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.