Sunday, February 11, 2018

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!





Saturday, January 27, 2018

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!

Friday, December 29, 2017

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.


Tuesday, December 26, 2017

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!


Sunday, December 3, 2017

A Big Move. A New Start. Managing the Same Health Challenges through It All

Why, hello, everyone!
You must have thought that I fell into a black hole lately, for not updating the blog for some time now. The reason of my temporary absence is that I have moved recently. Big, cross-country move. We moved from Utah to North Carolina at the end of October.

And we have been busy with everything that such a move entails: selling a house, buying a house, figuring out how to work remote, looking for a job (for my husband), registering cars in a new state, registering us as new inhabitants of the new state – you catch the drift. Busy, busy, busy, for the past several months.

Because I have the body that I have, I had to worry about many additional things in the moving process that had nothing to do with the bureaucracy of the move or its stress: while traveling across the US and not being able to cook for myself, will I be able to find enough food to live on for days with just fast food places as options? Will my INR get all messed up because my routine and my weather will change drastically? Not to mention my stress level. Will I be able to keep my Praluent cold enough in the camper's fridge, to be able to take it every two weeks, as scheduled? Will I find a new doctor/ cardiologist/ lipidologist, and how soon after I move?! And ultimately: will my heart be able to live through all the stress, the worry and the amount of work necessary for a move like this?!

Some of these questions have already been answered. And if this trip has taught me anything, it has taught me that I can fake being a 'normal' human being pretty well. Outside of the two weeks I took off from work, for the actual move, work has not missed a beat. The availability of the internet is pretty amazing in this day and age. The Praluent did fine in the camper, and for a while it was not even in the fridge: it was in its original Styrofoam box with its original icee for 3-4 days and it stayed cold. There was one dose due while we were on the road, and I took it in the camper. Now, if I were to travel abroad, I am not too sure how I would keep it cold for a while, but cross-country in a camper (in a car, too) would not be a problem.

I have read recently (http://www.empr.com/news/alirocumab-praluent-storage-refrigerator-room-temperature/article/687136/that the requirement that it must stay refrigerated has dropped, at least for a limited time frame, so you might want to look into that option, too.

My INR was 2.1, which is within my range, at one point when I did check it on the way. Once we arrived at the destination, it was fine, too, till I had to be on antibiotics for yet another UTI, and then it went to 2.9. This value is high for me, but way within normal range for many mechanical valve patients, so not too-too scary.


Testing my INR somewhere in America, in a campground. Accompanied by my morning bag of drugs. 

And that's the other thing I worry about while I travel: the UTIs which seem to always be connected to that! I have mentioned this before, I think, here: I have had up to 5-6 UTIs this year. The last one I just got over was accompanied by fever and chills and it was definitely the worst one yet. I have no idea why I have them so often, but they always seem to come sometime after or during a trip. And they make me crazy! I am thinking that either the infection will go to my heart (especially when I get fever and chills), or that the antibiotic will either kill my liver with everything else that I am taking alongside it or will make my blood so thin I would bleed to no end! These are real fears and real thoughts that keep me up at night. But somehow, the sun has risen every morning, in the East, like you would expect, and I am still here.

Life is a miracle, I tell you that much!

Once I moved, one of my previous caretakers (a lipid specialist and nutritionist) that I used to visit last time I lived in North Carolina recommended a new cardiologist close to my new home town. So, I have been able to actually see a cardiologist since I moved, even before I acquired a primary care doctor. The cardiologist did a cholesterol and a liver test, and the new numbers are included below. As you can see, my LDL is stubbornly hanging at 145 – and she said that in her opinion “the LDL can never be too low” and she wants it way, way, way under that number, and even lower than what the recommended range is. Of course I feel like therapy-wise they are throwing the kitchen sink at me, so what else could I possibly take?!


November 2017 Lipid Levels

She is scheduling me to see an endocrinologist who is also a lipid specialist. So, we will see what else he recommends.

In addition to the regular cholesterol levels, she also tested my apolipoprotein B (https://en.wikipedia.org/wiki/Apolipoprotein_B) which is another indicator for LDL cholesterol.
In the materials I have found online, it mentions that the normal ranges are within 40 and 125 mg/dl. My cardiologist's nurse emailed me that what they consider a normal range is between 54 and 133. Mine is 135. From what I have read, apolipoprotein B is supposed to go down to normal levels with statins. I never got it tested before now, so I am not sure what my sans statin value is, but apparently, even with a high dose of statins, mine is still high. I suppose that's understandable.

This new cardiologist being new to me, she is, like many doctors before her, pretty freaked out about trying to manage all this and my heart disease on top of it. So, she is deciding to have me see the endocrinologist lipid specialist for the cholesterol management, while she will manage my heart disease. I was spoiled before, with a lipid specialist and a lipid research geek for my cardiologist (all in one person), but we will have to see how this approach will work going forward.

All these years of testing my levels I have to say they are always new and always deja-vu for me, at the same time … I almost never expect my cholesterol levels to be anything but high and not to wow everyone. But one thing that is a surprise from time to time is my liver levels. They are about 90% of the time normal, but every once in a while they jump! Usually, they jump when someone is switching out my statin, but this time, there was no change in my cholesterol/ heart medication, and my ALT jumped to 102 (normal up to 54 U/L) and my AST is 54 (normal up to 41 U/L). When my liver enzymes jump, I get very scared, because everything I take has a potential of damaging the liver, and I know that the damage is irreversible. This time, there was no change in my medication at all, and I really don't know what caused them to be high (especially the ALT).

But this is why I continue to always check my liver, every 3-4 months, because you just never know when it decides to tell me that it cannot carry all these drugs' weight any longer. I just think it is so important to understand how these drugs we are on affect our entire body, not just the disease we are trying to manage with them.

My cardiologist's guess is that it's because the antibiotics that I took for my UTI for two weeks before I did the liver test. So, for now, she is repeating the test in a week to see if the enzymes go down. If not, we will start looking elsewhere for causes.

I will keep you posted!


Sunday, September 10, 2017

My Life. My FH. My Family.

How each member of my family deals with FH in their own way. A special installment for FH Awareness Month

I was never a momma's girl, like my sister. But there are two moments in my life when mom and I had a bond stronger than anything else. Stronger than any other bond that I have forged with anyone else.

The first one I can't remember, but it must be the day I was born. That first moment when she held me in her arms, as her first born must have been something very special. And it was special for me, too, I am sure, to see for the first time the face of the woman whose stomach I kicked for months.

The second one was the day my FH was essentially discovered. My mom was the person who was testing my blood, and trying to measure my cholesterol for the very first time in my life. As a biochemist tech in a hospital, it was her job to test the blood and see what the level was. I said “trying to measure my cholesterol” because she could not measure it right off the bat. Her machine was calibrated to only measure up to 5 mg/dl of cholesterol. Mine was much higher than that. The machine was giving her an error, meaning it was higher than what it could measure.

She had to do some magic chemistry tricks, with dilutions and what not, to figure out mathematically what my cholesterol was. So, my first value (she set it as 734 mg/dl) was a calculation, rather than a straight up measurement by a machine. It was my mom's math.

At the time, they did not call my condition “Homozygous FH”, or at least my parents were not calling it that. They were calling it “a very severe form of FH.” In those days (1983), they believed that my levels occurred in about one in a million individuals. Can you imagine: your first born has this very rare disease, she is one in a million people on Earth, and your hands, and your knowledge are the ones who testify it to the world?! That must have been the second pretty special moment for mom and me, too.

As they say, if you have found one FH patient, you have found a whole family of us. And this is always the case. We already knew my father had FH. His cholesterol has always been in the high 300's and low 400's. His father's cholesterol had always been in the mid to upper 300's. My father's father had his first stroke when he was 48. His second major one, at 50, left him paralyzed and bedridden for the next 12 years of his life. He died at 62.

My mother's mother (the only parent she knew really well and was still alive when I came along) had low cholesterol, so mom never tested her own. She always thought dad is the only one in the family with high cholesterol, and that he and grandpa gave it to me. It was not believed then that the “very severe form of FH” that I was diagnosed with could potentially come from both parents.

As mom grew older, she tested it, more of a curiosity than a necessity, she believes, and hers was always elevated, too. But she always thought it's because she eats badly. In Romania, the food is all home made, and very rich in fried oils, mayo, and red meats. It was not until I was finally diagnosed positively with Homozygous FH (this year, at age 42) that it was clear to us all that both of my parents have FH. She now believes it, too, as she finds lypomas on her skin, as she ages, and she has also been diagnosed with high blood pressure, cardiovascular disease, and heart failure.

And my family bonds don't stop just at my parents. My aunt, my father's sister, has always had high cholesterol, too, in the upper 200's, mid 300's. She has had over the years multiple stents inserted in her peripheral arteries, and about a month ago she had an ischemic stroke. She has a-fibrillation, tachycardia and arrhythmia – all caused by vascular disease because of high cholesterol. She is 67. She had her first stents put in in her 50's.

My sister has been formally diagnosed with FH a couple of years now. Because her levels are not astronomically high like mine, she, too, believed, it comes from her diet. But she lives one of the healthiest lives I know. She takes virtually no medication and she eats healthy, she runs and she lives in a huge city. She can't drive, and she has a one hour (one way) commute every day – she takes multiple public transport options and she walks. Her cholesterol is still higher than normal (low to mid 200's). It took some time for her to believe this is FH raising her cholesterol and not the chicken she was occasionally eating.

This year, her youngest son, who is six, was diagnosed as “borderline FH”, because his LDL is 170 mg/dl – somewhat high for anyone, but especially for a 6 year old kid with a very healthy diet.

And this is my real life FH family … as far as we know. So far, both my grandmothers, as well as my oldest nephew are the only immediate blood-related relatives that have not been affected by it.

It is interesting to see how we each decide how to live with it, and how we deal with it every day. There is the free will component in the way you deal with any disease and I have learned over the years that it is ours alone and it is not to be judged, even when you don't fully understand it. Or especially when you don't fully understand it.

I have lived my life with many doctors' appointments on my calendar. I always want to know the most there is to know about how much damage cholesterol has done to my arteries. I know my levels, I know my heart numbers, I know when I am due for my next investigation. I do an echo and a carotid ultrasound every year and I have cardiologist appointments every 3 months. Because I always know, I managed to have a very involved heart surgery that cleaned out my clogged up heart parts at 42 and undoubtedly extended my life by many years.

Without having been in the know about my levels, the damage that's being done to my arteries, and having been monitoring my heart and circulation closely, without having been on medication to keep my levels low (although still high by all other standards) for the past 20 years, I would not have made it to 42 and beyond, almost surely.

I also have chosen very early on to not have kids. I see no personal purpose into propagating this disease, and committing someone else, an innocent human being to the life I have lived. It's been a good life, but that is my judgment. Someone else might have seen it as a burden and I do not want to give that burden to anyone else, consciously. It stops with me, when I could control it.

My sister chose to have kids, and I think she is brave. She is also some days in a state of denial, because she still believes diet and exercise will keep her values healthy. She finds out the hard way that is not true. She does take drugs now, but not as religiously as she should – she does not tolerate statins as well as me, either, so it's not easy. She eats well, is not overweight, is very active and she sees her cardiologist every 6 months. So, she is definitely working to know more about her body and keeps a close watch on the disease.

My aunt only decided to do something about her cholesterol after the complications from it hit her hard. She did not take statins or heart medication before she had stents and the stroke. Although she is medicated now for the complications, she is still not medicated for her cholesterol. She does not keep a strict diet and she is overweight.

And then, there are my parents. Both of them have visible lypomas which give the FH away. They both have high blood pressure and cardio vascular disease. My dad has almost no peripheral circulation left in one leg and about 50% left in the other. My dad has been suspected of having mild strokes in his 50's already, but having not left visible signs in his mobility and mental behavior, they are not sure. My mom takes statins and Zetia occasionally, but not religiously. They both take heart medication. Their cholesterol levels are in the mid 300's constantly and their blood pressure is almost always high.

Both of them don't believe in diets, or exercise, because they think even with a healthy diet and an exercise regimen their cholesterol would still be high. They are correct, but I am sad, because their extra weight and fat diets are putting even more stress on their already strained hearts and brains. They don't even get regular physicals, and they only go to a specialist when something is definitely so wrong they can't function.

My parents are of a very old and very Balkanic mindset that we all owe a death to God and one day, sooner or later, we will die of something. They see no point in trying to push that death as far as we possibly can, at least the death from this disease. They also don't believe in 'quality of life' – their quality of life is miserable right now, but life 'is supposed to be hard' and 'you're supposed to be sick in your old age'. They are in their mid 60's and have been sick with the complications from cholesterol for most of their adult life and believe that after 50, you are certainly old and not expected to be healthy at all. Therefore, they see no point and no value in medication. They only take it when it's too late, and only to treat the symptoms of the disease, not to prevent any damage from it.

There is still to see how my little nephew will handle this, but my sister and I are trying to be good examples for him. So far, his mommy, my sister, is monitoring his numbers and watching his diet. He is too young for statins yet, and his levels are still borderline high. But this will be something he will have to watch in himself and possibly his children, too.

As you see: we all chose to deal with this very differently. You can do some things, and you can do nothing, but the effects of FH are pretty much the same. Now, they are known and they are manageable with medication, or, in my case, with surgery, too, but you have to be willing to manage them.

I have tried to use my own story to teach others about FH and share with them my journey, what to expect, what are some brighter spots of hope. My family knows about this journey way too well. But there is a difference between knowing and acting. If they have taught me anything is that you should share freely without putting strings on that sharing. However close to your heart some people might be you may never convince them to see what you see. I know – this is platitude.

I cherish every one of them for sharing this journey with me, so it feels less lonely. I also am deeply grateful that they gave me this disease – this has made me who I am today and I am happy and grateful that it has taught me so many things about my body, our health and how not to take even one breath nor one heart beat for granted.

And as chance would have it, there is yet another connection with my mom: The FH Awareness Month is September, which is when she was born. Happy birthday to mom and happy awareness to all of you! I found in my own life that being aware and knowing the risks, the numbers, the progress of this disease can ensure you are here, on the right side of dirt for many years beyond your original prognosis. If you learn about this disease now, I hope you find out all the people in your own families that might have it, and I hope you find the drive to manage it well into your advanced ages.

May your paths be adventuresome and leading you to good health and long years ahead! If you're still looking for more resources about FH, check out my Helpful Links section, or drop me a note. I'd be happy to share more!



Friday, August 25, 2017

Denied, and Then Approved. Again

If you have followed this blog regularly, you know that I have had trouble in the past being approved for my PCSK9 drug, Praluent. There is a whole process you have to follow to be approved for this drug by your insurance company, way long after a specialist doctor decides that it is your last resort.

I have outlined this struggle and the positive outcome from my last encounter with denial to be approved for the drug in this entry, earlier this year: https://livingwithfh.blogspot.com/2017/04/a-helpful-health-insurance-alas-there.html.

If you read that entry, you'll see that this insurance liaison helped me get my appeal approved, after having been rejected a few times. That man asked me to reach out to him directly should I ever encounter any problems with my approval again. I knew that offer was not going to be left hanging and that I will need his help again, and so I did just recently. But I am jumping ahead of myself!

So, after being approved for Praluent, finally, in the beginning of this year, I had to again obtain a pre-authorization from my insurance for my specialty pharmacy to be able to process my prescription renewal this August (after 6 months). It works like this: the insurance faxes a form to my doctor, and as the insurance representative and the pharmacy manager assured me last time, all they need to see is that my cholesterol level stays low on this drug, to ensure that the drug is still working. So, all they are asking the doctor on the form is what my LDL is, and what it was before starting therapy. Now, I would think that if the cholesterol level is not low, the doctor would not even prescribe the medication, right?! Why would they put me through a treatment that is costly (for anyone), risky (as we are yet to know the full spectrum of long-term side effects of this drug), if the drug is not working for me?! But I know: I ask silly questions!

So, this August, I get a letter from my specialty pharmacy telling me that my pre-authorization has been denied to renew my Praluent prescription, because my doctor has failed to send documentation that shows that there is a reduction in the LDL level while taking this drug – I paraphrased, but that was the gist of it. I was confused, because my LDL level before Praluent was 260, and my last level was 160. So I know my doctor has these levels and his office would have provided them, if needed.

I reached out to the same liaison from my insurance that helped me before, and the whole misunderstanding (again) was cleared up in less than half of a day. It turned out that all that the insurance company received from my doctor with their original request was the “fax form” (I imagine it to be like the fax cover letter with my general information on it), and not my medical records. When he intervened and they requested the information again, they received 26 pages of medical records. After reading those, they approved the pre-authorization for Praluent on the spot. When they received just the “fax form”, they turned around and denied me, without so much as to pause and ask themselves “hmm … maybe there was a fax line interruption and the rest of the pages are missing and we might need to call the doctor and make sure there is nothing else coming”, or something. Anything. I spoke about the broken processes in the medical world in another entry (http://livingwithfh.blogspot.com/2017/08/medical-non-care.html), but they abound, in my opinion, at every level.

This time, the insurance also approved me for a whole new year, instead of the original 6 months. Again, if you have read in the past: so far, I have been on Praluent for 18 months (give or take a couple of months when the drug was denied and I didn't take it), and I needed a pre-authorization from my insurance every 6 months in order for the specialty pharmacy to fill the prescription. After 18 months, the renewal is now required just every year.

I will have to say, it was helpful to have this relationship and be saved an appeal, or several.
I still believe that there are still a lot of rusty links in our processes. Maybe these medications are still very new, maybe the drug processing folks are still too little trained, what have you: if you are on any of these drugs, or any drugs where documentation from the doctor to verify your condition is still needed with every refill, be vigilant and act fast to stand up for your cause. I have made this connection with the insurance person through The FH Foundation who has introduced me to him as they knew about my difficulty to obtain an approval. Your employer (if you have your insurance through them) should have an advocate for your insurance that you can connect with for such cases. I will continue to do my share to push for more changes, more efficiencies to ensure they finally get the message and that filling such drugs gets to be routine for them, but sometimes, it might take a whole village.

The good moral of this story, however, is this: that the insurance companies and the specialty pharmacies, albeit marred in bureaucracies and lack of patient understanding at times, are willing to listen and learn. Or at least some of them are.