Emflaza (deflazacort)

The recommended dosage of deflazacort for the management of Duchenne muscular dystrophy in patients 2 years of age and older is approximately 0.9 mg/kg once daily. If the tablets are used, doses should be rounded up to the nearest 6-mg increment, using any combination of tablet strengths to achieve the required dose. If the oral suspension is used, doses should be rounded up to the nearest 0.1 mL.

When discontinuing deflazacort therapy after the drug has been given for more than a few days, dosage should be gradually tapered to minimize the potential for adrenal insufficiency.

Deflazacort is administered orally (as tablets or oral suspension) without regard to food. Do not administer the drug with grapefruit juice. The tablet and oral suspension formulations of the drug are bioequivalent.

Deflazacort tablets and oral suspension should be stored at 20-25degreesC, but may be exposed to 15-30degreesC; any unused portions of the oral suspension should be discarded 1 month after first opening the bottle.

No dosing information is available for this tab.

No dosing information is available for this tab.

The active metabolite of deflazacort (21-desacetyldeflazacort) is a substrate of cytochrome P-450 (CYP) isoenzyme 3A4. In vitro studies indicate that the active metabolite does not inhibit CYP1A2, 2C9, 2C19, or 3A4 (at concentrations up to 100 mcM), but exhibits weak (and not likely to be clinically important) inhibition of CYP2B6, 2C8, 2D6, and 3A4. In vitro studies indicate that deflazacort does not induce CYP1A2, 2B6, or 3A4 (at concentrations up to 10 mcM).

Deflazacort and its active metabolite are substrates of P-glycoprotein (P-gp), but do not inhibit P-gp in vitro.

The active metabolite of deflazacort is not a substrate of breast cancer resistance protein (BCRP); neither deflazacort nor its active metabolite inhibit BCRP in vitro.

The active metabolite of deflazacort is not a substrate or inhibitor of organic anion-transporting protein (OATP) 1B1 or 1B3, and is not an inhibitor of organic anion transporter (OAT) 1 or 3 or organic cation transporter (OCT) 2.

The active metabolite of deflazacort does not inhibit uridine diphosphate-glucuronosyltransferase (UGT) 1A1, 1A4, 1A6, 1A9, or 2B7 (at concentrations up to 100 mcM), but exhibits weak (and not likely to be clinically important) inhibition of UGT1A3 and 2B15.

There are no contraindication interactions for this drug.

There are no severe interactions for this drug.

There are no moderate interactions for this drug.

*Known hypersensitivity to deflazacort or any ingredient in the formulation.

There are no contraindication contraindications for this drug.

There are no severe contraindications for this drug.

There are no moderate contraindications for this drug.

Adverse effects reported in at least 10% of patients receiving deflazacort in clinical studies and more frequently than with placebo include Cushingoid appearance, increased weight, increased appetite, upper respiratory tract infection, cough, pollakiuria, hirsutism, central obesity, and nasopharyngitis.

There are no severe adverse reactions for this drug.

There are no less severe adverse reactions for this drug.

No enhanced messaging available.

Safety and efficacy of deflazacort have not been established in pediatric patients younger than 2 years of age.

Corticosteroids can have adverse effects on growth and development in children, particularly after long-term use. If prolonged therapy is necessary, the growth and development of children should be monitored closely.

Each mL of deflazacort oral suspension contains 10.45 mg of benzyl alcohol as a preservative. Administration of drugs preserved with benzyl alcohol has been associated with toxicity in preterm and low-birthweight infants. In these cases, large amounts (i.e., 100-400 mg/kg daily) of benzyl alcohol were administered. The lower limit for benzyl alcohol toxicity is unknown. Deflazacort oral suspension should not be used in pediatric patients younger than 2 years of age.

There are no adequate and well-controlled studies of deflazacort in pregnant women. Adverse developmental effects (e.g., cleft lip with or without cleft palate, intrauterine growth restriction, decreased birth weight) have been reported with maternal use of corticosteroids, including deflazacort, during pregnancy; in some cases, the underlying disease may have contributed to some of the observed effects.

Corticosteroids should be used during pregnancy only if the potential benefits justify the potential risks to the fetus. Some clinicians suggest that deflazacort should be avoided during the second and third trimesters because of the risk of fetal and neonatal adrenal suppression. Infants born to women who received substantial doses of corticosteroids during pregnancy should be carefully monitored for symptoms of adrenal insufficiency.

Corticosteroids are distributed into human milk and can suppress growth, interfere with endogenous corticosteroid production, or cause other adverse effects in nursing infants. The benefits of breast-feeding should be considered along with the woman's clinical need for deflazacort and any potential adverse effects on the breast-fed infant from the drug. Effects of the drug on milk production are not known.

The manufacturer makes no specific dosage recommendations for geriatric patients.

There is no experience with deflazacort in geriatric patients; Duchenne muscular dystrophy is generally a disease of children and young adults.