Cholesterol and Heart Health Update

My cardiologist is a funny dude. He keeps telling himself that I only need to see him every 6 months now, and then he can't make himself do it: he rescheduled me for 4 months again. I guess some of my symptoms and the recent test results made him nervous?! But let's not get ahead of myself.

This past week, I went in to see him after 6 months (since last November), which was the longest hiatus between two visits since my surgery. For the next appointment, he wants to see me in 4 months.

We looked over the new cholesterol numbers which are overall good, as shown below. 

 

You will notice that a couple of things are still off, though:

• the LDL (“bad” cholesterol) is a little high
• the HDL (“good” cholesterol) is too low
• as a result of the latter one, the cholesterol/ HDL ratio is still high

He is not thrilled with these numbers, although I am over the moon with them. If you don't remember, my LDL used to hang out in the 400's for years. To see the total cholesterol at NORMAL values is something I have not hoped to see in my life. But here it is.

If you want to see what drug and exercise regimen I currently am on, please visit this page: http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html

He talked to me about this new drug (also an injection, like Praluent) which is now in clinical research. It has not been approved yet, but he wants me to do the trial, to get that little bit of LDL down and that HDL up. I told him I would like to read about this new drug, but I made no commitments so far.

We also talked about my heart health. My blood pressure is still on its own internal roller-coaster. It can be as high as 150 over 50 one day, and as low as 110 over 35, another. Either way, the gap between the two numbers is what worries me. And the cardiologist. He still thinks that I have aortic insufficiency which is what he believes causes the weird blood pressure. This could also be worsened by the new aortic valve which is still a bit leaking ('mildly').

I have a couple of new symptoms, too.

I feel what I call a “claw” in my chest, over my heart almost daily. I think it might be in the chest wall, but I cannot be sure 100%. It feels like a sharp claw that just grabs hold of my chest. No idea where it's coming from. It does not feel anything like my angina which I had often right before the surgery. This is very different.

I still wonder if over a year from surgery now, my nerves are just now coming back into my chest wall, and I can feel more what went on in there. It just feels weird. The doctor did not seem concerned about this symptom and he said he could say positively that it is not heart related. He said it could be surgery related, but not heart related.

Another newer symptom is that my feet and legs seem to swell quite badly some days, especially in the evenings. I had swelling before, but it was very rare. Now it seems to be a pretty regular event. The swelling is painful, and I cannot quite pinpoint what causes it. He did a blood test for this, which is supposed to see if your heart is under stress, or to indicate whether you are in heart failure. The test is called a BNP (http://www.webmd.com/heart-disease/brain-natriuretic-peptide-bnp-test#1).

The normal levels for the BNP are under 190. Mine came back as 285. Always the overachiever, of course.

I am really worried about this, because coupled with the weird blood pressure and the fact that I tire easily when I exercise even a little bit, I am wondering if my heart is working right.

He did not seem to worry about this number, either. He said the value of 285 is “in no man's land”, and if the number would have been higher than 400, then, he would have known for sure that the heart is under severe stress. This number just shows, to him, that I have either aortic insufficiency, which he already suspected, or that my heart is still under the stress from the surgery last year. Either way, this is not why my feet swell up and this is not high enough to indicate heart failure.

I am trying to learn more about this test just because it puzzles me that it's so (seemingly, to me), high. If they give you a range, it must mean something, right?!

I have read that severe infections can also elevate the BNP, and I am currently trying to heal a stubborn UTI which seems to resist antibiotics, so I wonder if this threw off the number some, too. I have no baseline for this number, so I am not sure what it was before my surgery, or right after it. He said he will repeat this test in four months, when he sees me next.

Till then, continue with life as usual, I guess.

A Helpful Health Insurance?! Alas, There Is Such a Thing Indeed!

Why the insurance company finally approved my Praluent prescription and who is paying for it

I feel like I should have written these entries as part of a series: I should have named them all the same thing and then given them an episode number. But I didn't. Who knew it was going to become such a saga?!

If you are just now finding out about my struggles to stay on Praluent, due to my insurance denying my refill last year, then you must read the previous entries. They are, in order:

• The Struggles to Stay on Praluent. And They Are Not What You Might Think: http://livingwithfh.blogspot.com/2016/12/the-struggles-to-stay-on-praluent-and.html
• Between a Rock and a Hard Place with the Praluent Refill Authorization: http://livingwithfh.blogspot.com/2017/01/between-rock-and-hard-place-with.html
• Refill for Praluent APPROVED: http://livingwithfh.blogspot.com/2017/02/refill-for-praluent-approved.html

I suggest that you would read them in successive order, just to understand the whole story, as it progresses. First, I was denied when I needed to refill my prescription (please keep in mind the word “refill”: I had been approved by my insurance to take Praluent; but when the authorization needed to be renewed, after 6 months, I was denied); then, my nurse appealed the decision, to no avail – I got denied again; and lastly, I appealed their decision one last time and they eventually approved the refill, 6 weeks from my original request for a refill. All the details of what happened are in those three entries. If you are riding this roller coaster, as well, please read them and maybe some things will be helpful for you. But also, read on …

Just to be clear: patients of FH for whom statin therapy is not enough, or Homozygous FH patients, or FH patients who are allergic to statins depend on drugs like Repatha or Praluent (PCSK9 inhibitor drugs) to keep their cholesterol levels low. To some of us, this can be the difference between having a heart attack and avoiding one. So, if these drugs work for you, you want your insurance to keep them on their formulary and you want them to keep giving them to you. They are insanely expensive, so the insurance's help with paying for these drugs is paramount to being able to continue the therapy.

After my insurance approved my refill, I thought that was the end of my interaction with the insurance company and with the Specialty Pharmacy which is the entity that actually manages the filling of the prescription. But I was wrong.

As I mentioned before, the FH Foundation (https://thefhfoundation.org/) has reached out to me and wanted to investigate further into what happened with this refill. They work with various insurance companies to smooth out the process for patients that need these drugs

I can tell you I was not alone: several people that read the FH Facebook post mentioned that they had the same experience as mine, where they get approved the first time, and then the refills are problematic. My own cardiologist said that about 80% of his patients get denied for a refill and he cannot figure out why.

Well, after talking with the FH Foundation folks, they put me in touch with a person from my own health insurance who was investigating how they can improve this process for us, FH patients. He emailed me, and asked me a few basic questions about my account (name, date of birth, etc) and then he dug up my file and my history of requesting a refill. After several weeks of digging, he and a manager from the Specialty Pharmacy (you are always dealing with the reps from the Specialty Pharmacy when you fill this prescription) had a conference call with me to talk about what happened.

I went over the story, but it was fast apparent to me that they knew what the story was: I called in for a refill, was told that one would not be allowed without a pre-authorization; my nurse was required to fill out a form to require pre-authorization, and that was denied. Twice. Then, I filed one last appeal, and the insurance magically pre-authorized me for my refill.

To my surprise, the insurance company person as well as the pharmacy manager admitted that what happened was a series of mistakes on their end. The whole time I was calling and placing the order for a refill, they were treating me as a new case, not as a pre-approved case who just wanted a refill. When they did that, they also faxed the wrong form to my nurse: they faxed a form where they ask for all complete records that prove that I have FH. My nurse was half-completing that information, because she was assuming (like me, too), that she had already filled all that information out the first time she asked for the first approval and that the insurance company already has the information they need. But they needed that whole information thoroughly completed again, because in their mind, I was a new patient. This was a mistake made by the people working with my doctor to file the request for the pre-authorization.

According to both the insurance company and my Specialty Pharmacy, when you file for a refill, they require one form with just two questions on it: what was the LDL before therapy and what is the LDL now?! If they see that it dropped (and in my case, significantly), then they approve the refill, because they think the drug must be working. If not, they won't approve it. They don't need any of the previous information, about your family history, or your own history of CVD, or your previous and current medication, etc. They just need the doctor's office to tell them those 2 LDL levels and based on the trend of the numbers, they stop your therapy or continue it.

The people I spoke with apologized profusely and thanked me for being willing to help them “discover gaps in their process and training” so that they can fix this and so that no other patient should encounter this problem again. They also told me, quite surprisingly, again, that their full intent is to keep people like me on therapy, and not make them stop or skip the therapy. If you think about it, as expensive as these drugs are, they are still cheaper than paying for a heart attack or a stroke. So, it is in their interest, too, to keep us on the drugs.

I asked them what made them approve my last appeal and what made them investigate this case in particular. They said the appeal was approved because with the papers that I sent and the clear letter explaining my plea, they could tell right away that the Specialty Pharmacy reps were not listening to me. I asked if my doctor intervened more, and they said: “No. The only thing that approved your pre-authorization was your paperwork, that you submitted with your appeal.” In that, I added tons of documentation to prove my condition, medical history, my history with them, and I clearly stated that this was for a refill and not for a first time approval. When the appeal department got that, they approved it right away. You can read in this blog entry (http://livingwithfh.blogspot.com/2017/02/refill-for-praluent-approved.html) what all the papers were that I sent in with the appeal.

This conference call was the first time in my life, possibly, when I was not frustrated and exasperated, after talking with an insurance company. I was really grateful that they took the time to investigate and they discovered the mistakes in the process, and owned up to them. Hopefully, lots of people like me will have better experiences in the future.

My next pre-authorization is required again in August (after 6 more months since last time), but after that, it will only be required once a year, instead of every 6 months. I will definitely report back with how that next step will go. So far, all the monthly refills have come in pretty painlessly.

Another thing that I found was that the Praluent manufacturer is actually paying my copay, as I have yet not gotten a bill for this year's refills. They explained that sometimes, before they bill me, they look to see if the manufacturer offers a copay card. If they do, they apply it to my balance, and it just so happens that what they offer covers my balance. The manufacturer has never reached out to me, nor has it sent me any notification that they would be doing this. This has been completely behind the scenes, between the manufacturer and the health insurance. They mentioned that my insurance does not accept a copay card from all manufacturers, but they do from the maker of Praluent, as this is a preferred drug for my health insurance.

They also told me that when there is no copay card, and they must cover a significantly expensive drug like Praluent they go next to various foundations. Some foundations might cover the copay partially or fully, after a screening process. This was not my case, because the manufacturer's copay card was accepted. 

Both the insurance liaison as well as the pharmacy manager shared their contact information with me, so that I can contact them directly should this happen again. As I mentioned before: this has been an out of the ordinary experience with a health insurance company. So far, I have been pleasantly surprised by this follow-through and attention to details. Let's hope this holds and not just for me, but for everyone else struggling to get through to these institutions.

Please comment if you want additional information on what I have submitted, or if you have more questions about my interaction with the insurance company, the FH Foundation, or anything else. I'd be happy to help.

Easy journeys to all of you, and to all: much health!

The Roller Coaster of Cholesterol Numbers

If you have FH you are probably used to your numbers being low one month (yay!) and coming back up three months from then (drats!). Although you are doing nothing different, and you think you are managing your condition as you always have, the numbers fluctuate. 

Sometimes, I can kind of tell what causes my numbers to go up and down, but it's always just a guess. 

I know some doctors who would argue that the numbers even tell a story, but being on this roller coaster now for 36 years or so I can tell you that the numbers is the only gauge I have to refer to in order to understand how severe my condition is. So, yes, I still pay very close attention to the numbers.

Maybe you are familiar with my recent struggle to get Praluent approved again by my insurance, back in November - December (this is the last post that talks about it: http://livingwithfh.blogspot.com/2017/02/refill-for-praluent-approved.html). Well, in the process of getting that approved, I was out of Praluent for a couple of months. My doctor supplied me with Praluent samples but they were the wrong strength - they were 75 mg/ pen instead of my usual 150 mg/pen. He told me to just take one pen every two weeks and it will be better than not having the drug at all. 

Well, after a little over a month of taking the lower dose, my numbers went slightly up, as you can see in this picture, below. 

They are no longer within normal ranges, either. And this is, again, why it's so important to stick with a drug (and the strength that works), I believe, once we have found it. 

As you can read in the last Praluent post, I have been approved again for the correct dose of Praluent and I started taking that this week, so I hope the numbers will come down next time we check them, but there is no telling, of course. Like I said: sometimes it's no more than a guess. 

To keep ensuring that we all continue to have access to the medication we need and easily, I have also been working with the FH Foundation (https://thefhfoundation.org/) to help them understand the difficulties we encounter to get to the newest medication. They have been so supportive of me. They are doing just tireless and priceless work to work with drug manufacturers and insurance companies alike to support us and our cause and our need for ease of access to these medications. If you have not checked their site out yet, do it - it's a great resource for everything FH-related and they truly are great, compassionate advocates.  

I wish you all great numbers, great news and much health!

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.