Zokinvy

About Lonafarnib

• Lonafarnib is a farnesyltransferase inhibitor prescribed for patients aged 12 months and older, with a body surface area of 0.39m² and above.
• Lonafarnib belongs to the class miscellaneous metabolic agents which serves a dual purpose, aiming to reduce the risk of mortality associated with Hutchinson-Gilford Progeria Syndrome and providing treatment for processing-deficient Progeroid Laminopathies.
• The latter encompasses cases with a heterozygous LMNA (lamin A/C gene) mutation featuring progerin-like protein buildup, as well as those with homozygous or compound heterozygous ZMPSTE24 mutations.
• The selection of patients for Lonafarnib integration into their treatment plan is determined by their medical history, diagnosis, and the guidance of their healthcare provider.

Strength:

Lonafarnib hard capsules are delivered in a strength of 50mg and 75mg, respectively, for oral consumption.

Recommended Dosage:

For patients able to swallow capsules, take Lonafarnib capsules whole with water; do not chew. If unable to swallow, mix the capsule contents with Ora Blend SF® or OraPlus®. Alternatively, for those unable to use these options, mix with orange juice or applesauce (avoid grapefruit or Seville oranges).

Prepare each mixture fresh for each dose and consume within about 10 minutes. To prepare in Ora Blend SF, Ora-Plus, or orange juice, empty capsule contents into 5 mL to 10 mL of liquid, mix, and consume the entire serving. Mix capsule contents with 1 to 2 teaspoonfuls for applesauce, stir well, and consume the entire serving.

• The initial Lonafarnib dosage for patients with a BSA (Body Surface Area) of 0.39m² and above is 115mg/m², taken twice daily with morning and evening meals to minimize the risk of gastrointestinal side effects.
• Following four months of treatment, the dosage should be increased to 150mg/m², also taken twice daily with meals.
• Dosages should be rounded to the nearest 25mg increment. If a dose is missed, take it with food as soon as possible, up to 8 hours before the next scheduled dose.
• If less than 8 hours remain, skip the missed dose and resume at the next scheduled time.

Important:

Ensure you don’t miss any doses of Lonafarnib. If you happen to miss a dose, take it as soon as possible. However, if your next dose is approaching, skip the missed one and resume your regular schedule never take two doses together. Keep your doctor informed about all medications, including over-the-counter drugs, herbal supplements, and vitamins, as some may interact with Lonafarnib, potentially leading to severe side effects. If you have questions or concerns about taking this medicine , discuss them with your doctor. It’s crucial to adhere to these instructions carefully to maximize the benefits of the medicine while minimizing the risk of side effects.

Warnings & Precautions

• Lonafarnib can cause serious birth defects if taken during pregnancy. Pregnant women or those planning to become pregnant should avoid Lonafarnib.
• Lonafarnib may lead to liver damage, which can be life-threatening. Regular liver function monitoring is essential for patients using Lonafarnib.
• Lonafarnib can reduce blood cell counts, increasing the risk of infection, bleeding, and anemia. Patients should undergo regular blood count monitoring.
• Lonafarnib may trigger hypersensitivity reactions, including anaphylaxis. Patients should be monitored for such reactions during and after administration.
• Caution is advised when using Lonafarnib in patients with liver issues. Close monitoring for signs of hepatotoxicity is necessary.
• Patients with kidney problems should use Lonafarnib cautiously and be closely monitored for signs of myelosuppression.
• Lonafarnib can interact with other medications, including over-the-counter drugs, supplements, and vitamins. Patients should inform their doctor of all medications they are taking before starting Lonafarnib.
• Lonafarnib can cause serious birth defects, making it unsuitable for pregnant women. Immediate medical contact is necessary if pregnancy occurs during Lonafarnib use.
• It is unknown if Lonafarnib passes into breast milk, and because it may harm nursing infants, breastfeeding should be avoided by women taking Lonafarnib

Common Lonafarnib Side Effects:

• Serious liver damage
• Myelosuppression (a decrease in the number of blood cells)
• Hypersensitivity reactions, including anaphylaxis
• Dizziness
• Fatigue
• Infections
• Constipation
• Increased blood pressure
• Decreased weight
• Electrolyte abnormalities
• Hair loss
• Nail changes
• Skin discoloration

Use in Specific Population

Lonafarnib has not been studied in patients aged 65 and above. It is indicated for use in patients aged one year and older with Hutchinson-Gilford Progeria Syndrome (HGPS) and processing-deficient Progeroid Laminopathies (PL). Caution is advised when using the medicine in patients with liver or kidney impairment, and close monitoring for signs of liver damage (hepatotoxicity) and blood cell count reduction (myelosuppression) is necessary.

Storage and Handling

Before handling Lonafarnib capsules, ensure your hands are clean by washing them with soap and water. Avoid touching the capsules with wet hands. If you accidentally touch a capsule with wet hands, make sure your hands are completely dry before handling the capsule again.

• Store Lonafarnib capsules at room temperature (68°F to 77°F or 20°C to 25°C).
• Protect Lonafarnib from light and moisture.
• Do not store Lonafarnib in the bathroom or in other areas where it may be exposed to heat or humidity.

Keep Lonafarnib out of the reach of children and pets