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!