I was diagnosed with heterozygotic FH many years ago. My TC was in the low teens and I had xanthomas on the tendons of my hands and achilles heels. I had developed heart disease as a result of a number of years of stress and broken sleep due in the main to our first born coming into the World with biliary atresia and resulting cirrhosis of the liver.
Then followed 20 years of cholesterol lowering strategies which became 17 years of statinization culminating in 80mg of Lipitor over the final years of medication.
I am approaching the 2nd anniversary of my rebellion. The last time I had a fasting lipid blood test I was 3 standard deviations above the population mean, i.e. around 300. I am quite happy to remain at that level and have no xanthomas. In fact I now appreciate my good fortune in having above average cholesterol levels since this brings advantages in terms of health and longevity - better resistance to infections and lower risk of developing cancer.
Low cholesterol is a pathological state with serious long-term health consequences.
My only concession is that I take 500mg of SloNiacin (TM) each day in addition to the usual raft of supplements that we statin-damaged need.
Xanthomas are unsightly but I don't believe they are life threatening
I believe the monozygonomous form of FH is more problematic since the liver will have no receptors at all. Fortunately it is much rarer than the heterozygonomous form, which is the commonest hereditary disease (affecting 1 person in 500) according to the literature.