After 6 Years

It’s hard to believe that it’s been 6 years since my open-heart surgery. In some ways, that seems like a blink of an eye. A 6 year old would barely be even fit to start real school yet! But in the measurement of the time that open-heart surgeries recoveries are measured by, 6 years seems like a lifetime. 

February 11: 2016 (right before they rolled me into the OR) - 2022

I hated having such a huge surgery in February - my least favorite month. The month that, historically, has gotten people sick and killed among my loved  ones. A cruel, cold, dark (albeit short) month that’s not always been kind to me. But it had to be in February, because at the end of January, the month before, they found two arteries blocked in my heart (the LAD was at 99%, another one - an OM - was at 90%). The aortic valve was failing, in severe stenosis, and the aortic arch had an aneurysm. The cardiologist wanted the surgery done even sooner, if he could get me and the surgeon aligned on a date. 

I documented my surgery day, step by step, here: https://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html.

And the day before the surgery (the “pre-op” day), here:

https://livingwithfh.blogspot.com/2016/02/the-day-before-open-heart-surgery.html

If you ever have time to read it all, you will see that what was said the day before, what they prepared me for, mostly came true, but there were a few surprises. When I came out of the surgery, everyone who came into my room, ICU nurses, several surgeons who assisted me during the actual surgery (12+ hours), all the three PAs, breathing and physical therapists, and even my rock, my husband - all of them reminded me that I “had just been hit by a Mack truck.” You cannot possibly be very well hit by a Mack truck and expect every single thing to go “according to plan”. You cannot be hit by a Mack truck and plan for it, step by step, then hold God and the world accountable for every one of those steps. You are bound to be surprised, and thrown off the rails, and, oh, yeah, even shocked. 

The day of the surgery was a pretty peaceful day for me, consciously. Yes, my body went through hell; yes, it got killed, drained from all the blood, frozen for more than half an hour, and was brought back to life. But, mercifully, I don’t remember any of it. Sometimes I hear about people remembering bright lights and seeing their lives flash before their eyes, or floating above their bodies in the OR, but I experienced none of this. 

As peaceful as that day was, the days in the hospital that followed were intense! Just full of emotions, but more than anything full of new sensations, of discovering new parts of my body I didn’t know I had or parts that I didn’t know could hurt - my skin hurt. I could not wear ribbed tees or feel the creases in my hospital bed sheet because my skin felt like it was completely burnt ... The two days after the surgery, it took all I had to just stand up, held up by the nurses, beside my bed. Forget walking and balancing on my own. That didn’t come till it was almost time to go home, more than a week later ... I don’t think I ever walked during the hospital stay without holding on to the back of a wheel chair. And the breathing! Oh, my word! I could not breathe! I could not speak, I’d whisper. They wanted me to blow into this plastic tube and felt like that was my last breath, there it went into that stupid tube. At the end of that effort they’d all look disappointed because I never could blow strong enough for them! It was horrible. My lungs took such a beating! Even after three months of recovery, when I went back to work finally, my coworkers were saying that they could never believe I could speak so quietly. I don’t have a big voice to begin with but after the surgery, it was nothing but a whisper. 

Once I got home, the life “after OHS”, the life on my own, without hospital care and around-the-clock medical staff explaining every new symptom, started. I was terrified! Again, I had to relearn my body! I had to learn how to sleep again because no position felt comfortable. No bed or chair. I had to learn how to cough and laugh and sneeze again so as not to let my chest come loose ... I had to build an appetite again although nothing sounded good. I had lost 10% of my body weight in that surgery and I didn’t have that much to lose to begin with: I was 95 lbs at my lightest. 

I went through ER scares when I became too dizzy and light-headed, or when I cut my finger chopping veggies and was scared that the Coumadin might make me bleed forever ... I went through years (not even sure that’s even over yet!) of being scared that my INR will be too high (I’ll bleed to death) or too low (my mechanical valve would clot and I’d die of an embolism or something). Although I manage it better than ever before now, it is still a challenge to keep it tightly in my range, especially when seasons change or when I travel and add unusual, unfamiliar stress to my daily life. 

I went to physical therapy twice, first to build strength in my upper body, right after surgery, and almost two years later to build stamina to be able to walk for a mile at a time without my chest hurting too much and without feeling like I was going to faint from light-headedness. 

I stopped traveling the year of the surgery because I was afraid of getting an infection on airplanes. That was when my immunity was still fighting to heal my heart. I stopped traveling again for the past two years because being a cardiac patient with very high cholesterol for the rest of my life puts me in the “high risk” group for people who might get more severe or possibly lethal Covid. 

I do get out some and I meet with friends and even saw family across the country and in Canada last year. But I limit who I see and if I can’t meet people mostly outside I put it off for later. I am still nervous to fly. Maybe this coming summer ... We’ll see ... 

As much as I hate putting my life on hold, going through that surgery to rebuild my heart, with all its complexities, and then going through years of recovery to function somewhat normally again and being rewarded with a good life, all things considered, seems like the most precious gift. I don’t want to throw myself willingly at risks that are not absolutely necessary for survival, to endanger this beautiful gift. So, I’d rather wait out the pandemic, or not expose myself to other sources of more sickness or things that could damage my heart (or valve) again. In the whole scheme of things, it’s a small sacrifice.

I’ll have to say this though: my journey has been more lucky, more blessed than I have ever hoped for. So much more blessed than others’ paths - although there is no comparing journeys here. Everyone’s path is different and everyone feels pain and blessings at different temperatures. However, I can say, I am lucky to not have had too many complications in these past 6 years of renewed life. 

I wish I can tell you that the minute you walk out of that hospital after your heart surgery you’ll never look back and you’ll be as good as new with not a worry in the world about your health. But I’d be lying.  You will never be the same person you were before. In some ways you’ll be worse and in some ways you’ll be better. In some other ways, you’ll be much, much better. In almost all the ways, you’ll just be different. A different “you” that you’ll need to get reacquainted with. You’ll maybe be more hopeful, more appreciative of your health, with more respect for what your body can do and for its ability to miraculously heal, even after being hit by that Mack truck! 

I can tell you, though, this, for myself: I have zero regrets for anything related to this surgery. There has been no question in my mind that I needed this surgery. This was not elective: this was a surgery to save my life. My aortic valve had only a 0.49 cm opening, it was almost closed. My arteries were stenotic. My chest pain was constant. My ability to walk and exercise was very much reduced. I am very grateful that for my condition there was a course of action. There are so many afflictions out there that have no cure. So many diseases that come with a stopwatch that is set to expire in months, some in weeks or days. This was not the case with my heart disease: there was something they could do. I am grateful for that. 

I am also grateful for my team of doctors in Provo, Utah, at the Utah Valley Regional Medical Center. My cardiologist and my surgeon are my heroes, my true soldiers who fought as hard as me to get me back to “normal”. But the other members of the team, the PAs, the other surgeons, the medical staff, the technicians, and then the cardiologist I have had in North Carolina, after I moved, not to mention my family, my husband first and foremost (who tied my shoes and put on my socks after I got out of the hospital and when I could not bend over; who clipped my nails because the neuropathy in my hands could not work the clippers), my sister who is my biggest cheerleader - they all made me who I am today, when it comes to my heart and overall health. 

I have noticed that I can walk further and faster than ever before and that my stamina has improved little by little every year. I can now walk even in cold temperatures (30s-40s) or hot ones (80s) and can go longer than before. I walked a mile and a quarter last night in low-50s temperatures (with wind in my face that made it feel like the 40s) without even breaking a sweat. I used to be out of breath walking from my car to my office in 40F temperature before my surgery. Inclines are still a challenge to this day. I live in NC now and boy, there is not one street that’s not on some sort of a hill. Day to day, the progress you notice might be imperceptible, but when you look back at all these years, and compare what you could do then and what you can now, it’s pretty amazing ... 

A month ago my INR was 3.1 (too high since my range stops at 2.5). This past weekend it was 1.7 (within range but a little too close to my bottom of 1.5 for comfort). But I have learned how to live with this ever-swinging pendulum. I have bought a CoaguChek machine that measures your INR and I check it myself as many times a week as I want to just to know how to adjust my food accordingly. I usually check it once a week if no changes in diet, exercise, or stress level have occurred. I take it with me when I travel because it’s harder to plan for your food when you’re not home. This brings me a lot of peace of mind.

I have never, not once, regretted my choice of valve. It took sleepless days of reading just about everything there was to read on the internet about all the possible valve options that I had and when I found the On-X mechanical valve and learned about the benefits and came to peace about living on Coumadin (if you ever do!), I never looked back. I love knowing that at least if everything goes according to plan and I avoid infections and clots, my valve will outlive me and I won’t have to have my chest cracked open again. At least not for the valve, anyway. 

I got all sorts of other “work” done with my OHS: I had a quadruple by-pass, as well as endarterectomy of all the four arteries that they bypassed, and a new, grafted, ascending aorta. I also had an aneurism repair and additional endarterectomy of my aortic arch ... I get a yearly echo for my heart and both the current cardiologist and the surgeon read it and so far it all checks out, thank goodness. My ejection fraction is lower than before the surgery, but at 55% is still pretty good, considering. I still have high blood pressure, but we try to keep it under control with medication and exercise. The coronary artery damage that I might still have in my heart is believed to be in my very small vessels (which cannot be properly diagnosed with an echo; an angiogram is required for that and I am not due for a new one for another 3 years or so, barring any new symptoms, like acute chest pain, dizziness, collapse, etc). As long as I keep active, the belief is that I’ll help my body create new blood vessels that would build a natural bypass for any vessel that might clog in the future ... So far, every year I have been able to exercise more and more ... I am not a marathon runner, far from it, but my stamina has improved so much!

I look back on every anniversary and thank God, life, and the universe for the inspiration they all had to keep me in the light that fateful (in a good way!) day of February 11, 2016. Every day on this day, it’s my “other” birthday and I am grateful when I add another one to my heart age. I think my sister once said: “you are almost 47 in human years, but your heart is only 6.” That’s right! My heart, the engine of my body, is barely even old enough to go to school! Oh, the possibilities! 

Maybe having my Heart Day during Heart Month (February) was not a coincidence after all. I have made peace with that. 

Happy Heart Month to all the heart warriors out there! Embrace your body, embrace your heart and keep fighting the good fight. It’s always a good one when you keep that engine running! 

Much health to everyone! 

If you’d like to see my journey in pictures from my heart surgery day to today, you can do so here: https://wanderworldpics.shutterfly.com/22602. 

Meeting a New Vascular Surgeon

I opened up the year with a new-patient visit with my new vascular surgeon. 

Right before I headed to his office early on this month, I realized that I have gone more than four years without a vascular specialist monitoring my arteries. Considering the amount of disease in both my abdominal but mostly in my carotid artery, this sounds crazy to me! I used to have an amazing vascular surgeon when I lived in Utah. We moved to the Triangle region of North Carolina in 2017 specifically for the well-reputed healthcare we hoped to find here. And so far that has not proven to be the case. Provo, Utah offered a lot more quality care than all the specialists I have seen so far in North Carolina. The constant dismissal of my cardiologist that I should not see a vascular surgeon mostly contributed to this delay. But I persevered. 

It was also a matter of Karma, or chance, or call it whatever it was ... but even when he did refer me to a vascular surgeon, the week before my appointment was supposed to happen, she had to have emergency back surgery and my first appointment with her was canceled. I had to be referred to another vascular surgeon and they are busy people. It took months before I got another appointment. But I digress ... 

Prior to coming to this surgeon, I saw a cardiovascular interventionist cardiologist: they cath, but they don't cut. He was supposed to manage my arterial disease and my cardiologist was supposed to only manage my heart. I have written several times about how the vascular cardiologist downplayed my condition and it got to be so bad that I had to stop seeing him - I was not gaining any new information, I was going nowhere with understanding what kind of plan he had to manage my disease. So, I asked my cardiologist for another referral, this time to a vascular surgeon. 

You will find this a lot with this disease, especially if you are younger. People that are less knowledgeable in treating FH will not look past your appearance. They will not understand the danger that is developing in your arteries right as you speak to them. You think this is an exaggeration, but it is not. Like I said: you must persevere and find the best care for yourself. 

The new vascular surgeon ordered a couple of tests before he saw me: a CT scan of my abdomen (with contrast) and an ABI (Ankle–Brachial Pressure Index) of my leg - this measures your blood pressure in several spots on both of your legs before and after exercise. If the pressure is higher after exercise, this means that you have claudication (narrowing of arteries, or poor blood flow in your legs), or your abdominal aorta is not supplying your legs with enough blood. 

I have done this test several times before and it usually shows mild to moderate claudication, depending on who does the test. Not sure why he could not use the results from the same test my cardiologist did earlier this year, but he needed his own results. This time, they also sent me to what it ended up being the wrong lab: the exercise they have you do before checking your blood pressures the second time is to walk on a treadmill at a certain speed and with a certain incline. This lab had no treadmill. I was surprised about this but who am I to argue? They, instead, had me do calf raises (standing on your toes) for 3 minutes and they asked me if my legs were cramping. They were but I was pretty sure it was not from exercising too much but from muscular cramps of the legs not doing calf raises every day ... But they didn't care about that - they measured the pressures after this "exercise" and they were almost identical with the ones at rest. The test came back for the first time as "no claudication." 

The visit with the surgeon followed. I started by first asking him if he knows anything about FH or about HoFH specifically. Initially he said "no" and when I looked shocked he apologized and said he could not hear clearly what I said because of my mask and that he is very familiar with FH.

Just for safe measure, I explained to him my journey in a nutshell, from when I was diagnosed at age 8, through all the treatments, the open-heart surgery, the heart attack, etc. I told him how I have been disappointed in the care I have received with the current medical system for my arterial health specifically and I was hoping he will fix that. 

I explained to him my concerns with what I know the tests show - a narrowing abdominal aorta and several of its branches, narrowings in the carotids, etc. I also added that I am not looking for surgery at this time (he is a surgeon, I was thinking, the only way they know to fix things is to cut), unless it was absolutely necessary. I told him I am young but I have an old people's disease so it's tricky to do surgery unless it's really thought-out because things that just work for older people (like stenting) might not work for me. If I was wrong, I asked him to explain why. I told him that several doctors dismissed me as too young or not having enough disease to really be aggressive about watching the disease's progress and that cannot happen here. I need to be taken seriously. 

Some of the points he made and some of the learnings I gathered from our appointment: 

• Before we got to talking about my tests and to assess my situation, he started by saying that I should never let any doctor make me feel bad about standing up for myself. He said that is the "absolutely right thing to do as a patient: stand up for yourself and demand answers" so I can understand the situation. I was glad to hear that. 
• Then, he wanted to make another thing very clear: just because I had an incredibly involved open-heart surgery which showed more then severe disease in my coronary arteries and in my aorta, to the point that the aorta had to be replaced and the arteries had to be repaired and bypassed it does not necessarily mean that is the case of the aorta and all its branches "in all the other vascular beds in the body. That for whatever reason, and we don't know why, different beds see the disease at different speeds." This was news to me. I have generally been advised to always monitor all arteries, not just the heart. 
• He then said the ABI did not show signs of claudication which is good - this means the abdominal aorta sends enough blood to the legs which have good flow themselves. I challenged his statement, however: I asked him if "3 minutes of calf raises can be considered proper 'exercise' for a 46 year old" to judge the true condition of the leg arteries. He said it absolutely does not. He was not aware they did the test without a treadmill and he said the exercise must be in line with the patient and their condition and ability to exercise to show accurate results. So, he is sending me back to get the ABI done again in 6 months. (Never mind that I already paid for a test that is no good - no apology or do-over there). 
• When we got to the CT of my abdominal aorta, he did not go by the findings on the results, he brought up his own plan of action. He first said I did not have enough disease in the abdominal aorta right now to warrant any intervention. Then, he said my abdominal aorta is very small, just anatomically - it is slightly larger (by only 2mm) than the iliac branches which stem from it. Normally, the aorta should be twice the diameter of the iliac arteries. Add some calcification on top of this, you get a very narrow aorta. But the problem is not that it's stenotic, it's that it is too small to begin with. This is one of the reasons that does not make me a candidate for inserting a stent, if needed. He said the only thing he could do should I need surgery for an occluded aorta would be to do something similar to exploratory surgery (where they open up your abdomen) and replace the diseased portion of the aorta. This cannot be done laparoscopically or through any other of non-invasive kind of surgery. However, we are not at that bridge yet. 
• He also said that one of my celiac arteries (that distribute blood to different organs in the abdomen) is compressed by my diaphragm muscle (again, my anatomy, not any kind of arterial disease) which is preventing it to be fully open, but this would have to be addressed by a general surgeon and not himself. He also said there is nothing he recommends at this time for the celiac arteries, as well. 
• In conclusion the plan is:
• Do nothing now - the disease is not severe enough to suggest any kind of intervention.
• Repeat the ABI test in 6 months to get better "exercise" results. As long as there is good blood flow in the legs, he does not want to image the abdomen. He sounded pretty drastic to me when he said "even if the abdominal aorta were to become completely occluded, if we have good flow in the legs, we don't worry about it." I am still kind of noodling with this thought and will probably press for a better understanding of what's going on in the abdomen.
• Also, as part of his plan, he wants to do a carotid scan in 6 months to see the progress, if any, of that "vascular bed" which he intends to also monitor closely. We spoke about pre-stroke symptoms and he urged me to call him the minute I might think I experience them. (The cardiologist said he would monitor the carotids every two years, but the vascular surgeon wants this done every year).  
• He concluded by saying that everything I do for heart health is 100% beneficial for vascular health: keeping the cholesterol as low as I can, taking all the recommended medications for cholesterol and blood pressure, keeping the blood pressure as low as I can, eating a healthy, balanced diet, not smoking, not being overweight, and moving. "Moving, moving, moving. Moving is the best medicine for your arteries." - he said. 

He was mostly concerned with the legs - he kept saying that the one, most important test he will have to do every year with be the ABI test and that as long as the flow stays healthy in the legs, he trusts that the abdominal aorta is fine. 

I asked to be imaged every year for the abdomen and he insisted that is not necessary. I do have questions about the other branches, but I will just have to ask about them and ensure we know more as time passes, or listen to my body for new symptoms, which, of course, has always been the case. 

I was surprised that he did not do a physical exam this first time - he did not listen to my abdomen, or other arteries. He just discussed symptoms, the tests he's done so far and what the plan is for the future. I have to say, a physical exam among these kind of specialists seems to be more and more rare nowadays. I am not sure how you truly know a patient without listening to and feeling their body, but maybe I am old school. 

Although, he did seem thorough, there were things in the CT findings that he did not address. I realized how many things only after I came home. I find that this happens a lot: I go in, and we go over whatever the doctor wants to go over, and although I typically have a list of questions, I miss some of the points from the tests. In the future, I am planning to print every test they send me and highlight the portions on the test that I want more information on. I will bring this printed information with me to all the future appointments. 

Overall, I was somewhat pleased with the new doctor, although I did not feel like he did cross all the Ts nor dotted all the Is. But ... I have learned more from him than any other vascular specialist since I have moved here, and no one is perfect. After all the setbacks with the medical care here, since we moved back East, my bar was relatively low and, with all the missing information, he actually came above that. He did not dismiss me or my age and he provided what seemed to me scientific, non-biased information about what his plan will be. Never in our dialogue did he say "you're too young to worry about such things" which would have made me leave and never return like I did with the vascular cardiologist before him. 

It's a process. The first stone was laid down with this appointment. I am planning to give him (and his assistant) some time to prove me wrong. Or right. 

Staying Safe in Times of Covid when Having the Additional Risks of FH and Heart Disease

I see a lot of people that ask on social media what are the proper measures to take as an FH patient during times of Covid - how cautious we should be and why, whether we should get vaccinated, or allow our kids to be vaccinated and so on. Of course, I am not advising anyone about what they should do with their lives, but I thought it would be helpful to share some of the measures I have taken to stay safe so far during this pandemic. 

We are getting ready to enter the third (as in 3rd!) year of the Covid19 pandemic. At the beginning of this (March 2020), I started keeping a journal about the pandemic, where I’ve entered personal notes as well as copies of articles about important milestones during the pandemic, insights about the numbers at various points in time, in various countries that affect me, my family, my friends, my coworkers. That journal is now 275 pages and getting longer almost daily. That is, by far, the longest unified writing record that I have ever produced. And the story of the Covid19 pandemic is far from over. In those pages, I document in painful detail, at times, how we have learned so much about something that was virtually completely  unknown when everything started. And in the process how we have learned about ourselves, too, what kind of people we are and how we deal with pressure, hopelessness, sickness, and even death. 

For the past two years, I have started my days with a quick scan of the news to find out what is new in Covid19 research, cases, numbers, symptoms, etc ... I scan the headlines sometimes obsessively to see what is new. Learning the news first thing in the morning is how I have started every day since March 12, 2020 when the world closed up for the first time. 

I have paid close attention to this pandemic for many reasons, some of them more obvious than others, perhaps, but most of them are related to my being an FH and heart patient, and because of this, feeling a lot more vulnerable than others to this disease. Here are some examples of why I have been so keen on learning everything there is to know (so far) about Covid, for my own health first: 

• I have FH and research shows that Covid19 is more aggressive for and causes an increased number of acute myocardial infarctions (AMIs) in people with this disease - https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8289723/.
• Because of FH, I already have heart and cardiovascular disease and we know now that Covid19 causes disease in the heart and cardiovascular system that could lead up to severe complications and even death - https://www.ahajournals.org/doi/10.1161/CIRCRESAHA.121.317997. Adding more disease (or risks) on top of what FH already caused in my body sounds like a match made in hell to me. 
• I come from a family with lots of long-term lung disease and lung cancer patients: my mother’s aunt, my mom’s father and my mom all have had lung cancer. Her father also had emphysema and my mom also has COPD, lung fibrosis and asthma. We know now that Covid19 can cause long-lasting disease in the lungs and even death from lung disease complications - https://www.hopkinsmedicine.org/health/conditions-and-diseases/coronavirus/what-coronavirus-does-to-the-lungs 
• I have a mechanical aortic valve which puts me at a higher risk for developing blood clots. For this, I take blood thinners (Coumadin) that prevent my blood from clotting, but keeping the right balance of anti-clotting agents in my blood is a tricky business. If my INR (the number that measures the level of anticoagulant in your blood) dips too low, clotting can happen easily. We know now that some people with Covid19 develop blood clots that can lead to organ damage, heart attacks, stroke, and even death - https://www.nhlbi.nih.gov/coronavirus/blood. Again, this is an added risk to an already existing one for me. 
• Studies have already shown that people with heart disease, high blood pressure, and high cholesterol are at an increased risk for severe forms of Covid19. I have all these three diseases. 

For two years, I have tried to stay as informed as possible about how the disease progresses, how the vaccines work, which vaccine is more indicated than others for my specific case. What are the risks of unmasking, what are the risks for contracting Covid19 even if vaccinated, what are the risks for contracting the disease again, even if vaccinated or previously infected - are all questions I have had and have tried to learn the answers to online - the amount of resources is staggering but you need to qualify your sources and learn the reliable ones. I use the CDC, American Heart Association, The Family Heart Foundation, WebMD, Johns Hopkins Hospital, The Cleveland Clinic, to name a few. Again, I don’t endorse any source over another - these are just some of the sources I have found helpful that publish up-to-the-minute new information. 

The CDC reported early last year that there was a 1 in 100 chance of breakthrough infections with Covid19 (meaning 1 in 100 vaccinated people will still get Covid19). This number was back when the Delta variant was the more dominant one.  More recently, some studies show that at least 5 in 100 vaccinated (and boosted) people can still contract the disease. The increase is due to the Omicron variant which is far more contagious than previous ones. Considering that I have Homozygous FH which has an occurrence of 1 in 250,000 people ... the Covid19 numbers scare me, to say the least, because they sound like Covid is so much less rare than the disease that I already have. My odds have always been in the “rare” range for anything. Even when I start taking a new drug, I always develop the side effects listed under “less common” or “rare”. 

I don’t run my life by the numbers, the percentages, or the estimations that research spews out every day, but I use them as a guiding factor to adjust my behavior. So far, my husband and I have been so lucky to keep Covid at bay with the measures we have taken for our small household. 

I will have to say this has been the first time in my life when I am actually happy that we don’t live closer to family because this forces us to not congregate with people if we don’t need to. It’s easier to say “no” to friends than to family. We have very little need to see other people and we can see the few friends we do have around us separately from each other, in very small groups. And we are grateful for a handful of friends who understand our need for separation. 

Some measures my husband and I have implemented are:

• For the first year (2020, when we didn’t have any vaccines), we did not eat at a restaurant, not even outside. We only picked up food to go and ate it at home or in parks. 
• Also for the first year, we did not socialize with anyone indoors. We always socialized outside, in open air, and we did not share food, utensils, or drinks. We sat distanced from other couples and we never met with more than 2 other people (one couple) at a time. 
• We did not travel overnight in 2020. We took day trips to cities close-by, packed food and lawn chairs to spend time in parks, but never spent the night.
• Last year (2021) we both got vaccinated as soon as we were eligible (March 2021) and we got boosted as soon as we were eligible as well (November 2021). 
• In 2021, we started going to restaurants and eating outdoors on patios only, because we felt a bit more protected, having been vaccinated. But again, it was mostly just the two of us, or us and another couple. Never more than 4 people (including us) at the table. 
• We started traveling in 2021 again, but only by car. We have not boarded a plane since 2019. We spent some nights away from home in AirBnB’s or hotels that have a reputation for enhanced and deep cleaning (Marriott and Hilton). 
• In 2021 we drove up to Michigan and Canada to see family, finally. In Canada, we felt completely safe. We took 3 Covid19 tests before we were declared “OK” to stay in the country and all of them were negative (of course). I have to say, as inconvenient as they are, tests add an extra layer of peace of mind. Canada also requires proof of vaccination everywhere you go (museums, restaurants, hotels) which also added another safety blanket. They also require masks everywhere and they will escort you out of the establishment if you’re not complying. Things are very different in the US.
• We did dine in a couple of indoor restaurants (about 6 over 10 days) in both Michigan and Canada, but we went during less busy hours where the crowds were not very large, or we requested tables at the end of a restaurant, to stay distanced from everyone else. The staff was always accommodating when we made these requests. 
• To this day, we never go anywhere indoors without a mask. To me, this is just common sense. Even when the CDC told us that we could ditch the masks if we’re vaccinated, we still wore them because, again, to me, that was common sense: if there is even an iota of a chance to get a breakthrough infection after being fully vaccinated, why in the world would you want to risk it, especially when you know you already have so many other factors working against you should you contract this crazy disease?! 

Masks, vaccines, incredibly good hand hygiene, distancing, avoiding crowds and indoor gatherings - these are all the tools we have used so far to try to stay healthy. What started as a huge inconvenience and massive paranoia has become our lifestyle nowadays. 

I am not saying all this has been easy. It’s surely taken some time to make it into a routine. But at the end of every day, health is worth every effort, in my opinion. What do we have? What can we achieve without health? We hope that some of these (especially the distancing from our families and friends) are temporary, but who knows? What if they are not? Since the time this pandemic has started I keep telling myself: we can only make decisions with the information we have today. And we move along ... And that’s what we have been doing: a lot less planning for the (far) future, a lot more adjusting to what we know today. 

But I have to admit: it is getting harder and harder to stay guarded. Not because we’re getting tired of it all, not even because the variants seem to be getting more infections, but because people around us no longer play it safe. Everyone says they are tired of all the rules, they are tired of the iffiness and of the lack of certainty of what tomorrow’s new variant might bring, or what the vaccines might bring or fail to protect. More and more people don’t mask up anymore. North Carolina, where we live, seems to be a slightly safer state (at least in some parts) than most where more people wear masks rather than not. But Georgia, Michigan, Pennsylvania, West Virginia, Virginia, from what we have seen so far, are far from that. In these other states, the proportion of masked people is extremely low compared to those who wear masks. The number of people wearing the masks correctly is even lower. In the US as a country, the vaccination rates are low, too. Scaringly low ... 

And I don’t even begin to comprehend how people can even turn any other kind of ear but a deaf one to the misinformation about Covid being just a “regular” disease, “not much worse than the flu”. But maybe it’s just me - like I said, I have started every day of my life with the news, with the researched numbers, with how scary everything looks. Everywhere. In all countries. Across the world ... I have no other belief than this is a real and scary disease that is as easy to get as it is to breathe. 

I often tell myself: I never felt like I had to put my life on hold for FH. I always knew what the risks were and tried to stay healthy, listen to my cardiologist, take the drugs, and still do everything I wanted to do. Why am I putting so much on hold for Covid? Since I am doing everything I need to do (I think), why am I not moving on with flying across the world to see family, or going on a tour of the United Kingdom (like it was our plan in 2020), or the likes. The difference now is that unlike FH where you have just one person to control, I feel like with Covid, you have to control your actions and everyone else’s. I cannot control people spreading it around me because they don’t mask, they don’t wash hands, they don’t vaccinate, and they don’t keep their distance, or better yet they don’t stay home. How can I control that?! The one thing I can do is to stay away from them. And hence the (semi-)hermit-like life I continue to lead. 

I am not sure how long we’ll keep these measures in place. My personal (ideal) goal is to not change much until Covid19 drops to endemic levels, but I know that this might not be possible. My parents are aging fast and they live across the world. I am hoping that this summer I will go to visit them for what it could be my last time to see them alive. I have not seen them in 3 years and they look like they have aged by at least 15! I might take a huge risk, greater than I ever planned for, and jump on a plane (actually more like 3 planes one way) to go visit them. But I just never know what tomorrow might bring. 

We still live our lives from day to day, waiting for the next batch of news, the next findings in research, hoping for better, more efficient vaccines, better drugs to treat Covid, and so forth. I don’t click on things like “Covid numbers are soaring due to the Omicron variant” or “Covid numbers are surging because of holiday travel” because really, they offer nothing new. The times we live now are, I am afraid, not unprecedented anymore, to some extent. After two years, we have seen how it goes: numbers go up in the cold months, they go down in the warm months, variants happen and they can be more aggressive (delta) or less (omicron), but both can mess you up (long Covid) or kill you. And the measures for how to stay healthy, minus one or two iffy guidelines, have largely been the same. 

I try to stay calm and just read the facts and act accordingly, only with an extra amount of caution because FH and my heart disease makes me that much more vulnerable. Mostly - I try to keep people at arm’s length and act according to common sense and what science shows now. 

Most importantly: I try not to panic and to have much hope. Hope in science and research, hope in the power of medicine, and hope that one day people will become kinder and more careful towards others as well as kinder to their own selves. After all, how can one survive without hope?! 

Stay safe out there, folks! Be kind to yourselves and others. 

My Amazing Surgeon

I still remember the first time I heard the name of who was going to be my cardiac surgeon. My cardiologist recommended that my severely stenotic aortic valve could no longer be ignored and it had to be replaced: I needed open-heart surgery to fix this. I asked him if he recommended a certain surgeon, maybe someone he was routinely working with. His answer came out almost in one breath: "You will probably go to Dr. John Mitchell. Outside of being an amazing surgeon, he is also an incredible human being." 

I didn't know at the time that my cardiologist was underselling Dr. Mitchell. By a long shot. 

Now, if you read this blog frequently you know that I usually complain about doctors and seldom, if ever, praise them. However, this one is different. This is the man that not only saved my life, but gave me a new one! 

I came to know Dr. Mitchell during my surgery (in 2016) and in the long recovery afterwards. Even after leaving Utah, he insisted that I should come back for a follow-up consult every year. So, every time I've gone to Utah for work, I made sure I made an appointment just to ensure he has a look at my latest tests and he gives me his nod about what my next steps should be. 

He did a procedure on me that to this day, from everything I have read (and I have read a lot!), was incredibly complicated and incredibly brave (https://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html) . There were so many more things wrong with my heart that went way beyond just a stenotic valve: there were four major heart arteries almost all 100% blocked, a PVC-like aorta ('porcelainized'), a damaged aortic arch with an aneurism to repair - all because of what Homozygous FH did for many years of non-treatment and LDL levels in the 400-500 mg/dl range. He fixed it all in one operation, in an almost 12-hour surgery and he ensured me that what he did will last me "a long, long, very long time." 

Just like my cardiologist said: I have found out for myself: he has been an amazing human being, right along with being the most amazing doctor I have ever seen. And trust me, with FH - I have seen hundreds of doctors in my life. Not one other medical professional could ever come close to comparing. 

And just when I thought he could not be more amazing, I came across this story from May of this year: a former Army surgeon for 20 years, at the age of 61 now, he has rejoined the Army Reserves as a colonel, to serve the country one more time as a surgeon. He never stops to leave me speechless. 

I am so humbled that I had him as my surgeon. I am not sure how I got so lucky! Every day, I pray that he is blessed with health and hope and a long life, just like he gives his patients ... 

FH is a journey, full of twists and turns and so much unexpected that it all wears you down, at times.  Doctors are mostly unprepared or unaware of this disease. To find a doctor that is knowledgeable, on top of having a plan and being compassionate to boot makes you really feel like you hit the jackpot. It breathes life and hope back into your tired wings. 

I am forever indebted and forever honored to have had him as my surgeon. I owe the life that I have had for the past 5 years to him - and, if he is to be believed, the life of all the years to come, too. 

Read his amazing story here: https://www.deseret.com/utah/2021/3/21/22332684/doctor-reenlists-with-the-u-s-army-to-give-back-what-hes-learned 

A Mixed Bag: Some Good Things, Some Bad, and a Whole Bunch of Guessing, as Usual

Today was an odd appointment with my cardiologist, to say the least. It was my 3-month appointment (this is routine for me), where we were supposed to discuss the recent tests that he had ordered (a heart echo, a carotid ultrasound, recent blood work, and the results of my neurological tests) and, as always, assess if there are any changes needed in medication.

Right off the bat, he admitted that he didn’t review my tests before he walked in the room. He said he did see them when they were done (in September), but he had not reviewed them this morning before he walked in the room (intern in tow) to see me. So, he needed a minute. (My appointment was at 8:40 AM and he was already an hour late, so I guess: busy morning!)

My cholesterol went up slightly, as you can see below, but he said he will consider it a “lab error”. Well, which one was the error: the last one that showed it the lowest I have ever had it? Or this time, which is more in line with everything else we’ve done for the past year? No answer.

My AST (a liver enzyme) is elevated but only slightly (43 U/L and it’s normal between 15-41 U/L). But I have had it as low as 26, so … there is some reason for concern there. He said to repeat it in 3 months before our next appointment. We repeat the same tests before every appointment: a lipid panel, a liver and renal panel, a uric acid (because of the Nexletol/ bempedoic acid which elevates the uric acid and because in my 20’s I used to have gout attacks frequently).  The AST is part of the liver panel. He asked me if I want to do an extra measurement at 6 weeks but he said “he didn’t care; it was up to me”. OK, then … let’s just do them all at the same time which is in 3 months. (I love when he says “he doesn’t care” or “to him it’s six of this or half a dozen of the other”. Sounds so reassuring!)

My heart echo write-up mentioned for the first time “diastolic disfunction”. I asked him about this and he explained that what this means is when the heart fills up with blood, it increases in volume but it should not increase in pressure. In my case, there is some pressure that is measurable, but that it is “mild”. He said this is “normal” and “almost expected” in my case, having had a heart attack, open-heart surgery, and coronary vascular disease for many years. He said he is not extra concerned about it, as long as my aortic valve is clear (which it is) and my ejection fraction is good, which at 55% it is.

The narrowing of all my carotid arteries is increased compared to the measurements of two years ago, but the percentage is all the same – between 50-69%. This seems like a huge range to me, but that’s where they place my numbers.

For those more curious, here are my measurements for both the right (first) and the left (second) carotid arteries:

MEASUREMENTS – Right/ Left
------------------ -------------- --------------

Central Carotid Artery
CCA Proximal 249/ 19 cm/sec - 216/ 23 cm/sec
CCA Mid 168/ 21 cm/sec - 230/ 23 cm/sec
CCA Distal 141/ 19 cm/sec - 199/ 24 cm/sec

Internal Carotid Artery
ICA Proximal 136/ 24 cm/sec - 191/ 22 cm/sec
ICA Mid 189/ 36 cm/sec - 134/ 21 cm/sec
ICA Distal 160/ 30 cm/sec - 157/ 22 cm/sec

CCA/ICA Ratios 1.340 - 0.960

External Carotid Artery
ECA 550 - 260
Vertebral 93/ 16 cm/sec - 115/ 15 cm/sec
Subclavian 305 - 327

He said that the worst narrowing is in my External Carotid which is of least concern, because it’s the one that vascularizes the face which gets blood supplies from a “million other places” (his words), so there is no concern for no blood supply there.

I have an appointment with a vascular surgeon and he asked me to follow up with him for a second opinion on the carotid findings.

If it were not for me to mention the neurological test that he ordered to diagnose peripheral neuropathy, he would not have discussed it. I told him that the test showed that I did not have peripheral neuropathy. He was glad about that. He had suspected there was something neurologically wrong because my dizzy spells. Well, not sure what worked, but my dizzy spells are very mild now and very infrequent, and my muscle spasms and cramps are also much better, too. The dizziness definitely does not last for a whole day anymore. I started taking CoQ10 (my decision) which I guess must have made my muscle cramps less frequent, but I don’t think that it had anything to do with fixing the dizzy spells. In addition, my primary doctor diagnosed me with possibly anemia (low red cell count) and a B12 deficiency, so I started taking B12 vitamin supplements at about the same time as the CoQ10 – about 2-3 months ago. He agreed that this deficiency and the anemia could have caused the dizziness for sure. So, we’ll just continue with this treatment and the regular doctor is planning to check the B12 levels again at our 6 month follow-up.

We also talked about the heart symptoms: how’s the blood pressure, how’s the chest pain, how is the shortness of breath? How do I get along with the newest drug he put me on to treat all these (Amlodipine). I told him that the chest pain and shortness of breath are stationary, but I have more stamina when I walk (I can go further and on steeper inclines through the shortness of breath and the angina because I feel like my heart is getting enough blood supply). My neck still cramps, but after a longer walk. The blood pressure is medium-high (in the yellow-orange range on the machine) a lot more often than mostly high (red range), like it was before the Amlodipine. My gums are still very sensitive because of the Amlodipine but I am working with the dentist to use softer brushes, better paste to not irritate them too much.

After the physical consult, he said he thinks “I have more fluid than what he would like for me to have” and to back off the salt. This is the first time in my “heart-patient career” that anyone has said anything about salt, because typically my fluid is under control. He said my legs look fine but that my chest shows signs of too much fluid. He gave no reason as to why all of a sudden my fluid retention is higher, and no recommendation on what to stop or start doing (other than salt intake) to help with this.

As for the FH treatment, he said he would like to try the “twice a year siRNA PCSK9 inhibitor which might come out in the US sometimes next year” – his guess -  (he was referring to Inclisiran - https://www.novartis.com/news/media-releases/novartis-receives-eu-approval-leqvio-inclisiran-first-class-sirna-lower-cholesterol-two-doses-year) to replace the twice-weekly Praluent injections that I take now. I have asked him again (http://livingwithfh.blogspot.com/2021/07/who-knows-more-about-fh-you-or-your.html) about adding Evkeeza to the current treatment and he said “that would be another option as well”, but he made no recommendations about it. About this, I am puzzled: my LDL is nowhere near the “target” number of 70 mg/dl or lower, but he did not recommend adding anything else to my current drug regimen.

So, a mix of findings and if I were to summarize, I would say:

-          Heart function is stationary (no idea what the coronary arteries are doing because we would need a cath angiogram for that)

-          Arteries are showing advancing disease

-          Cholesterol (LDL) is still elevated, not at ideal levels for my disease and my history

-          Liver function a bit modified

-          Quality of life/ symptoms (dizziness, muscle cramps, chest pain and shortness of breath) somewhat improved.

I walk gently towards The Holidays with kind of a mixed bag and lots of unanswered questions. But … it’s better than six years ago when I was walking in with “you must have open-heart surgery in one to three months at the longest.” So, I’ll take it.

The Faces of My FH

 FH has many faces and many stories. I have homozygous FH (HoFH) which means that I inherited it from both my parents. As a matter of fact, both genes that came from them are the same exact gene, although my parents are not related, in any way, by blood.

My grandparents all came from huge families (think 10+ children). My parents have so many cousins they have not met all of them. This also means I have a lot of people on both sides of my family who have FH. And every one of them has a different story. A different story of their diagnosis, of their treatment, or lack thereof, of what the disease ultimately leads to. There are no two stories alike, and there are no two people that chose the same path in managing this disease (or not).

I see a lot of people with FH who are asking good questions about what to do when they are diagnosed; people who display all sorts of emotions, from sheer panic and depression to a nonchalance that I envy, in some ways, although I know that is not the proper course for a healthy and good-quality life when you have FH.

FH has been in my family’s life for generations – no one is shocked when they are diagnosed anymore. We’re all pretty much aware of what it is and what it can do to us: many of our aunts and uncles have suffered heart attacks, strokes, angioplasties, complications from diabetes and fat liver disease. Although we know all these things all too well, not all of us choose to receive treatment. More in the notes I drew below about my immediate family and their individual, unique stories.

My grandfather

Current age: deceased at age 65

Diagnosis age: as a young adult, after several of his older relatives and brothers and sisters were formally diagnosed with FH. At that time, they just spoke of “familial hypercholesterolemia” and did not dissociate between the HeFH and HoFH types. We believe he had heterozygous FH (HeFH).

Cholesterol levels: no one remembers for sure, but my parents think the total cholesterol stayed between 300-400 mg/dl.

Treatment: reduced fat diet; no drug treatment was available for cholesterol in Romania before 1990 when he died.

Complications: first stroke at 48, major stroke at 50 which left him paralyzed in one half of his body. He died at 65 after a massive stroke after having lived bed-ridden since he was 50 with the effects of the stroke and complications from diabetes. He also had coronary artery disease and high blood pressure.

My aunt

Current age: 71

Diagnosis age: as a young adult. At that time, they just spoke of “familial hypercholesterolemia” and did not dissociate between the HeFH and HoFH types. We believe she has HeFH.

Cholesterol levels: currently, the total cholesterol is between 200-300 mg/dl.

Treatment: no special diet, no treatment, by choice.

Complications: angioplasty (stent placement) in her thigh and upper-leg arteries in her 50’s; massive small-brain stroke at 67; high blood pressure, a-fibrillation, tachycardia.

My father

Current age: 69

Diagnosis age: in childhood, due to the fact that his father already knew about his diagnosis, my father was a sickly kid, and his mother (my grandmother) was a registered nurse who tested him for everything. At that time, they just spoke of “familial hypercholesterolemia” and did not dissociate between the HeFH and HoFH types. We believe he has HeFH.

Cholesterol levels: currently, his total cholesterol is 326 mg/dl.

Treatment: no special diet, no treatment, by choice.

Complications: several mini-strokes starting in his 40’s. High blood pressure in his 40’s. Diagnosed with coronary artery disease, peripheral atherosclerosis, peripheral neuropathy in his 50’s. His condition is further complicated by diabetes.

My mother

Current age: 68

Diagnosis age: 63. My mom’s cholesterol levels were in the upper 200’s all the way into her 50’s. She maintained that her cholesterol is not genetic, like my dad’s and it’s caused simply by bad eating habits. When she was 63, I had a genetic test that confirmed that I had Homozygous FH (HoFH). This was the clear indication that she, too, must also have FH. She suspects she inherited it from her father who died when she was 7. She had no further relationships with his surviving family, so the knowledge on her side of the family is very limited.

Cholesterol levels: currently, her total cholesterol is 313 mg/dl.

Treatment: no special diet, no treatment, by choice.

Complications: aortic valve stenosis, coronary artery disease, stroke at the age of 67. The cause for the stroke was unclear as she was also undergoing chemo treatment for lung cancer at the time. The doctor could not determine the cause of the stroke for sure – whether it was vascular or a complication of the chemo. She suspected it could be either one.  

Myself

Current age: 46

Diagnosis age: 8. My pediatrician felt an enlarged liver when I complained of pain in my upper abdomen. She sent me to get a complete liver and lipid profile, also knowing my family’s history of FH at the time. My mother found out the cholesterol level, as a hospital biochemist. At that time, they just spoke of “familial hypercholesterolemia” and did not dissociate between the HeFH and HoFH types.

At age 40, following a genetic test, I was diagnosed with HoFH.

Cholesterol levels: currently, my LDL is 107 mg/dl (the lowest it’s ever been). Before I started drug therapy at the age of 23, my LDL was 475 mg/dl. My total cholesterol was 526 mg/dl.

Treatment: no fat, vegan + fish diet, Lipitor, Zetia, Praluent, Nexletol.

Complications: diagnosed with tachycardia and arrythmia in my early 20’s; coronary and carotid artery disease at age 30; aortic valve stenosis at age 36. Open-heart surgery at age 40 to replace the aortic valve, ascending aorta, repair the aortic arch and repair and bypass four main coronary arteries.  

My sister

Current age: 43

Diagnosis age: 38. Although she knew her cholesterol was elevated, my sister did not get officially diagnosed and treated until this age. This was after my open-heart surgery which rang a bell of alarm for everyone in the family, I think.

Cholesterol levels: currently, her LDL is 108 mg/dl (total cholesterol is 201 mg/dl).

Treatment: low fat, white meat and fish diet, intense jogging (she is the runner in our family as she has been spared heart disease so far), Lipitor.  

Complications: no complications so far.   

My nephew

Current age: 10

Diagnosis age: 7.  

Cholesterol levels: last test showed an LDL of 170 mg/dl.

Treatment: all-inclusive diet, with less fried foods and lower fat, white meat.   

Complications: no complications so far.   

Whatever your story may be, what I believe firmly is this: it all starts with awareness: knowledge is power. You may choose not to do anything at all, but at least you know about the train that’ll be coming rather than one day be caught completely by surprise, way too late, when there might not be anything left to do or know anymore.

To honor the FH Awareness Day, these are the faces and stories of my FH family. What are yours? Do you know?!

Happy health, you all!

 

 

 

Good Numbers and a Slight Change in Drugs

I ended up getting double-checked for cholesterol this summer because in addition to my routine check from the cardiologist I also met with a lipidologist. I have been watching my cholesterol since I was 8 years old and let me tell you: I am yet to see a doctor who trusts someone else’s blood tests. They might rely on a CT scan result, or an MRI, but when it comes to blood – they will poke you again! However, in all fairness, the lipidologist was keener on checking the Apolipoprotein B and the Lipoprotein (a) than the whole lipid panel.

Here are the results:

June 2021 - cholesterol results

The bottom line is that the LDL number is the at the lowest level it’s ever been at 107 mg/dl. I wish I could say why, but not totally sure. My cardiologist thinks it’s the fact that we added Nexletol that made just a little bit more of a difference (in addition to Lipitor, Zetia and Praluent). But we added it in June of last year and it’s been higher then this in the meantime, although lower than before I was on it.

I did make two changes in my diet this year, too, which could have helped with the numbers as well: I eat a lot more nuts and seeds and I added more grains to my diet for about a month before those tests were taken. I also added more dark chocolate to my diet, which is a good antioxidant, as well (I don’t particularly like chocolate in general, but I have found that when it’s coupled with nuts, dark chocolate is actually bearable). Another bummer for me is that I cannot seem to get the HDL number up at all (despite the changes in the diet). It is actually going down even more. I am told that cholesterol medications bring all the cholesterol fractions down, and since I am on so many of them … there you have it.

I do complain about muscle tenderness (not so much as soreness because it hurts really bad only when I squeeze my muscles), and sometimes joint pain, both of which I have had for years now, but because I function just fine, I can stand up with no help nor pain, and am as independent as a healthy person would be at 46, we have not touched the drugs. We both want to see as much benefit as possible in the cholesterol numbers for as long as I can possibly tolerate the drugs. I know other individuals who make different choices here, but that is my choice. At least for now. And that’s just the thing: everyone should make the choice that is right for them. I know this sounds like a truism, but it bears repeating.

I was tentatively diagnosed with peripheral neuropathy this summer (EMG test to follow in a week or so for confirmation) which could be caused by muscle damage from statins. As a result, I added 200 mg of CoQ10 daily to my drugs to see if this will make a difference in the tenderness. But it might be that whatever damage the muscles have had so far might be irreversible, too – that, I don’t know for sure and no one seems to know. For now, I opt to be on the drugs and hoping for the best. The liver and kidney tests have almost always been normal.

The joint pain could be from my chronic inflammation (I try to keep this to a minimum through my diet), or it could be from Nexletol (which raises uric acid and causes gout eventually). As a result, we added the test to measure the level of uric acid to our quarterly “routine” tests. So, now, every quarter, I get a lipid panel, a liver panel, and  a uric acid. About once a year, the cardiologist or the PCP doctor also runs a complete metabolic panel to check for other issues, like anemia (which I have), or kidney issues. My uric acid has been creeping up on Nexletol, but it’s still within the normal range and I have not had any sign of a gout attack. We did not change the amount of Nexletol and I am still taking a full dose (140mg/day).