Adults Additional Treatment Considerations
ÎIf the patient is not at LDL-C treatment goal with the maximum available and tolerable dose of statin, then combine with ezetimibe, niacin, or a bile acid sequestrant (colesevelam preferred).
ÎDecisions regarding selection of additional drug combinations should be based on concomitant risk factors for myopathy, concomitant medications, and the presence of other disease conditions and lipid abnormalities.
Candidates for Low-Density Lipoprotein Apheresis
ÎLow-density lipoprotein (LDL) apheresis is a U.S. FDA approved medical therapy for patients who are not at LDL-C treatment goal or who have ongoing symptomatic disease.
ÎLDL apheresis is done weekly or biweekly and lowers LDL-C by up to 80% acutely and 30% chronically.
ÎIn patients who, after six months, do not have an adequate response to maximum tolerated drug therapy, LDL apheresis is indicated according to these guidelines:
• Functional homozygous FH patients with LDL-C ≥ 300 mg/dL (or non–HDL-C ≥ 330 mg/dL).
• Functional heterozygous FH patients with LDL-C ≥ 300 mg/dL (or non–HDL-C ≥ 330 mg/dL) and 0-1 risk factors.
• Functional heterozygous FH patients with LDL-C ≥ 200 mg/dL (or non–HDL-C ≥ 230 mg/dL) and high-risk characteristics such as ≥ 2 risk factors or high lipoprotein (a) ≥ 50 mg/dL using an isoform insensitive assay.
ÎHealthcare practitioners should refer candidates for LDL apheresis to qualified sites. Self-referrals are also possible. A list of sites qualified to perform LDL apheresis is in development and will be posted on the National Lipid Association website (www.lipid.org).
Women of Childbearing Age
ÎWomen with FH should receive pre-pregnancy counseling and instructions to stop statins, ezetimibe, and niacin at least four weeks before discontinuing contraception and should NOT use these medications during pregnancy and lactation.
ÎIn case of unintended pregnancy, a woman with FH should discontinue statins, ezetimibe, and niacin immediately and should consult with her healthcare practitioner promptly.
ÎConsultation with a healthcare practitioner regarding continuation of any other lipid medications is recommended.
• Functional heterozygotes with LDL-C ≥ 160 mg/dL (or non–HDL-C ≥ 190 mg/dL) and very high-risk characteristics (established CHD, other cardiovascular disease, or diabetes).
