When I Met My Extended FH Family and Learned a Thing or Ten

“There are no strangers here; only friends you haven't yet met.” (W.B. Yeats)

I have recently had the rare opportunity and honor to attend the training for FH Advocates organized by the FH Foundation which took place in Arlington, VA. If you're not familiar with this organization by now, you should be. They are doing great things to advocate for, educate and inform patients and medical staff about our condition. They have a terrific website, full of good information. If you or someone you know has been diagnosed with FH or is wondering whether they should be screened for it, and you don't know where to start, start there: thefhfoundation.org.

The training for advocates is just as it sounds: the staff and other resources hand-picked by The Foundation train people to be advocates for this disease. Individuals with FH, their family members or parents or anyone who has worked with people with FH can be an advocate. You learn everything from what this condition is all about, what makes it unique, what makes it like other conditions (here's a hint: nothing!) to what to say to patients, doctors, or the media when you advocate for it. There are two days of intense training followed by an elective day of going to Capitol Hill and speaking with senators and representatives about this condition, why it's important that people are aware of it, and how they could help bring awareness to their constituents.

I know, that sounds like a lot, and trust me, at the end of the three days my brain was absolutely fried. My brain was fried, but my heart was full. I am not sure how the FH Foundation staff has pulled this off, but they seemed to have painstakingly hand-picked the most amazing and nicest people on the planet. In the US, for sure. Never in my life have I met such wonderful folks: friendly, kind, humble, and giving. They shared each and every one of their experiences with FH, their heart stories, their family's stories as if we were olden friends, gathered 'round the ceremonial fire of cleansing, of letting go, and of taking in new life.

I have known that I have FH for 36 years now. Wow, just seeing that written down, it feels like a lifetime – which I am sure it is, for a lot of folks. In my family, everyone jokes about my cholesterol levels, because at 600 mg/dl, they call me a “champion”. My dad's family has it, so to us is just part of who we are (I suspect mom's family does too, but data is fuzzy on that side of the family). I never met anyone else outside my family, barring the folks I occasionally chat with online, that has FH. I have always wondered if other people can relate to my journey – with everything from the many doctor's appointments to being frustrated with the answers we receive, from insurance coverage challenges to what we should be eating every day, from the many a drug cocktails we feed ourselves every day to all the heart complications that ensue after some time.

I have found 30 or so complete strangers that shared the same questions and the same hopes and fears as myself. The most interesting thing to me was that we were all very different, coming from all four corners of the US, from different layers of the social web, of different ages (think 12 to 70 or so), at different stages in our medical journey, but all with a common denominator: we have FH and we're all living with it. For better or worse, through pain and through doubts, through successes and losses, we are here today to tell our story.

I think this, our being alive, is what unites us all into being advocates – because we want people to know, to be aware, and to find a way to manage this disease so that they, too, can continue living. We are all driven by this passion for life, and we want to continue with that, before any “events” get the better of us.

I have learned in my short time with these folks that we are all living proof that we love life, and we want to live it to the fullest. We all want to be here for our future. Whether we have 30 years to live, or 80 years to live, we all want to squeeze every drop of life out of this existence here, and we're ready for it. With needle marks on our arms, and stents in our hearts, we're doing it.

I grew up in a world that always puts the bleak and dark in front of any prospect: “Ah, well, you know, we all owe a death at the end.”, we say back home. They told me I was going to die by 25. But here, in Arlington, we talked about everything you can imagine except about death and about the end. We all want to be here for our kids, for our parents, for our nieces and nephews' graduations and weddings. Our collective spirit was smiling ear to ear the whole weekend.

Don't get me wrong: we are not delusional. We all have very grave stories to tell, but a grave outlook on everything would make this journey that much harder. And we're done with the hard parts. Those are a given. The hope and the lust for life is what we need – and from what I have seen, we all have plenty. It is this drive, and this spirit, this virtual smile on our collective face that should, I hope, drive us, as advocates, to spread the word about this condition.

Archbishop Desmond Tutu said in “The Book of Joy”: “...as we discover more joy, we can face suffering in a way that ennobles rather than embitters. We have hardship without becoming hard. We have heartbreak without being broken.” This is what comes to mind now, as I write this and think about these new wonderful friends I made.

You would think that someone who is taking 10+ drugs a day every day, and a couple of shots a month, someone who visits doctors more than they go out to eat, someone who gets so many stents placed in her heart that she has lost count, someone with quadruple bypass surgeries, replaced aorta, or valve surgeries in her 40's or 50's, someone who is in his early 20's and has been diagnosed with coronary artery disease in their teens, someone who is going in for apheresis twice a month and gets their blood drained three times over in one session is bleak, and cynical, and dark, and has lost hope. But this cannot be further from the truth.

This was the most energizing, the most fun, the lightest, along with the spiritually deepest group of people I have ever met. You can almost taste the maturity and wisdom that only hardships can teach us in the rooms these people filled this weekend. We are all great story tellers, and great listeners. I was grateful to each and every person in the room for the way they shared themselves with everyone. I have known all my life that battling disease leaves you naked and exposed. Raw, against the elements. There is power, strength, and true grit that you must amount when you go through life like this.

Apart from the human factor which was by far my favorite part of the weekend, this weekend has opened up new doors of understanding this condition for me. I thought, after 36 years, and virtually hundreds of doctors, I knew everything. But I have learned some new things, still. I have also realized that I have a long way to go before I truly know everything.

Here are some of the new (to me) learnings from this weekend:

1. There could be hundreds, maybe more, mutations for FH. Not two FH cases are alike. Depending on what receptor is missing or not working correctly in our bodies, we are all different cases of FH. This also includes members of each family within which the genes were passed.
2. Because of this, no two therapies are alike. Some of us are on statins, some are on pcsk9 inhibitor drugs, some are on apheresis, because they cannot take any drugs, and some are on a combination of all or some of these and more.
3. There are two types of diagnoses of FH: the genotype (looks at the mutations in your genes) and the phenotype diagnosis (looks at the levels of cholesterol). For a more in-depth description of what they mean, please refer to this site: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3104361/
4. A level of cholesterol of more than 400 mg/dl (mine is in the upper 500's – low 600's without therapy) could indicate more accurately a Homozygous FH. So, I am now not only more inclined to believe I have the HoFH kind, but also to be more sure that my mother probably has FH as well (her levels have been in the upper 200's and lower 300's all her adult life). Another possibility would be that I have a complex double HeFH mutation.
5. To know for sure what type of FH you have and what mutation, you must undergo genetic testing. I am looking into that with my cardiologist as my next step.
6. One in every 250 people has FH – so, it is not as rare as one thought. No one truly knows how rare HoFH is. The overwhelming lack of diagnosis of this condition is largely to be blamed for that.
7. Speaking of which: 90% of all FH people are undiagnosed, because the disease is easily confused with regular high cholesterol due to an unhealthy lifestyle. If your cholesterol is not lowered by diet and exercise, and you have a family history of high LDL cholesterol or cardiac events at an early age, then you should be screened for FH.
8. Apheresis is really amazing. I have met this wonderful lady whose LDL cholesterol is 220 mg/dl before apheresis, and this procedure takes it down to 22 mg/dl. Twenty. Two. I was in awe. I am still not running up to sign up for it, though. This is just a personal choice, of course, and I salute and bow to the people who have the gall to do this! It takes guts, folks!
9. I now know what a “high intensity statin” is, and that there are only two of them: Crestor and Lipitor – which are deemed to reduce LDL cholesterol by up to 50%. All others reduce LDL by less than 50%.
10. Along with coronary artery disease, aortic valve stenosis is also a complication of FH.

These are just the 10 that just came to mind. But my notebook is full of other goodies which I will share with my cardiologist.

I have also learned that all of us have encountered more than our share of clueless doctors, in want of a better word. We have all felt, at some point or another, like we are educating them on treating us. This was not totally surprising to me, but it does give me additional drive to continue spreading the awareness – not only through this blog, but with every doctor's visit.

I could write a whole book about how amazing this experience was. Not only how information packed, and numbers driven (I am a sucker for statistics) and educational it was, but also how life-affirming and spiritually rich it was. I will be forever grateful to the FH Foundation folks who are tirelessly fighting for us all, but also to all the strangers now become friends who shared themselves with us.

An old Tibetan saying goes like this “Wherever you have friends, that's your country, and wherever you receive love, that's your home.” Thank you all for welcoming me home.

The FH Foundation Advocates and some staff

Carotid Stenosis Improvement. Say What?!

To see the history of my carotid stenosis, all you will have to do is click the “carotid stenosis” label to the right of this blog. That should pull up all my previous posts on what the findings were in my many carotid ultrasounds, over the years. You will notice that since 2011, when I started documenting this, they have been worse every year. I believe I started with a 40-60% stenosis, which moved to 60-70% after a couple of years, and all the way close to a 90% blockage last year, before my heart surgery.

This week, I had my annual carotid ultrasound and vascular surgeon appointment, and for the first time ever, there is no progress in my stenosis. The narrowings are at the same percentage as last year, according to the PA. The vascular surgeon agrees, and even said “I will call them the same, because I am too nervous to really say they are a little better.” What?! “Better”?! They did not even bring up the one spot that had a 90% blockage last year. After 40+ years of clogging up my arteries and living with high cholesterol that has not been anywhere near normal limits, is there such a thing as an improvement or even a lack of change in my narrowings?! I am still in shock, and unsure whether to be thrilled about it or ask “what's next?!”

The only thing that is different this year is the fact that I have been taking a pcsk9 inhibitor drug (mine is 150 mg of Praluent every 2 weeks. I am not endorsing it, it is just what was prescribed to me). Drugs in this class promise to do wonders not only in our cholesterol levels, but also in reversing atherosclerosis. So, I guess the conclusion is: they are working.

I was told that the earliest you can see a change or reversal in the deposits on your arteries is after about 70 weeks since starting the pcsk9i drugs. I have about 61 weeks of Praluent behind me, and the verdict is that there has not been more damage added to my carotid arteries during this time. I will take that as a cautious win, of course, but I am still in shock.

Of course, I am still wondering what else (bad) is this drug doing to me besides improving my cardiovascular life, but I cannot dwell in fear of that. I am making the best decision with the information I have now, and will hope for the best.

There is still the risk of the carotid plaque having hardened the carotid wall, and continuing to do so, but at this time they said the blood velocity is good, so they are not suggesting surgery or repairs. Unless I develop an aneurysm, there is no surgery to be done, and because the narrowings are around 65%, they suggest no stents to be placed, either.

For now, I am just trying to let this bit of news sink in, because I am not used to ever getting good news about FH.

I hope everyone is healthy and having a good beginning of summer.

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