How FH Complicated My Health

I follow a number of forums and pages dedicated to FH and heart disease. Let me just say first that I am grateful that they exist. Back when I started this blog, there was no information out there about our disease and no way to reach out and meet other people like us. For this, I am deeply grateful. And I have spoken about this before (http://livingwithfh.blogspot.com/2018/09/from-obscure-past-into-bright-future-on.html)

Every once in a while I see a new person on these platforms that was newly diagnosed. One of their first questions is “if you do nothing about FH, would you automatically have any symptoms or develop any complications? Would you automatically get CAD or other arterial diseases?” And to be honest, I never know the absolute answer to these questions. I think doctors and other medical professionals should answer those and should back up their answers with research.

If I were to answer these questions for new patients myself, I can only speak about my own story and about my family's history with FH. In our world, you do get complications, symptoms, as well as CAD even when you do things to control the disease. That is for certain. That is our certain.

This year's been an odd one for me, with trying to understand and manage several complications. First, after two years since my heart surgery, I went back to cardiac rehab this year, to try to build some strength back into it. A stress test revealed that the bottom half of my heart (the left ventricle, really) gets very little if any oxygen during exercise. Because of this, I still have angina when I exercise which prevents me from going for a longer amount of time on the treadmill and from truly performing any cardio exercise. The idea is that with more exercise (as much as I can tolerate) more young blood vessels might appear to supplement the circulation of that part of the heart. The story of my cardiac rehab is a long one and maybe one day I will sit down to write it all. For now, I can only say I am trying hard to exercise regularly, but there are still limitations that prevent me from going so fast or so far.

Because my heart is somewhat under control, I had some time this year to address other complications that I have been living with for years now. One of them is the elevated enzymes of my liver as well as pain in the liver area. Two ultrasounds and a GI appointment later, they are still working on a diagnosis, but preliminarily they think I have something that they call tumefactive sludge in my gallbladder as well as non-alcoholic fatty liver disease. There are more tests to be done, but these are the first 'guesses' of the GI doctor. I asked him what could have caused this (they both lead to scary things like gallbladder cancer and liver cirrhosis) and the answer for both was “I am not 100% sure, but I think your Homozygous FH makes the most sense.” Like I said: we're still looking for a certain diagnosis, but based on my symptoms and enzyme levels, this is the deal for now.

Also after many years, I finally found the time to remove a lipoma from my neck. I know that non-FH people could get lipomas, too, but the amount of fat they removed from my neck is unnatural, in my opinion. It was more like a quarter pound of fat than just the size of a mole. I have had xanthomas removed twice before, and the contents of this year's lipoma looked very close to those, although the look under the skin was different than those. I cannot help but wonder if it would have ever gotten this big without the FH.

These are just some of the new things I have managed this year, alongside my existing complications that I have managed for many years now: the heart condition, the carotid stenosis, peripheral arterial disease. And all these have happened with having been on Statins for 20 years, Zetia for 10 and Praluent for two.

Like I said before: this is my story. Every one of us has a different kind of FH and a different body we're fighting it from. I am sure there are some people out there who are lucky and never experience any of these symptoms or complications, and are maybe considering not doing anything to try to manage this disease. However, from what I have seen myself and from what I have seen in my family, I am not going to take any chances. Yes, my life is not very easy even with treatment, but I cannot imagine how much different, how much more difficult would it have been have I done nothing. Or whether I would have been alive today.

In the closing of this year I hope, as always, for much health for everyone. I also wish everyone good doctors and teams to help them understand their specific condition and navigate the murky waters in compassion and tender care.

Happy New Year, everyone!

Between Tests, Doctors' Visits, and the Rest of My Life

It's been a while since we chatted. Life seems to have sped up there for the past few months. I guess it's good when you make plans but even better when you get to carry them out. But delay no longer.

I have been traveling, mostly. Some of it for work, and some of it for pleasure. Since the beginning of August, really, I have been hopping airplanes and Marriott hotels like it's my full time job. I am tired. I won't lie. I am doing it, mostly alone, but it's taking a toll on my stamina.

During this whole time, I have also worked full time and have carried on with my cardiac rehab appointments, sometimes as often as three times a week. Just writing this makes me yawn.

In September, my regular doctor did some blood tests to check for a few things, but especially my cholesterol and liver values.

The good news is that my cholesterol values are at an all-life low. It is truly unbelievable how well drugs work for me, and how lucky I am. Not even one hour of apheresis in my entire life, and the drugs I am on just dropped the cholesterol this much. My LDL is still not at the target that the doctors want, which is below 70, but I am happy with it. News like these don't happen often for HoFH patients!

The bad news is that my liver enzymes continue to be elevated. This trend started two years ago when I added the Coumadin, an anti-clotting medicine, after my heart surgery, as well as the Praluent injection. Now, my doctor is ordering a liver ultrasound to understand what is going on with it, if it is visible on an image. I am not really sure what the course of action will be to try to get the enzymes back to normal. Although I do have pain in my upper-right abdomen, I have virtually no other new symptoms from all this medication which seems to work so well. But … we will have to weigh the good with the risks and make some decisions pretty soon.

During one of my work travels, I had the chance to go back to Utah and meet with my surgeon for my yearly appointment. He insists that he still wants to see me every year till further notice. I love this, because I love him. What he has done for me, the life he has put into my remaining years is absolutely humbling. He was truly scared when he realized I had not only had my recommended yearly echo but also an angiogram since I saw him last year. He thought that something bad happened that required the angiogram. I explained that because I had moved to another state, I have a new cardiologist who is trying to understand my angina symptoms and explain my weird blood pressure numbers. Part of that quest was the angiogram to figure out whether there are severe blockages in my heart. (http://livingwithfh.blogspot.com/2018/05/another-cath-some-more-answers-and.html)

I shared with him that the same blood vessels that were 99 and 90% blocked before he did the surgery are now between 30-50% blocked. I asked him how could that happen, because as I said before, I cannot credit the Praluent alone with this huge improvement. He reminded me that not only did he bypass the blocked arteries, he also performed endarterectomies (https://en.wikipedia.org/wiki/Endarterectomy) on all of them. He also reminded me that it is clearly written in my operative report which I have. Now, if I didn't just chalk this up to a “fancy medical term” and I would have actually looked it up, I would have known. But I did not. Now, why didn't my current cardiologist know this, when he has my operative report himself, I am not sure. The cardiologist looked puzzled that I had bypasses at all because the main arteries were in good shape. Again: many thanks to my brilliant surgeon!

He said he not only bypassed them “he also cleaned them out for good measure.” I am telling you – the man is a saint! So, now the mystery of my virtually clean heart arteries is solved: endarterectomy + bypass grafts = functional arteries.

As I mentioned briefly, I have also been going to rehab since August, too. I am still very much underwhelmed with this program: there is not much monitoring, or explaining what is going on. We write our BP and pulse values in our charts ourselves. How long or fast we exercise seems to be up to us only. Whether it's because of my very fast lifestyle this year, running through airports with heavy carry-ons, or rehab, my stamina seems to be much improved and my breathing is also a little less shallow and my chest a little less painful when I exercise. We have this steep hill in our neighborhood and every time I climbed it until recently (maybe a month or so ago), my chest would tighten and my left upper body would be completely numb and painful. These symptoms are gone now, and I can climb the hill, still slow, but with no pain.

I am sure that since we moved closer to sea level (we were at 4000 ft in Utah, and we are at 500 ft now), my breathing has gotten deeper and my heart feels better overall. Every time I go to Utah for work now, I clearly see the difference between how I breathe at home and how I breathe there.

There are days when I want to call the cardiologist and quit rehab altogether (still have 17 sessions to go – argh!), but I am really hating having to argue with him. He believes very strongly in this rehab business and that this alone will improve the blood flow in my heart, grow new arteries, and improve the pump function. I might just stick it out. Or at least I will try my best to for as long as I can. Trying to balance work and travels with a very strict rehab schedule, though, might decide otherwise. If I truly saw the full benefit of this exercise alone, without what I do on my own, I would definitely put my health first and finish the 36 sessions that come with this program. But I don't see that going in for an hour and 45 minutes a couple of times a week and walking on a treadmill (which I can do at home, with no schedule, no driving for an hour one way, etc) makes that much difference for my health. We shall see …

I hope that all of you had a great summer and are having a beautiful fall. I hope you are ready to close out this year in health and peace.

Much health, to all!

From an Obscure Past into a Bright Future: on FH Awareness Day

I was diagnosed with FH when I was 8. That was in 1983, and it was back in Romania. Back there, it was a very rare and obscure disease, but they at least knew enough to know it is inherited from a parent and that you would have 50% chance to pass it on to your kids. My numbers were off the charts high, far more than my dad's and his dad's before me. They did not make the distinction between Heterozygous FH and Homozygous FH at that time, to understand that my numbers were so high that both my parents probably have it. They never suspected my mother. Her mother did not have high cholesterol so she didn't think she did, either, and she never tested hers till much later in life. After all, she was 30 when I was diagnosed. Unless you had a family history of high cholesterol, heart disease, or strokes, like my dad had, high cholesterol was an old people's disease. There were no effective drugs in Romania and apheresis was never offered to me.

I came to the US in 1998 and I was put on statins right away which slashed my numbers in half (I was in the upper 300's) – still high but lowest I had ever been. I struggled to learn more information about my disease. All the doctors I was seeing, although they said the name of the disease to me, never put the name of my disease on a medical form. I insisted with every visit that I have Familial Hypercholesterolemia, but they kept writing “dislipidemia” or “complex dislipidemia” or “high cholesterol” on my charts. I wanted to understand why my numbers are so big, and why some people (like my dad) had lower numbers. I wanted to understand what other treatments science was working on, I wanted to understand how other people with FH live and how their journeys were, but I found no information at that time.

Finally, after many years of a knowledge vacuum, I decided to start this blog, in 2011. I was hoping that at least I could document my own story and some other people that would just now find my blog and see some similarity between my story, my family's story, and theirs might learn something they were eager to know, like I was.

Shortly after that, the FH Foundation was founded (same year), but I did not learn about it till maybe 2-3 years later. Since then, lots of positive things have happened for me, and for many people with this disease:

• Thanks to the exposure to the FH foundation and the education that they do, I have learned about the difference between HoFH and HeFH after I was genetically tested for this. I know now that I am an HoFH patient and that my mother most positively has FH, as well. She is now religiously taking statins, although the CVD damage has already been done.
• Also thanks to the FH foundation I have met a lot of people like me and I understand more about everyone's challenges with this disease. I know now that we're not all alike. There are as many symptoms and medical resources as are patients, for the most part. There is no 'one size fits all' when it comes to the symptoms, the complications, or the medical resources we have available to us for treatment when it comes to this disease.
• The FH Foundation played a huge role in getting our disease a medical code, so now our claims can be properly filed with our insurances – our disease is not just a disease of “high cholesterol”; our disease is treated differently than just high cholesterol and our risk factors for heart disease and stroke are very different than the “regular” high cholesterol patients.
• Through the network of physicians and researchers that they work with, the FH Foundation continues to play a huge role in educating clinicians about this disease so they can be better prepared for patients like us.
• We now have websites, Facebook pages, Twitter feeds, Instagram accounts where people document their stories and share information with all of us.
• We now have an FH Awareness Day and Month! We have come of age!

And these are just some personal observations about benefits I have directly felt over the years where the FH Foundation has contributed directly to improve the knowledge and the awareness in our communities for this disease. 

My latest proof about how far this disease has come, in recognition alone, came just this past week when I received yet another write-up from my medical chart after a doctor's visit. This was not a note from the cardiology office, but from my regular doctor. I am including the picture below. This is the first time in my 43 years of life and in my 35 years as an FH patient when I see my disease spelled out for what it is on paper.

My latest list of diagnoses includes the correct one, finally: Homozygous familial hypercholesterolemia

Tomorrow is FH Awareness Day – another incredible proof of how far we have come. I am incredibly grateful to what every patient, every advocate, every clinician is doing to bring this disease out from obscurity and share the wealth of knowledge we now have about it. With every small step, we make a giant leap towards much better quality of life and possibly longevity that FH patients will have for many years to come. I am looking forward to many more years of accomplishments and recognition.

To all, good luck, much health, and let's all share the story for a brighter, more knowledgeable future ahead! Oh, and Happy FH Awareness Day, too!

Not All Cardiac Rehab Programs Are Alike

So, I am back in cardiac rehab – two years and 6 months after my surgery. It feels odd, and a bit tardy, but I only have so much energy to argue with my cardiologist.

The reason he sent me back is because I still have shortness of breath, I still have a chest squeeze and a pronounced numbness in my left arm, shoulder blade, and collar bone when I exercise even “a little bit”. Or whatever they consider a little bit. He doesn't think this is valve related, although the On-X mechanical valve I have is leaking a little bit. But not enough to make a difference in symptoms, he says. But enough to cause my large gap between my systolic and diastolic BP. He sent me back because he thinks the symptoms come from the fact that my smaller arteries are still clogged up with cholesterol (because of my FH) and my heart won't grow new vascularization unless it's very active. Since I am limited by my symptoms, I cannot exercise enough to grow new blood vessels, therefore the need for cardiac rehab, to recondition my heart and also monitor what it's going on when I do exercise.

And this is where the disconnect happens. I have just ended my first week in rehab (the second time around, like I said). This time it's a different state, facility, medical system than before and it is by leaps and bounds different from how my first experience was. This time, it feels like just going to a gym all by myself, with the only difference being that there are spectators around me watching my exercise. But that is all they are doing. Almost.

They do take our blood pressure when we walk in and when we walk out – and we need to record it in our folder ourselves. But they don't take our blood pressure as we exercise (they did the first time I was in rehab – several times, in fact).

We do wear a monitor that we hook up to our chests ourselves when we walk in (I pray every time that I do it right, but who knows?!). And they tell us that our heart “looks fine” because the monitor talks to their computer during our exercise. They said they would tell us to stop exercising if they see any EKG modifications. But it's like 10 of us and one nurse looking at the monitor and doing 10 other things, so I am not sure how they would be able to catch the modifications as they happen. She doesn't seem too focused. When I first went, we had an assigned person who would monitor just us the whole time we were exercising.

We have to pick our own machine that we want to exercise on, and we need to use that machine (treadmill, two kinds of bikes, elliptical machine) for 45 minutes. How fast, or steep we go is our call, but they do want us to reach our “maximum heart rates” which they base on our weight-height-age. After 45 minutes, we all go to the strengthening and relaxation room and exercise in a group as we all follow the physiologist in strengthening exercises for 20 minutes before we slow down during the relaxation time, which is roughly 10 minutes (that is a total of an hour and 15 minutes, if you didn't count).

During this whole time, you have to write down in your folder your own numbers: how fast you walked on the treadmill, your speed, your length of time and your RPE (Rate of Perceived Exertion) – in other words “how strenuous” you think your exercise was. They only take your blood pressure when you walk in and when you walk out, so when you are rested. They tell you what the highest heart rate was during your cardio exercises and you write that yourself in your folder. They ask you to take your own pulse at the end and record it in your folder (thank you, Apple watch! - almost everyone in the room is counting theirs themselves).

This was all done for me by the physiologist, and my blood pressure was taken 3-4 times during the cardio exercising the first time I was in rehab.

This past week, the physiologist I worked with the first day said there “is no need for them to take my blood pressure during my exercising, because by looking at my history she does not expect my blood pressure to drop.” She said that she expects it to rise with exercise, which is perfectly normal, but not drop. I was dubious, so dubious that I was speechless and didn't argue that nor told her about my jumping blood pressure for no reason at all. I also did not tell her about the regular huge gap between the BP numbers (which is my number one concern) and which I would like to see/ understand whether it can be cleared by exercise.

The second day I went in, the second physiologist asked me if I have questions and I told him that I was not expecting to be monitored so little in a program that is branded as a highly medically assisted one. I told him that the first time I was in rehab, my blood pressure was monitored closely and the numbers were not good, but they did show that my heart is not happy when it's pushed too far. I also told him that my cardiologist told me they would do a stress test before I would even start rehab so they'll know my limits, rather than tell me what my limits are based on my weight, height, and age. Given the condition of my heart, my limits should be lower. They never did a stress test before I started this. They said, however, that they based my limit on the stress test my cardiologist did in March (5 months ago). But even that one was different than the one they had done in December last year (9 months ago), so my heart is always changing, it seems. Don't they want to know what it's doing now?! Apparently not.

He apologized hurriedly and he said “oh, we can do the stress test any time during the program, no big deal.” But for now, he said, let's try to get your heart rate at least above 90 (my goal was 119, apparently, based on the last stress test they had access to see, but he wanted it at least above 90 given that the first day I was there it was only 86). Although I did put an RPE of 9 the first day when I exercised on the bike, indicating that I was very tired and breathless during that day, had my chest squeeze, he still wanted me to do more the second day.

So, I tried. I also told him one of my biggest concerns is my blood pressure and I am surprised that they do not monitor it during my cardio. He said he will monitor it that day closely. So, with me pushing myself and him taking my pressure, off I went.

My blood pressure climbed up to 155 over 55 (yes, the diastolic is always that far from the first number), and at that point I was out of breath completely. He said I was not at 90 yet, I was still at 86. I told him it's hard to really make my heart rate go really high because I am on atenolol, too, which is designed to keep the rate low. He then eased up and let me slow down. OK. Now, they got what they wanted: they stressed my heart out enough that I was a 10 on the RPE scale. My rate was 89!!

My left arm, shoulder blade, and collar bone were completely numb at this point – this is when he was really sold that I should slow down. And then, all of a sudden I started feeling very dizzy, very light headed and like I was going to faint. He took the blood pressure again, it was 142 over 50. Two minutes later, if that, he took it again: it was 106 over 34! So, in like 5 minutes, it went from 155 over 55 to 106 over 34. I thought I was going to faint!

They asked me if I should take a nitroglycerin, and I told them that usually I just wait it out and all this discomfort goes away on its own – so they left me be.

I didn't do the strengthening exercises that day. I just got some water and waited for my balance to come back so I can drive back home. I felt horrible the entire rest of the day.

The third day I went back, I took it very slow: my heart rate went up only to 79 (it is usually between 54-60 when I walk in), and I did reach an RPE of 7 on that third day, so I exercised within my own comfort level. I do not want to try to reach their weird goals because those are not MY goals! I know I am not a doctor, but I feel like my heart will never be an athlete's heart – this is not out of laziness and hypochondria, but it is out of being in touch with my body and knowing what it can and cannot do.

I still believe a stress test would have given them a more accurate picture of where I am today. Now. I flunk every test they give me – they all have a goal and I come way under that goal but my symptoms start way before I am anywhere near that goal. And when I do exercise, normally, on my own, I never push myself as hard as they always push me on the treadmill of a stress test (my goal for my age/ weight/ etc is 150 BPM – I have never been able to reach that). And even with me not pushing that hard, the symptoms are there.

It was the first time in rehab that they diagnosed my weird BP, and when they found that sometimes my MAP (Mean Arterial Pressure) is too low. And when this happened, I would start seeing symptoms, too – so the symptoms were heart related. This time, no one else in the room gets their blood pressure measured during their exercise till they actually can no longer do what they are doing. Definitely a different approach, maybe not as proactive as the first crew I was exposed to.

So, not so sure about this second go-around of rehab. I feel like they are not monitoring us closely enough to really understand each of our needs – had I not spoken to the second physiologist, they would have never found out about my jumping BP during exercise because the first person said “there is no concern that it would ever drop.” Now, he said (promised, even) that he would take it during my exercise every time I go in. He did tell me he would push me harder as time goes by but honestly, after the episode I had this week I wonder if that's even safe. Maybe the limitations in my heart will never get it working as hard as the doctors need it to work for the blood vessels to grow again, or the circulation to improve?!

I used to encourage people about going to rehab, because my first experience there was really good; it really gave me the confidence I needed after a very involved surgery that my heart CAN and will get stronger again. But now I understand why some people saw no value in theirs – because every place is different and some places are really not working with you to understand, explain, and monitor you so you know what is happening with your heart. Some places are really just a gym with heart monitors. But those who know me know that I'd rather know more than less.

All I can say is: always speak for yourself. If something does seem off, speak up, ask questions, be curious, know your numbers, challenge them – they owe you at least an explanation, even if they're not willing or able to make a change. At least that.

Much health to all!

Easiest Praluent Refill Approval in History

Caveat: this is not the norm! 

I have talked about this before (https://livingwithfh.blogspot.com/2017/04/a-helpful-health-insurance-alas-there.html), and as anyone taking Praluent or Repatha knows: ordering a new prescription or even a refill can be a pain in the rear-end. You always get denied by the insurance, always turned around, even when you know your reasoning for asking for these drugs is legitimate. They don't seem to care. It's not the first time when you realize that health insurance companies are not in the business of saving lives, but in that of making money. Pretty much a platitude, right?!

So, when it was time for me this month to renew my Praluent (this would have been my third renewal), I started an online journal. I wrote down the name of every person I talked to at the insurance company, at the specialty pharmacy, at my doctor's office and I was prepared to document every step of the way so I can show someone, anyone, where the process seemed to have been broken, should I be denied.

I took very detailed notes. I talked to the insurance first. They told me they cannot, by law, call my doctor; that they make up the list of questions / reasons for which I should be on Praluent, and that I have to call my doctor myself and have them write up this note with all the reasons that are approved by the insurance of why I should be on it. Then, the doctor has to fax this note to the insurance company. Then, the doctor has to also call the specialty pharmacy. When the pharmacy gets the call from the doctor that I was prescribed this drug, they cannot fill it unless the insurance approves that prescription. So, the pharmacy would call the insurance and the insurance would have had the questionnaire filled out from the doctor's office and would give them the OK (based on what the doctor answered) to fill the prescription. Or … they would deny refilling it.

If your head hurts, it should. The process is a spiderweb of convoluted bureaucratic loose ends and it is not designed for ease of access.

This year, I am also in a new state, and have a new cardiologist who is not the cardiologist that originally prescribed Praluent to me, two years ago. So, I was doubly nervous: I had to renew my prescription (so, renew, after originally having been approved) twice before and I was denied several times before during the renewal process. I knew this time would not be any different. Plus, not really being familiar with the staff of my new cardiologist, I was even more nervous that I would get denied so many times, an appeal would not even be possible anymore.

But sometimes, by the grace of Karma, or God, who knows?!, our worst fears are not met. I went to my new doctor and explained the maze to him. He smiled and told me to not worry about it, that the process has become easier over the years and a renewal is much easier than ordering a new prescription. I was dubious. I gave him the list of questions that the insurance needed answers for with the fax number for my insurance. Then, I gave him the phone number of the specialty pharmacy saying that I needed him to order the drug from the pharmacy. I was so scared he might get the two numbers confused: the insurance needed the questions answered and the pharmacy needed the prescription. And I talked to the doctor, but I know he is not the one actually doing all this, that his assistant or nurse might do this and between me telling him and him telling her what is needed something might get lost.

When it was time for my normal refill, I called the pharmacy to see if they had received a call from the doctor yet and could they please call the insurance for the OK. When I called them they said they did receive the call from the doctor and they also received the OK from the insurance and if I wanted, they could send me the new refill in a couple of days. I asked them, still dubious, how many refills I had with the newly renewed prescription, and they said 11 – that the new prescription is valid for the whole new year. I was in awe! This had to be the easiest Praluent refill in the history of Praluent.

To the time I write this, I am still not sure what changed: either my new doctor has had more experience with ordering these drugs than my previous one and he did tick all the possible boxes requested, or the health insurance/ specialty pharmacy business has gotten a little bit less rigid?

Not sure.

The couple of things I made sure that my new doctor had were:

• the results of my genetic test that shows I have HoFH
• the total Cholesterol and LDL Cholesterol numbers from before I started Praluent and the levels I am at now
• my history of CAD and heart surgery.

Hopefully that's all they needed and what he sent in.

Like I said: not sure what worked, but I am grateful it went well.

Now, the next big bump is renewing my benefits later this year: I am hoping that my employer will continue using the same insurance as we have now. Changing insurance companies at the end of this year would mean starting this process over with another company – and that, I know, and my doctor agreed, is much harder. Hoping for consistency going forward!

Good luck to all who are going through this process! These drugs have been a real live saver for me!

Then and Now. A Path to Recovery

I have been pondering upon life and the resilience we have, the stubbornness we have come equipped with from birth to embrace it. To fight on. To stay on the path of making ourselves stronger and to live.

This piece is for all the people out there who are just now finding out they have to have surgery, or for those who are about to walk into the pre-op appointment tomorrow and are wondering how will they ever come out of this daunting experience. And also for those who have put the surgery behind them but are now going through the hard first days and weeks of finding their new normal. This is also for everyone who, I know, can relate. This is for you all.

I was thinking recently about what all happened to me in early 2016 when I had my surgery. I walked into that hospital in Utah on my own two legs. I was rolled into the anesthesia room at around 7 AM with a smile on my face. They gave me that first (I think) IV shot of whatever … and I slipped away into the big slumber (I think, as I cannot remember anything till the next day around 2 AM when I woke up in the ICU with what seemed like a whole army of nurses around me).

I was thinking the other day about what all happened to my body in those hours, and it made me stop in my tracks. This really did happen. They took me to the OR and they stopped my heart. They cut my chest open, then my heart open (I think). They sunk me in buckets and buckets of ice to “freeze” me during the circulatory arrest procedure they had to do to trick my brain that it did not need that much oxygen to survive. They put me on this machine that pumped the blood and breathed for me for many hours. They removed my aortic valve, and then they removed my ascending aorta. At this point, there was no life-giving blood flowing through my arteries. Only some flowing through my veins, tricking my brain that it was OK not to breathe.

They replaced my aorta with a man-made graft and did the same for my aortic valve and root. They “cleaned out” (cut into the walls and removed the bad tissue of) my aortic arch which was thick with plaque. They “un-froze” me next and then started working on my by-passes. They removed veins from my left leg and sewed them onto my heart and did the same thing with the arteries from my chest – to bypass sick arteries. Somehow, through another big miracle, after they stopped the heart and lung machine my heart jolted itself on its own back into beating and making me a whole person again. Later on, my lungs started breathing on their own.

In essence, I was dead, really, if that machine would have stopped and the three surgeons, one anesthesiologist, one “heart-and-lung machine guy” (as my surgeon called him), three PAs and the army of nurses and other staff would have walked away on me. But they didn't. And I was breathing on my own by the time my husband saw me, in the middle of the night, and then when he left to go home.

When I got home from the hospital, after having a heart attack while in the hospital, 8 days after the surgery, I could not do a lot of things that are routine for any normally functioning human being. I could not open doors that were too heavy, including my refrigerator door. I could not tie my own shoes – I could not bend over at all. I could not clip my nails, because of the neuropathy in my fingers. I could not put on my socks. I took them off with my other foot. I could not wear a seat belt nor carry my purse on my shoulder or on my back. I could not wear a bra. I did not sleep on my back or my side; I slept sitting up for six months. After three months of medical leave, I went to work pulling a dolly that carried my purse and my lunch bag. I snuck into the building and in offices behind someone that would open the large, very heavy doors for me. I learned pretty fast where all the doors that let you push them open, rather than pull are, because I was afraid my chest would pop open if I struggled to open them myself.

Most of all. I was tired a lot. I was tired even after not doing anything at all. I was drained. I did not sleep well, but I watched a lot of TV, spent a lot of time on social media, and read a lot. I was not able to even cook, as a pot of water was too heavy to carry. I just rested. A lot. Well, I laid there – it did not feel very restful at all.

I remember writing during those early days of recovery that I cannot imagine being normal again, traveling, going about my normal life. And someone, a kind soul, told me that then I was praying for “good hours, but in time they will become good days and later good weeks.” I was dubious. But those words turned out to be true.

In the past two years and almost 5 months, I slowly grew stronger, just like that kind person predicted for me (or rather shared from experience). I started taking trips by plane again after a year. I visited three countries and went on a cruise a year after the surgery. This year, I flew across the ocean and visited my family in Europe. I carried my carry-on and my heavy backpack during security check-ins and switching gates in several airports. I drove across the USA last year, all by myself, when we moved from Utah to North Carolina. I did wear a seat belt with no trouble then.

I just took my first trip alone for a week, for work. I packed my carry-on with clothes, shoes, toiletries and gifts for a week, and had again a very heavy backpack with my purse and computer in it. Carried them all on my body, and lifted the heavy carry-on above my head and put it in the overhead bin with no problem, not even a sweat. My husband and I just finished staining our screened-in patio a couple of weeks ago on a Saturday. We worked together, side by side. We did about the same amount of work, although he did most of the “up high” areas, and I did the floor and the lower areas. It was 95F and about 70% humidity that day, and about half of that day we were in direct sunlight. I did fine.

I remember I did not plant a garden the year of my surgery because I could not sit or bent over in the sun at that time. Sunny days (even with no humidity) tired me so easily. While staining my deck I was in awe at how much different, stronger, my body has become in just two years and a little bit … I never hoped I could come this far. And I am not perfect, and there are still things I am working on two years and five months later, but I know one day I will reach most of them, just like I did all these other milestones …

If I have learned anything from this experience is that we are strong. We are built to fight, and we should give ourselves and our bodies more credit than we are inclined to to begin with. Thinking back at all these things that happened to my body and knowing how I feel today fills me with humility and gratitude!

I wish everyone happy summers, strong recoveries (they can be only as fast as you are comfortable with), and the faith that one day you will be whole again. I am just one living example that that is possible …

Much health and … keep on ticking!

CVD "Improvement" which Seems Truly Amazing

If you might remember and follow this saga, my new cardiologist suggested that I needed to have a heart catheterization, to find out more details about the status of the blood flow in my heart (http://livingwithfh.blogspot.com/2018/04/more-questions-than-answers.html) . This is because I complain of chest pressure, lack of air when I exercise, and general running-out-of-steam after so much as a few minutes of exercise. All these get worse and come on even sooner if I do this in the heat, or cold, or at altitude, or on an incline.

He did do a stress test first, and the test found that there is a portion of my heart (the bottom part) that does not get a proper blood supply when my pulse accelerates (so, during exercise).

So, this past week, he followed up with a cath. I was not crazy about the idea, especially when he said that they might insert a stent, in case they do find blockages in my arteries. They believe that these blockages might be giving me the symptoms. I was not thrilled about the idea of inserting yet another prosthetic in my heart (besides the grafted aorta and my mechanical aortic valve), and complicating not only my heart function, but also my treatment, especially my anti-coagulant treatment. If they had added a stent, I would have had to add Plavix on top of Warfarin, he said. Needless to say, I was not looking forward to this cath! But … I wanted it behind me!

This was my third cath in 13 years now. It was the first one when my regular cardiologist was not also the cath doctor. The way they do it at Duke, they have super specialized cardiologists, so the “office” cardiologist you see for your regular check-ups is not the “interventional cardiologist” that does caths and implants pace-makers, etc. This was new to me and I was very scared. The office cardiologist is the one I have worked with for the past months. I feel like he is the best one to know what is in my heart (literally and otherwise), and to really understand what he would be looking for in the cath test. But … I had no choice. I had to go to the hospital and meet with the cath cardiologist only a few minutes before the actual cath and then wheeled in on a stretcher to the cath lab.

I was, once again, like so many times in my life, very lucky. The cath doctor and I (and my husband) really clicked – he was a very compassionate and thorough man, and also a knowledgeable cardiologist, I thought. He had read up on my case and knew what to look for. My regular cardiologist was there, too, in and out of the hospital, and he did come into the cath lab and discussed the results as the cath was being performed. So, yes, I did feel like they were taking really good and thorough care of me, at least during the procedure.

The cath findings were somewhat of a shock to me, and I think to everyone else. Three main arteries in my heart had been by-passed two years ago when I had my open heart surgery (the LAD was one of them, and two others as well). An additional smaller branch of the LAD was also by-passed at the same time, giving me a quadruple by-pass on my heart resume. At that time, they saw 90-99% blockages in these arteries and hence the need for the by-passes.

Yesterday, during the cath, they found that all the by-passes have closed up and that my heart is still using my God-given blood vessels to function. Now, these arteries that once sported 90-99% blockages only show 30-50% blockages and are generally working fine on their own. At this time, the by-pass grafts have closed up and will never be used by the heart, while the original arteries seem clean enough to be functioning on their own.

The cath (and also the stress test) did show signs of mild ischemia (https://www.mayoclinic.org/diseases-conditions/myocardial-ischemia/symptoms-causes/syc-20375417) which could explain some of the symptoms.

They could see my main arteries and their immediate branches clearly during the cath procedure, but they could not see the very small arteries, the peripheral ones, the capillaries, because they could not find a catheter that was small enough to access the arteries at that level. They tried all the small catheters they had, but they could not find one small enough to access those smallest blood vessels nor to be mobile enough once they get to that level so they can really search for problems. However, they determined that I had good blood flow to the heart, overall, from the main arteries, and they should just leave it at that.

I, of course, have no explanation for why my ever worsening arteries (from many years of very elevated cholesterol running through them) have seen a turn for the better – and my only guess (and that is all that it is: a guess) is that Praluent might actually reverse atherosclerosis, like it has been speculated. I asked my cardiologist about this possibility and said at this point this would be pure speculation. He said whatever the reason is, he'll take it and he is happy with the blood flow he is seeing in my heart. I think this was the first time in my life when a cardiologist did not predict a gloomier, darker prognosis for my health going forward, and was actually pleased with the results.

This was by far the first time in my life when a result is actually better than everyone expected. I am thrilled because I think having my cholesterol as low as it's been on Praluent has everything to do with this. But I am so new to a good prognosis that I am not really sure what to do with myself. I must say, it's a good problem to have.

I still have tons of questions, like what is going on (still!) with my very weird blood pressure - they took my blood pressure five times at the cath lab yesterday before the machine could record a number (and then the number was 130 over 40), and is the Aortic Insufficiency (AI) caused by my leaking aortic valve the cause of these symptoms, perhaps?! But I will follow up with the office cardiologist in about a month on those. 

The next step is to basically continue to keep my cholesterol low (my most recent numbers are here: http://livingwithfh.blogspot.com/2016/07/my-current-cholesterol-numbers.html) and to go back to cardiac rehab. What he wants to get out of rehab is more understanding about my symptoms ( when they happen and what brings them on), and to give me more confidence to exercise in a controlled environment where I am monitored, and to learn how much I can actually “push myself.”

This “pushing myself” has been a matter of disagreement between a lot of my cardiologists: some of them say “do as much as you're able; don't do more than what you can, and when you reach that threshold, you just stop” and others say “push yourself beyond your limit, because I can guarantee that your heart is OK with it, based on what the tests show.” I want to believe the latter crowd, but I am human. So, typically, when I reach my limit, I stop.

And that's the verdict. At least for now. Now, I am determined to going back to my life and actually enjoy the rest of this year, of the summer, and beyond …

Happy Memorial Day Weekend, everyone! I hope you are all enjoying what's ahead. Much health and strength to everyone.

A Full Circle: Bringing FH Back to Romania

I was given a rare opportunity this spring, to speak to a class of Medical and Pharmacy students at the Medical School in my home town of Iasi, Romania. I did not even blink when my friend, who teaches English there, asked me to come and speak to her students (in English) about anything I wanted. She was thinking of this lecture more as their chance to enhance their English skills. I was thinking more as their chance to enhance their English skills while listening to something that might be interesting to them, like a medical system, or a rare disease. Or both.

My friend and I kept going back and forth about what the topic of my lecture would be, and we could not decide. So, I gave the power to the people: formulated three topics and let the students vote. They selected what I was guessing they might choose: “Managing a Genetic Disease in the USA. Navigating the American Medical System and Surviving to Tell the Story. A Patient's Perspective.”

So I proceeded to putting together a presentation about the medical system in the States, which is, as you might expect, quite different from that of a former Communist country like Romania. I presented our medical system through the lenses of FH, explaining the appointment and referral process, the tests that are necessary, sometimes mandatory, to perform every year, sometimes more often, the process of filling prescriptions, and a brief overview of what it all costs. I gave them an idea of what life with FH would cost in the USA if I had no insurance, or not as good as an insurance as my employer is providing for me. The whole concept of private insurance is completely foreign to Romania. The concept of a “pre-existing condition” is also.

I also talked to them a bit in depth about FH: what it is, why it is not widely diagnosed, but how it is believed to be widely common and widely devastating, as well. I talked to them about my own family. I also talked to them about the FH Foundation, and how I never knew anyone else outside of my family with FH until I met the folks at The Foundation. Kids nowadays are more familiar with the concept of a virtual family than I was when I was their age, but they were still impressed with this whole initiative and drive that The Foundation has to bring people together, to get us educated so we can get diagnosed and to help build bridges, and make resources available to us.

I was sure this will all be very interesting and captivating to them. What I did not foresee was that they would be more interested in the disease itself than the medical system that's helped me live with it. When I opened the floor for questions, they asked things like “So, how did you feel when you grew up almost all your life with being told you will die by the age of 25?” or “Do you think you would have made different choices in life had you not known about having FH at an early age?” or “So, do you feel less of a woman because you could not have children?” or “Was it hard for you to make the decision to not have any children?”

They also asked about the quality of my life, and the quality of my life after heart surgery. About the recovery time and “what hurt” and for how long after the surgery.

They also asked more “medical system”-related questions about how prescriptions are honored and the relationship a patient might have with a pharmacist in the US. But what shocked me the most was the “human” aspect of their questions, the interest in me, as a person, and as a patient.

At the end of it, I could not help but wonder: who beats the humanity out of the doctors by the time they get to actually practice? Because as students, at least if this one example showed me anything, they all come equipped with compassion, empathy, and the “human factor.” Or at least these students did.

It was an amazing opportunity and I would do it all over again. Speaking about my story, and the roads that got me here is always humbling and uplifting at the same time. I was honored to share myself and my experiences with these beautiful, eager minds and I look forward to more chances to do it again.

More Questions than Answers

Sometimes I wonder: “What is purpose of seeking to feel better? Have I not learned that I will never ever feel like I am running on 100% capacity, firing on all cylinders?! My HoFH will continue to do damage, my heart has had a major overhaul AND a heart attack. There is never hope for really feeling truly healthy and truly at full potential, as if I were a healthy person. So what is the point of all the tests and all the interventions that doctors still want to pursue?!”

And then there is this other little voice inside my head that says “Why not?! Medicine is so much more advanced than even 10 years ago. If there are ways, and procedures, and meds, why not seek the best life possible? Why not trust a doctor or two that do believe in the silver lining?!” The trouble with that is all the risks involved, and all the doubt, that despite all the advancement, I still have an unbelievably broken body, which is irremediably damaged for good, and forever, which will never ever run at 100% capacity, even with the best care.

Let me bring this in focus: I met with the second cardiologist since I moved to NC. He struck me as somewhat of a bully, a rushed maniac with a weird passion for treating hearts and somewhat of a bull in the China shop. Definitely not a boring guy! He initially was “guessing” based on what he has heard about my history (mostly from my previous cardiologist's notes) that the biggest problem with my heart is my AI (aortic insufficiency), caused by my (still) leaking aortic valve. Although, just like my previous cardiologist, he could not hear my AI (the leaking), he said the reason my blood pressure sometimes has “no bottom” (or a very low one) is because of the AI. He also guessed that my heart probably has fluid in it because of this and this is why my legs swell up sometimes. He also chalked the shortness of breath, the dizziness and other symptoms of fatigue and slowness to the AI as well.

He recommended a heart echo and then a nuclear stress test. After both were done, he called me and the conversation went into a completely different direction that before. He said “according to your echo, your heart is good.” That's it! He didn't say why, and he did not mention one word about my AI (which he believed to be severe before the echo, just based on the notes he read and on my symptoms). He said nothing about how badly my valve might be leaking, if at all, and whether there is a concern even about the leakage at all.

He, however, spoke a lot about the results of my nuclear stress test: he said when at rest, my heart gets an adequate amount of blood (and oxygen), and the vascularization is appropriate in my entire heart muscle. However, when my pulse raises to even as little as 120 (my goal was to hit 150 beats per minute, but I started getting short of breath and dizzy around 120, so they stopped the test), there is a corner in my heart (lower left ventricle to be exact) that does not receive blood at all. He said it's either a scar from an old heart attack (my MI was localized in the same area he saw on the test), or there is a blockage there that needs to be opened up.

So, his number one concern now is to get to the bottom of this mystery: he now believes that my shortness of breath, my dizziness, and all my other symptoms of fatigue come because of this area of my heart is struggling to work without proper oxygen.

He wants to do a cath next to understand which is the cause: if the cause of the lack of blood in that area of my heart is an old scar from the MI, I am not sure what he can do for that, really. But if the cause if a blockage, he wants to put a stent in to open it up. After which, he wants me (like he said the first time he saw me) “back in rehab”, because the heart has to start working better after the blood flow is opened up. The first time he mentioned rehab to me it sounded bogus, but this makes sense. Not sure what the course of action will be if we're not talking about stenosis but rather some other traumatic damage from my heart attack. He seems to be pretty certain that it's a stenotic spot where maybe a bypass was not done before, or where it was done and it didn't take. (have I mentioned that this doctor guesses a lot?! He does.)

I am, of course, not crazy about a stent, because that can also be a liability (stents can clog, too, with scar tissue). A little voice inside tells me also: “what is the point of all this? Can a stent really make you feel like a normal person? Can any procedure ever make you feel like a normal person? Of course not! So why add more side effects and worries, when you can just cope, like you have done for two years now, since surgery, and for 20+ years now since the first sign of cardiac problems?!” But not doing anything to help beaten heart (no pun intended) sounds just as crazy.

The doctor wants me to come up to the 150 bpm pulse and keep going with no symptoms for at least 15 minutes. But I have had angina before my surgery for years. Now, I have had early fatigue and shortness of breath after just a little bit of walking. Is the goal (for my age and weight, height, etc) of 150 bmp really realistic?! With everything that went on in my heart and with the constant abuse of high cholesterol (which will continue), is it really possible to hit the targets of a normal person?!

The one thing that motivates me to really look for answers and for a possible solution for getting my whole heart irrigated properly is that my symptoms kick in even without extensive exercise. They kick in after speaking for a longer period of time, or when I am nervous about a seminar I am presenting, or speaking in front of a crowd. So, simple things like these make me symptomatic. And to me symptoms means more damage to the heart, inherently. The heart cannot be healthy when it's struggling for air like this, when I do the simplest things. And I don't want it to quit on me when, say, I need to run across an airport dragging luggage during a short layover.

He wanted to do the cath within a week from my test (it's passed already), but personal conflicts for both me and the doctor have postponed this till a month from now. He wants me to increase my Atenolol to ensure my pulse rate stays low. In the meantime, he told me “not to go crazy: no jogging, no hiking steep hills, no pushing heavy carts. Just keep the pulse under 100, he said, or lower, as low as I can.

I am planning to have at least one more office visit with the doc before I go in for the cath, just to ask him all the questions I still have about all the tests he has done and about his overall opinion about my heart: some things that I knew I had from before (like the AI) – how severe are they? Should we worry about them? And also: is my heart ready for a stent?! What is the maintenance we need to consider when that will be implanted? What are the steps to take if I don't need a stent? If my heart is damaged otherwise from the MI, is there any remedy for that? If yes, what is it?! Lots of questions, still, as you can see …

I feel sometimes like trying to see this doctor is worse than trying to get approval for Praluent. You cannot get an appointment sooner than 6 months ahead. Now, because I am somewhat of an acute patient, I had to beg his office assistant (his nurse told me she cannot schedule anyone sooner than what the public calendar shows, and I have to work with his personal assistant) to schedule the appointment at least 2 months after my previous one. When we do meet, he is incredibly rushed and off to the next patient, so I have to really come prepared every time, with written questions to ensure I don't miss anything from what I want to know.

But we do what we have to do to get our answers, don't we?! A busy schedule, a rushed physician, or an inflexible insurance company should not stop us. Here's to resilience! 

I am very worried about what's in my heart, and I am doubly worried that he has little time to really know what's going on. Maybe he's used to this rushed schedule and to him is second nature to run off from one acute case to another … We can only hope …

I wish everyone smooth rides and easy questions. Much health and a beautiful spring, everyone!

New Numbers and a Slight Change in Drugs

My then new cardiologist recommended I'd see an endocrinologist for treating my cholesterol back in December (http://livingwithfh.blogspot.com/2017/12/the-tricky-balance-between-trusting.html). He tweaked a couple of my drugs then, in the hope of getting my LDL cholesterol down some more, and my HDL a tad up. 

At the time I was taking 5 mg/ day of Zetia and he doubled that to 10mg/ day. My previous cardiologist said there is little to no difference in the amount of Zetia you take for how much percentage of cholesterol that is lowered. He said taking 5 or even 2.5 mg is just as good as 10 mg. Since even the generic Zetia is $80 a month, I used to cut it in half to save on the money, since the benefit was not said to be great. But I gave it a shot and increased it at the advice of the endocrinologist. 

The endocrinologist also recommended I would add Metamucil to my diet which is also proven to have some benefit (being a fiber) in getting rid of some of the cholesterol. So, I also added that in December, as well. 

Last week I got new numbers and as you can see, they are not very much different that the ones in December. In fact, the total cholesterol, the LDL and the triglycerides are slightly worse. This time, these tests were not taking while fasting, so I think that matters, especially for the triglycerides. 

The endocrinologist also suggested that I would try Juxtapid, but after thorough research and hearing from folks who have taken it, and my liver enzymes going back up on their own, I decided not to go that route. I am way too nervous about the drug being too strong and damaging my liver to try it out. 

Since December, I switched my cardiologist and the new one might be able to manage my lipids himself, he said. We have not gotten to the part where we talk about my lipids, as he is more interested in figuring out my heart first. So, till we get to that point, I am deciding a few things on my own. Given the lack of improvement on the numbers in the past three months and the fact that my stomach is further upset by the Metamucil, I am deciding to: 

- stop the Metamucil
- go back to 5 mg/ day of Zetia

In the long run, I am also: 

- not signing up for Juxtapid (my new cardiologist agrees with this) 
- maybe canceling the appointment with the endocrinologist.

I have had some experience with most of these drugs and I just feel like the regimen and the diet (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html) I am on now has given me the lowest numbers that I could hope for, at this time. 

As always, this is a personal post and a personal decision. I always try to stay informed and weigh in what is best for my cholesterol as well as for my quality of life.