mine alone, and that also gives me strength.

Much health, to all!

Milestone: Breakthrough Drug Approved

I think my family has been dreaming of a day where they can breathe easy and tell themselves "there is something for our child to treat this disease with" since I was 6. That's 34 years ago, for those who are counting. Well, their day might already have come!

 
As you already know by now, the PCSK9 inhibitor drug to treat cholesterol and potentially reduce the risk for atherosclerosis, heart disease and strokes has been approved by the FDA: as stated in this article. 

Europe had approved it the week before.

I am personally excited about the opportunities out there, but although I have access to this drug therapy here in my state, and although I would qualify for it with no problem, I am actually putting off taking it. At $8000-$10,000/ yearly, Cost is one concern, for sure, but the biggest reason is just wanting to wait for a couple of years to see if the results are really what's expected of it, and waiting to see if more long term side effects will be revealed with time. 

I just pray and hope that I will have the luxury of waiting.

Wake up Call? I Hope so!

Not sure if having a cardiology appointment the day before Christmas Eve is a good or a bad idea. If the news they share with you is good, do you really want to have a carte blanche on eating as much ham as you want when you already have heart disease? And if the news is bad, do you really want to ruin your Holidays having a confirmation about something you already know is bad enough?! Either way you look at it: I don’t necessarily recommend it.

I had a recent echocardiogram done to check the status of my aortic valve’s condition, as this is supposed to be done every 2 years and I have not done this since 2011. The doctor appointment on December 23 followed that test, to talk about the results. I just figured there is worsening in the narrowing of my aortic valve, but that maybe the progress is probably slow, because I am medicated and I eat moderately healthy. I just figured “oh, this is just another routine echocardiogram where I have some damage, but it’s manageable”.

Well, there was progress on the worsening of the aortic valve’s condition. But the progress was much more advanced than I thought. Nothing makes more sense to me than talking in numbers. The doc said a normal adult aortic valve has an area of 3 cm squared. A section through my aortic valve is 1.1 cm squared, down from 1.3 cm squared from 2011. He did say that I am a “little person” so I can get away with a smaller opening, but if it goes lower than 0.9 cm squared, he “will have to do something” – meaning a valve replacement surgery. The velocity of my blood through the valve has gone up from 2.6 m/s to 3.5 m/s – which means it’s more of a struggle for my heart to pump blood through one of the main valves. Also, my left ventricle and atrium are enlarged, which shows the strain on my heart trying to pump through a narrowed passage.It's like looking down the barrel of a gun.

If you want a visual of what my valve looks like, I found this site that pretty much shows it: http://www.heart-valve-surgery.com/heart-surgery-blog/2010/07/02/aortic-valve-size-normal/ - and just like the second picture shows it: mine is bicuspid, too. This picture pretty much rings home and wakes me up with a jolt.

So, “happy holidays” to me, I guess. I have known about my cholesterol for 33 years now. But for the first time in my life, this news really, really woke me up! As we talked specifics about valve replacement surgery, how, when, open heart vs catheter surgery, etc, I kept telling myself “this is serious now. This is the time. This is the time when things get real”.

As much as I have loved foods, all my life, for the first time ever, I really started feeling a repulsion against all things cholesterol – even ham and bacon. Especially ham and bacon. I came home and started browsing the internet and my Dr. Ornish books for new recipes. I am working on new rules, 33 years later, and I am actually excited about kicking this in the a$$! Finally. I guess nothing wakes you up more than facing 40 and hearing that your heart is giving up. I am so blessed that I have done everything I ever wanted to do till this day. So grateful that I have not yet had a heart attack or a stroke. With every beat of the heart, and every blood drop, I cannot tell you how much gratefulness flows through my old, aged, and clogged up veins for everything that I have been allowed to do so far in my life!

So, I have stayed away from pretty much all protein that is not 100% fat free so far, in the past  couple of days – quite a feat if you knew what I had in my fridge – ham, eggnog, homemade mayo in my chicken and potato salad, and so forth. No, I don’t make all these all the time – only this time of the year. But no more! 

If I don't go on a drastic diet and a drastic (for me) exercise regimen now, I am looking at open heart surgery in 3 more years, if the progress stays the same. But it could go faster, given my advancing age. I am definitely not ready for that. 

 

I am not sure what my new “diet” is, but I can tell you I have cleaned up my fridge and freezer and simply threw away everything that was not fat free, low fat, or too much fatty meat. Done. Not looking back! Not this time. Just hope and pray it’s not too late!