Dear Dr. Roach: I am a 69-year-old woman who was diagnosed with familial hypercholesterolemia (FH). I used statins for over a decade but developed vulvar lichen sclerosus (LS), which only went away when I stopped the statins. They weren’t very effective; on a statin, my total cholesterol was still 226 mg/dL. Without medicine, it was 310 mg/dL. When I was put on Praluent by injection once a month, my cholesterol went down to 182 mg/dL. Although I don’t have many side effects, it is very expensive.

My question is: Do I need to be on the medication as long as I am exercising and eating well? I don’t want to have the expense, but I want to know if it is helping.

— E.L.

Answer: FH is a genetic condition of very high cholesterol levels that presents a high risk for coronary artery disease. The diagnosis can be made by genetic testing and looking for abnormalities in the gene for the LDL receptor. But it can also be inferred by family history, cholesterol levels, and the presence of abnormalities during a physical examination.

If you have a definite diagnosis of FH, your risk for developing heart disease without treatment is high. However, there is a big difference between a person with two abnormal FH genes (homozygous) versus those with one abnormal gene (heterozygous). Prior to effective treatment, people with homozygous FH could have heart attacks by the age of 10, and few lived past the age of 30.

Treatment of FH should be managed by a lipid expert. It usually includes high-dose statin drugs, which reduce the amount of cholesterol your body makes. It is often combined with ezetimibe, which blocks the absorption of cholesterol from food. If the combination of the two does not get the cholesterol levels under control, or if a person cannot tolerate a statin, then they may be prescribed a PCSK-9 inhibitor like alirocumab (Praluent), which causes the liver to remove the harmful LDL levels from the blood.

Incidentally, I have never seen statin-associated LS. I did read a paper that showed a higher risk for vulvar LS among women taking statins. The same paper showed a similar increase in LS among women who had gallbladder surgery or were overweight.

I can’t tell you exactly how big of a benefit Praluent is providing to your health. However, people with FH are at a higher risk for heart disease than a person who doesn’t have FH but has the same cholesterol levels. If the diagnosis of FH is certain, I advise you to continue taking Praluent, as it is likely to have a very large improvement in your chances of getting a heart attack.

There are a variety of programs to help people pay for Praluent. Treatment of FH is one of the clearest indications for this medication, so your doctor should be able to help you. I have been increasingly disconcerted by insurance companies failing to pay for medications that are clearly indicated, forcing patients and doctors to go to extraordinary lengths to make patients pay for medications that they should be taking.

Contact Dr. Roach at ToYourGoodHealth@med.cornell.edu.