More Questions than Answers

Sometimes I wonder: “What is purpose of seeking to feel better? Have I not learned that I will never ever feel like I am running on 100% capacity, firing on all cylinders?! My HoFH will continue to do damage, my heart has had a major overhaul AND a heart attack. There is never hope for really feeling truly healthy and truly at full potential, as if I were a healthy person. So what is the point of all the tests and all the interventions that doctors still want to pursue?!”

And then there is this other little voice inside my head that says “Why not?! Medicine is so much more advanced than even 10 years ago. If there are ways, and procedures, and meds, why not seek the best life possible? Why not trust a doctor or two that do believe in the silver lining?!” The trouble with that is all the risks involved, and all the doubt, that despite all the advancement, I still have an unbelievably broken body, which is irremediably damaged for good, and forever, which will never ever run at 100% capacity, even with the best care.

Let me bring this in focus: I met with the second cardiologist since I moved to NC. He struck me as somewhat of a bully, a rushed maniac with a weird passion for treating hearts and somewhat of a bull in the China shop. Definitely not a boring guy! He initially was “guessing” based on what he has heard about my history (mostly from my previous cardiologist's notes) that the biggest problem with my heart is my AI (aortic insufficiency), caused by my (still) leaking aortic valve. Although, just like my previous cardiologist, he could not hear my AI (the leaking), he said the reason my blood pressure sometimes has “no bottom” (or a very low one) is because of the AI. He also guessed that my heart probably has fluid in it because of this and this is why my legs swell up sometimes. He also chalked the shortness of breath, the dizziness and other symptoms of fatigue and slowness to the AI as well.

He recommended a heart echo and then a nuclear stress test. After both were done, he called me and the conversation went into a completely different direction that before. He said “according to your echo, your heart is good.” That's it! He didn't say why, and he did not mention one word about my AI (which he believed to be severe before the echo, just based on the notes he read and on my symptoms). He said nothing about how badly my valve might be leaking, if at all, and whether there is a concern even about the leakage at all.

He, however, spoke a lot about the results of my nuclear stress test: he said when at rest, my heart gets an adequate amount of blood (and oxygen), and the vascularization is appropriate in my entire heart muscle. However, when my pulse raises to even as little as 120 (my goal was to hit 150 beats per minute, but I started getting short of breath and dizzy around 120, so they stopped the test), there is a corner in my heart (lower left ventricle to be exact) that does not receive blood at all. He said it's either a scar from an old heart attack (my MI was localized in the same area he saw on the test), or there is a blockage there that needs to be opened up.

So, his number one concern now is to get to the bottom of this mystery: he now believes that my shortness of breath, my dizziness, and all my other symptoms of fatigue come because of this area of my heart is struggling to work without proper oxygen.

He wants to do a cath next to understand which is the cause: if the cause of the lack of blood in that area of my heart is an old scar from the MI, I am not sure what he can do for that, really. But if the cause if a blockage, he wants to put a stent in to open it up. After which, he wants me (like he said the first time he saw me) “back in rehab”, because the heart has to start working better after the blood flow is opened up. The first time he mentioned rehab to me it sounded bogus, but this makes sense. Not sure what the course of action will be if we're not talking about stenosis but rather some other traumatic damage from my heart attack. He seems to be pretty certain that it's a stenotic spot where maybe a bypass was not done before, or where it was done and it didn't take. (have I mentioned that this doctor guesses a lot?! He does.)

I am, of course, not crazy about a stent, because that can also be a liability (stents can clog, too, with scar tissue). A little voice inside tells me also: “what is the point of all this? Can a stent really make you feel like a normal person? Can any procedure ever make you feel like a normal person? Of course not! So why add more side effects and worries, when you can just cope, like you have done for two years now, since surgery, and for 20+ years now since the first sign of cardiac problems?!” But not doing anything to help beaten heart (no pun intended) sounds just as crazy.

The doctor wants me to come up to the 150 bpm pulse and keep going with no symptoms for at least 15 minutes. But I have had angina before my surgery for years. Now, I have had early fatigue and shortness of breath after just a little bit of walking. Is the goal (for my age and weight, height, etc) of 150 bmp really realistic?! With everything that went on in my heart and with the constant abuse of high cholesterol (which will continue), is it really possible to hit the targets of a normal person?!

The one thing that motivates me to really look for answers and for a possible solution for getting my whole heart irrigated properly is that my symptoms kick in even without extensive exercise. They kick in after speaking for a longer period of time, or when I am nervous about a seminar I am presenting, or speaking in front of a crowd. So, simple things like these make me symptomatic. And to me symptoms means more damage to the heart, inherently. The heart cannot be healthy when it's struggling for air like this, when I do the simplest things. And I don't want it to quit on me when, say, I need to run across an airport dragging luggage during a short layover.

He wanted to do the cath within a week from my test (it's passed already), but personal conflicts for both me and the doctor have postponed this till a month from now. He wants me to increase my Atenolol to ensure my pulse rate stays low. In the meantime, he told me “not to go crazy: no jogging, no hiking steep hills, no pushing heavy carts. Just keep the pulse under 100, he said, or lower, as low as I can.

I am planning to have at least one more office visit with the doc before I go in for the cath, just to ask him all the questions I still have about all the tests he has done and about his overall opinion about my heart: some things that I knew I had from before (like the AI) – how severe are they? Should we worry about them? And also: is my heart ready for a stent?! What is the maintenance we need to consider when that will be implanted? What are the steps to take if I don't need a stent? If my heart is damaged otherwise from the MI, is there any remedy for that? If yes, what is it?! Lots of questions, still, as you can see …

I feel sometimes like trying to see this doctor is worse than trying to get approval for Praluent. You cannot get an appointment sooner than 6 months ahead. Now, because I am somewhat of an acute patient, I had to beg his office assistant (his nurse told me she cannot schedule anyone sooner than what the public calendar shows, and I have to work with his personal assistant) to schedule the appointment at least 2 months after my previous one. When we do meet, he is incredibly rushed and off to the next patient, so I have to really come prepared every time, with written questions to ensure I don't miss anything from what I want to know.

But we do what we have to do to get our answers, don't we?! A busy schedule, a rushed physician, or an inflexible insurance company should not stop us. Here's to resilience! 

I am very worried about what's in my heart, and I am doubly worried that he has little time to really know what's going on. Maybe he's used to this rushed schedule and to him is second nature to run off from one acute case to another … We can only hope …

I wish everyone smooth rides and easy questions. Much health and a beautiful spring, everyone!

New Numbers and a Slight Change in Drugs

My then new cardiologist recommended I'd see an endocrinologist for treating my cholesterol back in December (http://livingwithfh.blogspot.com/2017/12/the-tricky-balance-between-trusting.html). He tweaked a couple of my drugs then, in the hope of getting my LDL cholesterol down some more, and my HDL a tad up. 

At the time I was taking 5 mg/ day of Zetia and he doubled that to 10mg/ day. My previous cardiologist said there is little to no difference in the amount of Zetia you take for how much percentage of cholesterol that is lowered. He said taking 5 or even 2.5 mg is just as good as 10 mg. Since even the generic Zetia is $80 a month, I used to cut it in half to save on the money, since the benefit was not said to be great. But I gave it a shot and increased it at the advice of the endocrinologist. 

The endocrinologist also recommended I would add Metamucil to my diet which is also proven to have some benefit (being a fiber) in getting rid of some of the cholesterol. So, I also added that in December, as well. 

Last week I got new numbers and as you can see, they are not very much different that the ones in December. In fact, the total cholesterol, the LDL and the triglycerides are slightly worse. This time, these tests were not taking while fasting, so I think that matters, especially for the triglycerides. 

The endocrinologist also suggested that I would try Juxtapid, but after thorough research and hearing from folks who have taken it, and my liver enzymes going back up on their own, I decided not to go that route. I am way too nervous about the drug being too strong and damaging my liver to try it out. 

Since December, I switched my cardiologist and the new one might be able to manage my lipids himself, he said. We have not gotten to the part where we talk about my lipids, as he is more interested in figuring out my heart first. So, till we get to that point, I am deciding a few things on my own. Given the lack of improvement on the numbers in the past three months and the fact that my stomach is further upset by the Metamucil, I am deciding to: 

- stop the Metamucil
- go back to 5 mg/ day of Zetia

In the long run, I am also: 

- not signing up for Juxtapid (my new cardiologist agrees with this) 
- maybe canceling the appointment with the endocrinologist.

I have had some experience with most of these drugs and I just feel like the regimen and the diet (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html) I am on now has given me the lowest numbers that I could hope for, at this time. 

As always, this is a personal post and a personal decision. I always try to stay informed and weigh in what is best for my cholesterol as well as for my quality of life. 

The Power of a Community

When I first started this blog there were virtually no resources to use as reference for my disease. All I knew is that I have a rare form of Familial Hypercholesterolemia, but at that time I didn't have a name for it. I knew my dad and his dad had a lesser form, and I knew what that did to them. But outside of my own family I had no resources to learn more about what this disease means for me, in the long run, and what are all the available tools where I can learn more about it, or learn more about managing it. More than that, I never knew whether my fears and my apprehension was mine alone, or those feelings were normal, as we are trying to understand this disease.

Most of my doctors were themselves in the dark about what it means and what to do with it.

Fast forward a few years later, I found a discussion group on Facebook (the Familial Hypercholesterolemia (FH) Discussion Group - https://www.facebook.com/groups/47098836197/) which made me feel a little bit less alone in the world. There, I started seeing comments and questions from people just like me who wanted to know more information or just wanted to share their journeys with others like them, and to be heard. A feeling I knew too well.

Fast forward yet some more time, and The FH Foundation was founded and even later than that I became personally engaged with it. They are great for a lot of reasons (just check out their website and learn for yourself: http://www.thefhfoundation.org/), but the one reason I love the most is, again, because they bring people like us together. We can learn from each other and feel that we are not alone.

Medicine has advanced and I find that now more doctors know, for the most part, how to deal with our disease, or at the very least they know what specialty care they can refer us to so we can find proper management for FH. It is with this advancement that I now know for sure that I have HoFH, because genetic testing is now more available than before. But if it hadn't been for these resources and these groups of people and their experiences, I would not have known what would have been possible for me.

Medicine advancement is great, for sure, but the strength we can find in the community of people that share the same experiences, fears, as well as successes and victories as us is invaluable! It's like a much needed virtual hug. 

Every day, I thank people who have put these types of forums and resources together for their time and their drive to fill a void that was very much painfully felt for people like us.

This week is Rare Disease Week (February 28 is Rare Disease Day). Because I have HoFH which is the rare form of FH, I want to use the platform that I have to thank all of you for sharing your experiences with me, over time. I hope my journey is also helpful to some of you. To all of you, I say: you are not alone. We have each other, and I hope you reach out and know this to be true. I hope you will use the platforms that you might have (groups, and social media accounts) to share with others and make other lost people feel less alone. The power of a community of like-minded people is truly priceless.

Much health and hope to all of you!

Two Year Anniversary Since OHS

It's been two years now, since my very involved heart surgery, and what I consider my re-birth, really. Every year on my anniversary, I read through my story which I wrote right after being released from the hospital, and I look at the pictures I took during my recovery year. It is a therapeutic remembrance, and it also puts things into perspective: if I am still worrying now, I need to see how bad off I was and how far I have come and I need to appreciate the life I have managed to live since then. The time I got after this surgery is bonus time which might not have happened without it. For that, I am forever grateful!

I will always be a heart patient. I wish I could be here today and tell you that you get your surgery and you're done worrying and you're done thinking every day about whether your heart is healthy or not. I don't care who your surgeons and your cardiologists are and how wonderful they are (and they are truly miracle workers), you will always wonder “is my heart truly all right?! Am I truly OK?!” At least I do. I wish I could say you're done seeing doctors and getting tests done, but it would be a lie.

But have you ever seen those trees that grow up crooked in the forest?! A brand new tree springs up, and they are straight and pretty when they're a sprout. And then, they hit a rock in their growth towards the skies, and the rock is right there, on top of them. But do they die?! No. They learn to grow crooked and around the rock. Their trunk bends around the obstacle and eventually they come back springing towards the skies, again, when they have grown past it. They will always look more interesting than the others which grew uninterrupted and straight. They will be crooked, but alive, and strong. Maybe that much stronger. This is how I feel, too: not perfect, not worry free, not whole but alive and with so much to live for!

Although there are plenty of things different for me, different than before the surgery and different than a healthy person, I can say that I feel like I do have a full life. I work full time, I travel, I write, I advocate, I walk (although not very fast, nor running), I eat what I want to eat, for the most part – a proof of this is all the 10 lbs I gained over the past year! I could be skinnier (I am 115 lbs) and less out of shape, and I wish my heart could do more to support that plan. Ever since I moved to lower altitude, my energy level is much better than when I lived at 4000+F. I still get tired, but I feel like I last longer than before.

How I have felt since surgery seems to be changing constantly. Some things have been the same since the first day after surgery (the numbness in my left arm, the shallow breathing, the raspy voice which sometimes completely vanishes, the difficulty remembering common words, the occasional stutter, the very weird blood pressure), but some things are new: the shortness of breath when I exercise or walk up the stairs is still lingering on, which is surprising to me, the occasionally high pulse (even on a beta blocker), the dizziness and ringing in my ears, the feeling of the earth opening up under my feet and having no stability at all. A new thing is also the fact that now, I can hear my valve even when it's not completely quiet in the room. My valve had been so quiet for the longest time after surgery. But now, I can hear it, and my husband, who would be sitting near me, can, too. It took almost two years to get to hearing it and I cannot tell you why that is.

Every now and again, I have this claw-like feeling in my chest, like someone is squeezing my heart really strongly. I take my blood pressure when that happens and it is as weird as it always is: either 160 over 50 or 130 over 40 – so nothing really strange there. I am not sure why this happens and this unsettles me, but so far no doctor has been able to explain this to me.

I do know that my heart is not 100% strong and healthy, and I have learned to accept that, like I said: I will always be a heart patient and I will always have to keep an eye on it. Diagnostically, they continue to find one thing or another: I have aortic insufficiency and my new aortic valve is still leaking (mildly, though). They also think that my body gets about 64% of the oxygen that it needs to get, something they call cardiac impairment. More tests are needed to determine this for sure and to determine the cause, so more will be scheduled.

I take 10 pills every day plus a shot every other week (I am treating my HoFH along with my heart disease). Sometimes I wonder what it would be like to just stop it all and just see what life would be after that. But I know that’s not possible. I know there is no life if I stopped this all. And I love life way too much to experiment that …

Another constant since my OHS is my relationship with Warfarin. Although I have learned to trust it more, and although I have been on a dose now for a year or so that has not needed to be changed because I am constantly in range, I do always fear that something (anything, really) will make the INR dip low or sky-rocket. I have learned that antibiotics make it sky-rocket and apples do, too. I have also learned that since on Warfarin my liver responds faster to other liver-poisoning drugs (like antibiotics or even pain killers).

I have stayed away completely from spinach and kale, but I still eat peas, green beans, broccoli and salad several times a week. It was hard to get used to this “consistency” thing – you have to have the same amount of greens every week. It was hard to figure out a cup of veggies every day in my diet, but I have learned to think in weeks instead of days when it comes to Warfarin: if I have those 4 cups of greens (or whatever it is) in a whole week, I call it good. I don't obsess so much over what I eat every day, or with every meal.

My life with INR is like that night-light you have in your bathroom that shines from under the door at night: you know you're settled for the night and it's nice and quiet and ready for slumber, but there is one light which will not turn off, as a reminder that it's not completely dark, after all. But you can still sleep through it, nonetheless.

I have not cut off a piece of any of my fingers anymore, and with an INR range of up to 2.5, I fear internal bleeding less and less. I am still very much afraid and paranoid about infections. I have become your worst nightmare germaphobe, but I can live with that. I get frequent UTIs which throw me in long psychoses, obsessing terribly over whether the infection will get in my blood stream!

My relationship with alcohol has changed, too: I drink mostly non-alcoholic wines and beers (yes, they make them!), and I have an occasional glass of something “real” which will always raise my pulse and make my heart beat so hard and fast I feel like it will pop my chest open. I don't like that feeling, so I stay away from it … I don't think this is a huge sacrifice since I feel great when I don't have alcohol – so, it's all good. Coffee is much the same as alcohol: I have only had decaf for two years now and some weeks I have no coffee at all. I seldom crave it anymore.

I keep telling myself that this is all a process, and things will change as my body changes and ages and develops. And it's true. I expect my symptoms to be different over time and changing, to some extent. But every time something new happens I wonder is it just a change? Or a change for the worse?! I wish I had a doctor I could trust again to explain all these to me, but not yet.

I have moved to NC late last year and the doctors that went through my heart surgery with me are not near me anymore. I tried to see a cardiologist in my new home town and it was a bad start. She did not get what I had gotten through and the seriousness of my heart condition at all. Now, I am starting anew with another cardiologist, but it will take a while to see him. I have learned that bigger cities means bigger waits to see people and do tests. I worry about not having a heart doctor at the moment. Having one I trusted and one that was involved was my security blanket. And I miss that dearly. I never underestimate the peace of mind that comes with a good doctor who knows you and gets your condition. Knowledge has always been power in my opinion, and I miss that!

But aside from all these symptoms and fears, I think life is good. With every morning when I see the sky first thing, I thank God and my surgeon for giving me another day. Life is what you make of it, they say, and with my limitations and nervous worries and all I am trying to make a good one. I love my husband, I love my family and although far from them geographically, I try to stay as close to them as Facetime and Skype allow. I am there for my team at work, sometimes for 10-12 hours every day (I work from home now, by choice, not because of a disability). I am planning trips and looking forward for more camping this year. There are so many trails unexplored out there! Life goes on, obstacles and all, crooked body and all. We just need to find that clear blue sky to rise towards. The rest is a miracle!

You can read about my surgery and hospital stay in this blog which I wrote two years ago: http://livingwithfh.blogspot.com/2016/02/open-heart-surgery-day-1-to-8.html

For a visual journey (through pictures) of my first year, you may scroll through these shots: https://wanderworldpics.shutterfly.com/22602

Much health to all and much hope!

Latest in My Heart Tests

I have talked before about my heart worries, about the fear that I have that all is not well in my heart after all (see this entry from last month: https://livingwithfh.blogspot.com/2017/12/the-tricky-balance-between-trusting.html).

Although most of the time I feel OK ( I have never in my life felt 'great', really), there are some symptoms that sometime worry me more than others. My neuropathy in my hand and arm, and the strange pains in my chest, also my weird voice that has gotten raspier and quieter with time, after my surgery are not worrisome to me that much. But the weird blood pressure which persists and the shortness of breath which feels like a lack of air when I try to exercise even a little bit do worry me. Is my heart truly OK?!

With a move across country and with trying to find the right doctor to figure this all out (and not really finding one I can truly trust so far) the worry seems to grow bigger. The new cardiologist I have seen in my new home state seems to be overwhelmed by the weight of my heart concerns, at least this is how I feel after having seen her for the past three months now. Anyway, this is about the new findings in some tests that she ordered rather than about my relationship with her as my caregiver. That is a topic for another post!

As I was mentioning in the blog linked above, because of these concerns I have, and because she finally listened, she ordered a holter monitor for a couple of days and a cardio-pulmonary stress test. A month or so later I finally received the results to both of these tests. Apparently, the delay was caused by her not being able to explain the results herself and waiting to find a more "senior" cardiologist to interpret them. How's that for not worrying?!  

She did not have much to say about the holter monitor, other than “my heart was beating faster when I recorded the discomfort I was having.” However, she did not say what mishe have caused my heart to beat faster, especially when most of the times when I recorded the discomfort were at rest. For those of you who have not had a holter monitor attached to your chest before, it is a device (http://www.heart.org/HEARTORG/Conditions/HeartAttack/DiagnosingaHeartAttack/Holter-Monitor_UCM_446437_Article.jsp#.WlzccqinHIU) that is connected to all these wires (like a mini-EKG machine). The wires are attached to your chest with stickies. It is the size of a mini-ipod or so, and you wear it for any amount of time (a day to a week). You cannot shower when you wear it. When you have any kind of chest discomfort, like palpitations, short of breath, chest pain or pressure, there is a button on it that you have to press to signal the holet that you are having an “event”. At the same time, you record the time this happened in a journal and you specify what you were doing at the time when this happened. When they receive back the monitor from you, they put the reading of the monitor together with your report to understand what really happens with the heart in the moments when you recorded the discomfort.

Outside of a brisk walk one day when I recorded shortness of breath, all the 'events' I recorded in my journal and signaled on the monitor were at rest. So, why my heart was beating faster, I am not sure and the doctor did not explain. But another bullet point has been added to my concern list, as you can imagine.

Then, she also ordered a cardio-pulmonary stress test. Although I have done many stress tests in my life, I had never done one of these before. It is not pleasant. Nothing hurts, but it is very awkward and very uncomfortable. As you are walking on a treadmill, they hook you up to an EKG machine to monitor the function of your heart. There are two techs in the room: one watching your lungs and one watching your heart. They put this mouth piece in your mouth and they clip your nose: you can only breathe in the mouth piece which is connected to this hose which measures your lung capacity and other variables. You cannot swallow your saliva during this and you run on a treadmill with this whole setup on you for as long as you can. They speed up your treadmill and they increase the angle of the incline slowly as you exercise.

The test is supposed to find out whether you have any kind of impairment to exercise, whether from the heart or from the lungs. The way my cardiologist explained, there could be three things that stand between you and a good cardio exercise: it could be physical de-conditioning, which is a fancy way to say that you're out of shape. It could also be a pulmonary impairment, where your lungs don't fill up with enough oxygen for whatever reason. And there can also be a cardiac or circulatory impediment which would mean that either your heart does not pump enough oxygen to your body (which could point to a 'bad pump function' of the heart) or the exchange between the heart oxygen and your body is limited, because of blockages in your circulatory (arterial) system.

They found no pulmonary impairment, according to the doctor, although some of the decoding I did does say that there is a limited oxygen exchange at the level of the alveoli. But it is unclear to me whether that is on the lung or the circulatory side.

They also found physical deconditioning, which I know is there, because after years of limitations, I can only push myself so much to do cardio exercising. So, I know I don't do enough to stay in shape.

What they found out more clearly, was that I have a mild to moderate cardiac impairment. Apparently, they can measure something called the VO2 max or the VO2 peak, which is the maximum amount of oxygen your body gets during exercise. For more on the VO2, see https://en.wikipedia.org/wiki/VO2_max.

Based on my weight, age, etc, the VO2 prediction is 31.30 mL/kg/min. At my level of extreme exercise, it is 19.90 mL/kg/min for me, which is at about 64% from where it needs to be. Related to this, the value of the METS (oxygen uptake in ml/kg/min) was only 7.43, and from what I read for a woman my age it needs to be around 9.5.

So, to unscramble all this: my body does not get enough oxygen when I exercise. The doctor says this could be due to either or both of these things:

• my aortic valve does not work sufficiently strong to push the oxygenated blood out to the body or my heart muscle doesn't work sufficiently to do the same thing.
• I have still blockages in my (heart or body) arteries that prevent my muscles (including the heart muscle) to get enough oxygen.

The next step would be more tests to figure out which of these two possibilities is actually at play.

But before I plunge into those, I must find a new cardiologist.

I am praying that whatever they find it does not involve more surgery and opening up more blood vessels, but … God willing, I'll deal with whatever when I get there. Should I be so lucky to get there before there is a bigger problem.

Much health, everyone, in the new year and beyond and may your diagnoses and plan of action be crystal clear!

About Juxtapid (Lomitapide)

My lipidologist has recently recommended a change in my drug regimen, to try to lower my LDL values even more (read about this here: http://livingwithfh.blogspot.com/2017/12/the-tricky-balance-between-trusting.html). He recommended I would start taking Juxtapid (Lomitapide). Saying that I am nervous about starting this very potent and very much dangerous drug is an understatement. 

I have gone online in the FH Foundation's Discussion Group on Facebook to ask other patients and care givers if they have any experience with Juxtapid. 

I have had a couple of answers, with folks saying they have been on it and that it worked great for the numbers, but the side effects was pretty severe. 

Out of all the answers, Leitha Jordan Brogan's was by far the most informative. She is a HoFH patient as well as a nurse. I thought adding her answers here about Juxtapid might help someone someday on their decision to go on it, or not. 

Read on, and Leitha, again: thank you for sharing! 

I started at 5 mg for 1 month, then 10 mg for a month, then 20 mg and my numbers finally normalized at 40 mg but that dose was hard on my gut. Dividing the dose lessened the discomfort but doubled the price. Yes!! Be concerned about the effects on the liver. I have fatty liver. The doctors don’t seem too concerned about it. When you first start Juxtapid the dietitian will counsel you about fat intake and based on your weight will calculate the appropriate percentage of fats for you. They teach based on “fat is fat” not, good vs bad fats. ALL fats will react exactly the same so, staying under the allowance for you personally will avert disaster. “Disaster” comes in the form of EXPLOSIVE diarrhea or vomiting like is depicted in a horror movie. (I wish I was joking). Like I said, you watch the fat grams and keep them under the allowance and never, ever eat closer than two hours to the capsule and you will probably be alright. I was very, very fortunate.

60 mg is the maximum dosage for Juxtapid and I know some of the folks taking it are at that dose. I don’t think I could tolerate it any higher than 40 mg and honestly, if I was to end up being able to go back on it, I don’t think I’d consent to 40 mg again.

When part of the company’s sales pitch is, “... but you’d still qualify for liver transplant...”, it behooves you to pay attention. It’s a serious medication for a serious disease. They don’t take any of the warnings lightly. The Compass program sets you up with a dietitian who is always a phone call away. The pharmacist is also easily reachable. You have a caseworker who is easy to reach for any questions. Your prescribing physician becomes your new, best friend and you will find yourself on a “high priority” status in that office. (If you don’t...something is wrong). You will have lab work that includes LFTs (liver function tests...AST, ALT, etc.) at very close intervals to begin with but is later reduced to about every three months (after you reach your titrated, effective dose). You will have frequent appointments with your doctor. You will receive mailings with low fat recipes to try. The “welcome kit” has a fat counter for every fast food and chain restaurant known and it’s easy to eat out while maintaining the fat intake. I was calculated to be allowed 37 fat grams in 24 hours so I divided them up between my meals. I allowed 10 for breakfast, 14 for lunch, and 13 for supper. I would simply adjust for planned outings or special occasions but I tried to keep the majority of fats around lunch because of knowing I would take the Juxtapid at bedtime. I set my alarms in my phone to remind me about the last food for the day, (to be consumed by 7 pm) and the dose of Juxtapid that I took at 9 pm. I never missed a dose that way.

Once I was finally at my acceptable dosage, I had very few issues. Breaks in taking Juxtapid causes you to have to re-titrate. When I had open heart surgery I had to stop it because of all the medication I was on in hospital. I tolerated going back on it okay but starting all over left me more open to the side-effects. I’m happy to answer any questions you may have. I spent 36 years as a nurse before I had to retire.

... the cost for Juxtapid was right around $78,000.00 per month for the divided dose. Ordinarily it would have cost around $34,000.00. The commercial insurance I had approved the prior authorization and it operated on a “co-pay” (fixed amount out-of-pocket) as opposed to a “co-insurance” (percentage based fee). I paid the highest co-pay of $40 per month. Once I was priced out of commercial insurance (premium went from $684/mo to $2288/mo), I was forced onto Medicare and it has no out-of-pocket maximum, operates on a co-insurance of 80/20, and allows no third-party assistance with premiums or services. My portion of the monthly fees for Juxtapid was between $4-5,000.00 per month. LDL Apheresis costs around $11,200.00 per month and between my Medicare and the supplement I purchased for a little over $700/month, I don’t owe anything. I just have to drive five hours to get there and can’t drive home so my husband takes two days every two weeks off from his job and he takes me from Florida Panhandle to Atlanta. It works and has no effect on the liver so, I’m not going to complain. I’m thankful it’s available. Best of luck to you in making your decision. If I knew I wouldn’t be jerked off and on Juxtapid because of the chronic need to re-authorize, I’d go back on it if it again became available. You may consider a trial of it. Nothing says you can’t decide it isn’t for you and stop it. I’m still in a 10-year study for it, lol. Even though I lost access they still want to follow my progress.

It’s a huge decision. I took Warfarin after my open heart. Ugh! I think I made it harder than it had to be. But the week I started Juxtapid I cried every time I tried to cook, and even just going with a friend for lunch. I finally found the Turkey, Bacon, Ranch at Tropical Smoothie was 17 fat grams. It’s a rather large (and tasty) sandwich and it saved my sanity. I ate half for lunch and took half home. It was about all I ate on my lunches away from home. I still like them. Best of luck. It’s an amazing medication but I certainly understand the hesitation.

The Tricky Balance between Trusting Your Doctor and Listening to Your Body

After 34 years or so of trying to find answers to my disease and its complications, you would think I am done searching. But, alas, that is not the case.

My newest dilemmas (yes, there are several):

• My liver enzymes are on the edge: if I add any medication to my current cocktail, they shoot up. Is this something to worry and watch?! (it is for me, at least)
• I need to exercise more, but I have symptoms that prevent me from doing much: shortness of breath, chest tightness, dizziness. Are these from the heart? Or is the source of this discomfort elsewhere? Can it be removed and how?
• My blood pressure is still odd (systolic too high, diastolic too low). Should it be medicated?
• My total cholesterol is 184mg/dl, the lowest it's ever been, but my LDL is still high, at 145. Should we try some other medication? Or should we say this is 'enough' for me, since my 'natural' numbers are in the 500's and stay on the regimen and the diet I am on now ?!

So, with these questions in mind, I proceeded to see my cardiologist and my new lipid specialist. I got some answers, but as it is usually the case with me, I don't agree with some of them. Or at least, I want to learn more before I blindly follow their advice.

As a reminder, this blog offers no advice to anyone. It is just a representation of my struggles, my fears, and the choices I make alone. It should never be read or interpreted otherwise.

The Liver Levels

Following up from the last entry (http://livingwithfh.blogspot.com/2017/12/a-big-move-new-start-managing-same.html), I have had my liver levels redone, because at the time I was writing that entry they were elevated. What had gone up before were my transaminases (which, I learned in the meantime, are a different measurement of liver health than your bilirubin – more in a sec). My ALT was 102 (normal up to 54 U/L) and my AST is 54 (normal up to 41 U/L). At that time, my cardiologist believed that they were elevated because on top of my usual cocktail I had taken about two weeks of antibiotics for a UTI. After a week from stopping the antibiotics and repeating the tests, the ALT is 59 (still a bit high) and the AST is normal, at 34.

As a reminder, my usual cocktail includes Lipitor, Zetia, Praluent, and Warfarin (amongst other things), all of which are known to affect the liver.

The cardiologist is not concerned at all that the medicine I am taking is affecting the liver, although she agrees that it seems that if we add anything else to it (like the antibiotic) the levels rise. However, she does not consider these values too elevated.

When I personally see numbers jump around past the accepted range (especially double, like the ALT), it makes me wonder whether something is wrong. I have not found out where to find the real answer, really. For right now, I just know that my liver is sensitive to adding more 'poison' to it: after reading a whole bunch of things about what really makes the transaminases go up, I am more careful with and aware about the drugs or foods that do affect the liver (the warning is usually printed on the labels) that I consider taking. So, I know if I take Tylenol, it could affect me; if I drink a glass of alcohol, it could also affect me, or the liver, rather.

This is yet another thing to watch, I believe, because in this case the liver damage is irreversible and potentially fatal. So, onward we go, checking the liver enzymes every 3-4 months, in line with when we check the cholesterol values. This is a decision I made despite my cardiologist saying that these could be checked once every year now. I just would rather not go off my life-long schedule of every 3-4 months which gives me reassurance that I know how my liver is doing at all times, given the tendency of these numbers to spike. As you know from reading here: I like to know more rather than less.

My Heart Discomfort and the Need to Exercise More

I had a little bit of a spat with my cardiologist (who is new to me, having just moved to this part of the country). We have been over my family history; she knows about my HoFH (which she stubbornly calls 'hyperlipidemia' – a term I am not crazy about because it is not specific enough.) She also knows about my heart surgery and its complex nature. She has not, however, seen any heart images yet, nor has she asked me what my diet was. She declared that my 'heart is just great' and suggested for a therapy going forward that I 'should look at my diet and exercise'. I was a bit floored, and I felt like I was not being heard.

This is one of the peeves I have with just about every other doctor that sees me: their failure to understand that just diet and exercise alone will not make a difference in my case. Plus, she has no true understanding of what my heart really is doing: yes, she has seen the transcripts from my previous echos and cath, but she has not done one recently herself.

She had also not asked me if I have any limitations in exercising – a thing which I brought up myself. She was puzzled as to why. I then shared that I am short of breath, and that I don't last very long as I walk on an incline, I have the dizzy spells, and tinnitus quite frequently. Should those be looked into?! I also asked her if the Aortic Insufficiency and the weird blood pressure could be things we're missing about my heart. I wished she should have found these, and she should have suggested that we should look more into them on her own, but I am happy she eventually listened to my list of concerns. By now, I am used to helping my doctors along when I see that they gloss over my case as just another 'lazy patient who needs to cut McDonald's out of her diet' (which I am far from, as you know).

I hate arguing with doctors. I really do. I do expect them, though, to step out of the text book and look at the patient as a unique human being, with unique characteristics and responses to medicine, as well as with a unique build which might be more or less responsive to whatever the 'book' tells them to prescribe.

After pushing for answers she agreed that she should hook me up to a holter monitor for 48 hours and also do a cardio-pulmonary stress test, just to understand more about my heart function during a normal day as well as during exercise. So, I hope we get a better picture of what this 'great heart' is really up to, so we can hopefully start on the right foot.

In the meantime, I agreed to push myself a little more each day, in my walks: go a little further, walk a little faster, and see if my heart takes it (although if history serves right, I tried this many times before, and there is always this big wall I hit). We moved down from The Rockies into the hills of North Carolina because my heart does feel better here. So, maybe, just maybe, my insufficiency, caused by who knows what, is less prohibitive of my movements here. I do know that the heart muscle is happiest and healthiest when it moves. So, I'll try more, if I can.

The 'Weird' Blood Pressure

My new cardiologist suggested kind of nonchalantly and very unconvincingly that I 'should be on an Ace-inhibitor, like Lisinopril'. I asked her why, and she said it would lower my blood pressure, but it 'is indicated that people with heart disease should be on one, just as a safe measure.' The trouble with this is that my blood pressure is not consistently high. It has higher spikes, and my diastolic value is always too low. Every time I took something to lower it, it made me dizzier than ever and I felt like fainting. So, we decided to continue to monitor the numbers and see what the consistent trend is: the high systolic (rare) or the normal systolic and the low diastolic (more often than not)?! So, just like before, we are just watching the blood pressure with no remedy. She thinks, as the cardiologist before her, that my Aortic Insufficiency is to blame for my odd blood pressure, but there is nothing to be done for that.

My LDL Level. Can It Be Lowered Even More?!

As I mentioned in the last post, this cardiologist will not be the one managing my lipids and their treatment. For that, she referred me to an endocrinologist who specializes in lipids.

Incidentally, I had met with this same endocrinologist almost 20 years ago when I first came to this country. He happens to be renowned for his work with lipids, so my PCP back then sent me to him as a 'know all' specialist who will figure our my cholesterol problem.

Back then, I had not been positively diagnosed with HoFH and I cannot remember what he guessed on my disease: he did agree I had FH, but I cannot remember if he picked a type. 20 years ago, his first move for my treatment was to cut my Lipitor dose way down and to prescribe Niacin (and later Niaspan) to me in addition to Lipitor. He is probably the biggest believer in Niacin out of any doctor I have ever seen. Niacin is, besides awful to take because of severe side effects, ineffective to me: my numbers do not change on Niacin.

Now, 20 years later, guess what?! He recommends cutting the Lipitor in half, and he once again prescribes adding Niacin to my drug regimen. I politely told him we have been down that path and that didn't work so well for me. So, I told him it's not even worth talking about it, I will just not do it. He said that the numbers are not so much of importance, but that the effects Niacin has on the artery wall, which are proven to be significant, are much more important. This is the second doctor that says this to me, which puzzles me so: why do we have numbers and why do we strive for a target if they are not important?!

Using the same logic, we could say that an LDL of 145 mg/dl is indeed good enough and we should stay where we are. Especially since at the last carotid ultrasound it was seen that there was no more additional damage done within the past year to my carotids. So, the artery wall is fine, why not stop here?!

He mentioned I could now be on a more advanced form of Niacin, called Enduracin which has less side effects. He asked me to consider this. I am planning to get educated about Enduracin, but … I am not sure what to think. So far, it looks like Enduracin is just a fancy name for Niacin: I am not coming up with anything different for it than what I have known about Niacin.

In addition to Niacin and cutting the Lipitor in half, he also wants to add Juxtapid (Lomitapide) (https://en.wikipedia.org/wiki/Lomitapide) to my 'cocktail'. This is an orphan drug approved only for HoFH and it is very potent. What it does is block fat from coming out of the liver. From what I have read, it is very 'poisonous' to the liver, potentially causing fatty liver disease, and high transaminases levels – and again, we come full circle: I must watch those, as well, so anything with known side effects of liver damage scares me.

He agreed that this is an incredibly potent medication which could damage the liver, the reason for which he will prescribe a low dose (5 mg) and he will cut my Lipitor dose from 80 to 40 mg. I told him I must read and document myself more about this drug, before he can start the proceedings with my health insurance to get me approved for it. By all means, if you are reading here and have some input on this drug, a personal experience with it, what the results were, etc, please do share.

He was not too concerned with the liver enzymes already being too high for me. This is his opinion on those:

1. Transaminases are not 'too high' unless they are consistently triple the upper acceptable value, which mine have not been (yet).
   And
2. He believes that elevated transaminases (ALT and AST) are not a good indicator of liver damage. That 'unless the bilirubin is elevated, then there is no liver damage.' And my bilirubin has been normal historically.

Again, deep down in my heart, I believe there are numbers for a reason and when they are not within guidelines they cause reason for concern. I am not used to just ignore numbers. It's just not how I operate, no matter how many specialists I am exposed to. I am always skeptical of just ignoring the levels for any test!

If I start taking Juxtapid, to prevent the liver from overloading with fat and to prevent stomach upset and other GI issues, I will not be able to eat any fats at all. My diet must be 100% fat free. I am not sure that this is totally humanly possible for anyone. For instance, the plant based spread I use sparingly as a butter substitute, or nuts, or the fish I eat a couple of times a week, or the little bit of olive oil in my cooking must be completely cut out. At that point, I think eating out would be completely prohibited, too, as we have no control over what is in those foods.

He also said that even before taking Juxtapid, adding psyllium (or Metamucil) to my diet will also remove any fat or cholesterol I am eating now, even in small quantities and it will lower the numbers by 10-15%. The percentage is small, but I will take it. Because my liver is so critical to me (if you have not gathered that by now!), I am willing to try things that are known not to damage it. I have way too many problems already to add liver failure to them, I think.

I also asked about whether it would be important to the raise my HDL which, for me, has always been low. It is 33 mg/dl now. He admitted that he does not know how to improve that. He said beyond a glass of wine and nuts daily, there is nothing medical that can improve the HDL.

Right now, I am in a 'research and wait phase': I am researching Juxtapid and Enduracin, and I am waiting the results from my cardiac tests (the holter monitor and the stress test). I am nervous about removing half of the dose of Lipitor from my regimen, because Lipitor has helped so much and I have gotten where I can tolerate the side effects from it now. Removing it, playing with the dose and introducing a drug that we're not sure it would work, or that it would be safe in the long run makes me very nervous.

And then, there is the Niacin. I really think that's an old school remedy, with little success for FH people, but … should I give it another go hoping that the drug has been improved over the years?! And that's just the thing: is it a drug or more of a supplement which is very little for a disease like FH which seems to be affected only by powerful drugs, not supplements and diets? With everything I am taking together, adding anything or taking anything away changes the balance not only in treating the cholesterol, but also in the way I feel and in the way I can (or cannot) manage all the side effects. There is also the Warfarin to keep in mind which is affected by anything new, and which is worse: it is all processed in the liver.

And then there is the nagging question: Are my numbers really the lowest they can ever be and we should just not bother the magical mix of drugs and diet we have found to achieve them?! Or is there such a thing as trying more/ harder drugs to get those numbers evern lower?! I wish it could be a clear-cut answer. But all of us who have navigated this ship before know that it's mostly a guessing game.

Happy Holidays to all, and I wish you all good health and easy decisions in the New Year!