The “En Garde” Life of an FH Patient

Happy New Year to all and here’s to much health, first. Here’s to everything else, second!

If you have read my blog before and if you are an FH patient yourself, you already heard me say this and you already know yourself that life with this disease is a constant checking in with your doctors, and a constant changing, adjusting of diet, medication, exercise, routine, etc … We always have some value to check, some artery to scan to see the progress of disease, a blood pressure, a calcium score to follow, or even new tests that we didn’t even start with when we started this journey.

Now, if you’re one of the “lucky” ones, like me, who has seen this disease affect other parts of your body, if you are a heart patient, or a fatty liver patient, or a kidney patient, then you have a full time second job to keep up with. But we all know this. I am just reiterating it for those who are new to it and might be wondering what it looks like.

But there is good news, you all! The good news is – if we do stay on top of these checks and appointments and scans and what not, we have a big change to a pretty regular life, otherwise. We live in a time where there is more and more known about FH and about what it can bring about if left untreated. We have places to research, we have The FH Foundation, and we have more and more medical professionals who recognize, understand and can manage it. Just remember this, too. The power is in the knowing, and there are infinite more places to find out information about it today than there were when I started 33 years ago in communist Romania!

Now, I’ll turn the focus on me and what the latest check-up has revealed. I saw my cardiologist in December. I did get the results to my blood tests shortly after that, but the rest of the tests that he ordered took a while. So I am posting this now so I can cover all the findings. I will have to give you a fair warning though and tell you that this might be boring. But, if you want to know the details of my every-day life, I hope you find something helpful here.

Before I saw my doctor in December, I had had a couple of months of really weird dizzy spells. I could be sitting at my desk, working, or on the couch, reading, and all of a sudden my head weighed 1000 lbs and the room started spinning. It is not like vertigo, it’s more in a heaviness and my knees sort of turn to jell-o. I mentioned this to him and he did several things:    

• he hooked me up to a holter monitor for 48 hours;
• he also ordered a ultrasound of my carotid arteries (which we know have narrowings of about 50-69%);
• he ordered an ultrasound of my abdominal aorta;
• he reduced my beta blocker drug (Atenolol) to 37.5 instead of 50/ day.

He figured that if there is an increased narrowing somewhere I guess it could cause the symptoms (hence the ultrasounds), and if there is anything “electric” wrong with my heart, the monitor would tell him. The atenolol could be slowing down my heart rate too much which could also cause the dizziness … I guess that was the reasoning behind all those.

Well, the carotids are stable – still at 50-69%, which they consider not yet bad enough to do anything about.  I am really happy about the fact that they seem to be stable for the past 4 years or so. Ever since my LDL dipped under 200 (which is still nor normal, but incredibly better for me), my carotids seemed to have slowed down the shrinking. Until then, every year they showed to be a little bit worse.

The distal abdominal aorta came back as narrowed as the last MRI showed – with a cross section of around 0.7 x 0.6 x 0.9 cm this time (I believe the normal diameter for a woman is around 1.7 cm). The abdominal aorta came out as less conclusive than the MRI showed it 7 months earlier, so because of this he is ordering an MRI next (but more about why that is needed in a second).

I reduced the Atenolol but for some odd reason, although my dizzy spells didn’t stop at all, my blood pressure started climbing right after I did that. I consistently got numbers like 167 over 59 and 165 over 55 and I felt even more dizzy and felt more heart palpitations than ever before. So, I had to take the Atenolol back to 50 mg/ day.

The holter didn’t show anything worth mentioning. Historically, though, the electrical function of my heart has been great. I have never had a-fib, and have never needed a pacemaker, or defibrillator, for a change!

Because of my blood pressure being so high lately, he is ordering this MRI of my abdominal aorta again. He wants to eliminate any suspicion of a severe stenosis before he does a nuclear stress test to look at my heart and before he medicates my blood pressure. So, those steps are next.

He also checked my cholesterol and my liver in December. My total cholesterol is up, slightly, as you can see in the picture below, but that is because my HDL is actually up, for the very first time in 5 years! I never quite buy the “eat nuts, they’re good for your HDL” talk, because I always think that fat is fat and your body doesn’t know the difference, but … I am here to tell you that the only thing I changed about my diet is the fact that now, I eat a handful of (very) dark chocolate – covered almonds once a day, almost every day (5-6 times a week). Now, the size of my hand is very small so don’t think that I am eating like a pound of chocolate almonds. It comes out at around 6-8/ day. This is the only change I made to my diet. Coincidence?! Not sure, but whatever it is, I’ll take it. I believe there is some research that says if you already have coronary heart disease and atherosclerosis the HDL does little to protect you against disease but I am pleased that the pesky LDL was not the one on the rise this time around.  

In addition to all this, he checked the level of my lipoprotein (a) for the first time which is slightly high, although he commented that “not high enough to be an issue.” I have included my level below along with the normal range. As I am learning about this new fraction, this value does not change during your lifetime (except it can increase with menopause for women), and does not respond to diet, exercise or medication. So, it is what it is, I guess, for the rest of your life. Here’s what the wiki says about it: https://en.wikipedia.org/wiki/Lipoprotein(a). 

I also like to know things like these just for my own education. Right now, there are no medications that affect this protein but it has been studied that it is one of the fractions that does contribute to CVD and atherosclerotic diseases. Maybe in the future there will be research that you could qualify for if you are one of the people with a higher value of the lipoprotein (a). All it takes is a blood test and you never know why it would be important to know.   

My liver values all came back normal, thank goodness! I always encourage everyone who is on any medication for cholesterol to demand that doctors check their liver values, because you just never know how these medications affect the liver and you do want to stay informed about what is happening in the liver before it’s too late.  

I went back to see my cardiologist this week because I am truly concerned with my blood pressure lately. We will follow up with an MRI of my abdominal aorta, like I said, and we’ll go from there. He wants to see my heart next but not if I have some sort of a severe stenosis in my abdomen. He believes my carotids look “clean enough” at 50-69% blockage, and he repeats what pretty much every other cardiologist has ever told me “when you are this young doing less too early is better than doing too much.” So, stents are not recommended for my age (almost 45) unless the stenosis is more severe than 90%.

He also checked my kidney values because the narrowest portion of my abdominal aorta is my infrarenal aorta. The kidney functions are fine except my BUN and Creatinine which are incredibly low. He did not comment on that at all (and from experience none of my other doctors do), but I researched this and one of the reasons they can be low is lack of protein in the diet. This would make sense, because I am almost 100% vegan and I eat about 1-2 servings of fish weekly. I do eat beans and nuts, but possibly not enough?! So, my next step is to incorporate more protein in my diet somehow. This will be a tricky business because protein messes up my INR level which is important to stay between 1.5 to 2 for my mechanical valve to be happy. Like I said: constant checking, adjusting, tweaking … and …

Onward we’ll go. I will probably follow up with one update after all the other tests are settled and we have stabilized or are on the way to stabilize that naughty blood pressure.

As they say in the heart patient world, folks: the best to you all, and keep ticking!

2019 - Another Year of Advocacy

Having a more-or-less terminal disease (isn't life one, too?) makes you appreciate every new day that lies in front of you. From the first breath you take when your eyes peel open and you look at the window to see the sun peeking in in the morning, you tell yourself: "Wow! What luck?! What blessing?!" It really is humbling ... 

I have been told since I was 8 that I will not make it past 18, or at the most past my 25th birthday. As I am entering the 45th year of my life, bruised and scarred and all, I am grateful for the years I have been given, and for the life I have lived. If I were to be born again today, knowing what I know now, I would not have had it any other way ... Like so many afflictions that sound and look like dead-ends, HoFH is a character building experience right alongside being hopeless and hard most days ... I like the hand I was dealt, although many doctors deemed it "a bad one." It's my hand. It's the only one I know, so I will take it. 

Apart from being selfishly grateful for another year, I am most grateful for the opportunities that I have had to share my journey and for all of you who have found comfort or help in my words. 

Looking back at the past year, here are some of the avenues where I have shared what I am living with every day: 

• I have continued updating this blog. You can find it by bookmarking it, subscribing to it by email or by following the News & Blog section on The FH Foundation's site: https://thefhfoundation.org/news-blog
• I have updated my Facebook page (https://www.facebook.com/LivingWithFH/) with pictures and events I participate in throughout the year, links to publications and articles that bring awareness and offer information about FH. I am truly grateful to you all who have become followers of this page this year. 
• I have also gained more Twitter followers if you follow me at @livingwithFH.  I post most of the articles I find, as well as events I participate in and links to posts on this blog at https://twitter.com/livingwithfh. I also share what I find about FH or heart disease here, as well as many initiatives that The FH Foundation participates in or advocates for. 
• In February, I wrote an article for the Circulation Journal of the American Heart Association about how I selected my valve when it was time for open-heart surgery: https://www.ahajournals.org/doi/10.1161/CIRCOUTCOMES.119.005523 
• In March, I was interviewed for The Health Monitor about what it's like to live with Homozygous FH: https://thefhfoundation.org/media/HealthMonitor_W1819.pdf?fbclid=IwAR2rzuB7OM-xOnxSmYJ8-KqgZ9dVlVRtfckd26Tku8HfWtwz6eqig-jIlxY
• In October, I participated in the FH Summit, and I spoke with representatives of pharmaceutical companies, geneticists, and doctors from the US and from around the world about our journey as patients with FH to hopefully create awareness about how not one treatment fits all and how what works for one country might not be true for another, and so forth. 

I can only hope that this is just a continued journey of advocacy and sharing and I truly hope you find help or comfort in knowing that, whether an FH or a heart patient, you are not alone. I humbly thank you for your input throughout the years, and for your guidance, as well as your readership. I wish you much health and a healthy and happy year ahead! 

The Stroke Family

How FH Has Affected Four Generations of Our Family

When some people are first diagnosed with FH, they have a hard time believing that they must be on medication to ensure protection of their arteries. There are folks coming to FH groups in disbelief, asking “if I don’t take my statins, or some other therapy, will I really get a heart attack? Will I really get a stroke? Will I be young when these things might happen to me?” Wouldn’t it be nice to know these things regardless of our diagnoses? Life, unfortunately, doesn’t work this way.

I, for one, can only tell you what has happened to the people in my own family who lived with untreated FH. You can view this information however you choose. I am not making a case for medications one way or another, here. I am just sharing what we have been witnesses to as a family. From what I have learned in the past few years, the story of my family repeats, more or less, in every family touched by FH. So, I am adding my voice to the chorus of folks that speak from experience here, in hope that this will be helpful to putting things into perspective for some.

I’ll start by saying that I have seen FH in my family with my own eyes for four generations now. It started with my father’s father and it stops, for now, with my nephew who is almost 9. I have been the first one to be medicated for it, but I started treatment when I was 23. Because I have Homozygous FH, however, even with all the medication available, my numbers are still out of the normal range and you might as well consider me an unmedicated Heterozygous patient now. So, what happened to the generations before me?

I was 3 years old when my grandpa, my dad’s father, had his massive stroke. He was 53. Before then, he had a smaller one, about 4 years prior, before I was born. When he had his first stroke, they learned that it happened because of his cholesterol which was at that time in the lower to mid 300’s. His first stroke was not debilitating, but the second one was. At 53, he was left bedridden from this second stroke. Half of his body (I can’t remember whether it was the left or right) was paralyzed. He walked around the house with a cane, and he never left the home after that, till he died from his last stroke at 65. During the time he was sick, he had many mini-strokes. My grandma being a nurse provided around-the-clock care to him and brought him ‘back’, she used to say, ‘many times.’ By bringing him ‘back; she meant she was doing everything she could to exercise various parts of his body that were becoming weaker and weaker with every stroke.

During all these years that he was sick, his brothers and sisters kept getting older and sick, as well. Out of the 10 living siblings he had, he had 5 affected by FH. He was the sixth one. Out of the 9 people who have passed so far, four of them died from complications from FH (strokes and heart attacks) and two of them died from a combination of complications from FH and diabetes. All of them died in their 50’s and 60’s. For the most part, all their cholesterol levels were in the lower to mid – 300’s, so as far as we can suspect, they all had Heterozygous FH. Most of them, like my grandpa, were not overweight. Grandpa was this stringy man, about 6 feet tall and maybe 150 – 160 lbs. He had been a construction engineer and he spent all his life on construction sites. His specialty was mountain access roads, viaducts and tunnels. He loved the mountains and being outdoors. I learned from my dad that my dad’s grandfather, grandpa’s dad, also died in his early 60’s from a stroke.

My dad’s cholesterol has always been, as far as he can remember, in the upper 300’s and lower 400’s. Grandma, his mother, did not have high cholesterol at all.

My dad’s first signs that something was wrong was in his 20’s. He had heart palpitations, high blood pressure and very elevated liver enzymes. He was diagnosed with a fatty liver at that time and high blood pressure. He refused any kind of medication until he was in his 50’s. And even then, he only started taking medicine for his heart, never for cholesterol. Being of old school upbringing and in a country where little is talked about cholesterol and its management, he does not believe in any of the cholesterol lowering drugs we have available here.

10 years ago, the lower extremities of his legs turned pretty much black from poor circulation. He was 57. He went to see a vascular doctor who determined that his arterial peripheral circulation was extremely poor, and he should probably have surgery to insert stents in his legs to open up the arteries. At that time, his blood pressure was something like 200 over 150 – and it stayed this way till very recently. He chose not to have surgery because he didn’t feel like his heart was going to be able to take the anesthesia.

About 15 years or so ago he had a minor stroke. He had minor symptoms of a stroke and when he saw a neurologist they confirmed that he probably had a stroke. This year, he had a second one. He is 67 now. This time, he has little feeling in his arms and legs, barely feeling the pedals under his feet when he drives and not feeling too sure he can safely shift gears. He gets very dizzy and has fallen several times even when just walking around the house. He is very stubborn to press on and he drives the car in this state and moves about town although not daily anymore. He is taking new medication now for better circulation as well as for improved nerve function. His cholesterol was in the upper 300’s last time he checked, and his liver functions are very high.

Dad’s only sister’s cholesterol has always been in the upper 200’s and lower 300’s. She had stents inserted in her legs about 10 years ago and her lower circulation improved after that. She had a stroke three years ago, when she was 66 which visibly slowed her down.

Dad also has another cousin whose father was grandpa’s brother. She had a significant stroke when she was in her 40’s. Like grandpa, half of her body was paralyzed, but in her case only the upper part. Her legs are still mobile, but one of her arms, her neck and half of her face were very affected by the stroke. Like dad, she also has a fatty liver.

Weight does not seem to be a factor in this disease, at least not in my family. Dad used to be very skinny until way into his 40’s. Now, dad and his sister are overweight. But his cousin is a 5ft – 110 lbs. woman and has been even lighter in her younger years. As I mentioned, grandpa was never heavy. I knew two of his brothers affected by FH and one of them was skinnier than him.

As far as everyone has been told so far, all the events happened because of atherosclerosis caused by FH. 

We did not find out for absolute sure that my mother also has FH until I was diagnosed with Heterozygous FH thanks to a genetic test at age 40. Her cholesterol has always been in mid to upper 200’s and she always compared her numbers to dad’s and mine and she decided hers are probably because of a poor diet. She was not overweight till she reached her late 50’s, so she never really looked into any medication for cholesterol. Thankfully, she has not had any events yet, but she does have a calcified aorta and aortic valve. She is taking a statin now with Ezetimibe to control her cholesterol. Because her mother did not have high cholesterol, we suspect that hers came from her father. He died when she was 7 from lung cancer and her mother never kept in touch with his family, so we don’t know what FH did on that side of the family.

My sister’s cholesterol is in the low to mid-200’s normally, but now she takes a statin and has brought it closer to the normal range. Her 9-year-old son has an LDL of 148 mg/dl with a total cholesterol of 223 mg/dl. They live in Canada and although they measure his cholesterol frequently, they don’t recommend that he should take a statin yet. We try to educate him as much as we can and even at 9, he reads labels and knows what foods are healthier to eat. Neither my sister nor my nephew is overweight and they are both extremely active. My sister is 40 and thankfully has had no events so far.

Another FH “marker” are xanthomas, but just like the varying weight, they are not 100% present in all of us. Grandpa, dad, mom and myself all have had xanthomas, but my aunt, my sister and my nephew do not.

You know my history, or can look it up on this blog to see when my complications appeared and what my levels are … All I can say is that complications from cholesterol do appear. That is a fact, as far as I can tell from my own experience and that of my own family. The ages at which some of them may appear may vary from one person to another. I do believe lifestyle has something to do with how well you manage those complications. I had a pretty massive surgery and an MI after that almost 4 years ago when I was 40. But I manage to stay pretty active after all that. I think a healthy diet and staying active helped me bounce back as much as possible (never fully) from all that.

If I could share a piece of advice at all, I would say: know your numbers and your heritage, learn from previous experiences and read the research and the testimonies. Always remember that there are not two people alike. So, whatever happens to one person might not happen to you. But I have found that learning from one another, from my family, and from the research available prepared me better for what did happen eventually. The rest is fate, luck, and the big unknown and there is a little bit of all of these in each one of us …

Stay healthy, you all! And make it a great Holiday Season!

Long Time, No Write …

How in the blue blazes has it come to be December already?? I cannot believe that my last blog was in July! We were eating peaches and getting our skin scorched in the sun and now, we’re watching the Christmas tree glittering in front of the fireplace. What a crazy idea, the passing of time?!

But passes it does, and it’s not asking us any for permission either …

And I don't even have a good reason for my long absence other than ... life. Life happens, you know, and lucky we are that and when it does! 

It’s been a busy year, personally, and for that I am grateful. It has also been a healthy year (or should I say, as I always do, “healthy for me”), and for that I am doubly grateful. We have travelled a little here and there, even went on a cruise in November. I only traveled for work one more time since I wrote here last. Travel is still one of the things I enjoy the most, and one of my more challenging things. I have the most trouble finding things to eat when I am away from home, because everything has to be vegan but not very green. The world still thinks that vegan means “a plate full of greens”, so it’s a bit challenging. I also have trouble sleeping and really getting comfortable. When I travel for work it’s always at very high altitude and I can hardly breathe and I tire even easier there. But I am so much in awe that I can still do it and live to tell the tale.

In October, my husband and I were lucky enough to be able to go to the FH Summit in Atlanta, GA. This was our first time although this has been happening every year for seven years now. The FH Foundation surely knows how to throw an educational event! We have learned so many new things, we have seen how much more people know about FH and how much more educated people (including doctors) are about this disease now. This Foundation has truly been doing a labor of love! We have learned about how doctors screen better nowadays for it, what therapies are out there in research or even approved but maybe not as well-known. I was the most pleasantly surprised at how incredibly approachable the doctors were at this conference, and how willing they were to learn from us, the patients, about what it means to live with this condition. If you or someone you know has FH, I would recommend attending, if you can, at least once. It will open your eyes and your hearts!

Besides the travel which is always draining for my heart especially, we have had a challenging year emotionally, too. My mother has still been battling lung cancer; my dad just had a stroke (more about this in a future post, I promise!), our last living kitty died while we were away on our cruise and my husband left a job and just started a new one which was a bit of a roller coaster, as well. Through and for it all, we are beyond grateful that we’re (almost) all still here and alive and managing our health the best we can. ‘Cause if you don’t have health how in the world can you do all the other commitments life throws at you?

Lately, I have had new symptoms of dizziness and light-headedness, low blood pressures and high, too. My INR nurse has noticed that around the holidays and when I travel I have a hard time keeping my INR in range (I still fight with this ONE number!). I mean, I have accepted that my cholesterol will always be high, but the INR cannot make up its mind! One day it’s 1.4, a week later it’s 2.9! Go figure! But this is what “normal” is for me now.

I wish I had more time for naps, as I feel drained almost always, but not really sure how to make time for that. The simple act of living for people like us means constant effort and awareness. I was telling my husband (as if he didn’t know quite well!) that there is not one thing that I put in my mouth that I don’t question: “oh, could it have butter? What about eggs? Could they have boiled this in chicken broth? Or since we’re in the South, could they have fried this in bacon?” I love eating at Oriental places because they never sneak in cheese into anything and they always steam their rice! At least that! There is no hour in the day that doesn’t pass without asking “oh, did I take my morning drugs? Is it time for Coumadin yet?” Or any week that I don’t think “is this Wednesday time for my bi-weekly Praluent shot?”

But we all, no matter who we are and what we’re up against, have a cross to bear one way or another.  We all have our own journeys. I am sure thousands of you have kids you worry about, parents you want to see living to an olden, ripe age. Jobs you hate and which stress you and yours out every hour of every day, houses you want to get out of or dying to get into … All I have this sick ol’ heart and my cholesterol. And this is OK, in the scheme of things, I guess. This … is manageable.

I promise to return much, much sooner with more updates about recent blood tests and heart and aortic tests, and also about what unfortunately happens as a complication of FH (will update you on my dad’s stroke). I promise I won’t be “on vacation” for months again.

Happy December to all till then! And never forget: make time for yourselves before you help others.

Why Are Cholesterol Numbers So Hard to Understand?

Although my cardiologist will tell you that I know more about cholesterol than he does, this is truly not a rhetorical question. I am really looking for the answer to it. 

After 36 years of watching these numbers, I am as puzzled by my cholesterol numbers as ever. The good news is – I am still at the better end of where the numbers need to be. But the bad news is, although I have seen some normal numbers while on my current regimen of drugs, the current numbers are not normal anymore, and they have not been this year.

If you have read the previous entry (https://livingwithfh.blogspot.com/2019/05/fh-is-pain-in-butt-literally.html), you know that my numbers were very low at some point last year (around September) and then they started inching back up. My cardiologist has exhausted all the guesses he has had so far (I think!) as to why this happened, since I have not made one change in my lifestyle nor medication.

We have exhausted guessing about the quality of the drugs maybe going down, or the fact that my body might be resistant to Praluent all of a sudden (maybe to the other drugs, too?!) – we still don’t have an answer to this last theory. As of late, he was trying to test the idea that maybe I was not injecting my Praluent properly, so for the past three shots (this is six weeks), he had me come into the clinic and let his nurse inject it “the correct way”. Which I did. Her correct day was not much different than mine: we both inject in the thigh, alternating thighs every two weeks. The numbers, as you can see below, have dropped a little, but not enough to really consider them being in a new range.

Sigh …

The guessing continues, and at this point this is where we are: guessing, or wondering, still.

The next step is to meet with him next week and try to understand what the immediate future plans hold. Some things we talked about were:

• Wait it out and do nothing. Continue with the current regimen and just keep the numbers as low as possible; they are still some of the lowest ever, so this is good.
•  Another option is to switch to Repatha. Maybe I respond better to it than to Praluent?!
  Maybe we have given Praluent a fighting chance and this is the best it can do for me.

At this point, this last option makes me angry and deflated, because this means a renewed battle with the insurance company to get Repatha approved, when I thought I was pretty much in the “safe zone” with the Praluent, since they have been approving it for three years. Sigh, again … Also, I am not sure what this would mean for my bottom line: for now, Praluent is clearly their preferred drug, because I have a $0 copay for it. Not sure how much Repatha would be.

I will wait for the appointment with the cardiologist next week to see where we go from here. But so far, so not so very good, I guess, you can say. I keep reading to see if others experience this “plateau” stage at all, and whether there is anything in the current research about how Praluent works (or stops working or changes the way it works) in the long run. So far, researching and staying tuned is about all I can do.

Much health, everyone! And many answered questions to all!

“FH Is a Pain in the Butt. Literally.”

The title needed quotation marks because it was suggested by my husband after my latest appointment with my cardiologist. I hope after reading this post you will concur with its appropriateness.

As we all know, managing FH is a journey of discovery. You have the condition, and then you have the complications. You have some treatment and then you have some side effects that also need to be managed. Some drugs work, some don’t, some drugs work for a while and then stop. And then they need other drugs to help them up and then their little helpers stop working … After 20+ years of managing this disease with drugs I am still puzzled about what does work and why. And about what makes drugs (or other things, like diet, my body and its hormones and its natural disposition) stop working with me to keep the numbers low. I am always learning and always starting over, it seems.

I know that a lot of people who are just now being diagnosed want to know the magic trick or cocktail they need to be on that will ensure they can manage the numbers and prevent events. But the truth is, as I have found it: there is no silver bullet. It’s what works for you, and that answer can take a while to figure out. Just make sure you get good doctors that understand the disease and why and how it should be managed differently than regular “high cholesterol” and try what is available out there (there are lots of options now!), and learn your body along the way and know what works for you. That would be my advice.

And with that, yes, I am myself still looking for that magic trick that would make my numbers normal – which has happened just once since I was first diagnosed 36 years ago. As you can see below, somehow, and we don’t know why, my total cholesterol and my LDL (which is more important) were within normal ranges once in September 2018. But since they, they have been going back up. Since January of this year, we have been trying to figure out why this is. In the words of my cardiologist, “I wanna know what happened to those 60 mgs of LDL! I wanna give them back!” – but we have not managed to find a reason for this.

We know that the numbers dipped to normal levels only after we added the Praluent. So, naturally, we think the Praluent might also be responsible for the numbers going back up, somehow. We went as far as contacting the Praluent manufacturer, but we have not gotten very far with them. In the beginning they said there is no known evidence that a patient could build resistance to the drug, but the second time (more recently) when we contacted them, they said there is a very small margin for resistance (I cannot remember if they said there is 0.3 or a 0.03% chance of building resistance – is what their research shows. They said “it is not zero, but it is very small”.) The course of action now is to go to the clinic and have the nurse inject the Praluent instead of me doing it on my own, just to ensure that I am administering it correctly. They also asked me where and how I inject the drug, and I told them: I have been doing it the same way, in the side of my thigh, alternating legs every 2 weeks about the same time of the day for 3 years now. Now, the manufacturer rep said it needs to be injected in the top (not side) of the thigh, so we will try that for a while and see if the numbers change. Again, a word of caution for folks out there: the cardiologist and the manufacturer both told me again that the drugs work best when injected in a muscle and not in a fat tissue; so, not in your stomach, but in your thigh or upper arm, where there is more muscle. At least, this is the advice they gave me.

I am not aware of any other changes that could trigger these numbers to go the other direction for me, and the cardiologist did not suspect anything else to be the culprit. So, we will see. Maybe it’s time to try Repatha instead?! Not sure …

In addition to this journey, there is the parallel journey of making sure that my heart and my arteries stay healthy, or whatever “healthy” means to me. So, to ensure that, the cardiologist ordered for the first time ever a complete body scan (with contrast) of all my arteries. I say “all”, but I mean only the arteries in the trunk of my body, if you will: from my neck to my pelvis – so, no brain, and no legs.

His idea was that if the damage in the arteries in my heart was so severe three years ago when I had my surgery, then he is wondering about the status of the rest of my arteries in my body – is there an aneurism anywhere else like I had in my aortic arch? Are there any blockages that need to be opened up like the four that needed bypassing in my heart?! What exactly is going on?!

The test showed, as you would suspect, atherosclerotic disease of various severities pretty much in every artery scanned. Very few had mild disease, but most of them had moderate, or moderate-to-severe disease. The worst (moderate to severe) are my thoracic and my abdominal branches of my aorta, where “the infrarenal abdominal aorta in particular is severely narrowed down to a minimum cross sectional diameter of 0.6 x 0.6 cm just above the takeoff of the inferior mesenteric artery.” I am told that this diameter would be equal to about a 70% blockage. The severity seems to match the one in my carotids, which is between 65-75%. Just like the carotids, the course of action here is to do nothing right now, and just to repeat with an ultrasound every year and ensure that the blockage does not become bigger. If it gets closer to 90% then surgery for a graft or a stent might be recommended.

The doctor said that if this blockage would cause symptoms, because of where it is located (right after the aorta leaves the kidney area and before it splits to go to my legs), I would notice a sharp pain in my buttocks. I don’t notice that, but I do have pain in my calves when I exercise – he said he doesn’t think that pain is related to this, because my buttocks would also hurt in this case. Hence, the title of this blog.

So, as of right now, these are the things I need to watch pretty much every year for as long as I live:

• Cholesterol numbers (every 3-6 months, typically, but we’re doing them more often now because we’re trying to figure out why they have gone back up)
• Liver enzymes (every 3-6 months)
• INR (weekly) for the health of my mechanical aortic valve
• Heart echo (yearly)
• Carotid ultrasound (every 2 years)
• Thoracic and abdominal aorta ultrasound (every 1-2 years – this is new and the cardiologist will confer with a vascular surgeon before he sets a definite schedule).

Of course, at any given time when a new symptom comes up, he orders other tests, as well.

Some might say that FH and all the complications that come from it is a full time job. I prefer to look at it as a life style: it is part of our bodies just as much as the color of our eyes. We, people who have it, learn to incorporate all this knowledge and all the tests in our daily routine. We need to know that along with vacation once a year, we need to make time for a couple or three echos and 3-5 blood tests. Plus, daily medicine and a healthier than average lifestyle. Knowledge is power and knowledge and taking advantage of the medical developments out there will give us a healthier and fuller life. None of us have control over the length of our lives, but I at least try to have some control over the quality of what I have left.

I am grateful that we have the tools that allow us to know and manage this disease. We can be mobile, aware, and do pretty much everything we want to do in and with our lives. There are millions of patients out there suffering from so many other afflictions that do not get this lucky.

Onward, all! It’s the only way …

The Heart Update: A Heart Event and New Heart Tests Results

A few weeks ago something really bizarre happened. On a Wednesday afternoon, my husband and I went for our usual bi-weekly or so walk in our neighborhood. We have what I call a “kick-a$$” (for me) hill right outside our driveway, about a mile long, round trip. This makes for good cardio. I have a landmark on the side of the road (a bright red fire hydrant) that is almost at the top of this hill about where my symptoms kick in: I start really losing my breath, choking up, my left arm gets numb, and the cramps in my legs start pulling at my muscles really tightly. These symptoms happen about 90% of the time when I do this routine walk, especially when I go at that hill fast. I normally do go fast because my cardiologist is of the belief that we really need to push our heart into cardio mode to grow new, healthy arteries. As my old ones are bad, I figured this is a good enough reason, but I almost always pay for it with these symptoms. They do ease up after I am done with the hill and walk back down, or wander around the neighborhood on more flat grounds.

I (sort of) digress, though. So, on this Wednesday, my symptoms start kicking in at around my landmark and they get worse as I walk. We make it to the top of the hill where there is a flat part of our walk, and where usually the symptoms kind of plateau or calm down. But on this one day, they were actually getting worse, even after was done climbing. On top of the usual cramps and numbness in my left arm and shoulder blade, I was now getting this incredibly sharp pain in my chest: like a huge eagle or owl was sitting on my chest digging their huge talons into my heart, or somewhere where my heart should be, somewhere behind my sternum.

We came back home and the chest claw was not letting up. My breathing was labored and I felt like I had SO MUCH acid reflux in my chest – I remembered very clearly that when I had my heart attack after my surgery, I thought I had acid reflux, with my chest full of fluid and that I was not able to burp it. That same feeling was coming back that day. I felt like I swallowed a basketball-sized water balloon and it was pushing into my lungs and chest walls or something.

Back home, after I rested for a bit, I took my blood pressure and it was 150/58 with a pulse of 58. I thought: well, something really did happen or my heart really did work out, since my pressure is clearly up! I kept feeling weak all evening, with the watery chest persisting and the claw coming and going. Because of all these new symptoms I did not even think to take a Nitroglycerin. I was just in awe: what could cause all this and what was happening?!

Next day, my weakness and full chest continued. My blood pressure kept climbing up all throughout the next day: 149/62 in the morning, 189/81 at lunch (what?!), 141/50 in the evening. All of these while at rest. I did not walk anymore. I was just around the house all day, doing light chores, but all these numbers and what I felt were scaring me. On Thursday morning, I jumped on the scale in my bathroom, as I usually do about 2-3 times a month. It read 120 lbs! This was 6 lbs more than the previous time that could not have been longer than a week and a half or two before! The feeling of bloating and puffiness in my core body was still there and I could not help but wonder if this was all fluid build-up. I had not changed my diet in any way. I was not lazier than normal, so  what could possibly make me gain 6 lbs in that short of a time?!

I have a pretty good relationship with my cardiologist and thought to text him a few times, but I also had an appointment scheduled with him the following week, so I thought I’d wait. I figured he’d send me to the ER if I texted him at that point and as much as that I figured out myself, but I did not want to deal with the ER. Not yet. I was hoping that I won’t get light headed or dizzy and as long as I didn’t feel that, I was going to wait this one out. So, I did.

On Friday, we had a weekend trip planned to Virginia to visit relatives and the symptoms continued during our drive up there. I took my bp monitor with me and kept records of it, because I wanted to show my cardiologist what was happening. The high blood pressure continued throughout the weekend. On Friday night my legs were so swollen, you could not see the bones in my ankles anymore. My feet  could not fit the clogs I was wearing except for the tips of my toes. On Saturday, we went to browse a Barnes & Noble store and I was walking around skimming through books and my chest claw came on again and I felt like I was going to fall down – it knocked the breath right outta me. I had to sit down. I was not dizzy, but very weak.

I continued to just take it easy and enjoyed our day up there – my husband got to reconnect with relatives he had not seen in 35+ years, so I was so wanting us to just enjoy our time and make new memories.

We came down on Sunday afternoon and I drove the whole way (about three and a half hours), just to take my mind off of this mess. I was absolutely exhausted when we made it back. I took a shower and napped. My blood pressure was still hanging out at 169/65 after my nap. My ears were bright red. I was afraid to jump on the scale again. My sleep was poor, as well: I just felt like suffocating when I laid down and I could hear my heart beating so strongly and my valve clicking louder than normal. I have a stethoscope, and I listed to my heart for new murmurs or swooshy sounds, but I could not hear anything new.

Next Monday, I woke up and I did check my weight again: it had dropped to 116! Just like that: from Thursday to Monday (in 4 days!) I had lost 4 lbs! I wish this would happen every week, believe me, but I knew this could not be just fat loss. Along with this mysterious weight loss, my bp was 115/50 that morning. The claw in my chest was gone and so was the full, watery feeling. All seemed to be back to normal. Just. Like. That.

On Wednesday I went in for a scheduled heart echo and for an appointment with my cardiologist. I explained all these to him and he has no idea what happened! He doesn’t want to go on guessing. He examined me, and heard no new sounds, either, and no sign of fluid. He did say that it does sound like I had fluid backed up but it was obvious to him it was not still a problem, as my leg swelling went down and my blood pressure too. He did check my C-reactive protein levels which could indicate an inflammation, I guess, and that came back normal. He just advised that next time I should take an extra-half of Atenolol (which I take daily) and see if the heart palpitations and the bp would go down. If that doesn’t happen, to call him or go to the ER. Of course.

The echo he ordered was just my yearly routine echo. It came back with almost no changes from the previous year, although it still shows disease, of course: there is annular calcification of my mitral valve, but the valve is fully mobile. My left atrium is moderately enlarged. My ventricle is normal and it squeezes strongly, the doctor said. My ejection fraction is still sitting at 55% which has been unchanged since my surgery, three years ago. I remember when it was 70%, even the year before my heart surgery, but I don’t think I will ever see that number again! He also said that by looking at the pictures, he thinks my aortic insufficiency has improved and it is not as bad as before. I was kind of wondering about that, because the gap between my blood pressure readings has gotten smaller. It’s still not what you would expect (the diastolic is still lower than what you want), but it has definitely closed up a bit. A good report, all things considered.

He also ordered an ABI test (or ankle-brachial index test - https://www.webmd.com/heart-disease/ankle-brachial-test#1) to determine if there is vascular disease in my legs, since they always get cramped when I walk on inclines or very fast. This test “indicated no significant arterial occlusive disease at rest and after exercise bilaterally.” They did make me walk on a treadmill, for the exercise, with an incline of 10 and a speed of 1.5 for 5 minutes. When I walk on the treadmill and when I do get symptoms, I usually walk with no incline and I reach a speed of about 3 mph. I usually get cramps about 10-15 minutes into the exercise, so during their test I did not get symptomatic at all. Not sure how to interpret this, but the cardiologist assured me that if there were any disease it would have been evident even in the case of a light, short walk with a steep incline like in the test they performed.

I wish I understood more about what happened that odd week. Since then, my blood pressure has stayed within normal ranges, or what normal is for me. I am back to my usual routine and I did go on my usual walk several times now, and the hill has not caused a problem yet. I am glad that it all appears to be cleared up now and I am back to my normal, but a nagging thought in the back of my head still tugs at me: was it a heart attack? What was that?! I guess, for now, I am grateful I am still here and back to my normal routine and looking forward to more normal days. Sometimes, this is plenty enough!