• Duchenne muscular dystrophy (DMD).

Contraindicated in:

• Hypersensitivity
• Active untreated infections.

Use Cautiously in:

• Hypopituitarism, primary adrenal insufficiency, congenital adrenal hyperplasia, thyroid dysfunction, or pheochromocytoma (↑ risk of endocrine abnormalities)
• HF, hypertension, recent MI, or renal impairment
• Peptic ulcer disease, diverticulitis, recent intestinal anastomoses, or ulcerative colitis (↑ risk of GI perforation)
• Myasthenia gravis (↑ risk of myopathy)
• OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk
• Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risks to infant
• Pedi: Children <2 yr (safety and effectiveness not established); oral suspension contains benzyl alcohol which can cause gasping syndrome in premature neonates and low birth weight infants.

CV: hypertension, peripheral edema, THROMBOEMBOLIC EVENTS.

Derm: acne, hirsutism, erythema, rash, skin striae, TOXIC EPIDERMAL NECROLYSIS.

EENT: cataracts, epistaxis, glaucoma, rhinorrhea.

Endo: Cushingoid appearance, ↓ bone density, ↓ growth (children), adrenal suppression, hyperglycemia.

GI: ↑ appetite, abdominal pain, constipation, nausea, PEPTIC ULCERATION.

Metab: ↑ weight.

MS: avascular necrosis of joints, back pain, myopathy.

Neuro: headache, depression, euphoria, insomnia, irritability, mood swings.

Resp: cough.
Misc: fever, (INCLUDING ANAPHYLAXIS)HYPERSENSITIVITY REACTIONS , INFECTION, KAPOSI'S SARCOMA.

Drug-Drug:

• May ↑ requirement for insulins or oral hypoglycemic agents.
• May ↓ antibody response to and ↑ the risk of adverse reactions from live virus vaccines.
• Concurrent use with neuromuscular blocking agents may ↑ risk of acute myopathy.
• Moderate CYP3A4 inhibitors and strong CYP3A4 inhibitors, including clarithromycin, fluconazole, diltiazem, or verapamil may ↑ levels of active metabolite and risk of toxicity; ↓ deflazacort dose.
• Moderate CYP3A4 inducers and strong CYP3A4 inducers, including rifampin, efavirenz, carbamazepine, or phenytoin may ↓ levels of active metabolite and therapeutic effect; avoid concurrent use.

Drug-Food:

• Grapefruit juice may ↑ levels of active metabolite and risk of toxicity; ↓ deflazacort dose.

• Oral suspension: 22.75 mg/mL;
• Tablets: 6 mg; 18 mg; 30 mg; 36 mg;

• PO (Adults and Children ≥2 yr): 0.9 mg/kg once daily. Concurrent use of moderate or strong CYP3A4 inhibitors: Give 1/3 the recommended dose.

Emflaza

• Exerts anti-inflammatory and immunosuppressive effects. Mechanism for therapeutic effect in DMD not fully known.

• Improved muscle strength.

Therapeutic Classification: anti-inflammatories, Immunosuppressant agents, n/a

Pharmacologic Classification: corticosteroids

Absorption: Rapidly absorbed.

Distribution: Unknown.

Metabolism/Excretion: Parent drug (prodrug) is rapidly metabolized by esterases to active metabolite (21-desDFZ), which is then further metabolized by CYP3A4 to inactive metabolites. Primarily excreted in urine.

Half-life: Unknown.

(plasma concentrations)

• Instruct patient and parents to take as directed. Do not stop taking without consulting health care professional; must be discontinued gradually. Advise patient to read Instructions for Use before starting therapy and with each Rx refill in case of changes.
• Advise patient to avoid live virus vaccines during therapy.
• Instruct patient to notify health care professional if signs and symptoms of infection, ↑ BP or fluid retention, behavioral or mood disturbances (manic or depressive symptoms), or rash occur.
• Inform patient of need for periodic bone mineral density and ophthalmalogic exams.
• Advise female patients to notify health care professional if pregnancy is planned or suspected or if breast feeding.

de-FLAZE-a-kort