Deflazacort is a corticosteroid medication that is used to treat various conditions such as asthma, allergies, rheumatoid arthritis, Duchenne muscular dystrophy (DMD), and inflammatory disorders. It works by reducing inflammation in the body, which helps to alleviate symptoms associated with these conditions.

In the case of Duchenne muscular dystrophy, deflazacort is often prescribed to help improve muscle strength and function. It has been shown to slow down the progression of muscle weakness in patients with DMD.

Deflazacort (Emflaza) is a corticosteroid and has anti-inflammatory and immune-modulatory effects. It is used to treat patients with Duchenne muscular dystrophy who are at least 5 years of age or older.

Deflazacort Uses:

• Duchenne muscular dystrophy:
  • Treatment of Duchenne muscular dystrophy (DMD) in patients 5 years and older

Deflazacort (Emflaza) Dose in Adults

Deflazacort (Emflaza) Dose in the treatment of Duchenne muscular dystrophy:

• For treating Duchenne muscular dystrophy, the typical dose of deflazacort taken by mouth is 0.9 milligrams per kilogram of body weight once a day.
• When you're using tablets, round up to the nearest possible dose, and when using suspension, round up to the nearest tenth of a milliliter.

Concomitant moderate or strong CYP3A4 Inhibitors (eg, clarithromycin, fluconazole, diltiazem, verapamil):

• If you're taking medications that strongly or moderately inhibit CYP3A4 enzymes (like clarithromycin, fluconazole, diltiazem, or verapamil), your doctor may advise reducing your deflazacort dose to a fraction of the usual dose.

Deflazacort (Emflaza) Dose in Childrens

Deflazacort (Emflaza) Dose in the treatment of Duchenne muscular dystrophy:

• To treat Duchenne muscular dystrophy in male children aged 5 years and older, as well as adolescents, the typical dose of deflazacort is approximately 0.9 milligrams per kilogram of body weight once a day.
• When administering the medication, round the dose to the nearest 0.1 milliliter when using oral suspension and round up to the nearest possible dose when using tablets, considering the available strengths.

Dosing adjustment for concomitant therapy with moderate or strong CYP3A4 inhibitors (eg, clarithromycin, fluconazole, diltiazem, verapamil):

• If the patient is also taking medications that moderately or strongly inhibit CYP3A4 enzymes (such as clarithromycin, fluconazole, diltiazem, or verapamil), the deflazacort dose should be reduced to a fraction of the usual dose.

Pregnancy Risk Category: N

• Deflazacort is capable of passing through the placenta during pregnancy.
• There have been reports of certain adverse effects associated with its maternal use, including orofacial clefts, intrauterine growth restriction, and reduced birth weight in newborns.
• Furthermore, newborns may experience hypoadrenalism as a result of corticosteroids used by the mother during pregnancy.

Use during breastfeeding:

• While specific information regarding the excretion of deflazacort into breast milk is not readily available, it's generally known that corticosteroids are excreted in breast milk.
• Therefore, caution should be exercised when considering the use of deflazacort while breastfeeding.
• According to the manufacturer's guidance, the decision to continue or discontinue breastfeeding during deflazacort therapy should be made by considering various factors, including the potential risk of infant exposure to the medication, the benefits of breastfeeding to the infant, and the benefits of treatment to the mother.

Dose in Kidney Disease:

• In cases of renal impairment, no dosage adjustment for deflazacort is typically required.
• However, it's advised to use the medication with caution in individuals with renal issues.
• This caution is mainly due to the possibility of altered drug metabolism and elimination in patients with renal impairment.

Dose in Liver disease:

• For individuals with mild to moderate hepatic impairment, no dosage adjustment is typically necessary when using deflazacort.

• In cases of severe hepatic impairment, there are no specific dosage adjustments provided in the manufacturer's labeling because this population hasn't been studied extensively. Therefore, caution should be exercised when administering deflazacort to individuals with severe hepatic impairment.

Common Side Effects of Deflazacort (Emflaza):

• Dermatologic:
  • Erythema
• Endocrine & metabolic:
  • Cushingoid appearance
  • Hirsutism
  • Weight gain
  • Obesity
• Gastrointestinal:
  • Abdominal pain
  • Increased appetite
• Genitourinary:
  • Pollakiuria
• Respiratory:
  • Cough
  • Upper respiratory tract infection

Less Common Side Effects of Deflazacort (Emflaza):

• Cardiovascular:
  • Cardiac Arrhythmia
• Central Nervous System:
  • Irritability
  • Abnormal Behavior
  • Psychomotor Agitation
  • Aggressive Behavior
  • Depression
  • Dizziness
  • Emotional Disturbance
  • Emotional Lability
  • Heat Exhaustion
  • Hypertonia
  • Insomnia
  • Mood Changes
  • Sleep Disorder
• Dermatologic:
  • Skin Rash
  • Atrophic Striae
  • Acneiform Eruption
  • Acne Vulgaris
  • Alopecia
  • Impetigo
• Endocrine & Metabolic:
  • Glycosuria
  • Hot Flash
  • Increased Thirst
• Gastrointestinal:
  • Constipation
  • Abdominal Distress
  • Nausea
  • Dyspepsia
  • Gastrointestinal Disease
• Genitourinary:
  • Dysuria
  • Testicular Pain
  • Urinary Tract Infection
  • Urine Discoloration
• Hematologic & Oncologic:
  • Bruise
• Infection:
  • Influenza
  • Tooth Abscess
  • Viral Infection
• Neuromuscular & Skeletal:
  • Back Pain
  • Back Injury
  • Limb Pain
  • Muscle Spasm
  • Myalgia
  • Neck Pain
• Ophthalmic:
  • Hordeolum
  • Increased Lacrimation
• Otic:
  • Otitis Externa
• Respiratory:
  • Nasopharyngitis
  • Rhinorrhea
  • Epistaxis
  • Hypoventilation
  • Pharyngitis
• Miscellaneous:
  • Fever
  • Accidental Injury
  • Mass

Frequency of side effects 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)

Contraindications to Deflazacort (Emflaza):

• Deflazacort should not be used in individuals who have a known hypersensitivity or allergy to deflazacort itself or to any component of the formulation.
• If a person experiences symptoms of hypersensitivity such as rash, itching, swelling, dizziness, or difficulty breathing after taking deflazacort, they should seek immediate medical attention.

Warnings and precautions

Suppression of the adrenals:

• Deflazacort can potentially lead to hypercortisolism or suppression of the hypothalamic-pituitary-adrenal (HPA) axis, which regulates the body's stress response and cortisol production. Suppression of the HPA axis may result in adrenal crisis, a serious condition characterized by insufficient cortisol levels.
• Therefore, it's important to taper and discontinue corticosteroids like deflazacort slowly and carefully under the supervision of a Doctor. Abrupt discontinuation can lead to adrenal insufficiency and withdrawal symptoms.

Anaphylaxis

• In rare instances, patients taking corticosteroids like deflazacort have experienced anaphylaxis, a severe allergic reaction that can be life-threatening.
• Anaphylaxis can cause symptoms such as difficulty breathing, swelling of the face or throat, rapid heartbeat, and a drop in blood pressure.
• If someone experiences these symptoms after taking deflazacort, it's crucial to seek immediate medical attention.

Immunosuppression:

• Long-term use of corticosteroids like deflazacort can suppress the immune system, increasing the risk of various issues.
• This includes a higher chance of getting secondary infections, activation of hidden infections, hiding signs of new infections like fungal ones, making existing infections last longer, or making them worse.
• It can also make vaccines that contain killed or inactivated viruses less effective.
• It's important to avoid exposure to chickenpox or measles while taking deflazacort, and close monitoring is needed for patients with latent tuberculosis or TB reactivity.
• Deflazacort should generally not be used in active tuberculosis, unless it's in conjunction with specific tuberculosis treatment.
• It's also not recommended for patients with active ocular herpes simplex or hepatitis B carriers, as it may reactivate these conditions.
• Travelers to tropical climates or those with unexplained diarrhea should be checked for amebiasis before starting corticosteroid treatment.
• Extreme caution is advised for patients with Strongyloides infections, as severe infections, spreading of the infection, and even death have occurred.

Kaposi Sarcoma:

• Prolonged use of corticosteroids has been linked to the development of Kaposi sarcoma, as reported in some cases.
• If signs of Kaposi sarcoma are observed during corticosteroid therapy, discontinuing the treatment should be considered.

Myopathy

• Acute myopathy, a condition characterized by muscle weakness and pain, has been reported in some cases with the use of high doses of corticosteroids.
• This is particularly observed in patients with neuromuscular transmission disorders and may affect muscles including those responsible for eye movement and breathing.

Ocular effects

• Prolonged use of corticosteroids like deflazacort can lead to several adverse effects on the eyes.
• These may include the development of posterior subcapsular cataracts, a type of clouding in the lens of the eye, as well as glaucoma, a condition characterized by increased pressure within the eye that can damage the optic nerve.
• It's important for chronic users of corticosteroids to consider routine eye examinations to monitor for these potential complications and to promptly address any changes in vision or symptoms of eye discomfort.

Psychiatric disorders:

• The use of corticosteroids such as deflazacort can sometimes lead to psychiatric disturbances, affecting mood and behavior.
• These disturbances may include symptoms like depression, euphoria, insomnia, mood swings, and changes in personality.
• It's important to note that preexisting psychiatric conditions may worsen with corticosteroid use.

Reactions to skin:

• Skin reactions, including serious conditions like toxic epidermal necrolysis, have been reported in some cases within the first 8 weeks of starting treatment with corticosteroids like deflazacort.
• Toxic epidermal necrolysis is a severe skin reaction characterized by widespread skin detachment and can be life-threatening.
• If a rash develops, it's crucial to discontinue deflazacort at the first sign of the rash, unless it's clearly determined not to be related to the medication.
• Prompt action and medical attention are essential to prevent the progression of severe skin reactions and ensure appropriate management.

Events that are thromboembolic:

• Higher cumulative doses of corticosteroids, like deflazacort, have been linked to an increased risk of thromboembolic events, which are conditions where blood clots form and block blood vessels.
• It's essential to exercise caution when prescribing corticosteroids to patients with a history of thromboembolic disorders or those at increased risk for such conditions.

Cardiovascular disease

• Deflazacort should be used cautiously in patients with cardiovascular disease, particularly those with conditions such as heart failure and hypertension.
• Corticosteroid use has been associated with fluid retention, electrolyte imbalances, and elevated blood pressure, which can exacerbate these conditions.
• Additionally, caution is advised when using deflazacort following an acute myocardial infarction (heart attack), as corticosteroids have been linked to an increased risk of myocardial rupture.

Diabetes:

• Deflazacort should be used cautiously in patients with diabetes mellitus.
• Corticosteroids like deflazacort can affect glucose production and regulation in the body, potentially leading to elevated blood sugar levels (hyperglycemia).
• Patients with diabetes may experience worsening of their condition or difficulty in managing their blood sugar levels while taking deflazacort.

Gastrointestinal Disease:

• Deflazacort should be used cautiously in patients with gastrointestinal diseases such as diverticulitis, recent intestinal surgery (anastomoses), ulcerative colitis, active or latent peptic ulcers, or abscesses, due to the increased risk of perforation (tearing or puncturing of the intestinal wall).
• If there's a possibility of impending perforation, abscess formation, or other pyogenic infections in the gastrointestinal tract, deflazacort should be avoided.

Hepatic impairment

• Deflazacort should be used with caution in patients with severe hepatic impairment.
• While specific dosage adjustments for severe hepatic impairment are not provided in the manufacturer's labeling, it's important to be cautious when administering deflazacort to individuals with severe liver dysfunction.

Myasthenia gravis:

• Deflazacort should be used cautiously in patients with myasthenia gravis.
• Exacerbation of symptoms, especially during the initial stages of treatment with corticosteroids, has been reported in some cases.
• Therefore, close monitoring of patients with myasthenia gravis is essential during deflazacort therapy.

Osteoporosis

• Deflazacort should be used cautiously in patients with or at risk for osteoporosis.
• High doses or prolonged use of corticosteroids like deflazacort have been linked to increased bone loss, osteoporotic fractures, and avascular necrosis (a condition where bone tissue dies due to reduced blood supply).
• Patients with existing osteoporosis or those with risk factors for the condition, such as older age, postmenopausal status, or a history of fractures, should be closely monitored during deflazacort therapy.

Pheochromocytoma:

• Deflazacort should be used cautiously in patients with pheochromocytoma, a rare tumor of the adrenal gland.
• Corticosteroids like deflazacort have been associated with cases of pheochromocytoma crisis, which can be life-threatening.

Renal impairment

• Deflazacort should be used with caution in patients with renal impairment.
• Fluid retention is a potential side effect of corticosteroid use, and in patients with renal impairment, this may pose an increased risk.
• Therefore, close monitoring of fluid balance and renal function is important during deflazacort therapy in patients with renal impairment.

Seizure disorders:

• Deflazacort should be used with caution in patients with a history of seizure disorders.
• Corticosteroids like deflazacort may lower the seizure threshold, increasing the risk of seizures in susceptible individuals.
• Therefore, close monitoring of patients with a history of seizure disorder is essential during deflazacort therapy.

Thyroid disease:

• Patients with thyroid disease should be monitored carefully while taking deflazacort, as changes in thyroid status may require dosage adjustments.
• In individuals with hyperthyroidism (overactive thyroid), the metabolic clearance of corticosteroids like deflazacort may increase, potentially leading to a need for higher doses to achieve the desired therapeutic effect.
• Conversely, in patients with hypothyroidism (underactive thyroid), the metabolic clearance of corticosteroids may decrease, necessitating lower doses to avoid excessive drug accumulation and potential side effects.

Deflazacort: Drug Interaction

Note: Drug Interaction Categories:

• Risk Factor C: Monitor When Using Combination
• Risk Factor D: Consider Treatment Modification
• Risk Factor X: Avoid Concomitant Use

Monitoring parameters:

Blood Pressure, Blood Glucose, and Electrolytes:

• Regular monitoring of blood pressure, blood glucose levels, and electrolyte levels is important during treatment with deflazacort.
• Corticosteroids like deflazacort can affect these parameters, so keeping track helps to detect and manage any abnormalities.

Following Prolonged Use:

• Bone Mass Density: After prolonged use of deflazacort, bone density should be assessed regularly.
• HPA Axis Suppression: Tests such as the ACTH stimulation test, morning plasma cortisol test, and urinary free cortisol test may be needed to assess suppression of the hypothalamic-pituitary-adrenal (HPA) axis.
• Growth in Children: Growth should be monitored in children to ensure proper development.
• Signs and Symptoms of Infection: Watch for any signs or symptoms of infection, as corticosteroids can increase susceptibility to infections.
• Cataract Formation: Regular eye exams should be conducted to check for cataracts, especially in patients on long-term treatment.
• Intraocular Pressure: Monitoring of intraocular pressure is important to detect any increase, which may occur with prolonged use of corticosteroids.

How to administer Deflazacort (Emflaza)?

Oral Administration:

• Deflazacort can be taken with or without food.

Tablets:

• Tablets should be swallowed whole.
• Alternatively, tablets may be crushed and mixed with applesauce.
• If mixing with applesauce, administer the mixture immediately after preparation.

Suspension:

• Shake the suspension well before use.
• Measure the prescribed dose using the provided dispenser.
• Mix the measured dose thoroughly with 3 to 4 ounces of juice or milk.
• Administer the mixture immediately after preparation.
• Do not mix or administer the suspension with grapefruit juice.

Mechanism of action of Deflazacort (Emflaza):

• Deflazacort is a corticosteroid prodrug, meaning it gets converted into an active form in the body.
• Its active metabolite, 21-desDFZ, works by binding to the glucocorticoid receptor.
• This binding triggers anti-inflammatory and immunosuppressive effects, which help to reduce inflammation and suppress the immune response.
• However, the exact mechanism by which deflazacort brings about its therapeutic effects in patients with Duchenne muscular dystrophy (DMD) is not fully understood.
• Further research is needed to elucidate the specific ways in which deflazacort benefits individuals with DMD.

Protein Binding:

• Approximately 40% of deflazacort binds to proteins in the blood.

Metabolism:

• Deflazacort is rapidly converted by esterases in the body to its active metabolite, 21-desDFZ.
• This active metabolite undergoes further metabolism by the enzyme CYP3A4, leading to the formation of several inactive metabolites.

Time to Peak Serum Concentration:

• The time it takes for deflazacort to reach peak levels in the blood is about 1 hour, with a range of 0.25 to 2 hours.
• This time may be delayed by approximately 1 hour if the medication is taken with a high-fat meal.

Excretion:

• Deflazacort and its metabolites are primarily excreted through the urine, accounting for approximately 68% of the administered dose.
• Around 18% of the excreted material is in the form of the active metabolite, 21-desDFZ.

International Brand Names of Deflazacort:

• Emflaza
• Aflazacort
• Alcoza
• Alfazcort
• Alnacort
• Arocort
• Arrumal
• Asfor
• Asodef
• Avantium
• Azacortid
• Calcorrt
• Calcort
• Carzoflep
• Clobax
• Cortiflo
• Cortimax
• Dazenar
• Defal
• Defas
• Defax
• Defcort
• Defla
• Deflaimmun
• Deflan
• Deflanil
• Deflazym
• Defzort
• Dezacor
• Dezartal
• DFZ
• Dispercort
• Flacort
• Flamirex
• Flantadin
• Flazacor
• Flazacort
• Flazal
• Landacort
• Lantadin
• Prandin
• Prism
• Refla
• Rosilan
• Servicor
• Setatrep
• Telacort
• Xalcort
• Zamen
• Zamene

Deflazacort Brand Names in Pakistan:

Not available.