It’s a Numbers’ and a Guessing Game

As I am watching the splash-down of Crew 9 coming back from the orbit back to earth on CNN (Suni Williams and Butch Wilmore), I am thinking: my cholesterol levels did a bit of a go-up-in-the-stratosphere-and-come-back-to-earth trip in the past few months, as well. 

To recap: I added Evkeeza (https://evkeeza.com/s/) to my drug therapy in June 2024. At that time, my LDL cholesterol was 238 mg/dl. After several months of treatment, the LDL went down to 67 mg/dl (and no, I am not making this up). Evkeeza’s site boasts around a 50% reduction in LDL cholesterol. Mine was 71%. My target LDL is 70 mg/dl or lower. I was at target for the very first time in my life. 

At that time, I was taking:

• 80 mg/ day  of Lipitor (a statin)

• 10 mg/ day of Zetia

• 150 ml/ twice a month of Praluent (an injection)

• Evkeeza (a monthly infusion) (the amount is based on your weight at the time of the infusion)

Because we saw these amazing results, my cardiologist believed that we could take away some of my other medications that might not be as effective and see if we could keep the low number on fewer drugs. He suggested that I would take just the following:

• 40 mg/day of Lipitor (eliminate 50% of the Lipitor I was taking)

• 150 ml/ twice a month of Praluent

• Evkeeza infusion (monthly)

• He eliminated Zeetia completely. 

And this is when my LDL cholesterol promptly took off back in space, as it were, climbing all the way to 142 mg/ dl (more than doubled) in two months. (https://livingwithfh.blogspot.com/2025/01/here-we-go-higher-again.html) 

So, we assessed. I increased the Lipitor back to 80 mg, but that only got the levels down to 121 mg/dl after a month. We then added back the Zetia and after one more month we got back down closer to my target (which is 70 mg/dl) to 74. 

So, the conclusion of this little experiment was that the magic sauce is in the combination of all the drugs together that makes a stronger difference. So, we are back on the same therapy we started with in June 2024. 

This is what FH is: a numbers’ and a guessing game. A trial-and-error adventure. And you must. Never. Ever. Give. Up! 

The biggest compliment I have ever received was from my heart surgeon earlier this year. He was pleased with my heart condition (after 9 years since surgery) and with the way I am managing my cholesterol. He said: “The main contributor to your health is only one person: yourself. You followed through. You looked for answers. You never stopped. Congratulations.” 

I felt so humbled! 

I think the world of this man, and of a handful of people in my medical team. I think the world of so many people who coached me through my journey (including The Family Heart Foundation - https://familyheart.org/, and friends who worked for drug manufacturers, my parents who had limited resources but taught me a healthy way of living, despite their own poor choices), so for a medical professional that I respect to share this was incredible! 

And this is what I want to tell you all who are managing this disease or who know of someone who does, from the bottom of my heart: never give up. Always look for answers. When you don’t like your doctor, change them. When the insurance says “no”, appeal their decision. You might have to do it multiple times, but don’t give up. When one medicine doesn’t work, look for alternatives. I have been at this for 42 years now and I have seen so much progress in medications that are allowing us to live longer and healthier lives. It’s possible and you can do it!  

My most recent journey of roughly 9 months (almost the same amount of time that Crew 9 spent on the ISS in orbit) is finally over and it is pictured below. And I hope it stays down on earth for a while. 

A former astronaut said on the TV tonight that the most he missed in orbit was the color green and everything about it - the trees, and the grass, etc ... I like to see myself back in the green too.

The Heart Month

US president Johnson announced that February was Heart Health Awareness month in 1964 “to give heed to the nationwide problem of the heart and blood-vessel diseases, and to support the programs required to bring about its solution.” To this day, we still fight against the number one killer of all people - heart and vascular disease. 

February is a peculiar month for me. In addition to celebrating Heart Month along with everyone else, I multiple-times celebrate it as a heart-disease survivor and a member of a heart-diseased family. 

Every February 11 I celebrate the anniversary of my open-heart surgery which took place in 2016 (https://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html). This year, I even got to travel back to Utah where my surgery was done and meet again with my surgeon. It was such a gift! 

After 9 years, and with many ups and downs, my heart has continued to change (you have probably heard the adage “once a heart patient, always a heart patient”). But despite the changes, I continue to live a full life (which is adjusted for me), and I am counting my blessings. 

February 20th is the anniversary of this very blog. I started this writing adventure on this day in 2011 out of a complete desert of news and information about Familial Hypercholesterolemia. There was nothing out there to give me a map on how to navigate this condition and how to live with it successfully - because I was determined I was going to live with it at any cost The only semblance of a map was what I had learned from my family and the prospects were not very happy - everyone in my family with FH had had hard lives, marred by lonely sick years, disabling symptoms, many complications, long, lingering suffering, and early deaths. I was determined there has to be another way. 

I wanted to document every step in my journey - all the tests, all the ups and downs of the treatments, all the successful and failed attempts at any medication so that others who I knew were out there could have a “real-life experience” reference to know what to expect through their own journeys. I figured that if I could reach at least one person like me, who was looking for their “tribe”, my job and the job of this blog is done. I have not put any advertising and any thought into making this blog more than what it has been from day one, but the handful of people that I know I have reached with my stories are such a gift to me! I thank you all for reading, and for your feedback over the years! I know (you have told me) you find a place of belonging here, and for that I am grateful. 

A year after I launched this blog, The Family Heart Foundation (then, The FH Foundation) (https://familyheart.org/) was founded and that truly threw our visibility as FH individuals into the stratosphere and amplified our voices infinitely. 

February 28th is also Rare Disease Awareness Day. I have known I have “a rare form of FH” since the age of 8 when I was first diagnosed. Officially diagnosed with HoFH at the age of 40 after my open-heart surgery, I am now officially a “rare disease individual”. 

Along with finding out that I am now in this cool crowd of “rare disease people”, finding out that I have a rare disease also helped my family: certifying that I had HoFH meant that both of my parents have FH. Until then, because we never knew about mom’s family history and because her levels were considered borderline (because they were always much lower than mine and lower than my father’s), no one ever suspected her of FH. She was 62 at the time we found out my HoFH diagnosis and she had advanced cardiovascular disease already.

This year, I also celebrate the fact that mom survived her first (that we know of) heart attack a month ago almost to today. She went into the ER with difficulty breathing. Being a lung cancer patient, everyone suspected it was her lungs, but it was in fact a blockage in her aorta that was not sending enough oxygen to her body. She needed a stent right away because the blockage was significant but given her many co-morbidities and her weakened health they advised against it. They kept her in the hospital for a week and treated her blockage with medication, then sent her back home with 6 new drugs among which a statin. She is 71. To me, she is still very young. 

FH is not a joke. It is a silent killer and left untreated and ignored it won’t ignore you, I can promise you that. There will be a time when it will speak. And it will be loud, and often not pretty. 

Below is a picture of me and mom about 6 days or so after my surgery. She came to babysit me while I convalesced but in an odd twist of fate I had to babysit her, as she got sick with the first symptoms that eventually lead to diagnosing her lung cancer. 

This year, as she had her own heart issues, I wished to God that I could have been there for her (she lives in Romania), but technology is all we had to keep us connected. 

I am grateful for the genes she gave me. Good and bad. Bad, to force me to learn how to be a fighter and not take one day for granted, and good to be stubborn and strong enough to fight that fight and overcome. 

She and I are both moving forward - scarred but more aware. Weak but still fighting. 

Everyone should celebrate heart month because, as my sister reminded me the other week, “we all die of heart disease.” It is the only organ that decides when they call it. 

Stay healthy, friends and heart warriors, and always know your numbers (cholesterol, blood pressure, pulse, and oxygen) and stay informed. I am living proof that advancements in medicine are working miracles and not only improving but also prolonging healthy lives nowadays and allowing us to build up new memories. Let’s hope this continues! 

Happy Heart Month to all! 

9 Years

When I went into my open-heart surgery, 9 years ago today, I never thought I would live to see 9 more years. My heart was in bad shape. The surgery ended up being even more involved than the plan outlined it to begin with. But more than that, I never thought I would live a full life after the good surgeon took apart my heart and stitched it back together. 

I knew I had more life in me, and I knew this surgery was not going to be the end of it. But I never imagined that 9 years later I would look back and say that I have lived a full life, either. 

But here I am. A life beyond any expectations.

A full job, all the trips I wanted to take. Buried one beloved pet. Buried one parent. Taking care of the second one from across the world. Watched my nephews turn into teenagers and young adults. Moved across the country. Zoomed over the ocean a few times, zoomed across the country for work, sometimes on my own. Medical bracelet on my right wrist, and tons of notes in my wallet that tell the world that I have a heart and a clotting condition (because of my mechanical aortic valve and because I must take Warfarin to prevent my blood from clotting and blocking the valve). Praying that whoever needs to know this information will find it. And off I go. Always cautious, always with all the risks in the back of my mind, always prepared for what would happen if I can no longer speak for myself, but I am not about to lock myself in a bubble, either. Time is always of the essence and time is short, for all of us. 

As I said 9 years ago, I say this again today: once you’re a heart patient, you are always a heart patient, but you learn to live with your limitations because there is no other way. Your limitations become your new boundaries, but you learn how to fit your dreams within those boundaries and you move on. 

There have been scares over the years. In the first couple of years, I went to urgent care when I cut my fingers in the kitchen and learned how to deal with the bleeding. I went to ER every time my blood pressure was off and I was dizzy, light-headed, or my pulse would race.

When I travel, my INR (coagulation marker) tends to go very high. I am supposed to keep it at 2.5 (for the upper limit), but it sometimes wanders off to 3.8. I am terrified I would bleed internally so I limit my physical activity for fear of bumping my head or something and I eat more greens to try to lower my INR.

I fell several times really hard. One time, I got a large open wound (as big as one whole thigh) in the muck of a dirty beach. I thought for sure I'd get some flesh-eating bacteria in my blood and the valve will have to be replaced, but it didn't happen (I drowned my wounds in hand sanitizer and alcohol which I keep in my car).

Covid just about paralyzed me with fear! With all the statistics of it damaging your heart, your lungs, and reputation of being harder on cardiac patients, I thought if I get it, it would surely kill me or at the very least land me in the ICU. But I have had it twice now (not for lack of protection as I am one of the most careful people I know - I still wear a mask in crowded places), and I have been very lucky to come out on the other end unharmed.

My valve is fine. But my heart is weakening because of my underlying cholesterol issues which continue to damage my coronary arteries. My heart muscle has thickened and I now have what they call “heart failure”. I am one of the lucky ones, though, because my ejection fraction (my "pump") is still high and strong - this is the more rare heart failure.

I learned to keep an eye on the “heart failure indicator” (called BNP, which is measured by a simple blood test) and with the proper medicine and the proper cardiologist I keep that number under control, as much as I can.

I still have neuropathy on my left hand, but there is nothing that can be done about that. I type fine with both of my hands, and sometimes I am spotty on the letters meant to be typed with my left hand, but I know to keep an eye on those and go back and fix them right away. It’s second nature now. I have some shortness of breath and the chest pains come and go, especially with exercise, extreme heat, or extreme cold. 

My blood pressure wanders upward sometimes, but most days it’s normal (staying on top of the meds is key). When I go to some place with an altitude higher than 2000 ft, my pulse races, but when I am at sea level, it’s a lazy 55-60 beats per minute. I have some dizzy spells, tinnitus, and something which I call “pixelated vision” at times (all these started happening right after my surgery and they are still here), where the image in front of me breaks up in what I can only describe as pixels. But again, all these have become old friends and we have learned to coexist. 

As always, today, I read some excerpts from my surgery report and some of my husband’s emails to my relatives and friends while I was in surgery for 12+ hours and hanging between life and death. 

More than anything, today I feel humbled. I feel lucky beyond bounds and humbled. I am not sure why God wanted me to live through this surgery and much, much more beyond. But I am looking forward every day to find out.  

Much health to everyone and as a heart warrior friend once said: “keep ticking!”

The day after my surgery, and today. 9 years is a long time.

 

 

Here We Go (Higher) Again!

Happy New Year, everyone! 

I wish and sincerely hope that you are all having a good year so far and that 2025 will bring you everything you set out to achieve - healthwise, and otherwise. 

To be honest, they always tell us that “less is more”, don’t they? I have always struggled with this saying and I have always believed it’s such a pile of nonsense. But for the case I am about to talk about here, less is indeed more. Unfortunately. Less drug means more cholesterol. 

My journey with Evkeeza has been great so far, at least from a standpoint of what my cholesterol levels have been - levels so close to my target of 70 mg/dl and even lower. In November, my LDL level was 67 mg/dl which is lower than my target. This is stuff which dreams and fantasies have been made of, for me. I never in 42 years did I dream that I would come anywhere close to my target (my “natural” LDL would be somewhere around 475 mg/dl). But there we are. Or rather, there we were in November. 

In December, at my last cardiology appointment, the doctor decided to reduce some of the other medication I have been on for years (https://livingwithfh.blogspot.com/2024/12/the-importance-of-advocacy.html) on the account that Evkeeza does more than it’s been expected to do. So, since November, I have stopped taking Ezetimibe and I have cut the Atorvastatin in half (40 mg, instead of the 80mg that I have taken for 20+ years). 

But at my January Evkeeza appointment, the numbers didn’t look so good anymore, as you can tell below. The LDL more than doubled (since November), to 142 mg/dl. 

Click on the picture for a higher view

(insert your losing move jingle here)

I typically get my infusions on Fridays, because I am usually tired the following day and I want to have the weekend to get some rest afterwards. So, Friday evening when the numbers were posted on MyChart, was followed by two non-working days where I could not reach my doctor. 

I made the decision on my own to double my Atorvastatin back up that Friday night. And I did. First thing on Monday, I emailed my cardiologist and told him that given the latest numbers, I would like to go back to my “usual” 80 mg of statin. He replied that he too was very disappointed to see the numbers creep up and that his advice is too, to double the Lipitor back up. 

So, here we g(r)o(w) again back to “the maximum tolerated (and allowed) statin dosage. 

It’s a guessing and a trial-and-error kinda game - managing this disease. I wish I can tell you that after 42 years of managing HoFH I got a handle on it and I have it under control, but I don’t. Dosage adjustments, trying new medication, trying combining new and old meds all the time, getting tested and then going back to square one, or making 2 steps in the right direction and one step back - it is all part of the lifestyle. 

The target is always: keep an eye on the numbers and adjust what you can regularly. That is all. I have not gotten discouraged because I am used to meds not working for me. My goal is to try the most of what I can try and tolerate and I have access to and just keep at it. The rest is up to whatever my body decides to do. I do my part. The rest is left to science and luck. 

For most of my life, I was not anywhere near “the target”. So, not hitting that is not proof of failure to me. Even with a small dip in the numbers, I am happy that it happened and I am always moving forward. I have learned that there are always things to try and always options. And when I hit a wall and there are none, all I have had to do was to wait for a few months or years and something else will pop up. And if I am really lucky, I might still be around to take advantage of it. 

I am just happy and have been lucky that there is research out there to always seek more ways for us to control this. 

Looking forward, hopefully, to the next blood test which will be sometime in February and which might show a more hopeful story. 

I wish you smooth journeys, full of silver linings out there! And make it a great 2025!