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James Underberg, MD, past-President of the National Lipid Association [NLA] and Clinical Assistant Professor at NYU Medical School discusses the guidelines for managing homozygous familial hypercholesterolemia [HoFH]. HoFH is an inherited lipid disorder that leads to extremely high levels of cholesterol [ie, less than 400 mg/dL or 10mmol/L] that is very difficult to control with standard lipid lowering strategies. As Dr. Underberg explains in this video, the rapid advances made in HoFH as well as the rarity of the condition means that most guidelines for this condition are fairly dated. Dr. Underberg also notes that while most lipidologists who manage people with HoFH are likely treating them in a similar manner, there is a need for newer guidelines that match the rapid advances that have been made in our understanding of this disease, as well as its treatment options. Currently, evolocumab and lomitapide are approved to treat HoFH patients and the U.S. Food and Drug Administration (FDA) is expected to make a decision about a third drug, evinacumab, very shortly.