Refill for Praluent APPROVED

This is a continuing story that I am following up from this previous blog post: http://livingwithfh.blogspot.com/2017/01/between-rock-and-hard-place-with.html. Please read that story first, to understand the full context, if you have not followed this from the beginning.

Briefly, I was approved for Praluent last May, in addition to the high dose Lipitor and Zetia I have been taking. In November (6 months since the original approval), my specialty pharmacy required that a pre-authoriaztion was needed in order for them to continue giving me the drug. My doctor's office filed the necessary papers twice with the pharmacy/ insurance company and they were denied the refill both times. Convinced that there was something missing on their paperwork, I decided to appeal a third time myself. I am giving all the reasoning behind why I thought this in the post I linked above. 

And now the update: after my third appeal, the pharmacy approved my refill of Praluent for another 6 months. 

And now the whole story. 

On January 27th, I filed my appeal which was the last one "internal appeal" remaining. I found out that they allow you to appeal three times, "internally", meaning you appeal to the same company that denies you. After that, you can file an "external appeal" which is sent to a third party that evaluates it. So you are no longer dealing with the insurance at that point, but with this other entity. 

When I filed my appeal, I included the following papers:

- the most recent denial letter from them;
- the most recent form faxed by my cardiologist's office to them, and I included the corrections on the form (if you read my previous post you'll see the list of the things they missed when they filed); this included the family history of FH which was not evident in my doctor's papers;
- a letter explaining my case: my long lasting diagnoses with FH, ASCVD, heart problems, atherosclerosis, etc, as well as the fact that I have been approved for this drug before; 
- my heart surgeon's and my cardiologist's phone number and address, in case they needed to confirm anything I was sending in;
- a list of all my diagnoses, which I have downloaded from my cardiologist's web portal;
- a list of all my medications, which I have downloaded from the same place;
- the  discharge papers from last year after my open heart surgery: in there, they list everything they found wrong with my heart (blocked arteries, defective aortic valve and aorta, atherosclerosis), as well as what they did to fix it (quadruple by-pass, replacement of the aorta and aortic valve, aortic arch repair);
- the cholesterol values before I was on Praluent (high 200's for the total cholesterol and mid-200's for the LDL);

Two days ago, I have found a letter from my insurance in the mail with my approval for another 6 months. They mention that they will require a pre-authorization for it again at that time. This time, I think I will cut to the chase and either send the information myself, or go personally to the doctor's office and make my nurse fax exactly what I sent this time. 

I am happy about this outcome, of course, because my numbers are unbelievable on this drug, so I would love to be able to continue the therapy. I am really excited that the drug is still on the market, after the lawsuit that has been developing over the patent (also linked to the previous post). 

Right now, I am just a phone call away from scheduling my next shipment. First, I want to explore some payment options as the $1400 / month bill is a bit high for me. If there are other options for payment or discounts to help with the payment, I would love to know about, and I am exploring those before I call in, but I should be ready to call in a week or less. 

I will update this site if there is any pertinent information about payment options. 

I want to thank the FH Foundation for reaching out to me personally and coaching me through this process. Their wisdom and care for us is amazing and I am forever grateful! 

Stay in touch and stay healthy, everyone!

Random Thoughts on My First Year OHS Anniversary

I have had my OHS a year ago today. I have written this piece for my Heart Valve Surgery site today. This site has been a great resource during my journey with OHS: http://www.heart-valve-surgery.com/journals/user/alinafh

I am really almost 42. But I like to think that today, my heart is one year old.

I am lucky – I get two birthdays, like most of you here. And my heart will always be almost 41 years younger than my whole body.

A year ago today I was not looking beyond that day. A year ago today, I could see nothing but darkness beyond February 11, 2016. Just uncertainty and darkness. It was a cold, foggy, quiet day and we started the day early. The day before, I had gone to the hospital for pre-op stuff and it scared me a little (yes, I am joking – it scared me to my wits!) All the warnings about how I would feel “after”, all the possibilities about infection, how I could not sit up on my own, how I had to breathe even with a cracked chest, how I won't be allowed to open my own doors for months; all the education done by the surgeon about how difficult my circulatory arrest would be. How it could render me stupid if my brain craved the oxygen. They were unbelievable pieces of information that made me feel like an experiment. I mean, how do you not fall apart when they tell you they'll stop your heart, cut into it and freeze it?! Then, replace a couple of its parts and rewire the rest?!

But there was something in my heart (no pun intended) that was bigger than all the warnings, larger than life itself. There was a reassurance and a peace that I could not even believe myself. I was serene, composed and calm (for those who know me, these are all things I am not, on a regular day). I was confident that this had to be done and I had the best team on my side to do it. Somehow, from somewhere, I got this reassurance. I cannot teach it. I cannot tell you that it comes in 2 lb packs at my grocery store, but I know that somehow, through fear, and prayer, and family holding hands together, and an incredible amount of hope, I found this peace.

So, on that February 11^th day, I walked into the hospital on my own two feet, with husband by my side, to have my chest cracked, my heart opened and frozen, and eventually returned back to life.

I sometimes sit and ponder upon all the bends in the road, all the bumps, all the highs and the lows I have been through in this past year. Even now, every day feels a little bit different than all of the ones since February 11^th at 2 or 3 AM when I woke up from surgery, nurses hovering over me telling me “Alina, you had a big operation!”. I remember those few minutes right after I came to: I felt like a little kid, peering from a small house out through a smokey window, with people looking in. Very eerie.

Funny how I got a new heart during Heart and Love Month. I went in for an aortic valve replacement, to begin with, and possibly a double by-pass. I walked away with my aortic valve and my ascending aorta replaced with an aortic graft composite (which includes the valve), with a repaired aortic arch and four bypasses. And just for safe measure, I had a heart attack while I was in the hospital, after the surgery.

And the long road towards recovery started only after I stood up from all that. Every day, I think back and I remember something about this year's journey.

I remember my first steps in the ICU – I thought they were smoking dope when they told me the day after surgery that I must make an effort to stand up and walk. Loopy as hell, with 100 machines hooked up to me and tubes coming our from everywhere (one or two in my neck, four in my abdomen, my catheter, IV), my first walk around the ward, then my breathing exercises, and the pain from my tubes – that was the worst of it, folks! The pain from those wretched tubes – I thought it would kill me! But I must tell you one thing: do not argue for the tubes to be taken out, no matter how bad it hurts! I had one in me for about 18 days after surgery – they sent me home with it. But they never had to do a puncture for built-up fluid, and my effusions healed nicely. I am grateful to that painful tube and that PA who talked me into keeping it in.

Then, there were the long days of trying to figure out how in the heck to sleep. I slept sitting up for months – definitely something to get used to; but I eventually became so tired that I didn't care anymore. Then, after several months, I slowly lowered myself down. Then, there were the fights with the insurance company who wanted me back to work in a month, even when the doctor recommended at least 3 months of recovery. Then, there were the ups and downs of figuring out my coumadin dose; then, the ups and downs of figuring up the dose of my beta blocker. Then, the skewed BP and pulse numbers – which are still skewed today.

Then, the OCD I developed about being clean and not touching anything in public. Seriously – I used to laugh at people who used hand sanitizer, and now I practically drink it. There were the occasional visits to the ER or Urgent Care, too. The sleepless nights when a tooth hurt and I was thinking my infection will attack my valve. It didn't.

Rehab was in there somewhere too. I thought I was going to throw up on my first day there, when they made me walk very slowly on a treadmill for 5 minutes! When I graduated, a couple of months later, I was walking 45 minutes and hardly breaking a sweat. But that could have been the coumadin – that thing makes me cold, to this day.

Then, the going back to work with people smiling and politely telling me I looked great, when I knew I didn't. I got down to 95lbs and I looked like a pole with stuff on when I went back. Plus, my skin was gray. I learned from a heart book I read this year that OHS patients have a gray complexion, because the heart pumps oxygen weakly right after surgery, and the main organs (liver, kidneys and brain) get the oxygen first with the skin being last. I was gray for a long while after the surgery.

There are literally hundreds of things I have been through this year that come to mind every day that make me wonder how in the heck do I still stand on my feet?! Things I never felt before that now I am used to as part of who I am: the dizzy spells, the weird double vision spells, the forgetful pump head and the stuttering, the hoarse voice, the wild dreams on pain killers, the permanent “Charlie horse” feeling on my numb left arm, the shallow breathing, the tightness in my chest, like a shirt two sizes too small … the blood popped vessels in my eye, the bruises right after surgery, the weirder than weird BP values …the fear of bleeding to death from coumadin, and the even greater, more horrid, overpowering and permanent fear of blood clots interfering with my new valve ... and I could go on for a whole day …

It's also been a year of trying to get back to normal. My normal is my work, my husband, my travels. I remember the travels this past year, full of fear and emotion, but also more vivid, more acute, filled with life, because every one of them was another achievement towards that elusive “normal. The mountains, the rivers, and people I met feel more real than before. I take them in and internalize them all more acutely than ever. This is what is important, for me, that, crutches or not, I get out there and live life. I smile at precious things, I frown at ugly ones, I walk, I see the sky, I love, I hate, I do what makes me human. I owe it to my doctor and to God who both put me back together to do my part and show them that their work was not in vain; that there is still life in this beating heart and curiosity in my brain, and a drive to discover.

It's really been a guessing game, every second of every day since February 12^th of 2016, when I started my life on this “other side”. Every day, I discover a new feeling in my body, a new pain, or numbness, or betterment, even. Every day, I am amazed at what medicine, but mostly at what very brave people can do to mend hearts. Every day, I visualize what went on in my chest and how these bionic pieces are now working with my heart to keep me alive.

It's amazing how much you learn about yourself and your body when you're listening. This past year has been a journey into that, for sure – into listening and paying attention closely and re-learning my body and who I am to some extent. I took all the freedom and symptoms from before for granted. But now, every single thing means something and it gets me closer to my core.

I don't hear my valve click, but I do hear my heart beat very strongly (stronger and louder than before) when it's quiet at night. With every beat I am walking closer and closer to my end, and I am aware now more of that end than ever before. I don't want any of these beats wasted. I want them full of meaning. Every precious beat is a gift. I want to sign a 'thank you' note to God for every beat I hear in my ear every night.

I still think of what happened every second of every day. I asked an OHS friend how long it took him to just never think about what happened in his chest, after having his aortic valve replaced. He said about 3 years. It's a long time, I say, but in a way, I never want to be forgetting it. I do hope that one day I will surprise myself with “Ha! I did not think of my bionic heart at all today.” but I am not rushing that day … Just like this year has taught me: one step at a time, in due time.

Today also ends my self-imposed ban on air travel. My first flight since before the surgery will be in a couple of weeks and we're shooting for a warm cruise. A little shy and nervous about it, but I am willing to let go a bit. I am ready to pack up with what I've got, the good and the bad, and head on to living life. It's the reason why I was given a second chance: the show must go on.

The toughest question I get asked is “are you feeling better?” Or “Are you 100% yet?”. If this year has taught me anything is that “100%” is an elusive number. I don't care who you are and what your life or health looks like, but none of us is ever 100%. But this does not stop us from living, does it?! I just want to scream at the top of my lungs that I am alive. Anything else is just bonus. I don't judge the way I feel anymore. When people ask how I am, or if I have a good day, I now always say that “Any day when I am alive is a good day”. And really, I mean that. From the wholeness of my heart. People look at me weird. But OHS has shaken me up to the point that my frame of reference is completely changed than before – I am more grateful and more aware. If I had to pick two words to describe me now, these would be them.

Just like river water, we are never the same person we were even a second ago. We are just different. But nonetheless, we owe ourselves to move on, eager to meet the next “different” us.

I heard it for years in yoga that we just inhabit this body, it's not ours, we just happen to find this host to carry us through this life. We're responsible to keep it clean and safe and sane, just like we would any abode. But I have not truly believed that till my heart stopped and got rebooted and I came out of it and read my surgery report and really bowed down in humility that this body indeed does not belong to me. And I am grateful it was given to me to care for … I hope I do it proud!

You will go through this and you will learn so much about you, first and foremost. About what makes you tick (literally). About how strong you are and how fragile and emotional you are, too. My dad always told me that heart patients are emotional – I have found this to be true. This site is proof of that – you all have big hearts and juggle big emotions. I have learned so much from you all. I also have learned that I am more emotional than I like to admit. I blame the heart for that.

You will also learn a lot about so many of the things that we cannot control. Especially, you will learn to trust: trust in God, trust in your doctors, your nurses, your family (even when they don't do the dishes just the way you like it, they are there for you, make your recovery easier, and keep you alive).

You will learn a lot about people, in general. About compassion or lack thereof, about caring and about science.

I have tried to live every second of the last year very much present and very much aware. I listen to my body more now. I know what it wants and when it wants it. I never ever put anything first before my body: if my body says I am tired, I stop. I left a job I loved because I was not able to close the door at 5 and just relax and give my body a break. I did not know how to relax before a year ago. Well I am glad to tell you – I love this relaxing stuff now. Some days, I am a total sloth – I just read, or paint, or knit, and let the dishes or the laundry wait another day. Some weekends are just lazy cat weekends with lots of comfort food and naps. Whatever I need, I give it to myself.

I have finally learned what the airlines tell us in every flight: put your own oxygen mask first, before you help someone else with theirs. In other words: you have to care for you, first, before you can be of any help to others.

I cannot believe this day has come. I cannot believe I have truly lived for 366 days (it was a leap year, last year) and muddled through this new me and have lived to tell you the tale. I was hoping that after all these days that big fog would be lifted and I would see the future more clearly. But it's not true. We can never see into the future, no matter how perfect we think our lives are. We can only see the now and here, and what we can touch in arm's reach. It's up to us whether we make this perfect, or wait for the fog to lift.

The now is the only guarantee I have, so I am permanently living inside it, cherishing it, loving it, as if it were all I got. And it is. I know you think this is a cliché, but there is no tomorrow without exhausting the now and today. This year turned this cliché into just fact, for me. The quality of tomorrow is measured only by what I put into the quality of today. So, I am trying my damnest to make it worth it.

I wish every single one of you much courage and faith. There is peace to be found and you will find it, right before your big day. And after that, there are amazing resources within your own self that will carry you through every day that follows. Trust in yourself, and trust in the power of medicine. We live in a fortunate age which is truly amazing and we are given all the ammunition to use that medicine to be whole. Not necessarily better (that's judgment), but whole and full.

And for those of you who can hear that click-clack of your valves, enjoy them! If that does not spell quite clearly “life”, I don't know what does. And isn't the sound of life the sweetest sound of all?!

Much health and strength to all of you, on my first second birthday! 

 

What a difference a year makes: February 11, 2016 and 2017

 

Between a Rock and a Hard Place with the Praluent Refill Authorization

… and why I am fighting for this still.

I have been sitting on this post for a while, not wanting to write it. There is so much negativity in the world today that my heart constantly aches. And I felt like this blog post will be negative, and I really don't want to add to that at this point in time, in our world, today.

But I felt, on the other hand, that I needed to continue with the saga of my trying to control my numbers and my advancing heart disease, and since a new chapter has been developing, I knew I needed to relate it. As usual, I hope this helps folks trying to stay healthy, out there.

A brief recap is needed: in December I was posting about how I was trying to refill my Praluent prescription and how the refill was denied twice, by the same insurance company that approved it 6 months before (see post from December here: http://livingwithfh.blogspot.com/2016/12/the-struggles-to-stay-on-praluent-and.html).

The doctor's (cardiologist) office faxed them all the information they requested, and they denied the refill twice. I received the denial letters on both occasions. What struck me as complete 'bogus' reasoning (for lack of a better word) was that in the first letter, they mention that I was denied the refill because I was not meeting 2 conditions, based on the information sent in by the cardiologist. After the nurse faxed them even more information, they sent the second letter of denial and this time, there were 5 (FIVE) conditions that they were looking for and I did not meet. So, it seems like for no reason, every time you give them what they want, they keep moving the finish line, raising the bar even higher. It definitely does not feel fair, but I am sure insurance companies are not in the business of fairness. But I digress.

I examined the list of the 5 conditions to see if I meet them. I am not going to reproduce them here, but if you want them, leave me a comment and I can share them with you.

They are written very much in 'lawyer talk' with 'either / or' and 'both or...' caveats, so they are a bit challenging to decipher, but I meet all five of them. This made me wonder if the nurse filled out some paperwork with mistakes in it, where she might have missed some of the conditions that I am sure I qualify for, unequivocally.

On my last week's appointment with the cardiologist, I asked the nurse if she can share with me what she sent to the insurance. She was not happy to share, but then again, this is not a happy nurse, usually. But she gave me a copy of what she sent, begrudgingly. She added that 'none of her patients ever gets approved for refills and from what she knows, about three quarters of all patients get denied for refills. They are approved for the first 6 months, and then get denied for the refills.” I asked the cardiologist, after she said all that, and gave me the papers, and he confirmed he statement. My cardiologist is also the head of the research program for Praluent and Repatha, for our state, so he works closely with patients just like me, as part of his daily practice.

When looking at the papers that the nurse filed, I could see she missed to check several boxes of conditions that I definitely have recorded in their files. The following things were missed and unchecked on the form she faxed to the insurance company:

• The diagnosis of Atherosclerothic Cardiovascular Disease (ASCVD);
• The ASCVD symptom of stable or acute angina;
• The ASCVD symptom of coronary or other revascularization;
• They checked that this medication (meaning Praluent) is being used in combination with another PCSK9 drug, which is incorrect;
• They checked that there is no presence of tendinous xanthomas in me, or any relative, which is incorrect.

However, they have checked many other boxes which still meet the 5 conditions as stated by the second letter I received:

• They sent my confirmed diagnosis as HeFH (heterozygous familial hypercholesterolemia);
• For the symptoms of ASCVD, they checked acute coronary symptoms and history of myocardial infraction;
• They mentioned that I have been on high dose of statins coupled with zetia for more than 12 months (since 2011);
• They showed that my LDL is higher than 100 mg/dl on statins (the value they used is 184 mg/dl and that was on statins, zetia and one month of 75 mg of Praluent) in addition to the ASCVD;

All I am thinking is that all the things that were missed could have proven to be even more convincing to the insurance company that I am really in need of this drug. However, I know this sounds wimpish and weak, but I cannot get my nurse to re-fax the complete information to the insurance. I have never found her to be extremely helpful, but on top of that, she is also so sick of dealing with the insurance companies and so sick of them continuously denying the applications.

I talked with the cardiologist and he agreed that several things were not checked, and he would ask her to fax it again. With me present, he asked her and she refused. She said “everything is on the paper and everyone gets denied. She (meaning me) is not the only one.” End of story. She also said she included the ICD codes which testify ALL of my diagnoses, but I looked up the codes on the fax sent to the insurance and two of them are for “mixed hyperlipidemia” and one of them I cannot find (I29.10). None of them say coronary heart disease, revascularization, myocardial infraction, AVR, or HeFH.

So, right now, I am pretty much stuck in between an incomplete application filed by my cardiologist's office and an insurance who refuses to renew the drug, even though they qualified me as a patient who needs the drug 6 months before.

What I know for sure is that I cannot have the cardiologist's office appeal this yet again. At least I don't know how to make them do it. But, I still have 90 days left from the last denial letter when I can appeal on my own, and I am getting the papers ready to be sent in today. Fingers crossed.

I am not sure if I could get an approval on my own, but the insurance papers say that I can appeal myself, and add more proof. I will use the papers already signed by the doctor, and add information that is missing in the paper which I can obtain online, from their web portal. Hopefully, this will add to the case.

And now the reason why I am fighting for this so much. During my lifetime, I have seen my total cholesterol levels in the 700's and 500's. For most of my adult life, my numbers have been (on lots of maximum dose of many drugs) in the 300's or, at best and rarely, upper 200's. After six months of Praluent, my numbers are finally in the normal range. 

My numbers, since April 2016, which was the last test before I started Praluent.
(click to enlarge picture)

When I saw those results, I was worried that something else might happen to me. I was worried that these new drugs are so powerful that they are going to render me dead if I quit them – they really did reverse my FH, judging by the numbers, and with virtually no side effects. I was elated at seeing the new values, but also scared, as if something powerful took hold of me. I felt like Bradley Cooper in Limitless. The drug is definitely working, but what happens when I stop taking it?!

There is also a belief that with improving the numbers, the cardiovascular disease will be reversed, too. Praluent (and Repatha) are too new to know by how much it would be reversed, but the studies are being done now, so we'll hopefully know soon.

All I am thinking, every day, is how my heart surgeon described the damage he had found in my heart arteries, and in my aorta and aortic valve, all because of cholesterol build-up. My major goal in life has always been to keep those numbers down, but I could never bring them so low that they would not affect my heart. And after an aortic valve replacement, an aortic graft, an aortic arch repair and a quadruple bypass, I know what all that build-up does to your heart. I was so incredibly lucky to have survived this!

Now, that I know there is something out there to bring those numbers in the normal range and possibly prevent further damage to my heart, carotids, brain, I don't want to leave anything un-turned to ensure I get access to it.

I feel like there are still things I need to explore, and it all starts with the first step. That step, for me, is to appeal once again and add more evidence to my case so that the insurance takes yet another look at my file.

And because when it rains it usually pours, this news came out just a few weeks ago: https://www.bloomberg.com/news/articles/2017-01-05/amgen-wins-ban-on-sanofi-s-sales-of-praluent-cholesterol-drug . Right now, Sanofi is appealing this decision, so there is a delay in this ban being implemented yet. For right now, there is still Praluent on the market and my cardiologist is supplying me with samples. But even if they are continuing to sell, being dependent on one doctor's sample supply is not a viable solution for treatment. I will continue to rebut their decision to not revew this and I will continue to update everyone on how it's going.

I know this fight is not mine alone, and that also gives me strength.

Much health, to all!

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!

Nine Months and Counting

Two days ago I celebrated 9 months since my surgery and things are well. I have no idea where all this time has gone! I know I was home for three full months after surgery, which (for someone who has never had medical leave before and never took more than 3 weeks of vacation at a time) seemed like an eternity. But now, I can hardly remember ever being home. I have been back to work for 6 months now, and it's all a routine again. 

I have no words to describe how incredibly grateful and humbled I am that I am here today, typing this. Although I am not the same person, physically (in so many ways) that I was a year ago, I feel well, and I am adjusting to my new reality. Living with Coumadin is not as scary as it was in the beginning. I go to the clinic every month and I take my INR myself at home, about 2-3 times in-between my clinic appointments. My INR is usually either within the range, or  higher. My range is only 1.5 to 2, so when it's 2.3 or 2.1, it doesn't concern me that much. 

My left arm has been numb since the surgery, and it's actually getting worse, for some reason. Only the pinkie and the 2 next fingers were numb, and now my whole hand and wrist are numb. No idea why. My breathing is shallow (see previous post for details), but I am learning to live with it. 

Believe it or not, I still have "pump head". I have days when words just don't come out of my mouth. I KNOW what I want to say, but I cannot say the words - and it looks like I am stuttering. I read that this could last for 5 years in some people. 

We are trying to make some plans for a cruise or some tropical vacation for later this winter, and I am a little nervous about flying again and going into different territory, outside the range of my doctors, but ... life is short. And I want to live it. And God has been so generous so far, that I feel encouraged to put this new lease HE has given me to good use.
I already started  shopping for Christmas, and we just remodeled a couple of rooms in our home. In other words: life is moving on, even after heart surgery. 9 months ago I could not have conceived I would be doing all these things in this much time. But life's a living surprise. And I am so amazed and in awe of it all! 

I asked one of my friends who has had his aortic valve replaced too about 6 years ago when will I just go through ONE day without thinking about what's going on in my chest. He said to give it at least 3 years. So, I'll try to be patient and still obsess over my heart, BUT ... I want to do things, too. So, I'll push through each day, one foot in front of the other. Worry or without. 

Another friend of mine asked me if I am going to yell at my surgeon when I see him next time (next month) for messing up my breathing and my arm, but IS SHE KIDDING??? The man practically killed me and brought me back to life in one piece! He gave me a functioning and better heart, and probably added years to my life. I have no words for the gratitude I owe him forever. I truly hope he lives forever and he gets to help many, many people. And I hope God is really good to him! I would not be here much longer if it were not for people like him, brave enough to fix hearts! 

I pray for all of the people who have to face heart surgery and are in some leg of this journey. I hope all of them have smooth rides, good teams and a lot of hope and faith! You all can do this. Medicine is amazing nowadays. Much health, strength and hope.