Generic drug: deflazacort
Brand name: Emflaza
What is Emflaza, and how does it work?
Emflaza (deflazacort) is a prescription medicine used to treat the symptoms of Duchenne Muscular Dystrophy. Emflaza may be used alone or with other medications.
Emflaza belongs to a class of drugs called Corticosteroids.
It is not known if Emflaza is safe and effective in children younger than 2 years of age.
What are the side effects of Emflaza?
Emflaza may cause serious side effects including:
- hives,
- difficulty breathing,
- swelling of your face, lips, tongue, or throat,
- mood changes,
- eye pain,
- excess thirst,
- increased appetite,
- increased urination,
- tiredness,
- blurry vision,
- fever,
- chills,
- cough,
- sore throat,
- little or no urination,
- slowed growth in children,
- swelling of ankles or feet,
- trouble sleeping, and
- skeletal weakness (if used for an extended time)
Get medical help right away, if you have any of the symptoms listed above.
The most common side effects of Emflaza include:
- nausea,
- indigestion, and
- weight gain
Tell the doctor if you have any side effect that bothers you or that does not go away.
These are not all the possible side effects of Emflaza. For more information, ask your doctor or pharmacist.
Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
What is the dosage for Emflaza?
Assessments Prior To First Dose Of Emflaza
- Administer all immunizations according to immunization guidelines prior to starting
Emflaza. Administer live-attenuated or live vaccines at least 4 to 6 weeks prior to starting
Emflaza.
Dosing Information
- The recommended oral dosage of Emflaza is approximately 0.9 mg/kg/day once daily. If tablets are used, round up to the nearest possible dose. Any combination of the four
Emflaza tablet strengths can be used to achieve this dose. If the oral suspension is used, round up to the nearest tenth of a milliliter (mL).
Discontinuation
- Dosage of Emflaza must be decreased gradually if the drug has been
administered for more than a few days.
Important Preparation And Administration Instructions
- Emflaza Tablets and Oral Suspension can be taken with or without food. Do not administer
Emflaza with grapefruit juice.
Emflaza Tablets
- Emflaza Tablets can be administered whole or crushed and taken immediately after mixing with applesauce.
Emflaza Oral Suspension
- Shake Emflaza Oral Suspension well before administration.
- Use only the oral dispenser provided with the product. After withdrawing the appropriate dose into the oral dispenser, slowly add the
Emflaza Oral Suspension into 3 to 4 ounces of juice (except grapefruit juice) or milk and mix well. The dose should then be administered immediately. - Discard any unused Emflaza Oral Suspension remaining after 1 month of first opening the bottle.
Dosage Modification For Use With CYP3A4 Inhibitors And Inducers
CYP3A4 Inhibitors
- Give one third of the recommended dosage when Emflaza is administered
with moderate or strong CYP3A4 inhibitors. For example, a 36 mg per day dose
would be reduced to a 12 mg per day dose when used with moderate or strong
CYP3A4 inhibitors.
CYP3A4 Inducers
- Avoid use with moderate or strong CYP3A4 inducers with Emflaza.
What drugs interact with Emflaza?
CYP3A4 Inhibitors And Inducers
Moderate Or Strong CYP3A4 Inhibitors
- The active metabolite of deflazacort, 21-desDFZ, is a substrate of
CYP3A4. - Co-administration of deflazacort with clarithromycin, a strong CYP3A4 inhibitor, increased total exposure to 21-desDFZ by about 3-fold.
- Therefore, give one third the recommended dosage of Emflaza when moderate or strong CYP3A4 inhibitors (e.g., clarithromycin, fluconazole, diltiazem, verapamil, grapefruit juice) are used concomitantly with
Emflaza.
Moderate Or Strong CYP3A4 Inducers
- Co-administration of deflazacort with rifampin, a strong CYP3A4 inducer, significantly decreased the exposure of 21-desDFZ. Avoid concomitant use of strong (e.g., efavirenz) or moderate (e.g., carbamazepine, phenytoin) CYP3A4 inducers with
Emflaza.
Neuromuscular Blockers
- Patients receiving corticosteroids, including Emflaza, and concomitant
therapy with neuromuscular blocking drugs (e.g., pancuronium) may be at
increased risk of developing an acute myopathy.
Is Emflaza safe to use while pregnant or breastfeeding?
- Corticosteroids should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus. Infants born to mothers who have received substantial doses of corticosteroids during pregnancy should be carefully observed for signs of hypoadrenalism.
- There are no adequate and well-controlled studies with Emflaza in pregnant women to inform drug-associated risks.
- Systemically administered corticosteroids appear in human milk and could suppress growth, interfere with endogenous corticosteroid production, or cause other untoward effects.
- The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for
Emflaza and any potential adverse effects on the breastfed infant from
Emflaza. There are no data on the effects on milk production.