“COVID-19: How it worked for us” - a guest blog

 Becky is my team mate. She lives in Utah. She and her daughter and husband were diagnosed with COVID19 around Labor Day (September 7, 2020). This is her story, in her own words, and what she wants the world to know about having had COVID19. 

September 8, 2020: Becky’s announcement in a chat message to our team that her family has been diagnosed with COVID19 (a day after diagnosis)

All three <members of our family> that live here have coronavirus. (My son escaped to college in time.) We're still trying to figure out what exactly that means, like, logistically. But it's a fair bet that I'll be in and out in a pretty random/sporadic schedule. We're okay, mostly. We don't have any health risk factors, and we DO have health insurance,  but this is going to be a weird week for sure.

September 17: Becky’s note to our team after having been sick for 10 days

<rant> I'm pretty frustrated about this. I made good choices, wore my mask every time I left the house, didn't go anywhere I didn't need to go. It didn't matter, because other people didn't wear masks, and then my daughter brought it home. Now I'm sitting here unable to carry things up and down the stairs or EAT CHOCOLATE because somebody else thought it was no big deal. I would be exponentially more pissed if I or anyone I loved would have actually had a bad case. I 100% support a mask mandate and other nanny state behavior and I wish they would shut up and DO it already. I apologize, and I will not interject politics again.</rant>. 

October 8: She sent me this account privately in a Google doc (a month and a day after diagnosis) 

On this day, I had asked her if I might be too paranoid to still obey all the guidelines and restrictions and to still be very guarded and isolated. She said plainly she will send me her full story (below) and then she added: “In short: you are NOT paranoid!”

People are listed in the order that we caught it. Each person’s story is separate, but in real life we all caught it within a few days of each other, so we overlapped a lot.

One of the weirdest things was that we all had completely different sets of symptoms, although we were all definitely sick. The only thing that happened to all of us was losing smell and taste.

Victoria

Age 17, no known risk factors

Main symptoms: Mostly shortness of breath

Tori was the first to get it. We think she got it from school, because when she went back to school after isolation, someone she sits near in one of her classes had only returned from COVID isolation a few days before. Logically, they would have also had it a few days before her. We wondered if she’d caught it from friends, but all the friends she’d been in contact with had to get tested (because they’d been exposed to her). Only one had it, and he had symptoms a few days after her, so we think he caught it from her instead of the other way around. It’s also possible she got it from work (grocery store), but it doesn’t sound like any of her coworkers had it, and there’s no way to know about customers.

The first day (September 4), she had a fever and felt yucky. Her fever was only 100.something, and it never really got any higher than that. She had some pretty bad muscle pain that day too. She called in to work and they told her she had to get tested before she could work again. So I took her down and got her tested. Results back 24 hours later, positive. She only felt rotten for a couple of days, but she was short of breath for like a week. At one point (I want to say day 4?) she wasn’t feeling so bad, but she was getting short of breath. Her oxygen levels were okay (we had a finger tester) but a doctor friend said he’d probably take her in. I took her to the ER because her regular doctor and insta-care didn’t want us to come in and spread germs. The ER x-rayed her for pneumonia (she didn’t have it) and gave her a prescription for an inhaler. She used it every few hours for a couple of days, then less and less and now I think it’s been more than a week. She did lose taste and smell, but seemed to have it mostly back in a couple of weeks. She says it’s hard to know exactly if it’s all back, because she didn’t pay enough attention before she lost it, and it’s hard to compare and judge. She was 90% better in 2 weeks, and I would say is totally better now (October 8).

NOTE: Of the 6 friends that had to be isolated and tested because they’d been exposed to her, only 2 had gone into self-isolation when she told them they’d been exposed. 4 had to be told to isolate by the health department even though they knew they’d been exposed, and 2 were actively mad at Tori for giving their names to the health department because they’d have to miss work and not get money while they were tested. This is probably why, at least in Utah, cases are going up among young people: because at least some of them are idiots.

NOTE: HOORAY FOR HEALTH INSURANCE AT THE ER

Joshua

Age 45, no known risk factors

Main symptoms: Coughing, headaches

Josh caught it a couple of days after Tori. We think he caught it from her. As soon as she was sick we isolated her, brought food to her room, wiped everything down with Lysol, everything, but it was already too late. He had a cough for a couple of days, but it was so smoky here (in Utah) that we’d just assumed that he was coughing from that. Then when Tori tested positive we thought “uh-oh” and he got tested. He was of course positive, although before we heard back about his test the health department called and said they were going to put us down in the computer as positive anyway since we lived with Tori.

The cough was about the same as a cough from a regular cold. It lasted for about a week. Nyquil helped some. The headache was pretty bad and lasted for, I want to say, 5 days (off and on, but mostly on). The health department said to take Tylenol, but you can’t take much of that. Ibuprofen they said might not hurt, but other articles said it might, so we tried to avoid that. We didn’t have any aspirin. The headache was mostly in the forehead, like a really horrible sinus headache, but his nose was clear. Josh was never short of breath.

Josh was tested on September 7, and went backpacking on September 26, so he was feeling completely better by then. He’d been feeling better for a while, but I think if you go hiking in the mountains and overnight backpacking and you’re just fine, you’re pretty much completely healthy.

Becky

Age 45, no known risk factors*

Main symptoms: Muscle pain, fatigue (sleepy), fatigue (weak), shortness of breath, lack of smell

*My family has a history of autoimmune disorders, but I don’t have one that I know of. Also I should probably mention that my cholesterol tends to be a bit high naturally (despite diet and exercise) but not enough for medication (190s).

This one has more details because it’s the one that I saw from the inside, so to speak.

My first symptom was a scratchy throat. I started feeling it on the day Josh was tested, so I went and got tested even though it was just a little scratchy throat. At that point, we thought Tori had it, and Josh maybe had it, and I didn’t have it, so we were all living in separate parts of the house and not interacting at all. The health department said to assume I had it, and that was nice because then at least we could talk to each other and watch movies together and stuff. Then I got a positive test result even though I wasn’t feeling very bad yet. Then I started to feel rotten. 

I did get the same sinus headache, but only for a couple of days. I was a little bit congested, but not very, even though I apparently snored a lot. I had some bathroom trouble one day. But the main symptoms for me were:

Muscle pain. So much pain. More than I’ve ever had without an actual injury. It was mostly concentrated in my hips and legs, but even my teeth hurt. And it lasted for like five days. Tylenol helped some, but again you can’t take it very often. It just made the pain a little less painful for a couple of hours. Tori said she was in a lot of pain too, that first day, and that her friend also commented on how much pain he was in. It surprised me because for how much pain it was, you’d think it would be in the news more.

Fatigue (sleepy): It’s hard to know where the fatigue was coming from because there was a lot going on. I was sick, which makes you tired, but also I wasn’t sleeping well. The pain kept me awake, and also I was extremely stressed out because we all had COVID. That was the first week. The second week I slept like 12 hours a day. The third week I had trouble sleeping again, but I think that was just stress and bad luck. This week my sleep schedule’s out of whack so I don’t know if I’m still having fatigue or not.

Shortness of breath: This one’s weird, because it didn’t show up until I was mostly feeling better. I was sick for two weeks, then improving for one week, and then the shortness of breath kicked in and I’m way worse than I was a couple of weeks ago. I went to the library yesterday and by the time I got back to the car I was breathing like I’d been running. I breathe hard after gathering up laundry. I breathe hard when playing Guitar Hero. It’s ridiculous.

Fatigue (weak): I suspect this is a side effect from the shortness of breath. I was okay right after I started feeling better but now I’m so weak. I can’t get through a grocery trip without crying when I get back to the car. I can’t go for a walk because it’s hard to get back up the hill to home. If I overdo things, it feels like really low blood sugar or something where you can feel your muscles desperately searching for energy and there just isn’t any. It’s super frustrating for a few reasons. It’s frustrating to have to depend on people to do stuff I feel like I should be able to do myself (shopping, chores). It’s frustrating to be stuck at home because it’s so boring here. When video games are too tiring, all that’s left is movies and needlework. I’m running out of movies, and needlework has its own way of driving you slowly crazy. That’s probably not useful info to you but ARRRRGH I’M SO FRUSTRATED.

Losing smell and taste: You can function just fine without smell and taste, but it’s very weird and surprisingly depressing. Whether we want to admit it or not, comfort food cheers people up, and when you’re already stuck at home feeling rotten, losing taste feels like kicking you when you’re down. Also there was this weird not-quite-smell-not-quite-feeling that was there for the first week or so that was weird and unpleasant. And I was like, well, none of us can smell so hopefully we don’t get a gas leak or anything this week! That lasted longer than any other symptom for any of us, and it came back in bits and pieces, so I could smell flowers, but not cooking meat, and cloves smelled weird because I think I was only detecting some of the chemicals or something. Weird and unpleasant.

I don’t know why I’m having trouble so long after the rest of the family is better. Bad luck I suppose. I have a checkup on the 25th (of September) so if it’s still going on then I’ll find out more. 

Michael

Age 19

Michael left for college on August 19, and didn’t ever show symptoms, so that was a huge relief that he just missed it completely.

October 9: Becky’s note to me (a month and 2 days after diagnosis):

Update on coronavirus in case it's useful to know: I did have to go to the insta-care clinic this morning because my breathing took a turn for the worse. They x-rayed me (I'm fine) and gave me an inhaler and also some pretty heavy doses of prednisone (steroid) for the next five days. I do feel a little better this afternoon, so here's hoping it helps. 

October 23: another update from Becky (a month and 2 weeks after diagnosis)

I went into the ER Friday because I was having trouble breathing and my chest hurt. I probably didn’t need to go to the ER, but I was freaking out. They took another x-ray and an EKG and tested me for blood clots (my mom died from blood clots). That was all fine. The doctor told me I have “Reactive Airway Disease” which is apparently what they call asthma when you haven’t been specifically tested for asthma. They put me on some pills (generic Singulair) and some powder that I have to breathe in (Advair) which are both asthma medications. The good news is that they seem to be working! The doctor said that I’d probably be on them for several months.  I still get winded and tired easily, but I don’t have to lie down nearly as much, and when I’m not active I feel totally fine, which is new and exciting for me.

I asked her if she had ever been diagnosed with asthma before or any other lung disease. She answered: No, I don't think so. I can't remember having anything like this before. I get hay fever sometimes, but I don't think that involves your lungs. I do have a cousin with asthma, but no one in my immediate family has it. I'm just glad the doctor figured out what was going on and how to treat it. I'm not thrilled about possibly having asthma, but it's way better than having breathing problems that I don't know what's happening or what to do, if that makes sense.

December 2, 2020: I asked Becky for an update on this day. This is her note ( 5 days shy of 3 months after diagnosis)

Becky’s been wonderful: she is one of the hardest-working people I know. Incredibly smart, too, and even more, incredibly humble. On the virtual calls we have, though, she always looks very tired. So, I checked in with her and asked her today how she feels and whether the doctors have any idea when her life will be back to normal. This was her answer: 

My doctor said 3-6 months. 3 months is next week so I'm pretty sure I'm not going to make that goal.  If I'm not noticeably better in February I'm supposed to go back and she'll start sending me to specialists. It wouldn't do me any good to go to specialists yet because I might still get better by myself. Personally, I don't know if I will. I haven't made any improvement in a while, so right now it's less "rest so I can get better" and more "rest because this is what life is like now." 

Honestly it's kind of rough, emotionally. I am trying very very hard to be patient and optimistic, but some days that's kind of hard to do. I don't mean to complain. I really don't. I know that other people have it much worse. No one I know has died from COVID, and my sister has MS and has been dealing with this sort of thing for years. But I don't love it, and I wish people would understand that this is just not worth the risk. I do have LOTS of support, so that's good. I have no idea how I would cope if I was alone.  

... it's been nearly 3 months, I'm still on Advair and Singulair, and I'm still only able to walk around the block, and not even every day. If I do more than that it's a bad time.  

I thank Becky tremendously for letting me share this with you all and I respect her courage for fighting this, as well as her selflessness for sharing herself so kindly, honestly, and thoughtfully with the world. She is my hero! 

Much health to you, Becky, and thank you, again, for the gift you have given us of your story. 

Trying to Achieve the Best Results with the Least Amount of Compromise. A Continuous Guessing Game.

Speaking about the benefits and side effects of Amlodipine and Nexletol            

If you remember earlier in the year, I started two different therapies: Amlodipine, to regulate my blood pressure and Nexletol (or Bempedoic Acid), an add-on drug to my cholesterol-lowering therapy. I have written before about Nexletol and what side effects I experienced as I started it (https://livingwithfh.blogspot.com/2020/07/personal-side-effects-of-bempedoic-acid.html). And I also wrote about how well it worked for lowering my LDL levels (https://livingwithfh.blogspot.com/2020/09/results-after-latest-addition-to-my.html). The truth is: it worked really well. Better than the cardiologist expected it to work. That was the great news! 

 

Amlodipine worked well for lowering the blood pressure, too. It was amazing to see how much the blood flow to my heart, brain, and legs increased as I could clearly tell when I was able to exercise longer and more strenuously, or in more strenuous conditions, like in extreme heat, during this past summer.

 

However, I also noticed that while I was on both drugs for the past few months, I developed more side effects or some of the existing ones got worse. Some of the more troublesome ones were:

• A hoarse voice, sometimes to the point of losing it altogether.
• Muscle spasms for no reason, even at rest.
• The joint pain I speak about in the entry linked above got worse.
• Extremely sensitive gums, to the point that even a soft-bristle brush would hurt and make them bleed.
• Slower than normal urination.

I would not call any of these side effects debilitating, by any means, but I was worried that they might be telling of other, more serious problems that might develop underneath. So, at my appointment later last month, I mentioned all these to my doctor. He knew pretty surely that the gum problems were caused by Amlodipine – I also knew this, as this is a well-documented side effect for this drug, publicized everywhere I could find. However, he didn’t know which drug was causing all the other symptoms.

 

To address these things, he ran some tests first, to ensure my liver and kidneys are fine. The tests came back normal for both. He also ran a Uric Acid test to see if I started to develop gout – Nexletol (Bempedoic Acid) is known to cause gout in some cases, and my sensitive and painful joints were a concern. The levels were normal here, as well.

 

Then, he took me off the Amlodipine for two weeks. The gums got better, but none of the other symptoms budged. He’s prescribed something else for lowering the blood pressure (Losartan), but he also has me monitor my blood pressure more or less daily. So far, the highest I have had it (and it’s not consistently this high) was around 145/60. Usually, it’s around 135/55, as an average, but sometimes lower than that. He said I don’t need to take anything for blood pressure unless I start feeling weak, dizzy, or if I have palpitations. I don’t experience these, so at this time I am taking nothing. However, I do feel like I am more winded and lack enough blood flow to exercise more vigorously. My chest becomes tighter and my carotids and calves also become more cramped when I exercise now. Although I can still exercise fairly well (I went for a 3 mile hike this past weekend at altitude and in cold weather and did fine) I will talk with him about this change at our next appointment. For now, no blood pressure meds, at least not on a daily basis. I have a prescription of Losartan, if needed.

 

After that experiment, he took me off the Bempedoic Acid for a month. That month will be up sometime in the beginning of November. Since I stopped it (about a week and a half – two weeks ago), my urinary problems are all cleared up, my joints feel better but still not pain-free, my voice is better, but still not completely hoarseness-free, and there is no change in my muscle spasms. The one month is not up, so I am still watching.

 

We will see what this experiment will show and what he’ll recommend next. I asked him if I could take half of a Bempedoic Acid and try to build it back up slowly (given that there is only one strength for it, so he can’t recommend a lower dose). He said he’ll look into whether we can cut this pill for a lower dose. I guess the drug is new enough where this is not known?! We have made no more decisions beyond trying to figure out what a break from drugs will look like in terms of well-being.

 

For those of you still looking for the perfect therapy for FH, I can say: it’s more of an art than a science, really. All my life, I have tried different drug combinations and different doses, and decided what the best regimen is for me with the least amount of negative impact. I have never found a side effect-free regimen. Maybe other patients have, but I cannot say that was my experience.

 

Right now, I am still on Lipitor, Zetia, and Praluent for cholesterol lowering. I am also aware that because Nexletol is no longer in the picture, my LDL levels are probably climbing back up. I am also wondering if the muscle spasms and the hoarse voice could not be from the Praluent, and just recently have become more pronounced and bothersome. They are also side effects mentioned in relation to Praluent (I have taken Praluent for 4 years now). With so many drugs at play and all of them coming with their own side effects, it’s hard to pinpoint which one is the culprit. 

Sometimes I wonder if it’s the combination of two or more of the drugs that cause the side effects. Unfortunately, I have not found a simple answer here. My goal is to get my numbers (cholesterol and blood pressure) closest to their healthy targets with the least amount of compromise of quality of life. And to this purpose, I am still very much on a journey instead of feeling like I have reached a destination.

 

What Being Cholesterol and FH Aware Means to Me

Those of you who follow this blog should already know my story (http://livingwithfh.blogspot.com/2011/03/my-story.html). Just in case you don’t, here’s a short summary: 

My parents knew that my dad’s side of the family had FH long before I was born. Dad had it, and his father had it, as well as most of his uncles and aunts on his father’s side. When I was 8, my pediatrician recommended that my parents would check my cholesterol level as well, because my liver was enlarged. My LDL was 525 mg/dl and my total cholesterol was 734 mg/dl. 

My mom fainted when she found these numbers, as she was the lab tech that actually read them for the first time. They didn’t have much hope that I would survive my teens. No one had ever seen such levels in a small child in 1983 Romania and with no medication or procedures on the market, they had little hope that I’d make it very long. Even in Communist Romania, with virtually no access to cholesterol-lowering drugs, they did know one important thing about FH: that it leads to premature heart disease or strokes at an early age. My grandfather had his first stroke at 48 and then, two years later, another massive one which left him bedridden. Even at my fragile age of 8, I had grown up to see my grandfather decay fast and I understood so much. 

My parents kept giving him as an example to me, of what will, for sure, happen to me, only much, much earlier in life because my cholesterol levels were so much higher than his. Even at that age, I learned what cholesterol meant (a white, fat substance that clings on to your blood vessels and organs and prevents them from working right), and what one can do about it: extremely severe diet (at the time, my parents would try anything so I was on a no-meat, no-fat, no-oil diet for about two years before they gave up on that because it was not really working for my numbers), and medication (in the beginning they gave me a white powder-drug called Cholestyramine which is a binder drug, but that didn’t help much either). I think I was 9 or 10 when I could tell you very eloquently what atherosclerosis,  dyslipidemia, and familial hypercholesterolemia meant. Google them, please! 

I went from doctor to doctor, from specialist to specialist till I was about 14 or 15. Everyone shrugged. No one knew what else to do. When I was in my late teens (19 and in college), I started to have heart symptoms: mainly tachycardia and arrhythmia so they put me on a beta blocker. They also did my first echo at that time and they saw modifications then, although I am not remembering exactly what kind. All they told me was “this is perfectly normal given your disease, but it’s serious.” 

I moved to the US when I was 23 and my first priority was to get on a treatment. Since then, I have tried every statin there is, and added much more to my regimen of drugs. Even with everything I have been throwing at this disease (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html), my LDL cholesterol has only recently (this month) been within normal ranges but  still not at my ideal target (below 70 mg/dl). 

Even with much access to medication and specialists in the United States, my heart problems have increased over the years: my aortic valve and my aorta replaced, my aortic arch repaired for an aneurysm and four by-pass grafts of the major arteries of my heart. I also had a heart attack following my surgery. All this before my 41st birthday. It was then when I had access to genetic testing and finally my “very severe case of FH” had its own name: Homozygous FH, which is the rarer and more severe form of FH. This meant that my mom also has the disease. Because her levels had always been in the upper 200’s and because she doesn’t have much knowledge about her family history she never suspected it when she compared her levels with dad’s and mine. In her late 60’s now, she has just been diagnosed with a slew of heart problems, among which a stenotic aortic valve and severe atherosclerosis of most major arteries of her heart. 

In addition to my heart problem, I have moderate to severe stenosis in my carotid arteries and at least one instance of severe stenosis in one branch of my abdominal aorta. For now, we are keeping a close watch on these areas, with yearly exams and visits to a vascular specialist. 

I would not be sitting here writing this for you all today if it were not for the fact that I have been aware early in my life about what cholesterol means and what FH can lead to. I have taken every treatment that seemed to match my body and my needs and have made choices in my life that allowed me to stay as healthy as I can. Sometimes those choices went against the popular expectations, like choosing to not have children because I simply didn’t want to pass this legacy on, and I didn’t know if I was going to be around for them. 

However, I have had a full life otherwise.  I am proud of my family and their support, and proud of my marriage, my travels and my work. I would have had a different story, or not been here to tell you a story at all if it were not for the fact that very early on, I knew what this means and how to manage it and I started doing that as soon as it became possible. By then, I knew exactly what specialists I needed to see and what questions to ask. Awareness is key, with FH. 

September is cholesterol awareness month and September 24th this year is FH awareness day. If you know a little bit about your family history and you know you have people in your family either with high cholesterol (especially when they lead an otherwise healthy life) or with heart disease or stroke, urge your doctor to check your cholesterol and know what the numbers are and what they mean (http://livingwithfh.blogspot.com/2016/07/my-current-cholesterol-numbers.html) . Do this especially if you have children or think you might have them one day. Knowing early will allow you to start treatment early and slow the progression of atherosclerosis. Your life is worth so much more! 

Stay healthy and stay aware. 

Results After the Latest Addition to My Combined-Drug Therapy

I wanted to follow up on a couple of blog posts that I have published recently. In June, my cardiologist thought it would be a good idea to add one more drug to my cholesterol lowering regimen. I talk about that decision in this entry (https://livingwithfh.blogspot.com/2020/06/how-hard-is-it-to-manage-my-hofh.html). Up to that point, I had been on 80 mg/ day of Lipitor, 10 mg/ day of Zetia and 150 ml/ twice a month of Praluent (injectable).

Since June, he added 180 mg/ day of Nexletol (or Bempedoic Acid). This month, he repeated the lipid panel and the results are listed below.

As you can see, the drop in LDL cholesterol is fairly dramatic, for me. I have not had my appointment with my cardiologist yet, I have just gotten the results for the tests, so we have not discussed in detail what it all means. The appointment is in a couple of weeks yet. However, I do know that although my LDL and total cholesterol levels are within limits that are considered normal for healthy people, because of my history and my underlying disease, my targets are much lower. Ideally, several specialists I have seen and whose school of thought I have followed over the years, consider that my target level for LDL cholesterol should be 70 at the most. They say that a level around 30-40 mg/dl would be ideal. So, as you can see, I have a long way to go. Honestly, I don’t have too much hope that with what I know is on the market now I will be able to reach those lowest levels.

I am, however, happy with this new result, as lower can only mean better for cholesterol and less disease for my arteries, in general.

If you are curious to see if Nexletol added any side effects to my life, I have talked about them in this entry (https://livingwithfh.blogspot.com/2020/07/personal-side-effects-of-bempedoic-acid.html).

The “lesson”, if you will, of the journey with Homozygous FH, and sometimes with Heterozygous FH, too, is that at times one drug might not be the silver bullet for lowering and managing the cholesterol numbers. You might need to explore a combination of drugs and that combination is always as unique as every one of us. What I have found is that I tried different drugs and only this very combination of these four drugs in these dosages seem to do the most work to lower my numbers.

Good luck in finding that sweet spot to all of you. I hope it happens quickly and with minimal side effects and inconveniences.

Much health to all!

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

Personal Side Effects of Bempedoic Acid (Nexletol)

I have been taking Nexletol (Bempedoic Acid) for a month now. As I mentioned in my previous entry (http://livingwithfh.blogspot.com/2020/06/how-hard-is-it-to-manage-my-hofh.html), this was an add-on medication for me to my other three medicines: Atorvastatin (80 mg), Zetia (10 mg) and Praluent (15 ml injection). I wanted to share here some side effects I personally have experienced so far. Not to discourage you from taking the drug, but just to share my experience in case you have questions about this when you are prescribed the medication. Again, this is a personal experience and that alone. Not an advice of any kind, medical or otherwise.

If you read that previous blog entry, you’ll also find out that my LDL is currently 193 mg/dl before the Nexletol. This latest drug is supposed to lower my LDL by 15%. This would bring the LDL down to 164 mg/dl which is still not optimal, but I guess the thought here is “every little bit helps.” Homozygous FH numbers are stubborn!

The one side effect I noticed right after (within days) I started taking the Bempedoic Acid was a horrible sore throat. It was incredibly painful and made my voice raspy and hoarse. Lozenges which normally soothe my throat when it is sore, did absolutely nothing to help it this time. The only thing that helped was ice-cream. This would normally be good for most people, but I am not keen on ice-cream. I know, crazy! Anyway, that lasted for about two weeks and it went away. Along with that, my nose was dripping like I had allergies or the sniffles. That also cleared up in a couple of weeks. I read that one of the side effects of Nexletol  is “upper respiratory tract infection”, so I chalked it up to that and I was happy when it went away.

A third side effect that seems to have been lingering now for a month is soreness in my joints, especially in my ankles. This has been somewhat steady, and it’s not getting worse. It is mostly like something between a dull pain and tenderness. My joints have been more painful this year, for some reason (could be age?!), but since the Nexletol, they are slightly more so. This has not cleared up, like I said, but it’s very bearable, so unless this becomes more disabling, I am willing to stick it out for two more months when the next cholesterol check is scheduled. I am curious to see what benefit I do get from adding this new drug. If I can tolerate it and my liver can tolerate it with no other trouble, I am willing to stay on it. We shall see.

Much health to everyone who has been on the journey of managing FH for a while or is just starting today. I hope you’re all safe!

How Hard Is It to Manage My HoFH Cholesterol Numbers?!

I have expressed this … sort of mild desperation before: I wish I knew what causes my cholesterol numbers to go up. There are virtually no changes in my diet (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html), nor lifestyle, and as many people with FH know: diet wouldn’t much matter for the FH cholesterol numbers anyway. So, why my LDL oscillates anywhere between 140 and 190, I am not sure. Of course, both numbers are above the normal range and not anywhere close to what is considered desired for me (a top value of 70 mg/dl), but I would still like to know how to keep that number closer to 140.

The most recent blood tests showed the numbers you see in the picture below. Since March, if there is one change in my life is the fact that I probably deal with a lot more worry. I don’t know whether this also translates into a lot more stress, but it would be a fair assumption. Of course, we all have that, especially lately: insecurities about our health, our jobs, the economy in general, the loss of freedom that we have all felt because of the stay-at-home restrictions all add up to stress, I am sure. But, on the flip side of that: I have had lots more time to walk and exercise, a lot more than ever before. Walking, finding a trail and hiking are virtually the only times I get out of the house. And you would think those would translate into better numbers, but … not for me, I guess. I really would like to know what it would take to make the numbers stay low, once they do reach the 140’ish bracket.

My current levels

My liver functions, which I always insist on having checked when we check the cholesterol levels, are normal: in the past, I had those numbers jump up a bit, too. Usually, the cardiologist would send me home with “ah, well, that’s FH!”. Sure, there are other things they can “try”, like Juxtapid (https://en.wikipedia.org/wiki/Lomitapide) or LDL apheresis (https://en.wikipedia.org/wiki/LDL_apheresis), but for personal reasons that have to do with their side effects and overall impact on the body, as I understand it, I have chosen against them. And my doctor knows this.  

This time, though, he said something more than “ah, well!”: there is a new drug on the market that he would like me to try: Nexletol, or Bempedoic Acid (https://en.wikipedia.org/wiki/Bempedoic_acid). To recap, I will take the following cholesterol-lowering drugs going forward: Atorvastativ (80mg), Ezetimibe (10 mg), Praluent (150 mg), and Nexletol (180 mg). 

I have researched this drug to understand by how much it reduces the LDL levels and the numbers are anywhere between 17 and 21%, depending on your source. My doctor put the number at a conservative 15%. So, even with the fourth drug added to my cocktail, my  LDL number won’t be within normal range, generally, and I am still far off from my targeted range – 15% reduction would put me around 164 mg/dl (124 mg/dl when the cholesterol dips to the 140 range). The lowest numbers I can achieve is definitely the purpose, overall, even if they are not within the range.

Of course, with FH, we live and die by the numbers: the importance of the numbers being the lowest they can is paramount in reducing the risk for worsening my heart disease and atherosclerosis. 

My current drugs

I am happy that there is still research out there that continuously looks for alternative therapies for FH and I will take this new drug, as it so far promises little to no side effects. I hope the lowering of the numbers will happen for me as well – there have been other drugs that I have tried that have not worked so well for me, so this is always a possibility.

I plan to still measure my values as well as my liver values every 3-4 months. This has become pretty much routine for me. I will report back, as usual, when I know more about how this affects me, in general, and how it affects my levels, as well.

Much health to you all!