Could Praluent Stop Working?!

Happy New Year, everyone! I hope you are all healthy and ready to take another one on with all that it might bring us.

I was happening to read this article the other day and it made me chuckle that they actually look at cholesterol numbers this way: https://www.wsj.com/articles/high-cholesterol-it-must-be-january-11547814600. The article is worth a read, but to summarize: apparently, our cholesterol goes up over the holidays, and if we were to measure it in January we would see that, because we fall off the wagon and we eat all those rich, unhealthy foods over the holidays, and too much of them.

I chuckled to myself because diet is not really a factor for my cholesterol numbers, this time of the year or ever, because:

1. I have HoFH which is really not influenced much by diet, if at all
   and
2. I don't change what I eat when we're around holidays. I can't because I am allergic to all those rich foods that could raise my cholesterol (like meats, dairy, eggs and the likes).

But what do you know, I went in for a cholesterol check and these were my numbers:

<insert funny, deflating sound of disappointment here>

So, diet is a moot point for me, so what could be the culprit for these crazy numbers?! As you can tell, the jump is huge.

Truth be told, the numbers I got last time surprised me as being way too low. Since I started Praluent, my Total Cholesterol hangs around 190's and my LDL around 140-150s. So, I saw the results in September as a fluke, too. But these new numbers are scary, considering the full cocktail I am on. For a list of all my medications, see http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html.

My doctor and his nurse have been trying to figure out what happened. The following has been their inquiry process to try to get to the bottom of this change:

1. Did I stop the Praluent or any of my other medications? No, I did not change even one thing about my medication. No change in the time, dose, pattern of injecting the Praluent.
2. Did I not use my thigh to inject the Praluent? Did I use my stomach? No, I did not. I have always used my thigh, alternating between the thighs from one injection the to next.
3. Did I inject every 1^st and 15^th of the month “to be exact in the break between the shots”? I corrected them: I always inject the Praluent on a Wednesday every two weeks. This (and not the 1^st and the 15^th of every month) will always ensure an equal amount of days between the shots. The nurse agreed with me. She, apparently “never thought about it, but I was right.”
4. Did I not refrigerate my Praluent? Nope. The first thing I do when it comes off the UPS/ FedEx truck is to put it in the fridge. ( you will find literature out there that says that even if you do not refrigerate it, it should be OK to use for up to 30 days, but I don't believe that, really).

Besides the detective work that the doctor and the nurse did with me, I called a friend who is a medical researcher and has been involved in researching Praluent particularly since it was in clinical trials. I asked them if there is any evidence of developing resistance to the medication after a while. This happened with the statins for me, and I had to add additional drugs to support the statin. The answer was: nothing like it has ever been found and they have had people that have been on Praluent now for 6 years (double of the amount of time I have been on it) and they never noticed resistance.

Well, all this work was interesting, but I still had no answers. I did notice one difference in the shot, though: the last two shots that I took right before the cholesterol test were excruciatingly painful. I always whine that they hurt, but those two times I literally screamed and started crying instantly. They burned and hurt like crazy. So, I told the nurse that. She said, “well, that must be it. You got a bad batch of Praluent.”

Now, how would I know for sure? She asked if I had the LOT number from those injections that hurt (the last ones) and of course I don't anymore, but I am keeping closer records now. She gave me a number for the My Praluent line to speak with the manufacturer about what happened: the fact that the injection hurt more than usual (they consider this to be a “reaction” that needs to be reported to Regeneron/ Sanofi), and the fact that my numbers went up with no other explanation.

I called the number (which actually redirected me to Sanofi who is marketing the drug, after selecting the extension for “reporting an adverse reaction”) and they, too, were not able to help very much.

They asked me the same questions that my doctor has already asked. In addition, they also suggested that maybe “I need to increase the dose, as the current dose stopped working”. Umm... that is not possible because I am already on the 150mg pen which is the highest dose approved for Praluent. So, not a go there.

They only are guessing that somehow, one of the entities handling the drug (could be the warehouse, or anyone in the transportation of the drug from any leg of the trip until the drug got to me) mis-stored the box with the pens in it. The drug has to be refrigerated but not frozen. With the low temperatures we have been seeing for the past several months, who knows? Maybe it froze on the truck to one place or to my house?! Without a LOT number, they cannot do much and right now they do not have any recalls recorded. So, they are not aware of any certain batches that were bad, so the mishandling must be the problem here.

They said I will get a lot of paperwork in the mail to fill out and I should send it back to them and this is what they will use if they need to build a case for a recall, in case they get similar reports from other patients.

They also said if they do have a recall they notify all the doctors that are ordering the drugs (as you know, doctors work very closely with the distributors of Praluent and Repatha, so they have close records for those doctors, I would imagine) and the doctor should notify me immediately. I already know my doctor is extremely efficient in letting me know about recalls, as another medical device I order (and of which my doctor is aware) had a recall a few months back and the doctor emailed me on a Sunday, the day the FDA announced the recall.

So, unfortunately, we don't know what happened, but we have some guesses. Now, the plan is to continue with the Praluent, as it arrives from the specialty pharmacy for the next month (so, two or three more shots) and then repeat the test in the beginning of March. Hopefully, the numbers will go back down to “my” normal.

Another interesting thing did happen, however. After talking with the My Praluent folks (who are not the same as my specialty pharmacy), I had to order a new refill for the Praluent. I called the pharmacy and ordered it, and nothing was changed on that end – the refill went smoothly and routinely. I ordered it to be delivered with no signature required, which is what I always select. The day when the order shipped I got a call for the very first time in three years (since I have been filling this drug) from a bot. They wanted to remind me that I had placed an order for Praluent and that I did not require a signature. And they wanted to ensure that I open the package and I store it properly just the minute it arrives at my door. Now, this was an interesting coincidence that I received this very specific indication after doubting the storage and packaging of this drug like never before, wasn't it?! I am trying to figure out what prompted the call this time: is it more than one person complaining that all of a sudden the drugs stopped working? Or was it the brutal temperatures we're dealing with nowadays, the polar vortex and the below human temps we have seen everywhere in the country?! Like I said: I called My Praluent to tell them about the mishap, and not my specialty pharmacy – so how could the two actually be concerned about the handling of the drug right at this very moment when I was doubting exactly that.

I guess I will never know. As you can tell, I have tons of questions and only a guess, at best, for an answer. I will continue with what drugs I have, and we'll see what numbers I'll get in March. Hoping for some good news …

Much health and a warm rest of the winter to all!

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!