Ask for hepatic and renal function tests (baseline alkaline phosphatase, alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total bilirubin).

Before starting treatment, pregnancy test in females in the child-bearing age.

In the first year, assess transaminases every month or before any rise in the dosage (whichever happens first), and afterward every three months minimally and before increasing the dose.

Check other medications that patients might be administering; adjusting doses or alternative treatments might be required. 

Check signs of severe diarrhea or hepatic toxicity.

For nurses:

Assess abnormalities in the ordered labs and reports. 

Monitor for and inform the patient to report severe diarrhea or any signs of hepatic toxicity, for example, loss of appetite, serious abdominal pain, jaundice, dark urine, or light-colored feces.

Lomitapide could elevate transaminases. In a clinical trial, 34% of patients showed an elevation in ALT or AST about three times the upper limit of normal (ULN), or even more. There were no significant clinically-relevant elevations in alkaline phosphatase, total bilirubin, or the international normalized ratio (INR).[9][10]

Lomitapide can elevate liver fats as well, with or without a related transaminases elevations. Following 26 and 78 weeks of therapy, the median absolute rise in liver fats, from a baseline of 1%, was 6% assessed through magnetic resonance spectroscopy (MRS). Lomitapide therapy-associated liver steatosis could be a risk factor for advanced liver disease, involving both steatohepatitis or cirrhosis.

Before starting therapy, assess AST, ALT, alkaline phosphatase, and total bilirubin and routinely AST and ALT as required afterward. During treatment, modify lomitapide dosage if the AST or  ALT is three times the ULN or more. Stop lomitapide in case of clinically meaningful hepatic toxicity.

For the risk of liver toxicity, lomitapide is prescribed only via a controlled program below policy for assessing and mitigating risk. Prescribe lomitapide only to patients who are confirmed either clinically or by laboratory results to have HoFH. Lomitapide efficacy and safety remain unconfirmed in patients diagnosed with hypercholesterolemia other than HoFH.

High lipid profile is a risk factor for atherosclerosis and could cause asymptomatic coronary artery or peripheral arterial diseases.

• Dyslipidemia causes involve a sedentary living with diets rich in calories, saturated and trans fats, cholesterol, and/or familial (genetic) abnormalities in metabolizing fats.
• Diagnosis is through measuring lipid levels in serum (assessing total cholesterol, TGs, and HDL cholesterol and quantified LDL cholesterol and VLDL).
• Screening exams should take place at 9 to 11 years old, then at 17 to 21 years old and 2 to 8 years old (for a significant family history of serious hyperlipidemia, premature coronary artery disease or other risk factors); adults should have screening starting at 20 years of age, and then every five years.
• Total and HDL cholesterol are assessable while not fasting; however, most patients should have all lipids assessed during fasting, often for 12 hours for optimal validity and precision.
• The American Heart Association and the American College of Cardiology recommend treatment for all patients in the four key risk groups and for those who have raised lipid profiles and other risk factors combinations to decrease atherosclerotic cardiovascular disease risk. 
• Management relies on the particular abnormalities in lipid profile. However, it should always cover optimizing compliance, changing lifestyle (diet modification and physical activity), controlling diabetes and hypertension, quitting smoking, and sometimes in cases with an elevated risk of myocardial infarction or death because of coronary artery disease, low-dose of daily aspirin.
• Lomitapide is a microsomal triglyceride transfer protein inhibitor, which affects secreting TG-rich lipoproteins in the intestine and the liver. The dose starts low and step-by-step, raised almost bi-weekly. Patients should adhere to diets with less than 20% in fat calories. 
• Lomitapide might lead to gastrointestinal side effects such as diarrhea, raised liver fats, and increased hepatic enzymes.  Efficacy and safety in patients who lack homozygous familial hypercholesterolemia remain unproven.
• Apheresis is also a frequently used treatment in patients suffering from the HoFH who have limited or no response to medications.
• However, elevated levels of HDL have links with lower cardiovascular risk, and it is unclear if therapies that raise the levels of HDL cholesterol would reduce cardiovascular events or death risks.