Orfadin Oral
Generic Name: NITISINONE - ORALPronounced: (nih-TIS-ih-known)
Orfadin Oral Uses
This medication is used to treat a certain inherited disorder (hereditary tyrosinemia type 1, also known as HT-1). Usually seen in children, this condition is caused by a shortage of a certain natural substance that is needed to break down a nutrient (tyrosine) found in food. This effect causes a build-up of too much tyrosine and related substances in the liver. This build-up can lead to very serious (often fatal) liver damage, as well as possible damage to the kidneys and nervous system. Nitisinone works by helping to prevent the formation and build-up of several toxic substances that cause damage to the liver, kidneys, and nervous system. This drug must be used along with a diet low in protein, tyrosine, and phenylalanine.
How To Use Orfadin Oral
This medication should be taken by mouth on an empty stomach (at least 1 hour before a meal) twice a day or as directed by the child's doctor.
For children who are unable to swallow capsules, the capsules may be opened and the contents mixed in a small amount of water, formula, or applesauce immediately before use. Make sure the child swallows the entire dose.
Dosage is based on the patients weight, medical condition, and response to treatment. The manufacturer recommends a daily maximum dose of 2 milligrams per kilogram of body weight.
This medication should be used regularly to get the most benefit from it. Patients should take it at the same times each day.
Nitisinone can increase tyrosine levels, an effect that can cause eye problems. Before using this medication, patients should have an eye test (slit-lamp examination) so that later the doctor can see whether there have been changes. (See also Side Effects and Precautions sections.) It is very important that patients taking this medication follow a special diet low in protein, tyrosine, and phenylalanine. Consult your doctor, pharmacist, or dietician for more details. (See also Drug Interactions.) Tell the doctor if the patients condition worsens after 3 weeks.
