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 ... 

Supporting an Organization with a Heart

Please read and, if you are able, consider making a tax-deductible gift (or buy a shirt) to this amazing organization. The fundraiser is still running until June 15, 2024.

I thank you deeply in advance! 

Imagine that you are a child  and you are 8 years old. Your parents tell you that you have this thing in your blood called “cholesterol” and you have way, way, way too much of it (about 7 times more than everyone else).  They talk to you in terms in which you can understand: “Honey, you know how grandpa cannot walk because he had that brain accident (a massive stroke)? You know how grandpa needs to check his blood levels every month and his blood pressure with the machine every day? Do you know how he will get to spend all of his days in bed for the rest of his life? Well, you have the same problem as grandpa. But all this happened to grandpa when he was 50. All of this and more will happen to you when you will be 25.” 

They tell you that you have a disease that gets passed on from generation to generation, called “familial hypercholesterolemia”, but that your kind of it is “much, much more severe and more rare” (“you’re probably one in a million people” - they say) than your dad’s, and your grandpa’s (dad’s father), and all of dad’s uncles and aunts. 

You don’t really understand, and you don’t really think that 25 is really that soon. You are 8 now. You got plenty of time to grow and be an adult by 25 and do so many things, go to college, even! 

Then, it gets worse: your parents are desperate and they keep taking you to doctors to figure out what to do with you, to treat you somehow. The doctors tell you not that you will be paralyzed like grandpa, but that you will be dead by 25. Others are even more grim, they foresee that you’d be dead by 18. 

And you feel special, in a way, like a kid would, but as you age you become more curious (not to mention desperate, too): why doesn’t someone do something to fix this? Are there any people out there that have exactly what you have and did they die at 18 or 25? Did they get married, did they have kids? How did they survive this? Anyone?! 

And for years, you don’t meet anyone who has what you have, outside your own family. But none of the people in your family are this “rare”, nor this “severe”.

For years, you don’t have access to all the new medications, because you either live in the wrong country, or you go to the wrong doctors who don’t know to give them to you. 

When you go to doctors and you tell them that you have “familial hypercholesterolemia”, they write down “genetic dyslipidemia” which confuses the heck out of you - but they don’t have the right disease name in their medical records, so they use what they can. You have a  disease that is so rare, there is not even an accepted proper name for it .... 

Over the years, I had to find my own way through the maze of various drugs most of which didn't work, and the armies of specialists that tried to treat me but most of whom failed. But then in 2016, right after I had my 13-hour open-heart surgery which fixed my heart after the cholesterol had wreaked havoc on it for 40 years (getting on some treatment in my early 20s allowed me to cheat death and I made it kicking and screaming into my 40s), I learned about The Family Hearth Foundation. 

This is where not only did I learn more than I had ever known before about my disease, but I also met my tribe - people that not only had “familial hypercholesterolemia”, but people who had the same form of the disease (the “more severe and more rare” kind), the “one in a million” people. I have learned from them how they have lived and treated it. I finally learned the proper name for my disease (“homozygous familial hypercholesterolemia”) which taught me that not only my dad but also my mom has this disease - something we didn’t know till I was 40. Learning about genetic testing through the resources at The Foundation, I asked my doctor at the time to allow me to be genetically tested and the result of that lead us to my mom’s diagnosis, and later to my sister’s and nephew’s diagnosis, too.  

I learned about treatments that are designed for my disease particularly and I have learned from others how they felt after following the different treatments for it. I learned about the side effects, the do and don’t, the gotchas for each treatment. I learned how expensive, or painful, or full of side effects they are. This was gold to me - having a real-life connection with people like me.

Through the association they have with specialists, medical professionals, researchers, geneticists, the Family Heart Foundation provided me with science and facts about my disease. Through their membership and community reach, they have given me a family - people like me who are fighting to survive every day. Listening to their stories, meeting them would not have been possible without the open forum that The Family Heart Foundation provides tirelessly through their Ambassador programs, through their Summit events, through their HoFH gatherings, through their webinars and interviews, and through so many more resources. 

They save lives by advocating for things like early screening of all family members, they save lives by finding the right medical resources for people that are newly diagnosed and don’t know where to turn.  They educate family doctors, nurse practitioners how to diagnose patients early so they can prevent major events like heart attacks and strokes which are the most common complications from unmanaged cholesterol levels. Ultimately, they have fought for our disease to be recognized by the proper name: through their tireless advocacy, we finally got an ICD-10 code of our own in 2016. So, now we are real. In every way.

They even bring together small, confused little children  like I was, that go through tough treatments at an early age and they build a community for them and their parents, too, so they would feel less alone and less confused. 

FH is often an invisible disease until something terrible happens. It is estimated that one in 250 people (and someone in their family, too) have it and yet about 70% of these people don’t know they do. Some of you have four or five times more Facebook friends than this, so you do the math. The Family Heart Foundation won’t  rest until every person is diagnosed and has a plan towards health, if they choose to. 

It is because of all this and more that I support The Family Heart Foundation every year. I am proud to be an ambassador for this organization that gives such hope to droves of hopeless individuals who are scared and lost when they first learn their diagnosis. 

Words can never express how grateful I am for everything they do and for the appreciation I have for  the impact that they have had on so many of us, including on my own family. 

Thank you! 

Here’s the link to their current campaign, if you would like to support a cause that saves lives and builds hope. 

What Happens to a Prior Authorization When You Switch Doctors?

The drug in this story is Evkeeza, a drug used for HoFH patients in addition to other cholesterol-lowering therapies and which requires prior authorization from the insurance company before your doctor’s lab can administer it to you. This is an infusion drug, which involves you going to the infusion center and getting an infusion the strength of which is calculated based on your weight for an hour every month. 

So, what happens to the prior authorization approved by the insurance company for the doctor that originally prescribed the drug when you switch doctors?! 

To make a really long story short, the answer to this question is: the prior authorization given to your previous doctor is null and void. Your new doctor has to apply for a new one with the same insurance (if the doctor agrees to prescribe it to you), since the doctor’s office is responsible for providing the treatment (not just prescribing the drug). 

To make a really long story even longer: after waiting for two and a half years with the first doctor to even understand what this drug is, get it prescribed and approved, and waiting for the first doctor’s pharmacy to understand what the drug is and how to order it, you now have to wait even longer for the new doctor to apply for the same prior authorization (hope they don’t make a mistake on the request form), and hope that you get approved the second time. 

So, now your two and a half years are more like three ... 

Evkeeza has been approved since February 2021. I asked my first doctor in the summer-fall of that year if he thinks he can get me approved for it. He said he had never heard of it and will research it. It took him till the fall of 2023 (that is two whole years if you’re not counting) to actually prescribe it to me. After his office figured out what the drug is for and how to fill out the paperwork for the prior authorization, I got approved for treatment (for 6 months) in February 2024. Since then, I have been waiting on the first doctor’s pharmacy to figure out where to purchase this from. And this puts us in May 2024. Again, if you’re not counting, two months (soon to be three) have passed from my 6 month-approval, so even if I got the infusion tomorrow, I would only be eligible to get 4 infusions, not 6 which is what I was approved for.  

I called the maker of the drug (Regeneron) and asked if they could help get the drug to the first doctor’s pharmacy. They responded with “Why, yes, yes we can, but we need to know your doctor’s information.” I sent that to them in March 2024. Since then, I have had no communication from the manufacturer, the doctor, or the pharmacy that I can start my treatment. 

Finally, this month (May 2024) I am moving to a new doctor who has heard of the drug, has gotten it into their infusion center and is even treating two patients with it. Score, on my end. Except ... the previous prior authorization does not work for the new doctor. 

His office applied for it last week and now we wait for the insurance to re-approve it (although the patient, my coverage and my medical condition and cholesterol levels have not changed) yet again (hopefully). 

Since this is a new doctor and they don’t have my whole history of diagnoses, treatments tried before and current therapies, I sent the new doctor the following:

• My cholesterol levels with and without any therapy

• My current cholesterol levels (my current LDL is 250 mg/dl - far from my target of 70 mg/dl)

• My surgical report from the open-heart surgeries from 8 years ago (that documents the massive coronary artery disease already present in my arteries)

• My genetic test results (testifying that I have HoFH)

• The prior authorization approval from my insurance for the previous doctor

I spoke with the new doctor’s team and they said they feel confident that the approval will be granted but that it might take about a month (from what they saw before) to receive it. 

I also spoke with a nurse with whom I am connected from the insurance company and she said although she is not in charge of the decision, because she knows my history, she also feels like this is just a formality and that it will be approved. She also can check on the status of the application, so this is a good resource to have in your corner, if your insurance offers it. 

And thus, bureaucracy wins. Healthcare takes a step back. 

In the meantime, I wait - not too patiently, I’ll have to say. And also, more than anything: I hope that once all this is said and done and I do get the treatment, finally, the treatment actually works ... 

Here’s hoping indeed! 

Time to Turn Over a New Leaf

As you might remember, I posted in January of this year (https://livingwithfh.blogspot.com/2024/01/even-after-41-years.html) how defeated I felt when the blood tests showed that my total cholesterol was sitting at 423 mg/dl, while my LDL cholesterol was at 358 mg/dl. These are no numbers to feel "safe" at when trying to survive with FH or cardio-vascular disease ... 

I also mentioned in January that the cardiologist promised that he has additional things he wants to try, like switching up drugs or trying to approve me for a new drug (Evkeeza) which is an infusion that I would get every month. In January, all he did was to switch my statin drug back to Lipitor (which has worked for me for 20 or so years) but only at a lower dose (40mg instead of the 80mg I used to take before my liver levels decided to go crazy last year). He was hoping that the lower levels will protect the liver more. 

In February, he repeated the blood tests, and my cholesterol levels did go down slightly, at 350 mg/dl for the total cholesterol and 279 mg/dl for the LDL. My liver levels were also normal. The cholesterol levels were still far from ideal! In February, I also received a call from the infusion center from my cardiologist's medical system that informed me that the medical system has not only not heard about a drug called Evkeeza, but they don't even know where to buy it from. So, in the meantime, despite the fact that my insurance approved me to be on this specialized drug that requires prior authorization, despite the fact that a doctor in their system recommended it, they cannot administer it to me because their pharmacy is not sure where to buy it from. 

LDL Cholesterol still high in February 2024

(with 40 mg Lipitor, 300 mg Praluent, and 10 mg Zetia)

I called the manufacturer (Regeneron) directly to ask what are my options here, and they just wanted to talk to my doctor. They asked that I send them the insurance's approval for the drug, and the information about my cardiologist. I did that about two weeks ago and I have heard nothing. I have heard nothing from the drug's manufacturer, nor from the infusion center (who promised they would apply to their pharmacy system in February to find out how they can order the drug). I have also heard nothing from my cardiologist about adding more drugs, or switching anything up in my regimen. 

So, it's time once again, to take the matters in my own hands and move on. Some things I have changed myself for now: 

• With the help of my family doctor, I got a liver ultrasound done because I wanted to see if there is any visible damage to my liver or any significant (or new) fatty liver disease that might explain the elevated liver levels. The ultrasound confirmed once again the presence of tumefactive bile in my gallbladder, but no other changes to the liver. This gave me some reassurance that there is no damage done to the liver yet. I also have been told over the years that the really dangerous levels of AST or ALT are in the thousands and mine have not been that high. At their highest, they were still three times the recommended levels (at 125 and 95 U/L), but not as high as in the thousands. So, I am hoping we are still in some sort of a safe zone. 
• I am staying off the Bempedoic Acid (Nexletol) which has been shown to have some impact on liver levels. After all, I have been on Lipitor for 20+ years with virtually no liver levels elevations and it's been only in the past could of years (since adding the Bempedoic Acid) that the levels have been creeping slowly up. I also know from experience that other drugs can make the statins more toxic, so I am hoping that eliminating a drug that is known to also be toxic for the liver will help the statin work again, more "cleanly". 
• I have increased the Lipitor dosage from 40mg back to 80mg which is where it's been for 20+ years. 
• I am looking for another cardiologist (finally!) in another medical system, hopefully one that can accommodate the Evkeeza infusion. I will ask them to recheck my liver values once I see them in May. I have waited for two years now for Evkeeza and even when I got the insurance's approval (usually the hardest part of the whole process!), my current team cannot provide the treatment for me. I also am the only one who keeps going back asking for answers, changes, more appointments, more tests - without not much initiative from the doctor. The one thing even worse than the cardiologist is the vascular team who does not treat my abdominal severe stenosis very seriously. So, it's time for a new team. After six years of allowing this current team multiple chances with no changes for the better in my health, it's time to move on. 
  At a recent seminar offered by the Family Heart Foundation, we were told that in our case "Time is plaque" - and that is so true! Time is always of the essence in FH. The more we wait around with improper (or not enough) treatment, the worse our arteries are getting and the sooner we move towards events that we might or might not survive.  

For now, I am waiting (not so patiently) for my appointment with my new cardiologist and will take it from there. 

I chose this new practice because I know they are the only clinic in my area that does LDL Apheresis, so I hope that this means that they must understand lipids to a higher degree. 

When I called the clinic to make a cardiology appointment, I asked that I need to see someone who specializes in lipids and ideally someone who specializes in hard-to-treat lipids, like those from familial hypercholesterolemia because this is what I have. I also asked if I could see an interventional cardiologist, one that also works in the cath lab and can understand cardiovascular damage done to the heart arteries. 

The receptionist made the appointment with this specific cardiologist based on my specific request. So, I am hoping I can turn over a new leaf and start a new journey to lower levels, first, and better health for my arteries, ultimately ... 

Will report back! Much health to all, in the meantime! 

Eight Years Later

I read a meme on the internet the other day. It said "You're only as old as your heart is." So, I must be 8.

It’s that time of the year again - February 11th ... 8 years ago today, I was dead. They made me dead. Only to bring me back to life, if I (and if they) was (were) lucky ... And lucky I was and lucky I am today, too.

Eight years ago today, I had my open-heart surgery. My big, scary, always-had-known-I-would-need-it-one-day surgery ... Every year, on this date, I skim through my operative report and I feel humbled, small, and oh, so beyond-words grateful for everything that came together to save my life and give me a new lease (renewed now for the 8th year) on life ...

I went into the surgery with 3 diagnoses. After the good man, Dr. John Mitchell from the Provo Utah Valley Hospital and his team, were done with me (after 12 and a half hours or so), they put down on paper 5 diagnoses ...

Some scary stuff! Each one of the 5 diagnoses is enough to independently kill someone. I, ever the overachiever, needed to have not one but 5 risks for cardiac death ...


But God and fate were not done with me. I do remember when going in that I told my family: I am not scared that this will kill me. I feel like there is so much more life after this for me. I feel like it’ll be hard, but I will make it ... That premonition or guess has come true ...


There has been so much life after that day ... There have been trips, and new jobs, and meeting new people, and spending time with our families and friends. There has been learning and trying new things, shooting (maybe) millions more pictures, and squeezing more kitties ... There has been building of memories, watching nephews grow and become almost young people now.


There have been some of the biggest heartaches of my life too. It’s like they prepared me for what was to come by fixing my heart to be able to take more sadness: loss of jobs, loss of friends, moved across the country, our kitty died alone when we were traveling, a parent got cancer, we buried another parent ... But my heart keeps going ...


Just like the good doctor said in one of the days after the surgery while I was still in the hospital: it was “up and at them after I left the hospital”. I guess I never quite felt like “up and at ‘em” to me, exactly (I felt as slow as a snail and as weak as a feather), but compared to that day, when I was practically dead, with the heart-lung machine breathing and beating for me, it must have felt like “up and at ‘em” even if all I did was little as getting out of bed in the morning ...


But I have been lucky to do much more than that ...


After learning my new routine in the first couple of years, I have been able to have a new life, a different life than before, but still a good one.


The operation (or, as you can see from the operative report excerpt above the “operations”) did change my entire life’s routine: the anticoagulant forces me to manage my food even more closely than I normally do, it also has me OCD paranoid about measuring my INR (blood “thickness” levels) weekly and I never leave the house for more than a week without my INR machine; I get tired easier than before, still; I have learned how to live with permanent neuropathy in my left arm which still feels like my hand is permanently stuck in a bucket of ice; my chest still feels different than ever before, including my spine and ribs - it cramps and it hurts at certain times; my medical wires left in my chest forever after the surgery still poke into my chest wall at times (and I have not figured out why and when they act out); my blood pressure has been on a new and more noisy roller coaster than ever before, my heart muscle is stiffer and it needs extra meds to relax ... and I could go on and on ... Living through increased inflammation and through several years of Covid that threatened to kill people like me, with a cardiac condition and with high cholesterol, has meant yet a new way of life. A new lesson in living this new, changed life ...


Yes, things have changed. But what I now know for sure is that we are adaptable. We learn to grow around the boulders put in our path, and still are able to shoot towards the skies, kicking and screaming ...


I have a much better understanding of limitations and of giving up things to feel “normal” ... But I also cherish every second of every accomplishment of just going through the day and the night and waking up to a new morning day after day ... There is no price I can place on this blessing and this gift called life ...

First day after the surgery, in the ICU. And 8 years later ...

Even After 41 Years ...

Happy new year, everyone! I truly hope for a better and healthier year, for all of us! 

It’s a rainy and unseasonably warm evening here in North Carolina tonight. It’s around 65 at 8PM which is unreal for a January day ... But there you have it ... The fog is rolling in, making it feel very British ... 

Last year ended on a not so good note for me, healthwise ... My liver values (AST and ALT) started going up and my cardiologist and I have no idea why. We have not changed anything in my drug regimen, nor in my diet. Outside of a (much) higher level of stress, we cannot figure out what could be making my liver enzymes go up, and - at the same time - my cholesterol levels as well. Maybe a sign that whatever drugs need to be processed in the liver are not being processed well anymore. Or who knows?! 

Since September of last year, when my LDL levels jumped from 125 to 144 mg/dl and my AST/ ALT levels jumped from 47/44 to 125/95 U/L(normal values are up to 41/49 U/L), we have been trying to switch drugs around to see which one of them if any, are causing the jump in the liver enzymes first ... 

We know that taking the statin drug (Lipitor) away from the regimen makes the enzymes come to completely normal levels. Leaving the statin in but taking the Bempedoic Acid (the other drug in my cocktail that is known to modify liver enzymes) away makes the enzymes close to the normal levels but not fully normal - still a little elevated. 

We never tested for the cholesterol levels during this time of starting and stopping the drugs, because we figured it would be high since we were reducing the amount of drugs in the mix. In December, the doctor replaced the statin (Lipitor) with a less strong one (Pravastatin) for a month, then he did a cholesterol check in January. The liver enzymes were totally normal, but the cholesterol ... oh, wow! 

At that time, while taking Pravastatin (40mg), Ezetimibe and Praluent, my cholesterol values were 423 mg/dl (total cholesterol) and 358 mg/dl (LDL cholesterol). Both the doctor and myself were shocked. Shocked and defeated that the Ezetimibe and the Praluent were not making more of a difference. Last time my LDL cholesterol was anywhere near this level (it was 344 mg/dl) was in 2014. 10 years ago! 

My big LDL jump to 358 mg/dl - January 2024

It’s been over 20 years since I have measured my cholesterol with no drugs at all. In 1999, my total cholesterol was 526 and my LDL cholesterol was 475 mg/dl with no drugs. That is, if you will, my baseline ... As you can see above, being on three drugs is not moving me  much more away from those numbers. None of the promised “up to 50% reduction” in LDL cholesterol with Praluent is happening in my case ... 

As many of you know, an LDL cholesterol of 300+ and a total cholesterol of 400+ spells more than “trouble” for anyone (not just for an FH patient), especially one with a massive history of cardiovascular disease, open-heart surgery, multiple bypasses, aortic valve replacement and a heart attack ... 

I have always felt like a timed bomb but seeing these numbers again in my chart is scary. Also, given the fact that I am no longer 24 like I was in 1999, but almost 49 now ... this doubles the worries. 

For now, the cardiologist put me back on Lipitor (40mg instead of the 80 mg I was on when the liver values were going up) and I continue to take Ezetimibe and Praluent. We need to test for both the liver and the cholesterol in February and make a decision what to do then. 

He says “he has other options”: he can add the Bempedoic Acid back in for another drug, if the liver can take it - we’ve been testing for liver enzymes every month since September. The lab people and I are on a first-name basis now. We all know what each other has done for Thanksgiving and Christmas. I know that Bill, the nurse, has a step-mother-in-law with dementia and Michelle, the front-desk receptionist has a pitbull lady dog and that her birthday is in April, like mine ... 

The cardiologist is also wanting to start a new drug, Evkeeza - an infusion that works differently than any other drug I am on, but not before we stabilize the liver ... I have asked to try for this drug for years now, and if you have read here before, you know the stories ... 

I have always felt like a Guinea pig since I was 8 and they discovered my FH. And things sometimes never change, no matter how far science goes ... So here’s back in the spinny wheel for a while to try to get my numbers down again and keep my liver (and the rest of me) healthy, otherwise ... And as always: there are options. I can’t help but feel almost back at square one, though which is a bit of a let-down after 20+ years of doing so much better than this ... 

More to come, I am sure, in future posts. 

Much health to all of you! 

The Wins and Up-hill Journeys of 2023

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Happy New Year!