How Hard Is It to Manage My HoFH Cholesterol Numbers?!

I have expressed this … sort of mild desperation before: I wish I knew what causes my cholesterol numbers to go up. There are virtually no changes in my diet (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html), nor lifestyle, and as many people with FH know: diet wouldn’t much matter for the FH cholesterol numbers anyway. So, why my LDL oscillates anywhere between 140 and 190, I am not sure. Of course, both numbers are above the normal range and not anywhere close to what is considered desired for me (a top value of 70 mg/dl), but I would still like to know how to keep that number closer to 140.

The most recent blood tests showed the numbers you see in the picture below. Since March, if there is one change in my life is the fact that I probably deal with a lot more worry. I don’t know whether this also translates into a lot more stress, but it would be a fair assumption. Of course, we all have that, especially lately: insecurities about our health, our jobs, the economy in general, the loss of freedom that we have all felt because of the stay-at-home restrictions all add up to stress, I am sure. But, on the flip side of that: I have had lots more time to walk and exercise, a lot more than ever before. Walking, finding a trail and hiking are virtually the only times I get out of the house. And you would think those would translate into better numbers, but … not for me, I guess. I really would like to know what it would take to make the numbers stay low, once they do reach the 140’ish bracket.

My current levels

My liver functions, which I always insist on having checked when we check the cholesterol levels, are normal: in the past, I had those numbers jump up a bit, too. Usually, the cardiologist would send me home with “ah, well, that’s FH!”. Sure, there are other things they can “try”, like Juxtapid (https://en.wikipedia.org/wiki/Lomitapide) or LDL apheresis (https://en.wikipedia.org/wiki/LDL_apheresis), but for personal reasons that have to do with their side effects and overall impact on the body, as I understand it, I have chosen against them. And my doctor knows this.  

This time, though, he said something more than “ah, well!”: there is a new drug on the market that he would like me to try: Nexletol, or Bempedoic Acid (https://en.wikipedia.org/wiki/Bempedoic_acid). To recap, I will take the following cholesterol-lowering drugs going forward: Atorvastativ (80mg), Ezetimibe (10 mg), Praluent (150 mg), and Nexletol (180 mg). 

I have researched this drug to understand by how much it reduces the LDL levels and the numbers are anywhere between 17 and 21%, depending on your source. My doctor put the number at a conservative 15%. So, even with the fourth drug added to my cocktail, my  LDL number won’t be within normal range, generally, and I am still far off from my targeted range – 15% reduction would put me around 164 mg/dl (124 mg/dl when the cholesterol dips to the 140 range). The lowest numbers I can achieve is definitely the purpose, overall, even if they are not within the range.

Of course, with FH, we live and die by the numbers: the importance of the numbers being the lowest they can is paramount in reducing the risk for worsening my heart disease and atherosclerosis. 

My current drugs

I am happy that there is still research out there that continuously looks for alternative therapies for FH and I will take this new drug, as it so far promises little to no side effects. I hope the lowering of the numbers will happen for me as well – there have been other drugs that I have tried that have not worked so well for me, so this is always a possibility.

I plan to still measure my values as well as my liver values every 3-4 months. This has become pretty much routine for me. I will report back, as usual, when I know more about how this affects me, in general, and how it affects my levels, as well.

Much health to you all!