Deflazacort

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling. [DSC] = Discontinued product

Suspension, Oral:

Emflaza: 22.75 mg/mL (13 mL) [contains polysorbate 80]

Emflaza: 22.75 mg/mL (13 mL [DSC]) [contains polysorbate 80, sorbitol]

Tablet, Oral:

Emflaza: 6 mg, 18 mg, 30 mg, 36 mg [contains corn starch]

Pharmacology

Mechanism of Action

Deflazacort is a corticosteroid prodrug; its active metabolite, 21-desDFZ, acts on the glucocorticoid receptor to exert anti-inflammatory and immunosuppressive effects. The precise mechanism by which deflazacort exerts its therapeutic effects in patients with Duchenne muscular dystrophy is unknown.

Pharmacokinetics/Pharmacodynamics

Metabolism

Rapidly converted by esterases to 21-desDFZ (active metabolite); 21-desDFZ metabolized by CYP3A4 to several metabolites (inactive)

Excretion

Urine (~68%; 18% as 21-desDFZ)

Time to Peak

1 hour (range: 0.25 to 2 hours); delayed by 1 hour with a high-fat meal

Protein Binding

~40%

Use: Labeled Indications

Duchenne muscular dystrophy: Treatment of Duchenne muscular dystrophy (DMD) in patients ≥2 years of age.

Contraindications

Hypersensitivity to deflazacort or any component of the formulation.

Dosage and Administration

Dosing: Adult

Duchenne muscular dystrophy: Oral: Usual dose: 0.9 mg/kg once daily. Note: Round up to nearest possible dose when using tablets; round up to nearest tenth of a mL when using suspension.

Concomitant moderate or strong CYP3A4 Inhibitors (eg, clarithromycin, fluconazole, diltiazem, verapamil): Reduce deflazacort dose to ¹/₃ of usual dose.

Dosing: Geriatric

Refer to adult dosing.

Dosing: Pediatric

Note: Vaccinate patients based on current immunization schedules prior to therapy initiation; any live-attenuated or live vaccines should be administered at least 4 to 6 weeks prior to starting deflazacort.

Duchenne muscular dystrophy (DMD): Children ≥2 years and Adolescents: Oral: ~0.9 mg/kg/dose once daily; doses should be rounded based on product formulation: Oral suspension: Round dose up to nearest 0.1 mL (tenth of a mL), Tablets: Round up to the nearest possible dose based on tablet strengths using any combination of available tablet strengths. Note: DMD primarily affects males, and rarely females; therefore, clinical trial experience is limited to the male population.

Discontinuation of therapy: It is necessary to gradually decrease deflazacort if administered for more than few days.

Dosing adjustment for concomitant therapy:

Moderate or strong CYP3A4 inhibitors (eg, clarithromycin, fluconazole, diltiazem, verapamil): Children ≥2 years and Adolescents: Reduce deflazacort dose to ¹/₃ of the usual dose.

Moderate or strong CYP3A4 inducer:Children ≥2 years and Adolescents: Avoid use.

Administration

Oral: Administer with or without food.

Tablets: Administer whole or may crush and mix with applesauce (administer applesauce mixture immediately).

Suspension: Shake well; measure prescribed dose with provided dispenser, mix thoroughly with 3 to 4 ounces of juice or milk and administer immediately. Do not mix or administer with grapefruit juice.

Dietary Considerations

Grapefruit juice increases the total exposure to the active metabolite of deflazacort.

Storage

Store at 20°C to 25°C (68°F to 77°F); excursions permitted between 15°C to 30°C (59°F to 86°F). Discard unused suspension 1 month after opening the bottle.

Drug Interactions

Acetylcholinesterase Inhibitors: Corticosteroids (Systemic) may enhance the adverse/toxic effect of Acetylcholinesterase Inhibitors. Increased muscular weakness may occur. Monitor therapy

Aldesleukin: Corticosteroids may diminish the antineoplastic effect of Aldesleukin. Avoid combination

Amphotericin B: Corticosteroids (Systemic) may enhance the hypokalemic effect of Amphotericin B. Monitor therapy

Androgens: Corticosteroids (Systemic) may enhance the fluid-retaining effect of Androgens. Monitor therapy

Antacids: May decrease the bioavailability of Corticosteroids (Oral). Management: Consider separating doses by 2 or more hours. Budesonide enteric coated tablets could dissolve prematurely if given with drugs that lower gastric acid, with unknown impact on budesonide therapeutic effects. Consider therapy modification

Antidiabetic Agents: Hyperglycemia-Associated Agents may diminish the therapeutic effect of Antidiabetic Agents. Monitor therapy

Aprepitant: May increase the serum concentration of Corticosteroids (Systemic). Management: No dose adjustment is needed for single 40 mg aprepitant doses. For other regimens, reduce oral dexamethasone or methylprednisolone doses by 50%, and IV methylprednisolone doses by 25%. Antiemetic regimens containing dexamethasone reflect this adjustment. Consider therapy modification

Axicabtagene Ciloleucel: Corticosteroids (Systemic) may diminish the therapeutic effect of Axicabtagene Ciloleucel. Management: Avoid use of corticosteroids as premedication before axicabtagene ciloleucel. Corticosteroids may, however, be required for treatment of cytokine release syndrome or neurologic toxicity. Consider therapy modification

Baricitinib: Immunosuppressants may enhance the immunosuppressive effect of Baricitinib. Management: Use of baricitinib in combination with potent immunosuppressants such as azathioprine or cyclosporine is not recommended. Concurrent use with antirheumatic doses of methotrexate or nonbiologic disease modifying antirheumatic drugs (DMARDs) is permitted. Consider therapy modification

BCG (Intravesical): Immunosuppressants may diminish the therapeutic effect of BCG (Intravesical). Avoid combination

Bile Acid Sequestrants: May decrease the absorption of Corticosteroids (Oral). Monitor therapy

Calcitriol (Systemic): Corticosteroids (Systemic) may diminish the therapeutic effect of Calcitriol (Systemic). Monitor therapy

Cladribine: May enhance the immunosuppressive effect of Immunosuppressants. Avoid combination

Clofazimine: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Monitor therapy

Coccidioides immitis Skin Test: Immunosuppressants may diminish the diagnostic effect of Coccidioides immitis Skin Test. Monitor therapy

Conivaptan: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Avoid combination

Corticorelin: Corticosteroids may diminish the therapeutic effect of Corticorelin. Specifically, the plasma ACTH response to corticorelin may be blunted by recent or current corticosteroid therapy. Monitor therapy

Cosyntropin: Corticosteroids (Systemic) may diminish the diagnostic effect of Cosyntropin. Monitor therapy

CYP3A4 Inducers (Moderate): May decrease serum concentrations of the active metabolite(s) of Deflazacort. Avoid combination

CYP3A4 Inducers (Strong): May decrease serum concentrations of the active metabolite(s) of Deflazacort. Avoid combination

CYP3A4 Inhibitors (Moderate): May increase serum concentrations of the active metabolite(s) of Deflazacort. Management: Administer one third of the recommended deflazacort dose when used together with a strong or moderate CYP3A4 inhibitor. Consider therapy modification

CYP3A4 Inhibitors (Strong): May increase serum concentrations of the active metabolite(s) of Deflazacort. Management: Administer one third of the recommended deflazacort dose when used together with a strong or moderate CYP3A4 inhibitor. Consider therapy modification

Deferasirox: May decrease the serum concentration of CYP3A4 Substrates (High risk with Inducers). Monitor therapy

Deferasirox: Corticosteroids (Systemic) may enhance the adverse/toxic effect of Deferasirox. Specifically, the risk for GI ulceration/irritation or GI bleeding may be increased. Monitor therapy

Deferasirox: Corticosteroids may enhance the adverse/toxic effect of Deferasirox. Specifically, the risk for GI ulceration/irritation or GI bleeding may be increased. Monitor therapy

Denosumab: May enhance the adverse/toxic effect of Immunosuppressants. Specifically, the risk for serious infections may be increased. Monitor therapy

Desirudin: Corticosteroids (Systemic) may enhance the anticoagulant effect of Desirudin. More specifically, corticosteroids may increase hemorrhagic risk during desirudin treatment. Management: Discontinue treatment with systemic corticosteroids prior to desirudin initiation. If concomitant use cannot be avoided, monitor patients receiving these combinations closely for clinical and laboratory evidence of excessive anticoagulation. Consider therapy modification

Desmopressin: Corticosteroids (Systemic) may enhance the hyponatremic effect of Desmopressin. Avoid combination

Echinacea: May diminish the therapeutic effect of Immunosuppressants. Consider therapy modification

Erdafitinib: May decrease the serum concentration of CYP3A4 Substrates (High risk with Inducers). Monitor therapy

Erdafitinib: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Monitor therapy

Estrogen Derivatives: May increase the serum concentration of Corticosteroids (Systemic). Monitor therapy

Fexinidazole [INT]: Corticosteroids (Systemic) may enhance the arrhythmogenic effect of Fexinidazole [INT]. Avoid combination

Fingolimod: Immunosuppressants may enhance the immunosuppressive effect of Fingolimod. Management: Avoid the concomitant use of fingolimod and other immunosuppressants when possible. If combined, monitor patients closely for additive immunosuppressant effects (eg, infections). Consider therapy modification

Fosaprepitant: May increase the serum concentration of Corticosteroids (Systemic). The active metabolite aprepitant is likely responsible for this effect. Consider therapy modification

Fusidic Acid (Systemic): May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Avoid combination

Grapefruit Juice: May increase the serum concentration of Deflazacort. Avoid combination

Hyaluronidase: Corticosteroids may diminish the therapeutic effect of Hyaluronidase. Management: Patients receiving corticosteroids (particularly at larger doses) may not experience the desired clinical response to standard doses of hyaluronidase. Larger doses of hyaluronidase may be required. Consider therapy modification

Idelalisib: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Avoid combination

Indacaterol: May enhance the hypokalemic effect of Corticosteroids (Systemic). Monitor therapy

Indium 111 Capromab Pendetide: Corticosteroids (Systemic) may diminish the diagnostic effect of Indium 111 Capromab Pendetide. Avoid combination

Isoniazid: Corticosteroids (Systemic) may decrease the serum concentration of Isoniazid. Monitor therapy

Ivosidenib: May decrease the serum concentration of CYP3A4 Substrates (High risk with Inducers). Monitor therapy

Larotrectinib: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Monitor therapy

Leflunomide: Immunosuppressants may enhance the adverse/toxic effect of Leflunomide. Specifically, the risk for hematologic toxicity such as pancytopenia, agranulocytosis, and/or thrombocytopenia may be increased. Management: Consider not using a leflunomide loading dose in patients receiving other immunosuppressants. Patients receiving both leflunomide and another immunosuppressant should be monitored for bone marrow suppression at least monthly. Consider therapy modification

Loop Diuretics: Corticosteroids (Systemic) may enhance the hypokalemic effect of Loop Diuretics. Monitor therapy

Macimorelin: Corticosteroids (Systemic) may diminish the diagnostic effect of Macimorelin. Avoid combination

Mifamurtide: Corticosteroids (Systemic) may diminish the therapeutic effect of Mifamurtide. Avoid combination

MiFEPRIStone: May diminish the therapeutic effect of Corticosteroids (Systemic). MiFEPRIStone may increase the serum concentration of Corticosteroids (Systemic). Management: Avoid mifepristone in patients who require long-term corticosteroid treatment of serious illnesses or conditions (e.g., for immunosuppression following transplantation). Corticosteroid effects may be reduced by mifepristone treatment. Avoid combination

Natalizumab: Immunosuppressants may enhance the adverse/toxic effect of Natalizumab. Specifically, the risk of concurrent infection may be increased. Avoid combination

Neuromuscular-Blocking Agents (Nondepolarizing): May enhance the adverse neuromuscular effect of Corticosteroids (Systemic). Increased muscle weakness, possibly progressing to polyneuropathies and myopathies, may occur. Consider therapy modification

Nicorandil: Corticosteroids (Systemic) may enhance the adverse/toxic effect of Nicorandil. Gastrointestinal perforation has been reported in association with this combination. Monitor therapy

Nivolumab: Immunosuppressants may diminish the therapeutic effect of Nivolumab. Consider therapy modification

Nonsteroidal Anti-Inflammatory Agents (COX-2 Selective): Corticosteroids (Systemic) may enhance the adverse/toxic effect of Nonsteroidal Anti-Inflammatory Agents (COX-2 Selective). Monitor therapy

Nonsteroidal Anti-Inflammatory Agents (Nonselective): Corticosteroids (Systemic) may enhance the adverse/toxic effect of Nonsteroidal Anti-Inflammatory Agents (Nonselective). Monitor therapy

Ocrelizumab: May enhance the immunosuppressive effect of Immunosuppressants. Monitor therapy

Palbociclib: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Monitor therapy

Pidotimod: Immunosuppressants may diminish the therapeutic effect of Pidotimod. Monitor therapy

Pimecrolimus: May enhance the adverse/toxic effect of Immunosuppressants. Avoid combination

Quinolones: Corticosteroids (Systemic) may enhance the adverse/toxic effect of Quinolones. Specifically, the risk of tendonitis and tendon rupture may be increased. Monitor therapy

Ritodrine: Corticosteroids may enhance the adverse/toxic effect of Ritodrine. Monitor therapy

Roflumilast: May enhance the immunosuppressive effect of Immunosuppressants. Consider therapy modification

Salicylates: May enhance the adverse/toxic effect of Corticosteroids (Systemic). These specifically include gastrointestinal ulceration and bleeding. Corticosteroids (Systemic) may decrease the serum concentration of Salicylates. Withdrawal of corticosteroids may result in salicylate toxicity. Monitor therapy

Sargramostim: Corticosteroids (Systemic) may enhance the therapeutic effect of Sargramostim. Specifically, corticosteroids may enhance the myeloproliferative effects of sargramostim. Monitor therapy

Sarilumab: May decrease the serum concentration of CYP3A4 Substrates (High risk with Inducers). Monitor therapy

Siltuximab: May decrease the serum concentration of CYP3A4 Substrates (High risk with Inducers). Monitor therapy

Simeprevir: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Monitor therapy

Siponimod: Immunosuppressants may enhance the immunosuppressive effect of Siponimod. Monitor therapy

Sipuleucel-T: Immunosuppressants may diminish the therapeutic effect of Sipuleucel-T. Management: Evaluate patients to see if it is medically appropriate to reduce or discontinue therapy with immunosuppressants prior to initiating sipuleucel-T therapy. Consider therapy modification

Somatropin: Corticosteroids (Systemic) may diminish the therapeutic effect of Somatropin. Monitor therapy

Stiripentol: May increase the serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Management: Use of stiripentol with CYP3A4 substrates that are considered to have a narrow therapeutic index should be avoided due to the increased risk for adverse effects and toxicity. Any CYP3A4 substrate used with stiripentol requires closer monitoring. Consider therapy modification

Tacrolimus (Systemic): Corticosteroids (Systemic) may decrease the serum concentration of Tacrolimus (Systemic). Conversely, when discontinuing corticosteroid therapy, tacrolimus concentrations may increase. Monitor therapy

Tacrolimus (Topical): May enhance the adverse/toxic effect of Immunosuppressants. Avoid combination

Tertomotide: Immunosuppressants may diminish the therapeutic effect of Tertomotide. Monitor therapy

Thiazide and Thiazide-Like Diuretics: Corticosteroids (Systemic) may enhance the hypokalemic effect of Thiazide and Thiazide-Like Diuretics. Monitor therapy

Tisagenlecleucel: Corticosteroids (Systemic) may diminish the therapeutic effect of Tisagenlecleucel. Management: Avoid use of corticosteroids as premedication or at any time during treatment with tisagenlecleucel, except in the case of life-threatening emergency (such as resistant cytokine release syndrome). Consider therapy modification

Tocilizumab: May decrease the serum concentration of CYP3A4 Substrates (High risk with Inducers). Monitor therapy

Tofacitinib: Immunosuppressants may enhance the immunosuppressive effect of Tofacitinib. Management: Concurrent use with antirheumatic doses of methotrexate or nonbiologic disease modifying antirheumatic drugs (DMARDs) is permitted, and this warning seems particularly focused on more potent immunosuppressants. Consider therapy modification

Trastuzumab: May enhance the neutropenic effect of Immunosuppressants. Monitor therapy

Upadacitinib: Immunosuppressants may enhance the immunosuppressive effect of Upadacitinib. Avoid combination

Urea Cycle Disorder Agents: Corticosteroids (Systemic) may diminish the therapeutic effect of Urea Cycle Disorder Agents. More specifically, Corticosteroids (Systemic) may increase protein catabolism and plasma ammonia concentrations, thereby increasing the doses of Urea Cycle Disorder Agents needed to maintain these concentrations in the target range. Monitor therapy

Vaccines (Inactivated): Immunosuppressants may diminish the therapeutic effect of Vaccines (Inactivated). Management: Vaccine efficacy may be reduced. Complete all age-appropriate vaccinations at least 2 weeks prior to starting an immunosuppressant. If vaccinated during immunosuppressant therapy, revaccinate at least 3 months after immunosuppressant discontinuation. Consider therapy modification

Vaccines (Live): Corticosteroids (Systemic) may enhance the adverse/toxic effect of Vaccines (Live). Corticosteroids (Systemic) may diminish the therapeutic effect of Vaccines (Live). Management: Doses equivalent to less than 2 mg/kg or 20 mg per day of prednisone administered for less than 2 weeks are not considered sufficiently immunosuppressive to create vaccine safety concerns. Higher doses and longer durations should be avoided. Consider therapy modification

Warfarin: Corticosteroids (Systemic) may enhance the anticoagulant effect of Warfarin. Monitor therapy

Adverse Reactions

>10%:

Dermatologic: Erythema (8% to 28%)

Endocrine & metabolic: Cushingoid appearance (33% to 60%), hirsutism (10% to 35%), weight gain (20% to 28%), obesity (central, 10% to 25%)

Gastrointestinal: Abdominal pain (including upper abdominal pain: 18%), increased appetite (14%)

Genitourinary: Pollakiuria (12% to 15%)

Respiratory: Cough (12%), upper respiratory tract infection (12%)

1% to 10%:

Cardiovascular: Cardiac arrhythmia (≥1%)

Central nervous system: Irritability (8% to 10%), abnormal behavior (9%), psychomotor agitation (6%), aggressive behavior (≥1%), depression (≥1%), dizziness (≥1%), emotional disturbance (≥1%), emotional lability (≥1%), heat exhaustion (≥1%), hypertonia (≥1%, hypertonic bladder), insomnia (≥1%), mood changes (≥1%), sleep disorder (≥1%)

Dermatologic: Skin rash (7%), atrophic striae (6%), acneiform eruption (≥1%), acne vulgaris (≥1%), alopecia (≥1%), impetigo (≥1%)

Endocrine & metabolic: Glycosuria (≥1%), hot flash (≥1%), increased thirst (≥1%)

Gastrointestinal: Constipation (10%), abdominal distress (6%), nausea (6%), dyspepsia (≥1%), gastrointestinal disease (≥1%)

Genitourinary: Dysuria (≥1%), testicular pain (≥1%), urinary tract infection (≥1%), urine discoloration (≥1%)

Hematologic & oncologic: Bruise (6%)

Infection: Influenza (≥1%), tooth abscess (≥1%), viral infection (≥1%)

Neuromuscular & skeletal: Back pain (7%), back injury (≥1%), limb pain (≥1%), muscle spasm (≥1%), myalgia (≥1%), neck pain (≥1%)

Ophthalmic: Hordeolum (≥1%), increased lacrimation (≥1%)

Otic: Otitis externa (≥1%)

Respiratory: Nasopharyngitis (10%), rhinorrhea (8%), epistaxis (6%), hypoventilation (≥1%), pharyngitis (≥1%)

Miscellaneous: Fever (9%), accidental injury (≥1%, face), mass (≥1%, neck)

Frequency not defined.

Central nervous system: Myasthenia (associated with long-term use)

Neuromuscular & skeletal: Bone fracture (long bones including the fibula as well as greenstick fractures), decreased bone mineral density, osteopenia (associated with long-term use), tendon disease (associated with long-term use)

<1%, postmarketing, and/or case reports: Abnormal serum calcium (negative calcium balance), acute pancreatitis (especially in children), acute peptic ulcer with hemorrhage and perforation, amyotrophy, anaphylaxis, anxiety, avascular necrosis of bones, carbohydrate intolerance, change in serum protein (negative protein balance), chorioretinitis, cognitive dysfunction (including confusion, amnesia, delusions, hallucinations, mania, or suicidal thoughts), corneal thinning, decreased serum potassium, edema, exacerbation of epilepsy, hemorrhage, hypersensitivity, hypokalemic alkalosis, increased intracranial pressure (with papilledema in children), leukocytosis, negative nitrogen balance, peptic ulcer, pseudotumor cerebri, scleral thinning, thromboembolism (especially in patients with underlying conditions associated with increased thrombotic tendency), toxic epidermal necrolysis, vertebral compression fracture, vertigo, wound healing impairment

Warnings/Precautions

Concerns related to adverse effects:

• Adrenal suppression: May cause hypercortisolism or suppression of hypothalamic-pituitary-adrenal (HPA) axis. HPA axis suppression may lead to adrenal crisis. Withdrawal and discontinuation of a corticosteroid should be done slowly and carefully.
• Anaphylaxis: Rare cases of anaphylaxis have occurred in patients receiving corticosteroids.
• Immunosuppression: Prolonged use of corticosteroids may increase the incidence of secondary infection, cause activation of latent infections, mask acute infection (including fungal infections), prolong or exacerbate existing infections, or limit response to killed or inactivated vaccines. Exposure to chickenpox or measles should be avoided. Vaccinate patients based on current immunization schedules prior to therapy initiation; any live-attenuated or live vaccines should be administered at least 4 to 6 weeks prior to starting deflazacort. Close observation is required in patients with latent tuberculosis and/or TB reactivity; restrict use in active TB (only in conjunction with antituberculosis treatment). Avoid use in patients with active ocular herpes simplex. Hepatitis B reactivation can occur in patients who are hepatitis B carriers. Amebiasis should be ruled out in any patient with recent travel to tropic climates or unexplained diarrhea prior to initiation of corticosteroids. Use with extreme caution in patients with Strongyloides infections; hyperinfection, dissemination and fatalities have occurred.
• Kaposi sarcoma: Prolonged treatment with corticosteroids has been associated with the development of Kaposi sarcoma (case reports); if noted, discontinuation of therapy should be considered (Goedert 2002).
• Myopathy: Acute myopathy has been reported with high dose corticosteroids, usually in patients with neuromuscular transmission disorders; may involve ocular and/or respiratory muscles; monitor creatinine kinase; recovery may be delayed.
• Ocular effects: Prolonged use may cause posterior subcapsular cataracts, glaucoma (with possible nerve damage), and increased intraocular pressure. Consider routine eye exams in chronic users.
• Psychiatric disturbances: Corticosteroid use may cause psychiatric disturbances, including depression, euphoria, insomnia, mood swings, and personality changes. Preexisting psychiatric conditions may be exacerbated by corticosteroid use.
• Skin reactions: Toxic epidermal necrolysis has been reported within 8 weeks of starting treatment; discontinue at first sign of rash, unless rash is clearly not drug-related.
• Thromboembolic events: Higher cumulative doses of corticosteroids have been associated with an increased risk of thromboembolism. Use caution in patients with a history of or at increased risk for thromboembolic disorders.

Disease-related concerns:

• Cardiovascular disease: Use with caution in patients with heart failure and/or hypertension; use has been associated with fluid retention, electrolyte disturbances, and hypertension. Use with caution following acute MI; corticosteroids have been associated with myocardial rupture.
• Diabetes: Use with caution in patients with diabetes mellitus; may alter glucose production/regulation leading to hyperglycemia.
• Gastrointestinal disease: Use with caution in patients with GI diseases (diverticulitis, fresh intestinal anastomoses, ulcerative colitis, active or latent peptic ulcer, abscess or other pyogenic infections) due to perforation risk. Avoid use if there is a probability of impending perforation, abscess, or other pyogenic infection.
• Hepatic impairment: Use with caution in patients with severe hepatic impairment.
• Myasthenia gravis: Use with caution in patients with myasthenia gravis; exacerbation of symptoms has occurred especially during initial treatment with corticosteroids.
• Osteoporosis: Use with caution in patients with or who are at risk for osteoporosis; high doses and/or long-term use of corticosteroids have been associated with increased bone loss, osteoporotic fractures, and avascular necrosis.
• Pheochromocytoma: Use with caution in patients with pheochromocytoma; cases of pheochromocytoma crisis, which can be fatal, have been reported with corticosteroids.
• Renal impairment: Use with caution in renal impairment; fluid retention may occur.
• Seizure disorders: Use with caution in patients with a history of seizure disorder.
• Thyroid disease: Changes in thyroid status may necessitate dosage adjustments; metabolic clearance of corticosteroids increases in hyperthyroid patients and decreases in hypothyroid ones.

Concurrent drug therapy issues:

• Drug-drug interactions: Potentially significant interactions may exist, requiring dose or frequency adjustment, additional monitoring, and/or selection of alternative therapy. Consult drug interactions database for more detailed information.

Special populations:

• Pediatric: May affect growth velocity; growth should be routinely monitored in pediatric patients.

Dosage form specific issues:

• Benzyl alcohol and derivatives: Some dosage forms may contain benzyl alcohol; large amounts of benzyl alcohol (≥99 mg/kg/day) have been associated with a potentially fatal toxicity (“gasping syndrome”) in neonates; the “gasping syndrome” consists of metabolic acidosis, respiratory distress, gasping respirations, CNS dysfunction (including convulsions, intracranial hemorrhage), hypotension and cardiovascular collapse (AAP 1997; CDC 1982); some data suggests that benzoate displaces bilirubin from protein binding sites (Ahlfors 2001); avoid or use dosage forms containing benzyl alcohol with caution in neonates. See manufacturer's labeling.

Other warnings/precautions:

• Discontinuation of therapy: Withdraw therapy with gradual tapering of dose.
• Immunizations: Patients should be brought up to date with all immunizations before initiating therapy. Complete necessary immunizations ≥4 to 6 weeks prior to initiating therapy; live vaccines should not be given concurrently with deflazacort.
• Stress: Patients may require higher doses when subject to stress (ie, trauma, surgery, severe infection).

Monitoring Parameters

Blood pressure, blood glucose, electrolytes

Following prolonged use: Bone mass density, assess HPA axis suppression (eg, ACTH stimulation test, morning plasma cortisol test, urinary free cortisol test), growth in children, signs and symptoms of infection, cataract formation, intraocular pressure.

Pregnancy

Pregnancy Considerations

Deflazacort crosses the placenta. Orofacial clefts, intrauterine growth restriction, and decreased birth weight have been reported following maternal use. Hypoadrenalism may occur in newborns following maternal use of corticosteroids in pregnancy; monitor.

Patient Education

What is this drug used for?

• It is used to treat Duchenne muscular dystrophy (DMD).

Frequently reported side effects of this drug

• Increased hunger
• Common cold symptoms
• Passing a lot of urine
• Constipation
• Back pain
• Nausea

Other side effects of this drug: Talk with your doctor right away if you have any of these signs of:

• Infection
• High blood sugar like confusion, fatigue, increased thirst, increased hunger, passing a lot of urine, flushing, fast breathing, or breath that smells like fruit
• Blood clots like numbness or weakness on one side of the body; pain, redness, tenderness, warmth, or swelling in the arms or legs; change in color of an arm or leg; chest pain; shortness of breath; fast heartbeat; or coughing up blood
• Cushing disease like weight gain in upper back or abdomen; moon face; severe headache; or slow healing
• Adrenal gland problems like severe nausea, vomiting, severe dizziness, passing out, muscle weakness, severe fatigue, mood changes, lack of appetite, or weight loss
• Severe headache
• Dizziness
• Passing out
• Vision changes
• Electrolyte problems like mood changes, confusion, muscle pain or weakness, abnormal heartbeat, seizures, lack of appetite, or severe nausea or vomiting
• Severe loss of strength and energy
• Irritability
• Tremors
• Fast heartbeat
• Confusion
• Sweating a lot
• Shortness of breath
• Excessive weight gain
• Swelling in the arms or legs
• Skin changes like acne, stretch marks, slow healing, or hair growth
• Bone pain
• Joint pain
• Severe abdominal pain
• Black, tarry, or bloody stools
• Vomiting blood
• Mood changes
• Behavioral changes
• Stevens-Johnson syndrome/toxic epidermal necrolysis like red, swollen, blistered, or peeling skin (with or without fever); red or irritated eyes; or sores in mouth, throat, nose, or eyes
• Signs of a significant reaction like wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat.

Note: This is not a comprehensive list of all side effects. Talk to your doctor if you have questions.

Consumer Information Use and Disclaimer: This information should not be used to decide whether or not to take this medicine or any other medicine. Only the healthcare provider has the knowledge and training to decide which medicines are right for a specific patient. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a brief summary of general information about this medicine. It does NOT include all information about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to this medicine. This information is not specific medical advice and does not replace information you receive from the healthcare provider. You must talk with the healthcare provider for complete information about the risks and benefits of using this medicine.