Patient selection
Use NCEP guidelines for drug initiation
Patient age and LDL level at which statin therapy is initiated may be influenced by the presence, magnitude, and number of other cardiovascular risk factors, as well as the presence of cutaneous xanthomas
Consider patient and family preferences
In general, therapy should not be initiated before 10 years of age in boys or before the onset of menses in girls. Patients should ideally be at Tanner stage II or higher
Ensure that patient does not have contraindications for statin therapy (e.g., significant hepatic disease)
Initiation and titration
Choice of statin should be based on patient and family preference
Begin therapy with the lowest daily dose, usually at bedtime. Measure baseline CK, ALT, and AST
Patient should report potential adverse effects, especially myopathy; if myopathy is present, its relation to recent physical activity should be assessed, treatment should be stopped, and CK should be measured; monitor the patient for resolution of myopathy and any associated increases in CK; medication may be restarted once symptoms and laboratory abnormalities are resolved
Advise female patients of concerns related to pregnancy and the need for contraception
Advise patients about drug interactions
Measure fasting lipoprotein profile, CK, ALT, and AST after four weeks and compare with laboratory-specific reported normal values
If target LDL levels are achieved and no laboratory abnormalities exist, continue therapy; recheck at eight weeks and three months
If laboratory abnormalities are noted or symptoms are reported, temporarily discontinue treatment and repeat blood work in approximately two weeks; when abnormalities resolve, treatment may be restarted with close monitoring
If target LDL levels are not achieved, double the dose and repeat blood work at four weeks; continue stepped titration through the maximum recommended dose until target LDL levels are achieved or there is evidence of toxicity
Monitoring
Monitor patient growth, sexual maturation, and development (Tanner staging)
Monitor fasting lipoprotein profile, CK, ALT, and AST every three to six months
Monitor and encourage patient compliance with lipid-lowering dietary and drug therapy; serially assess and counsel patient for other risk factors, including weight gain, smoking, and inactivity
Counsel female patients about contraindications in pregnancy with statin therapy and the need for abstinence or use of contraceptive measures; refer patient to a subspecialist, if appropriate