The Struggles to Stay on Praluent. And They Are Not What You Might Think.

Well, it was OK while it lasted. My Praluent prescription from my insurance's 'specialty pharmacy' that is.

So, it went like this: back in May, I was approved by my insurance to be on Praluent (which is a PCSK9 inhibitor drug, one of the latest drugs approved for the treatment of FH when all drugs fail.) They required, at that time, proof that my cholesterol levels are not normal (they were in the mid-300 range), and that my doctor tried everything else on the market and that I am currently on the highest dose approved of all medications there are on the market, and my levels are still high. All that was documented and sent to the insurance company, and they approved me to be eligible for Praluent, based on my life-long history of FH and current (at the time) elevated levels of cholesterol.

I have received the injections monthly from them since May. For some reason, the “authorization” that clears that I should be on these drugs expired in November (so, only 6 months from the initial approval). So, when I ordered my refill for December, they told me they can't ship it, because it needs to be approved, yet again, by the insurance company.

I asked them what that entails. They gave me a number to have my doctor call them and give his blessing that I still have to be on Praluent. My nurse called the number and she was given a fax number where she should fax the prescription written by my cardiologist, in essence, their approval that yes, I still need to be on it. This is important, because I was later told that the 'deliberation process' for the insurance company on this authorization is slower when the request is faxed in, rather than when it is phoned in. She did phone it in, but she was advised to fax it. This was only one kink in the many that followed during this process.

So, the nurse faxed the doctor's approval that I need to be on this drug. The insurance company denied it. I didn't know this and she didn't get a confirmation or denial over the phone or fax. I found out when I called again, after a couple of days, to see if I can refill the prescription.

At that time, I asked why was the request from the doctor denied. They said because the doctor failed to file with the request my medical history and the proof that my levels are high and the proof that I have taken everything else on the market and nothing seems to work. I was puzzled. This is a refill. All this information has been submitted and approved before, in May, when they first screened me and approved me for it. If for my entire life nothing worked, and Praluent was the only thing to try … what could have changed in the meantime?! In short: once you are born with FH which is not curable by the meds on the market, as we know them, outside of Praluent … what could possibly change in 6 months?! I was puzzled. And lost. And frustrated. But insurance companies are not here to be logical. We all know that!

So, I phoned my nurse again, to ask her to please resend the papers with the proof that I am truly in need of these drugs as nothing else works, and with my numbers to see if we can get it extended for 6 more months. She was shocked, of course. She did not understand why she would have to do that again, when it was clear that with my diagnosis (FH), I qualify for this drug “for life” as she put it, just as I did only 6 months ago. But, because she wants to help, she called again. Faxed the information again.

I will save you the boredom of reading through the story of my 2 additional phone calls to the insurance company when I argued why they are not reading my nurse's fax and why they are not giving me my December refill already. It took a week of calling back, being online for an hour and having no resolution at the end. This is for a refill, you guys, not an original approval.

Anywhoo … On December 27, I called again and they told me they denied the distribution of Praluent to me, because after checking the papers from the doctor, they decided that I do not qualify for Praluent. I asked what is the reason, they just said: 'no reason is specified on the denial, other than you do not qualify'. I asked how did I qualify for 6 months and to tell me what changed. They denied comment. I asked what is my recourse here and they said the doctor has to file an appeal and he would be the one to fight with the insurance for this approval.

I am not about to have the doctor go through all this. I think my next step is to wait for the actual 'denial letter' (they normally send one) and then contact our plan administrator and see if she can fight for this.

My only guess is that my nurse sent them my latest blood tests, where my total cholesterol was 200 (much improved from mid 300's, and almost normal, right?!), so they probably decided it's not bad enough for Praluent. But, again, insurance companies are not here to be logical: they do not see that the only reason my cholesterol is back hovering around normal ranges is because I have taken Praluent every 2 weeks for the past 6 months! This little, tiny, insignificant detail does not cross their dirty little minds. Sigh.

So, beware that even when you are approved for this drug (and at this point, I think all of us need to go through the approval period), there is no guarantee that you will be approved forever.

Right now, my Plan B is to continue Praluent by getting it in free samples from my cardiologist's office. I cannot help but feel like I am doing something illegal, which is totally the wrong way to feel.

I hope this is just a temporary kink in the system till the insurance companies learn how to deal with this very new, very powerful and very expensive drug. I hope they learn to see the cause-and-effect of using this drug and the need for it continuously to stay on top of our numbers, and that they revise their processes so more lives and hearts can be saved.

A Challenging and Blessed Year

Two years ago, right before Christmas, my cardiologist at the time called me to share with me the results of my latest yearly echo. Things were starting to get worse. My aortic valve was still shrinking, and it was 1.1 cm2 in area now. I remember his words: “I am not sure when you're going to have surgery, but if it dips below 0.9 cm2, it will be time. You're very close to that.”

My aortic valve was born healthy, but years of calcification from FH caused it to collapse and shrink and become dysfunctional.

A year went by, my cardiologist then retired and referred me to a new one. So, last Christmas, the new cardiologist calls me with the yearly echo results, around December 20^th and says: ”It's time. Your aortic valve is now 0.4cm2, and we must operate to replace it as soon as you can. Don't wait for longer than 3 months. Tops.” I requested a repeat of the echo just to make sure they measured right. The repeat echo was done on Christmas Eve of last year. The second echo confirmed the findings: the area of the aortic valve was too small to be deemed safe, so surgery was imminent.

I am sure a lot of you who have been faced with heart surgery can relate to what was going on in my heart (literally and figuratively) around Christmas last year. I was scared. I was in a lot of ways hopeless and very, very afraid. I live in a small state (barely 2 million people), with very rare good medical care. I didn't know where to go and how to ensure I'd have the best surgeon, the best nurses, the best care possible. I didn't want to travel for this surgery, because I wanted the people who operated on me to be close by, during my recovery. I was terrified. How I didn't go into the abyss of some sort of depression is beyond me. Christmas and New Year were bleak last year. I know I was supposed to be hopeful, and part of me knew this was coming for most of my life, but this was right in front of me, staring and in the way – I could not divert it or go around it any longer. This was it.

When it was finally time for surgery, they found out from an angiogram that my heart was even in rougher shape than they initially thought: on top of replacing my aortic valve, they have repaired my aortic arch as well as replaced my ascending aorta. And if that was not enough, they also did a quadruple bypass on the left side of my heart. It felt, for a while, like my heart was hit by a Mack truck. In essence, it was! But the name of the truck was 'cholesterol' instead. 

This is an X-ray of my heart, in a lateral pose. You can see the artificial parts inside the heart, as well as the steel wires that helped my sternum heal. 

I won't go into the details of my past year – you can always read my past blog posts to find out how it went down. But I am sitting here today, typing this and, for the first time in a long time (2 years) I feel like the monkey on my back has taken a hike.

I can now make plans, and actually enjoy Christmas and New Year's without wondering what the monkey will do to me if I don't 'feed' it. It's an incredible feeling of peace and gratitude.

The tech from my last year's echo said: “It's pretty bad, but you have to wrap your head around this huge surgery a little bit, and then, you'll bounce back after a short time. I see people do it all the time.” I honestly thought she was on crack: how the heck do you 'wrap your head' around your heart being stopped and being cut in two, and parts of it being removed and replaced with God knows what? And how the double heck do you 'bounce back' from that?! Looking back at this year shows me that she was mostly right.

What cholesterol does to us in unequivocally bad. When I hear people say that watching your cholesterol and how it can affect your heart and brain is a myth, I just want to jump right out of my skin and give them a good shake. Cholesterol damage is real and I have this past year to prove it. Send me a note and I'll ask my surgeon to call you and tell you what state my heart and aorta were in at only 41 years of age. And this is also after 20 years of medication for lowering cholesterol. I am not sure what state they would have been in without medication.

I visited with my surgeon this past week. He got together with my cardiologist and they both read my latest echo together. The surgeon called me this week with his interpretation of my results, which, he said, agree with the cardiologist's opinion, too, for the most part: my replaced On-x aortic valve is doing great and he said “it will last you for the rest of your life, as long as you don't clog it.” It does still have a leak, more than what he normally sees on these types of valves, but the leak is trivial. He is not concerned about it. He said it all comes down to how I feel, and I feel great. My left ventricle (on which he placed 4 bypasses and which had an MI while I was recovering in the hospital right after surgery) is “incredibly strong” and my ejection fraction is back to around 70% which he says it's almost the number for a “healthy heart”.

I still have a very low systolic BP, which he cannot positively diagnose. He says as long as I am not dizzy and otherwise feel OK, the number won't matter much. I know the cardiologist is concerned about the number, but I have felt great, too. I do have occasional spikes in BP which I also had before the surgery, but they are short lasting. Alcohol seems to cause this sometimes, too. But that is something easy to live without, or with very little of. It is a miracle to me to not have angina anymore. I believe my first bout of angina was when I was in college at 21 and it was happening weekly, sometimes daily (depending on what I did) for 20 years now, before my surgery. I have not had that since surgery. This tells me my heart is now on the mend and has been freed to work as it should.

I have been lucky beyond belief that I have managed to go back to work after 3 months of medical absence and I have not taken many sick days since (7 months ago). Ironically, the only sick leave I took after the surgery was for stomach flu. I have had only one ER visit (for low BP and dizziness), and only one Urgent Care visit to stop a bleeding finger which I chopped while cutting up onions. I have been incredibly blessed with not only good doctors, but a great family and husband who has done pretty much everything around the house so I can rest and take it easy.

I have taken this past year “off” from traveling which I love, just so I will be gentle to my heart. We did take road trips but I have now flown. Being 'grounded' was not all bad. I have gotten more in touch with my crafts and they have helped me lift my spirit. I have also spent some time learning and finding new beautiful places around our home, which I never would have made time for otherwise.

My surgeon was shocked that I have not flown yet. He said: “Go out there, and live your life! This is why we did this, so you can have a good, happy, meaningful life. Go and enjoy!”.

It felt so freeing to hear him say that. And as a good patient that I am, I am about to follow orders.

As you all know, I have a new life and new routine after this surgery. I watch what I eat constantly, and I am hooked up with apps that tell me how much Vitamin K is in anything. Both my doctors always tell me that the only ONE thing that I must ensure I do to protect the valve is to 'not clog it.' I have other food allergies, too, and watching what I eat and where I eat it is almost second nature now. I don't think about having to think about it. It just happens as routine. I have 10 medications I have to take every day, for the rest of my life. This includes prescription and non-prescription drugs and supplements. I have one shot I take for cholesterol, every two weeks. I go to the clinic for my INR check every month now, and I check it at home about every week. I have a cardiologist appointment every 3 months (for now), and I meet with the surgeon after every echo (about 6 months now). It's all in my planner and every “thing” I have to do is just part of my daily routine, like brushing your teeth or doing your laundry.

My regimen of drugs and doctor's appointments include a double focus nowadays: the cholesterol numbers as well as how the 'repaired' heart is doing. Cholesterol has always been in the front and center focus, but now, the heart is very much there, too, as you can imagine. We're no longer trying to prevent affecting the heart with what the cholesterol is doing. That's already happened and has had some mending. Now, we're watching how the mending is doing as well as whether there is further injury to it from the still slightly elevated cholesterol. My new numbers (http://livingwithfh.blogspot.com/2016/09/numbers-come-back-to-their-normal.html) look pretty close to normal, however, and I am hoping that I can keep taking the new PCSK9 drug I am on. Its affordability, though, is a huge challenge at this time. As long as my cardiologist can supply me with samples, I pray that this will help the numbers stay low.

One thing that still worries me about caring for my heart, are infections, because we have so little control over those. I have had an infected tooth for a while (doctor doesn't know how long, because it has not really hurt, so I didn't complain about it). They have treated it for now, to let me go through the holidays, but I must have the tooth pulled to prevent the infection for really drilling into my sinus and spreading in my body. I am more vigilant now, when it comes to my teeth – I go to the dentist for every new throb, or pain, or sore gum. I just don't want to risk some pesky bug going into my blood stream.

But outside of all these, which are truthfully my new normal, I am happy and I feel complete, and so lucky. I thank God every day for giving me such a challenge. I never knew I could do these things before I had to do them, and coming out on the other side of it is like being born again, truly. You know how a puppy scared of water feels like when you throw him in the lake and he realizes he can swim and he'll be fine, after all?! That's exactly how I feel.

For Christmas this year, I feel peaceful and healthy, despite all the limitations that I was left with after surgery (consult previous posts here, too). I feel ready to enjoy my family, my memories and my foods and to plan for what it is ahead. After all, planning the rest of my life is quite a chore.

Merry Christmas to all and much health and strength in the new year!

And lastly, I am sharing with you my “Open Heart Surgery Year” album – there are pictures in here when I am at my worst (not awake yet from surgery) all the way to the newest picture which I took about 2 weeks ago, with our Christmas tree. If you hate bruises and cuts, maybe it's not for you: https://wanderworldpics.shutterfly.com/22602

Most Days, I Am Fine.

After a lifetime of being on a low fat/ no fat diet, and now, after two full years of being a pretty religious vegan pescetarian (not by choice, but by intolerance necessity), I must say that I am mostly used to it. I don't crave meat anymore, even bacon, and I always tell myself that there is no need to eat “other things” since I am always full and fulfilled when I do eat what I can.

I refer to it as “not being able to color with all the crayons in the box, but still able to color, and thus still having the fun”. And I pretty much believe that. I should have probably been a vegan long before I turned 39, but I didn't have the need to until meat and animal products made me literally sick.

I am very used to my diet now, to the point that when a restaurant sneaks something in my food, I can tell immediately and I do feel sick, not happy about the inadvertent breaking of the rules.

Like I said, I don't feel restricted in any way and I don't wish “what if.” To me, those would be a waste of time and energy. I am pretty much settled in my new reality – which has been always changing over the years. Until today. And you knew this was coming, right?!

I have loved sushi, all my life. I didn't grow up eating it (we didn't have such diversity in communist, and post-communist Romania), but once I had it in The States (around 2002 or so), it was love at first bite. I have eaten it till last year in December, when my aortic valve was getting so bad that it needed surgery. I was afraid to eat any raw fish, because of all the bacteria that could be introduced into the body and damage the valve with it.

After the valve was repaired, I cannot eat it now for more than one reason: raw meat contamination is just one of them, but also, I have not found conclusive information on what the content of Vitamin K is in the seaweed wrapper, and that can throw off my INR, since I would be eating it just occasionally. Another reason for not eating it is because  I cannot eat soy (intolerant, as well), and there is a lot of soy contamination with any sushi, for sure.

So, today, our office ordered sushi for one of our office work lunches, and not only that but we had our own sushi chef on site, too. Every roll I saw leaving our kitchen was amazing – huge portions, too, and all you can eat. I thought I was going to faint. Today, I can say that it was the first time, in … ever since I can remember, that I wish my diet would be different and that I could eat sushi once again.

I ate my home-made and home brought whole grain pasta with chickpea “meat balls” and home-made tomato sauce. Yummy, too, but … not sushi. Some sacrifices we have to make, for our health.

I wish your journeys through this are easy and successful.
Much health to all!