State-of-the art treatment can reduce LDL by up to 75%

Most of the estimated 42 million Americans with high cholesterol can successfully lower it with diet and exercise — or, when necessary, with statins or other cholesterol-lowering drugs.

But what if your cholesterol levels do not improve substantially with these standard therapies?

About one in every 500 Americans has an inherited (genetic) predisposition to high cholesterol — a condition known as familial hypercholesterolemia (FH), which is marked by LDL “bad” cholesterol levels ranging from 150 mg/dL to 1,000 mg/dL.

Dietary changes may have some positive effect on people with FH but typically do not lower LDL levels to a normal range. Cholesterol-lowering medication is sometimes sufficient for people with FH — but not always.

What most people don’t know: A high-tech treatment that filters LDL from the blood can reduce LDL levels by as much as 75% in people with FH whose cholesterol is not controlled with standard therapies. The procedure also can be used by others, including people who cannot tolerate statin drugs due to side effects.

SKYROCKETING LDL

On the surfaces of a healthy person’s cells, there are LDL receptors that remove LDL from the blood. Lower levels of LDL cholesterol reduce the risk for atherosclerosis (the accumulation of cholesterol and other fatty substances on artery walls). A genetic mutation in people with FH results in a greatly reduced number of LDL receptors — or none at all.

People who inherit a defective gene from one parent (the heterozygous form of FH) typically have cholesterol levels of 250 mg/dL to 500 mg/dL, while those with two defective copies of the gene (the homozygous form) can have cholesterol readings as high as 1,000 mg/dL. Genetic tests are available to detect the defective genes, but most doctors diagnose FH based on such factors as very high LDL levels and the presence of fatty deposits on certain parts of the body.

Important red flag: Cholesterol levels in people with FH may be so high that they develop xanthomas (deposits of cholesterol that accumulate). These occur most often in the Achilles tendons (backs of the ankles) but also over the knuckles, elbows, knees and bottom of the feet. They’re most commonly seen in people with FH who have LDL levels above 200 mg/dL. If you have any such deposits, see a doctor for an evaluation.

MEDICATION TO TRY

Most people with FH can achieve normal — or nearly normal — cholesterol levels with the use of medication. Typically, more potent statins are prescribed at the upper end of the daily dose range — for example, simvastatin (Zocor) — 40 mg to 80 mg… atorvastatin (Lipitor) — 80 mg… or rosuvastatin (Crestor) — 40 mg.

Good news about side effects: Even though statin-related side effects, such as muscle pain, are more likely to occur when high doses are used, people with FH who take such doses of these drugs don’t appear to have more side effects than individuals without FH who take lower doses.

Most patients with FH require combination therapy — treatment with a statin plus one or more additional cholesterol-lowering drugs, such as ezetimibe (Zetia)… bile-acid resins, such as cholestyramine (Questran)… or high-dose niacin.

“DIALYSIS” FOR LDL

A relatively new procedure, known as LDL apheresis, filters LDL from the blood — similar to the way dialysis filters toxins from the blood when the kidneys are unable to do so. LDL apheresis can reduce LDL levels by at least 50% and sometimes by as much as 75%.

How it works: At an outpatient clinic, a needle attached to a catheter is inserted into a vein in the arm. Over a period of about 90 minutes, up to three quarts of blood are withdrawn from the body and passed through a series of filters that remove the LDL. The “cleansed” blood is then returned to the body through another vein.

Who can benefit: LDL apheresis is recommended for people who don’t have atherosclerotic cardiovascular disease and whose LDL levels are 300 mg/dL or above and who can’t significantly lower their LDL after maximum therapy, including medication. Additionally, if you have been diagnosed with atherosclerotic cardiovascular disease and your LDL level is 200 mg/dL or above after maximum treatment, you may benefit from LDL apheresis. Patients with cardiovascular disease whose LDL levels are above 200 mg/dL and who cannot tolerate the side effects of statins also are eligible.

The results of apheresis are immediate. Cholesterol levels are tested before and after the procedure. It’s not uncommon for LDL to drop from levels greater than 300 mg/dL to as low as 35 mg/dL. The procedure also causes a reduction in C-reactive protein and fibrinogen, substances that increase the risk for blood clots.

Not a cure: Because apheresis does not eliminate the underlying genetic defect in people with FH, LDL levels start to rise immediately after the procedure is completed. Patients who opt for LDL apheresis must repeat the treatment every two weeks, possibly for the rest of their lives.

LDL apheresis is very safe. There is a potential risk for unwanted bleeding (both internally or from the needle site) because the blood thinner heparin is used to keep blood flowing during the procedure. However, this type of bleeding rarely occurs because the patient’s “bleeding times” (how fast small blood vessels close to stop bleeding) are frequently tested, and the dose of heparin is adjusted as needed.

Doctors don’t yet know how effective LDL apheresis is at reducing cardiovascular disease, but patients who receive the therapy often report a rapid reduction in cardiovascular symptoms, such as leg discomfort (caused by insufficient blood flow) and chest pain from angina.

People who are eligible for LDL apheresis should ask their doctors where they need to go for the procedure. It is currently offered at more than 40 medical centers across the US. Each treatment costs, on average, $2,500 to $3,000 and is covered by Medicare and most insurance plans.