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Fosdenopterin

Brand Name: Nulibry
Dosage Form: intravenous injection powder (9.5 mg).
Drug class: Miscellaneous metabolic agents

What is Fosdenopterin?

Fosdenopterin reduces the risk of death for children with MoCD Type A. The molybdenum cofactor deficiency (pronounced "moe LIB-den-um") is a rare disorder that affects the brain function of newborn babies. It gets worse with time. MoCD can cause severe developmental delays as well as life-threatening health issues. Symptoms usually appear within one week of birth. Fosdenopterin can be used in other ways not mentioned in this guide.

Side effects of Fosdenopterin

If your child shows any of these symptoms of an allergic reaction, you should seek immediate medical attention: hives, difficulty breathing, swelling of the lips, face, tongue, or throat.

If your child is suffering from:

  • A virus
  • Severe or persistent nausea or diarrhea.
  • Around the catheter, you may experience bruising, swelling, or discomfort.
  • Cough, mucus, and difficulty breathing
  • A seizure;
  • Signs of an ear infection include fever, earache, drainage, and fussiness.

If your child is experiencing a serious burn, such as blisters, skin redness, or burning, you should seek immediate medical attention.

Side effects of fosdenopterin include:

  • Problems with the IV line or catheter
  • Fever;
  • Vomiting, diarrhea;
  • Cold symptoms like stuffy noses, sneezing, and coughing.

There may be other side effects.For medical advice regarding side effects, call your physician. You can contact the FDA at 1-800-FDA-1088 if any side effects arise; for reporting purposes.

Similar/related drugs

Nulibry

Warnings

Please read all the instructions on the label or package of your medication. Inform your child's doctors about their medical conditions, allergies, and medications.

Before you take this drug

Your doctor will run a genetic test on your baby to determine if it has MoCD. Fosdenopterin may be given even before the results of genetic testing are known. If the test indicates that your child does not have MoCD, treatment will stop.

How to take Fosdenopterin?

Read and follow all instructions that accompany your medication.Sometimes, your doctor will change the dose for your child. Follow the instructions on the medication. Fosdenopterin can be administered using an infusion device attached to a catheter inserted into a vein. You can learn how to use the infusion pumps by yourself with the help of a healthcare provider. Keep a journal of when you give your child each dose. You may need to keep a record of the doses and vials you use. Bring this information with you every time you bring your child to the doctor. Please read and follow all instructions for use that come with your medication. If you don't understand the instructions, reach out to your physician or pharmacist for help. Fosdenopterin should be mixed with a liquid (diluent) before use. If you are using injections on your own, make sure that you know how to mix and store them. Do not mix the medicines.

Only prepare an injection when it's time to give it. You should finish each infusion within four hours of mixing the medicine and diluent. If the medicine is cloudy, has changed color, or contains particles, do not use it. For new medicine, call your pharmacist. Fosdenopterin dosage is based on body weight. If your child loses or gains weight, the dose may need to change. Fosdenopterin, unmixed, should be stored in the freezer. Each vial should be kept in the original carton to protect it from light. Mix the diluent with the medicine after it has reached room temperature. The medicine can be warmed in your hands for between 3 and 5 minutes. The medicine should not be heated. Store mixed medicines in the fridge or at room temperature. Allow the mixture to come to room temperature if it has been stored in the refrigerator. After mixing the medicine, you must inject the mixture within four hours.

Details on dosage

Adult dose for combined molybdoflavoprotein enzyme deficiency:
0.9 mg/kg IV, once daily
Comments:
The dosage is determined by the actual weight of the patient. Infuse with 1.5 mL/min using non-DEHP tubing and a filter of 0.2 microns.
Based on a well-controlled and adequate clinical study in pediatric patients, the safety and efficacy of this drug for adults with molybedenum deficiency type A (MoCD) have been determined.
Use: To reduce mortality risk in patients with moCD type A.
The usual pediatric dose for combined molybdoflavoprotein enzyme deficiency is:
Patients younger than one year: The dosage is determined by gestational age.
Preterm babies are those born at a gestational age of less than 37 weeks.
Initial Dosage: 0.04 mg/kg IV, once per day
Dose after one month: 0.7 mg/kg IV, once per day.
Dose after three months: 0.9 mg/kg IV, once per day.
Age of gestation: 37 weeks or more (term neonates).
Initial Dosage: 0,55 mg/kg intravenous once per day
Dose after one month: 0.75 mg/kg IV, once per day.
Dose after three months: 0.9 mg/kg IV, once per day.
Patients older than 1 year: 0.9 mg/kg intravenously once per day
Comments:
The dosage is determined by the actual weight of the patient; use non-DEHP tubing with a 0.2-micron filter. Volumes less than 2 mL will require a slow intravenous injection using a syringe.
If a diagnosis is suspected, genetic testing should be done to confirm it. The drug can then be discontinued if the diagnosis cannot be confirmed.
Use: To reduce mortality risk in patients with MoCD type A.

What happens if I miss the dose?

You should give the first injection as soon as possible, and then the second injection six hours later. Then you can resume your regular daily schedule. Give no more than one dose at a time.

What happens if I overdose?

Call 1-800-222-1222 for poison help or seek immediate medical attention.

What should be avoided?

Fosdenopterin can cause your child to sunburn more easily. Avoid sun exposure or tanning bed use. Wear sunglasses, protective clothing, and a hat to protect your child when outdoors. If your child is older than 6 months, you can also apply sunblock with an SPF of 30 or more.

Interaction with other drug

Fosdenopterin may be affected by other drugs, such as vitamins and herbal products. Inform your doctor of all the medicines your child is taking, including any new ones.