FH Advocate for Awareness- Alina

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

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Blog Post from My Journey with Familial Hypercholesterolemia and Heart Disease

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.
  3. 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!

    To view original post visit:
    http://livingwithfh.blogspot.com/2017/12/the-tricky-balance-between-trusting.html

    Blog Post by A.W.

    About this Blog

    In this blog I will follow my everyday journey of living with familial hypercholesterolemia (or FH). I am sharing my own experience with this inherited disorder, and how I manage it daily – from what literature I read on the topic and what my doctors say to how I live my life (what I eat, what medicine I take, how I exercise, etc). This is solely a personal account that might or might not offer some insight on what to expect when diagnosed with this condition. This blog does not offer advice, in any way, to anyone suffering from this disease.

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