For individuals with FH, bile acid sequestrants remain an effective, safe option for the treatment and can be particularly beneficial for statin intolerance. ^[1]

Secreted by the liver, bile is a fluid in the body that aids in digestion. It also helps to carry waste out of the body.

Since bile is composed, in large part, of cholesterol, bile acid sequestrants lead to the loss of cholesterol in the stool, and ultimately in the blood stream. They work by binding bile acids in the intestine, keeping them out of circulation and depleting the liver of the substance.

This in turn helps to reduce LDL-cholesterol (bad cholesterol) from the blood stream by an estimated 18 to 25 percent, which is important for those with FH, as plaque build-up can lead to heart attacks and even death.

Some people may be prescribed bile acid sequestrants alone, or in combination with other drugs to maximize its effectiveness.

Bile acid sequestrants are available under the following name:

• Cholestyramine (Questran, Prevalite)
• Colestipol (Colestid, Flavored Colestid)
• Colesevelam (Welchol)

The medications can be used for pregnant women and for children with homozygous familial hypercholesterolemia (HoFH), a rare, rare yet severe form of FH.

Consult a lipid specialist to determine if bile acid sequestrants are the right medications for you.

[1] Familial hypercholesterolemia & Pregnancy. National Lipid Association. https://www.lipid.org/sites/default/files/10-_underberg.pdf.