deflazacort (Rx)

Brand and Other Names:Emflaza
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

tablet

  • 6mg
  • 18mg
  • 30mg
  • 36mg

oral suspension

  • 22.75mg/mL

Duchenne Muscular Dystrophy

Indicated for Duchenne muscular dystrophy (DMD)

0.9 mg/kg/day PO qDay

If tablets are used, round up to the nearest possible dose; may use any combination of tablet strengths to achieve calculated dose

If the oral suspension is used, round up to the nearest tenth of a milliliter (mL)

Also see Administration

Dosage Modifications

Coadministration with CYP3A4 inhibitors

  • Moderate or strong CYP3A4 inhibitors: Give one third the recommended deflazacort dose
  • Example: A 36-mg/day dose would be reduced to 12 mg/day when used with moderate or strong CYP3A4 inhibitors

Coadministration with CYP3A4 inducers

  • Moderate or strong CYP3A4 inducers: Avoid use

Renal impairment

  • Mild, moderate, or severe: No dose adjustment required

Hepatic impairment

  • Mild or moderate: No dose adjustment required
  • Severe: Not studied

Dosage Forms & Strengths

tablet

  • 6mg
  • 18mg
  • 30mg
  • 36mg

oral suspension

  • 22.75mg/mL

Duchenne Muscular Dystrophy

Indicated for Duchenne muscular dystrophy (DMD)

<5 years: Safety and efficacy not established

≥5 years: 0.9 mg/kg/day PO qDay

If tablets are used, round up to the nearest possible dose; may use any combination of tablet strengths to achieve calculated dose

If the oral suspension is used, round up to the nearest tenth of a milliliter (mL)

Also see Administration

Dosage Modifications

Coadministration with CYP3A4 inhibitors

  • Moderate or strong CYP3A4 inhibitors: Give one third the recommended deflazacort dose
  • Example: A 36-mg/day dose would be reduced to 12 mg/day when used with moderate or strong CYP3A4 inhibitors

Coadministration with CYP3A4 inducers

  • Moderate or strong CYP3A4 inducers: Avoid use

Renal impairment

  • Mild, moderate, or severe: No dose adjustment required

Hepatic impairment

  • Mild or moderate: No dose adjustment required
  • Severe: Not studied
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Interactions

Interaction Checker

and deflazacort

No Results

     activity indicator 
    No Interactions Found
    Interactions Found

    Contraindicated

      Serious - Use Alternative

        Significant - Monitor Closely

          Minor

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            Adverse Effects

            >10%

            Cushingoid appearance (33%)

            Weight increased (20%)

            Increased appetite (14%)

            Upper respiratory tract infection (12%)

            Cough (12%)

            Pollakiuria (12%)

            1-10%

            Nasopharyngitis (10%)

            Hirsutism (10%)

            Central obesity (10%)

            Erythema (8%)

            Irritability (8%)

            Rhinorrhea (8%)

            Abdominal discomfort (6%)

            Eye disorders: Lacrimation increased (1-4%)

            Gastrointestinal disorders: Dyspepsia, nausea, gastrointestinal disorder (1-4%)

            General disorders and administration site conditions: Thirst (1-4%)

            Infections: Hordeolum, impetigo, influenza, otitis externa, pharyngitis, tooth abscess, urinary tract infection, viral infection (1-4%)

            Injury, poisoning and procedural complications: Back injury, contusion, face injury, fibula fracture, greenstick fracture, heat exhaustion (1-4%)

            Investigations: Glucose urine present, heart rate irregular (1-4%)

            Musculoskeletal and connective tissue disorders: Back pain, muscle spasms, myalgia, neck mass, neck pain, pain in extremity (1-4%)

            Nervous system disorders: Dizziness, psychomotor hyperactivity (1-4%)

            Psychiatric disorders: Affect lability, aggression, depression, emotional disorder, middle insomnia, mood altered, mood swings, sleep disorder (1-4%)

            Renal and urinary disorders: Chromaturia, dysuria, hypertonic bladder (1-4%)

            Reproductive system and breast disorders: Testicular pain (1-4%)

            Respiratory, thoracic, and mediastinal disorders: Hypoventilation, rhinorrhea (1-4%)

            Skin and subcutaneous tissue disorders: Acne, alopecia, dermatitis acneiform (1-4%)

            Vascular disorders: Hot flush (1-4%)

            Postmarketing Reports

            Blood and lymphatic system disorders: Leukocytosis

            Cardiac disorder: Heart failure

            Eye disorders: Chorioretinopathy, corneal or scleral thinning

            Gastrointestinal disorders: Acute pancreatitis (especially in children), hemorrhage, peptic ulceration, perforation of peptic ulcer

            General disorders and administration site conditions: Edema, impaired healing

            Immune system disorders: Hypersensitivity including anaphylaxis

            Metabolism and nutrition disorders: Impaired carbohydrate tolerance with increased requirement for antidiabetic therapy, negative protein and calcium balance, potassium loss and hypokalemic alkalosis when coadministered with beta2-agonist and xanthines

            Musculoskeletal and connective tissue disorders: Avascular necrosis, muscle wasting, negative nitrogen balance, tendonitis and tendon rupture when coadministered with quinolones, vertebral and long bone fractures

            Nervous system disorders: Aggravation of epilepsy, increased intracranial pressure with papilledema in children (pseudotumor cerebri) usually after treatment withdrawal, vertigo

            Psychiatric disorders: Anxiety, cognitive dysfunction including confusion and amnesia, delusions, hallucinations, mania, suicidal thoughts

            Skin and subcutaneous tissue disorders: Toxic epidermal necrolysis

            Vascular disorders: Thromboembolism, in particular in patients with underlying conditions associated with increased thrombotic tendency, benign intracranial hypertension

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            Warnings

            Contraindications

            Known hypersensitivity, including anaphylaxis (see Cautions)

            Cautions

            Known hypersensitivity; instances of hypersensitivity, including anaphylaxis, have occurred with corticosteroid therapy

            Alterations in endocrine function

            • Corticosteroids can cause serious and life-threatening alterations in endocrine function, especially with long-term use
            • Corticosteroids produce reversible hypothalamic-pituitary-adrenal (HPA) axis suppression, with the potential for the development of secondary adrenal insufficiency after withdrawal of corticosteroid treatment
            • Acute adrenal insufficiency can occur if corticosteroids are withdrawn abruptly, and can be fatal; the risk is reduced by gradually tapering the dose
            • Adrenal insufficiency may persist, however, for months after discontinuation of prolonged therapy; therefore, in any situation of stress occurring during that period of discontinuation, corticosteroid therapy should be reinstituted
            • Cushing syndrome (hypercortisolism) occurs with prolonged exogenous corticosteroid exposure; symptoms include hypertension, truncal obesity and thinning of the limbs, purple striae, facial rounding, facial plethora, muscle weakness, easy and frequent bruising with thin fragile skin, posterior neck fat deposition, osteopenia, acne, amenorrhea, hirsutism and psychiatric abnormalities
            • Corticosteroid may increase blood glucose, worsen pre-existing diabetes, predispose those on long-term therapy to diabetes mellitus, and may reduce the effect of antidiabetic drugs; monitor blood glucose at regular intervals and if needed, antidiabetic treatment should be initiated
            • Corticosteroid metabolic clearance is decreased in patients with hypothyroidism and increased in patients with hyperthyroidism Pheochromocytoma crisis, which can be fatal, has been reported after administration of systemic corticosteroids

            Immunosuppression and increased risk of infection

            • Increased risk of new, exacerbation, dissemination, or reactivation of latent infections, which can be severe and at times fatal; signs and symptoms of infection may be masked
            • Monitor for development of infection and consider withdrawal of corticosteroids or reduction of the dose of corticosteroids as needed
            • Varicella zoster virus exposure: Prophylaxis with varicella zoster immune globulin (VZIG) may be indicated; if chickenpox/varicella zoster develops, treatment with antiviral agents may be considered
            • Measles exposure: Prophylaxis with immunoglobulin (IG) may be indicated
            • Hepatitis B virus reactivation can occur in patients who are hepatitis B carriers undergoing treatment with immunosuppressive drugs including corticosteroids; reactivation can also occur with resolved hepatitis B infection
            • Fungal infections
              • If a systemic fungal infection develops, withdrawal of corticosteroids or dose reduction is recommended
              • Amebiasis: Corticosteroids may activate latent amebiasis; rule out latent or active amebiasis before initiating corticosteroids in any patient who has spent time in the tropics or other patients with unexplained diarrhea
              • Strongyloides (threadworm) infestation: Corticosteroid-induced immunosuppression may lead to Strongyloides hyperinfection and dissemination of widespread larval migration, often accompanied by severe enterocolitis and potentially fatal gram-negative septicemia; withdrawal of corticosteroids or dose reduction is recommended

            Alterations in cardiovascular/renal function: Monitor for elevated blood pressure and sodium levels, and for decreased potassium levels

            Gastrointestinal perforation: Increased risk with certain GI disorders (eg, active/latent peptic ulcers, diverticulitis, fresh intestinal anastomoses, ulcerative colitis); signs and symptoms may be masked

            Behavioral and mood disturbances: May include euphoria, insomnia, mood swings, personality changes, severe depression, and psychosis

            Effects on bones: Monitor for decreases in bone mineral density with long-term use

            Ophthalmic effects: May include cataracts, infections, and glaucoma; monitor intraocular pressure if used for >6 weeks

            Serious rashes: Discontinue at the first sign of rash, unless the rash is clearly not drug related; toxic epidermal necrolysis reported within 8 weeks of starting treatment with deflazacort

            Myopathy may occur with concomitant neuromuscular blocking agents or disorders of neuromuscular transmission (eg, myasthenia gravis)

            Kaposi sarcoma reported in patients receiving corticosteroid therapy, most often for chronic conditions; discontinuation of corticosteroids may result in clinical improvement

            Oral suspension contains benzyl alcohol and is not approved for use in children aged <5 yr; serious and fatal adverse reactions including (gasping syndrome) can occur in neonates and low-birth-weight infants treated with benzyl alcohol-preserved drugs

            Observational studies have shown an increased risk of thromboembolism (including VTE), particularly with higher cumulative doses of corticosteroids

            Drug interaction overview

            • The active metabolite of deflazacort, 21-desDFZ, is a substrate of CYP3A4; decrease deflazacort dose by one third if coadministered with moderate or strong CYP3A4 inhibitors (see Dosage Modification)
            • Coadministration of deflazacort with moderate or strong CYP3A4 inducers significantly decreases exposure of the active metabolite; avoid coadministration
            • Coadministration with neuromuscular blocking drugs (eg, pancuronium) may increase risk of acute myopathy
            • Caution if coadministered with other drugs that decrease serum potassium; monitor serum potassium or use an alternant drug
            • Immunosuppressive doses of corticosteroids may interfere with efficacy of live or live-attenuated vaccines
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            Pregnancy

            Pregnancy

            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

            Lactation

            Systemically administered corticosteroids appear in human milk and could suppress growth, interfere with endogenous corticosteroid production, or cause other untoward effects

            Consider the developmental and health benefits of breastfeeding along with the mother’s clinical need for the drug, and any potential adverse effects on the breastfed infant from the drug or from the underlying maternal condition

            Pregnancy Categories

            A:Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

            B:May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

            C:Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

            D:Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

            X:Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

            NA:Information not available.

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            Pharmacology

            Mechanism of Action

            Corticosteroid prodrug, whose active metabolite, 21-desDFZ, acts through the glucocorticoid receptor to exert anti-inflammatory and immunosuppressive effects

            The precise mechanism by which deflazacort exerts its therapeutic effects in patients with DMD is unknown

            Absorption

            Peak plasma time: ~1 hr (fasting); ~2 hr (high-fat meal)

            Distribution

            Protein bound: 40%

            Metabolism

            Rapidly converted to the active metabolite 21-desDFZ by esterases after oral administration

            21-desDFZ is further metabolized by CYP3A4 to several inactive metabolites

            Elimination

            Excretion: Predominantly urinary (~68% [active metabolite accounts for 18% of this]); elimination is nearly complete by 24 hr post dose

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            Administration

            Oral Administration

            May take with or without food

            Tablets

            • Round up to the nearest possible dose; may use any combination of tablet strengths to achieve calculated dose
            • Tablets can be administered whole or crushed and taken immediately after mixing with applesauce

            Oral suspension

            • Round up to the nearest tenth of a milliliter (mL)
            • Shake suspension well before measuring dose
            • Use only the oral dispenser provided with the product to measure the dose
            • After withdrawing the appropriate dose into the oral dispenser, slowly add the suspension into 3-4 ounces of juice or milk and mix well
            • The dose should then be administered immediately after mixing
            • Do not mix or administer with grapefruit juice

            Discontinuation

            • Dosage must be decreased gradually if the drug has been administered for more than a few days

            Storage

            Store at controlled room temperature (20-25°C [68-77°F]); excursion permitted between 15-30°C (59-86°F)

            Oral suspension: Discard any unused oral suspension remaining after 1 month of first opening the bottle

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            Formulary

            FormularyPatient Discounts

            Adding plans allows you to compare formulary status to other drugs in the same class.

            To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.

            Adding plans allows you to:

            • View the formulary and any restrictions for each plan.
            • Manage and view all your plans together – even plans in different states.
            • Compare formulary status to other drugs in the same class.
            • Access your plan list on any device – mobile or desktop.

            The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

            Tier Description
            1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
            2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
            3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
            4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            NC NOT COVERED – Drugs that are not covered by the plan.
            Code Definition
            PA Prior Authorization
            Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
            QL Quantity Limits
            Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
            ST Step Therapy
            Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
            OR Other Restrictions
            Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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