Description

Zokinvy (lonafarnib) is a farnesyltransferase inhibitor. It is a prescription medication used to reduce the risk of mortality in patients with Hutchinson-Gilford Progeria Syndrome (HGPS), a rare genetic disorder that causes premature aging. Zokinvy is also used to treat processing-deficient progeroid laminopathies, a group of rare genetic disorders that cause premature aging and other health problems.

Uses

Zokinvy is used to treat the following:

• Hutchinson-Gilford Progeria Syndrome (HGPS)
• Processing-deficient progeroid laminopathies with either heterozygous LMNA mutation with progerin-like protein accumulation or homozygous or compound heterozygous ZMPSTE24 mutations

How to use

Zokinvy is taken by mouth, with or without food. The recommended starting dosage for patients with a body surface area (BSA) of 0.39 m2 and above is 115 mg/m2 twice daily with morning and evening meals. After 4 months of treatment, the dosage is increased to 150 mg/m2 twice daily with morning and evening meals. Round all total daily dosages to the nearest 25 mg increment.

If a dose is missed, take it as soon as possible with food, up to 8 hours prior to the next scheduled dose.

Dosage

The recommended dosage of Zokinvy is based on body surface area (BSA). The starting dosage for patients with a BSA of 0.39 m2 and above is 115 mg/m2 twice daily with morning and evening meals. After 4 months of treatment, the dosage is increased to 150 mg/m2 twice daily with morning and evening meals.

Mechanism of action

Zokinvy works by blocking the farnesyltransferase enzyme. Farnesyltransferase is an enzyme that adds a farnesyl group to proteins. This process is called farnesylation. Farnesylation is necessary for the proper function of many proteins, but it is also required for the activity of progerin, a protein that is responsible for the premature aging seen in HGPS and other progeroid laminopathies. By blocking farnesyltransferase, Zokinvy prevents the farnesylation of progerin, which reduces its activity and improves the symptoms of HGPS and other progeroid laminopathies.

Storage conditions

Zokinvy should be stored at room temperature (68°F to 77°F; 20°C to 25°C). Keep out of the reach of children.

Precautions

Zokinvy should not be used in patients with a known allergy to lonafarnib or any of the other ingredients in Zokinvy.

Zokinvy may cause serious side effects, including:

• Gastrointestinal problems, such as diarrhea, nausea, and vomiting
• Liver problems
• Decreased white blood cell count
• Decreased platelet count
• Decreased red blood cell count
• Increased risk of bleeding
• Increased risk of infection

Zokinvy may interact with other medications. Tell your doctor about all the medications you are taking, including prescription and over-the-counter medications, vitamins, and herbal supplements.

Contraindications

Zokinvy is contraindicated in patients with a known allergy to lonafarnib or any of the other ingredients in Zokinvy.

Side effects

The most common side effects of Zokinvy include:

• Gastrointestinal problems, such as diarrhea, nausea, and vomiting
• Liver problems
• Decreased white blood cell count
• Decreased platelet count
• Decreased red blood cell count
• Increased risk of bleeding
• Increased risk of infection

Other side effects of Zokinvy may include:

• Muscle pain
• Joint pain
• Fatigue
• Fever
• Headache
• Rash
• Hair loss

Overdose

There is no information on overdose of Zokinvy in humans. If you suspect an overdose, contact your doctor or poison control center immediately.

Additional information

Zokinvy is available in capsule form. It is available in the following strengths: 25 mg, 50 mg, 75 mg, and 100 mg.

Zokinvy is a prescription medication. It is available from select pharmacies.

Please note that this is not a complete list of all the information about Zokinvy. Please talk to your doctor