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Zokinvy

Generic name: lonafarnib (LOE na FAR nib)
Brand name: Zokinvy
Drug class: Miscellaneous metabolic agents

Medically reviewed by Judith Stewart, BPharm. Last updated on Feb 9, 2021.

What is Zokinvy?

Zokinvy (lonafarnib) a prescription medicine used in people with certain rare genetic conditions that cause premature and rapid aging. Lonafarnib is for use in adults and children at least 12 months old whose bodies have reached a certain size (a body surface area of 0.39 square meters).

Zokinvy is used to lower the risk of death in people with Hutchinson-Gilford Progeria Syndrome (HGPS).

Zokinvy is also used to treat certain types of Progeroid Laminopathies that are "processing-deficient."

This medicine is not for use in people with non-HGPS Progeroid Syndromes or with Progeroid Laminopathies that are "processing-proficient."

Warnings

Tell your doctor about all your current medicines and any you start or stop using. Many drugs can interact, and some drugs should not be used together.

Lonafarnib can harm your unborn baby. Females who are able to become pregnant should use effective birth control during treatment with Zokinvy.

It is not known if Zokinvy is safe and effective in children under 12 months of age.

Before taking this medicine

You should not use Zokinvy if you are also using certain other medicines.

Many drugs can interact and cause dangerous effects. Some drugs should not be used together with Zokinvy. Your doctor may change your treatment plan if you also use:

You may need to stop using Zokinvy for 10 to 14 days before and 2 days after using certain other medicines. Do not stop taking Zokinvy without your doctor's advice.

To make sure Zokinvy is safe for you, tell your doctor if you have ever had:

  • kidney disease; or

  • eye problems.

Lonafarnib may harm an unborn baby. Use effective birth control to prevent pregnancy, and tell your doctor if you become pregnant.

This medicine may affect fertility (ability to have children) in both men and women. However, women should still use birth control to prevent pregnancy because lonafarnib can harm an unborn baby.

It may not be safe to breastfeed while using this medicine. Ask your doctor about any risk.

How should I take Zokinvy?

Take Zokinvy exactly as prescribed by your doctor. Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose.

Zokinvy is usually taken daily with morning and evening meals. Follow your doctor's dosing instructions very carefully.

Take this medicine with a full glass of water.

Swallow the capsule whole and do not crush, chew, break, or open it.

If you cannot swallow a capsule whole, open it and sprinkle the medicine into 1 to 2 teaspoons of Ora Blend SF, Ora-Plus, orange juice, or applesauce. Swallow the mixture within 10 minutes after mixing. Do not save it for later use.

Do not mix the contents of a Zokinvy capsule with grapefruit juice or Seville orange juice. Read and carefully follow any Instructions for Use provided with your medicine. Ask your doctor or pharmacist if you do not understand these instructions.

Lonafarnib doses are based on body surface area (height and weight). Your dose needs may change if you gain or lose weight or if you are still growing.

Your doctor may need to check your vision on a regular basis.

Store at room temperature away from moisture and heat.

Dosing information

Usual Adult Dose for Hutchinson-Gilford Syndrome:

Initial dose: 115 mg/m2 orally twice a day
-After 4 months increase to 150 mg/m2 orally twice a day
Maintenance dose: 115 mg/m2 or 150 mg/m2 twice a day.

Usual Pediatric Dose for Hutchinson-Gilford Syndrome:

12 months and older with a Body Surface Area (BSA) of 0.39 m2 and above:

Initial dose: 115 mg/m2 orally twice a day
-After 4 months increase to 150 mg/m2 orally twice a day
Maintenance dose: 115 mg/m2 or 150 mg/m2 twice a day

Comments:
-The total daily dose should be rounded to the nearest 25 mg increment.
-For patients unable to tolerate a dose of 150 mg/m2 twice a day due to repeated episodes of vomiting and/or diarrhea resulting in dehydration or weight loss, may decrease dose to 115 mg/m2.

-Limitations of Use: Not indicated for other Progeroid Syndromes or processing-proficient Progeroid Laminopathies since based upon its mechanism of action, it would not be expected to be effective.

Uses:
-To reduce the risk of mortality in Hutchinson-Gilford Progeria Syndrome (HGPS).
-For the treatment of processing deficient Progeroid Laminopathies with either:
--Heterozygous LMNA mutation with progerin-like protein accumulation
--Homozygous or compound heterozygous ZMPSTE24 mutations.

What happens if I miss a dose?

Take the medicine as soon as you can, but skip the missed dose if your next dose is due in less than 8 hours. Do not take two doses at one time.

What happens if I overdose?

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

What to avoid

Grapefruit and Seville oranges may interact with lonafarnib and lead to unwanted side effects. Avoid the use of grapefruit products and orange marmalades.

Zokinvy side effects

Get emergency medical help if you have signs of an allergic reaction to Zokinvy: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Call your doctor at once if you have:

  • new or worsening vision problems (such as decreased night vision);

  • severe ongoing nausea, vomiting, diarrhea;

  • loss of appetite that causes weight loss;

  • high blood pressure - severe headache, blurred vision, shortness of breath, nosebleeds, pounding in your neck or ears;

  • kidney problems - little or no urination, swelling in your feet or ankles, feeling tired or short of breath;

  • high potassium level - nausea, weakness, tingly feeling, chest pain, irregular heartbeats, loss of movement;

  • low potassium level - leg cramps, constipation, irregular heartbeats, fluttering in your chest, increased thirst or urination, numbness or tingling, muscle weakness or limp feeling;

  • low sodium level - headache, confusion, slurred speech, severe weakness, vomiting, loss of coordination, feeling unsteady;

  • low calcium level - muscle spasms or contractions, numbness or tingly feeling (around your mouth, or in your fingers and toes); or

  • signs of infection - fever, chills, cough, rash, swelling, diarrhea, pain or burning when you urinate.

Common Zokinvy side effects may include:

  • an electrolyte imbalance (such as low levels of potassium, sodium, or calcium in your blood);

  • infection;

  • increased blood pressure;

  • stomach pain, nausea, vomiting, diarrhea;

  • decreased appetite, weight loss;

  • headache;

  • feeling tired;

  • muscle or joint pain;

  • cold symptoms such as stuffy nose, sneezing, sore throat, cough; or

  • abnormal lab tests;

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

What other drugs will affect Zokinvy?

Sometimes it is not safe to use certain medications at the same time. Some drugs can affect your blood levels of other drugs you take, which may increase side effects or make the medications less effective.

Many drugs can interact with lonafarnib. This includes prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible interactions are listed here. Tell your doctor about all your current medicines and any medicine you start or stop using.

Further information

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use Zokinvy only for the indication prescribed.

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.