Monday, September 21, 2020

What Being Cholesterol and FH Aware Means to Me

Those of you who follow this blog should already know my story (http://livingwithfh.blogspot.com/2011/03/my-story.html). Just in case you don’t, here’s a short summary: 

My parents knew that my dad’s side of the family had FH long before I was born. Dad had it, and his father had it, as well as most of his uncles and aunts on his father’s side. When I was 8, my pediatrician recommended that my parents would check my cholesterol level as well, because my liver was enlarged. My LDL was 525 mg/dl and my total cholesterol was 734 mg/dl. 

My mom fainted when she found these numbers, as she was the lab tech that actually read them for the first time. They didn’t have much hope that I would survive my teens. No one had ever seen such levels in a small child in 1983 Romania and with no medication or procedures on the market, they had little hope that I’d make it very long. Even in Communist Romania, with virtually no access to cholesterol-lowering drugs, they did know one important thing about FH: that it leads to premature heart disease or strokes at an early age. My grandfather had his first stroke at 48 and then, two years later, another massive one which left him bedridden. Even at my fragile age of 8, I had grown up to see my grandfather decay fast and I understood so much. 

My parents kept giving him as an example to me, of what will, for sure, happen to me, only much, much earlier in life because my cholesterol levels were so much higher than his. Even at that age, I learned what cholesterol meant (a white, fat substance that clings on to your blood vessels and organs and prevents them from working right), and what one can do about it: extremely severe diet (at the time, my parents would try anything so I was on a no-meat, no-fat, no-oil diet for about two years before they gave up on that because it was not really working for my numbers), and medication (in the beginning they gave me a white powder-drug called Cholestyramine which is a binder drug, but that didn’t help much either). I think I was 9 or 10 when I could tell you very eloquently what atherosclerosis,  dyslipidemia, and familial hypercholesterolemia meant. Google them, please! 

I went from doctor to doctor, from specialist to specialist till I was about 14 or 15. Everyone shrugged. No one knew what else to do. When I was in my late teens (19 and in college), I started to have heart symptoms: mainly tachycardia and arrhythmia so they put me on a beta blocker. They also did my first echo at that time and they saw modifications then, although I am not remembering exactly what kind. All they told me was “this is perfectly normal given your disease, but it’s serious.” 

I moved to the US when I was 23 and my first priority was to get on a treatment. Since then, I have tried every statin there is, and added much more to my regimen of drugs. Even with everything I have been throwing at this disease (http://livingwithfh.blogspot.com/2016/07/my-current-drug-regimen-and-diet.html), my LDL cholesterol has only recently (this month) been within normal ranges but  still not at my ideal target (below 70 mg/dl). 

Even with much access to medication and specialists in the United States, my heart problems have increased over the years: my aortic valve and my aorta replaced, my aortic arch repaired for an aneurysm and four by-pass grafts of the major arteries of my heart. I also had a heart attack following my surgery. All this before my 41st birthday. It was then when I had access to genetic testing and finally my “very severe case of FH” had its own name: Homozygous FH, which is the rarer and more severe form of FH. This meant that my mom also has the disease. Because her levels had always been in the upper 200’s and because she doesn’t have much knowledge about her family history she never suspected it when she compared her levels with dad’s and mine. In her late 60’s now, she has just been diagnosed with a slew of heart problems, among which a stenotic aortic valve and severe atherosclerosis of most major arteries of her heart. 

In addition to my heart problem, I have moderate to severe stenosis in my carotid arteries and at least one instance of severe stenosis in one branch of my abdominal aorta. For now, we are keeping a close watch on these areas, with yearly exams and visits to a vascular specialist. 

I would not be sitting here writing this for you all today if it were not for the fact that I have been aware early in my life about what cholesterol means and what FH can lead to. I have taken every treatment that seemed to match my body and my needs and have made choices in my life that allowed me to stay as healthy as I can. Sometimes those choices went against the popular expectations, like choosing to not have children because I simply didn’t want to pass this legacy on, and I didn’t know if I was going to be around for them. 

However, I have had a full life otherwise.  I am proud of my family and their support, and proud of my marriage, my travels and my work. I would have had a different story, or not been here to tell you a story at all if it were not for the fact that very early on, I knew what this means and how to manage it and I started doing that as soon as it became possible. By then, I knew exactly what specialists I needed to see and what questions to ask. Awareness is key, with FH. 

September is cholesterol awareness month and September 24th this year is FH awareness day. If you know a little bit about your family history and you know you have people in your family either with high cholesterol (especially when they lead an otherwise healthy life) or with heart disease or stroke, urge your doctor to check your cholesterol and know what the numbers are and what they mean (http://livingwithfh.blogspot.com/2016/07/my-current-cholesterol-numbers.html) . Do this especially if you have children or think you might have them one day. Knowing early will allow you to start treatment early and slow the progression of atherosclerosis. Your life is worth so much more! 

Stay healthy and stay aware. 

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