Indication and Dosage of Cerdelga

 INDICATIONS

CERDELGA is indicated for the long-term treatment of adult patients with Gaucher disease type 1 (GD1) who are CYP2D6 extensive metabolizers (EMs), intermediate metabolizers (IMs), or poor metabolizers (PMs) as detected by an FDA-cleared test [see DOSAGE AND ADMINISTRATION].
Limitations Of Use

   named  Patient program who are CYP2D6 ultra-rapid metabolizers (URMs) may not achieve adequate concentrations of CERDELGA to achieve a therapeutic effect [see Clinical Studies].
    A specific dosage cannot be recommended for those patients whose CYP2D6 genotype cannot be determined (indeterminate metabolizers) [see Clinical Studies].

DOSAGE AND ADMINISTRATION

Patient Selection

Select patients with Gaucher disease type 1 based on their CYP2D6 metabolizer status. It is recommended patient genotypes be established using an FDA-cleared test for determining CYP2D6 genotype.

Important Administration Instructions

•     Swallow capsules whole, preferably with water, and do not crush, dissolve, or open the capsules.
•     CERDELGA can be taken with or without food.
•     Avoid the consumption of grapefruit or grapefruit juice (strong CYP3A inhibitors) with CERDELGA [see DRUG INTERACTIONS].
•     If a dose of CERDELGA is missed, take the prescribed dose at the next scheduled time; do not double the next dose.
•     For patients currently treated with imiglucerase, velaglucerase alfa, or taliglucerase alfa, CERDELGA may be administered 24 hours after the last dose of the previous enzyme replacement therapy (ERT).

What is CERDELGA and how is it used?

CERDELGA is a prescription medicine used for the long-term treatment of Gaucher disease type 1 (GD1) in adults.

CERDELGA is not used in certain people with Gaucher disease type 1. Your doctor will perform a test to make sure that CERDELGA is right for you.

It is not known if CERDELGA is safe and effective in children.

What are the possible side effects of CERDELGA?

See "What is the most important information I should know about CERDELGA?"

    CERDELGA, used with certain other medicines, may cause changes in the electrical activity of your heart (ECG changes) and irregular heart beat (arrhythmias). Tell your doctor if you have new symptoms such as palpitations, fainting, or dizziness.

The most common side effects of CERDELGA include: tiredness, headache, nausea, diarrhea, and pain in the arms, legs, back, or stomach (abdomen).

Tell your doctor if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of CERDELGA.

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

How should I store CERDELGA?

    Store CERDELGA at room temperature between 68°F to 77 °F (20°C to 25 °C).
    Keep CERDELGA and all medicines out of reach of children.

General information about the safe and effective use of CERDELGA.

Medicines are sometimes prescribed for purposes other than those listed in a Medication Guide. Do not use CERDELGA for a condition for which it was not prescribed. Do not give CERDELGA to other people, even if they have the same symptoms you have. It may harm them.

If you would like more information, talk with your doctor. named patient india You can ask your doctor or pharmacist for information about CERDELGA that is written for health professionals.

Zavesca Indications & Dosage

 Zavesca is a glucosylceramide synthase inhibitor and is indicated as a monotherapy for adult patients with mild to moderate type 1 Gaucher disease. For these patients, enzyme replacement therapy is not a treatment option (for example, due to allergies, Hypersensitivity or poor venous access).

Dosage and administration

Management Instructions

Treatment should be guided by a doctor who has a good understanding of Gaucher's disease.

The recommended dose for adults with type 1 Gaucher disease is one 100 mg capsule orally three times a day. If a dose is missed, the next Zavesca capsule should be taken at the next scheduled time.

In some patients, due to adverse reactions such as tremor or diarrhea, it may be necessary to reduce the dose to 100 mg capsules once or twice a day.

Patients with renal insufficiency

For patients with mild renal insufficiency (adjusted creatinine clearance 50-70 mL/min / 1.73m²), the administration of Zavesca should be started with a dose of 100 mg twice a day. For patients with moderate renal insufficiency (adjusted creatinine clearance rate of 30-50 mL/min / 1.73m²), the administration of Zavesca should start with a daily dose of 100 mg capsules. Zavesca is not recommended for named patient basis with severe renal insufficiency (creatinine clearance <30 mL / min / 1.73m²)

ZAVESCA capsule uses

 

 Zavesca (miglustat capsules, 100 mg) is an inhibitor of glucosylceramide synthase, which is the first glucosyltransferase responsible for the synthesis of most glycosphingolipids.

Zavesca is an N-alkylated imino sugar, which is a synthetic analog of D-glucose.

The chemical name of miglustat is 1,5-(butylimino)-1,5-dideoxy-D-glucitol, the chemical formula is C10H21NO4, and the molecular weight is 219.28.



Miglustat is a white to off-white crystalline solid with a bitter taste. It is highly soluble in water (>1000 mg/mL is free base).

Zavesca is available in hard gelatin capsules, each capsule containing 100 mg of miglustat orally. named patient program, Each Zavesca 100 mg capsule also contains sodium starch glycolate, povidone (K30) and magnesium stearate. The ingredients in the capsule shell include gelatin and titanium dioxide, and the shell is printed with edible ink composed of black iron oxide and shellac.

ORENCIA SIDE EFFECTS

 Because clinical trials are conducted under widely varying and controlled conditions, it is impossible to directly compare the adverse reaction rate observed in the drug clinical trial with the incidence in the clinical trial of another drug, and may not be predictable in clinical practice The adverse reaction rate observed in the wider patient population.

Like all therapeutic proteins, it has the potential for immunogenicity. The detection of antibody formation is highly dependent on the sensitivity and specificity of the assay. In addition, the incidence of positive antibodies (including neutralizing antibodies) observed in the assay may be affected by several factors, including the assay method, sample processing, sample collection time, concomitant medications and underlying diseases. named patient supply For these reasons, comparing the incidence of abatacept antibodies in the studies described below with the incidence of antibodies in other studies or other products may be misleading.

Clinical research experience of Orencia intravenous injection in adult RA patients

The data described in this article reflect the intravenous use of Orencia in patients with active RA in a placebo-controlled study (Orencia patients in 1955, 989 placebo patients).named patient medicines The double-blind placebo-controlled period of the study was 6 months (258 ORENCIA patients, 133 placebo) or 1 year (1697 ORENCIA patients, 856 placebo). Some of these patients received biological DMARD therapy, such as TNF blockers (204 ORENCIA patients and 134 placebo patients).

Most patients in RA clinical studies receive one or more of the following ORENCIA concomitant drugs: methotrexate, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, TNF blockers, azathioprine, chloroquine, gold, hydroxy Chloroquine, leflunomide, sulfasalazine and anakinra.

The most serious adverse reactions are serious infections and malignant tumors.

The most common adverse events (occurring in ≥10% of patients treated with ORENCIA) were headache, upper respiratory tract infection, nasopharyngitis, and nausea.

The most common adverse event (interruption or discontinuation of ORERNCIA) of clinical intervention is caused by infection. pharmaceutical consulting companies The most frequently reported infections that caused dose interruptions were upper respiratory tract infection (1.0%), bronchitis (0.7%), and herpes zoster (0.7%). The most common infections leading to discontinuation are pneumonia (0.2%), local infection (0.2%) and bronchitis (0.1%).

DOSAGE AND ADMINISTRATION ORENCIA

Adult rheumatoid arthritis

For adult patients with RA, ORENCIA can be administered by intravenous infusion or subcutaneous injection.

ORENCIA can be used as a monotherapy or with DMARDs other than TNF antagonists.
IV regimen

Orerncia lyophilized powder should be diluted and used after dilution [see adult psoriatic arthritis], and intravenous infusion of 30 minutes according to the dose based on the weight range specified in Table 1. After the first intravenous administration, intravenous infusion should be given at 2 and 1. 4 weeks after the first infusion and every 4 weeks thereafter.

Subcutaneous dosing regimen

ORENCIA 125 mg in a prefilled syringe or in the ORENCIA ClickJect™ auto-injector should be injected subcutaneously once a week [see preparation and dosing instructions for intravenous infusion], and can be started with or without an intravenous dose . named patient program For patients starting treatment with an intravenous loading dose, ORENCIA should be started with a single intravenous infusion (according to the weight category listed in Table 1), and then the first 125 mg subcutaneous injection should be given within the same day of the intravenous infusion.

Patients transitioning from Orencia intravenous therapy to subcutaneous administration should be given the first subcutaneous dose instead of the next scheduled intravenous dose.
Juvenile idiopathic arthritis

For patients with juvenile idiopathic arthritis (JIA), ORENCIA can be administered by intravenous infusion (6 years and older) or subcutaneous injection (2 years and older). No intravenous administration was given to patients under 6 years of age.

ORENCIA can be used as a monotherapy or concurrently with methotrexate.
IV regimen

ORENCIA should be administered as a 30-minute intravenous infusion based on body weight. Pediatric patients:

 People weighing less than 75 kg should be administered ORENCIA at a dose of 10 mg/kg [see Adult Psoriatic Arthritis] After the adult intravenous administration regimen (see Table 1), Orencia should be given a body weight of 75 kg or more, and the maximum dose should not exceed 1,000 mg.

After the initial administration, ORENCIA should be administered 2 and 4 weeks after the first infusion and every 4 weeks thereafter. named patient basis,The unused part of the sample bottle must be discarded immediately.
Subcutaneous dosing regimen

Orencia for subcutaneous injection should be started without an intravenous dose, and the dose based on the weight range specified in Table 2 should be used.

ORENCIA INDICATIONS

INDICATIONS

Adult rheumatoid arthritis (RA)

ORENCIA® is suitable for alleviating the symptoms and signs of adult patients with moderate to severe active rheumatoid arthritis, causing significant clinical reactions, inhibiting the progression of structural damage and improving physical function. In addition to tumor necrosis factor (TNF) antagonists, Pharmaceutical Consulting Company OERNCIA can also be used as a monotherapy or in conjunction with disease-modifying anti-rheumatic drugs (DMARD).

Juvenile idiopathic arthritis

ORENCIA is indicated for alleviating signs and symptoms of adolescent idiopathic arthritis with moderately to severely active polyarticular joints aged 2 years and older. ORENCIA can be used as a monotherapy or in combination with methotrexate (MTX).

Adult Psoriatic Arthritis (PsA)

ORENCIA is designated for the treatment of adult patients with active psoriatic arthritis (PsA).

Important usage restrictions

ORENCIA should not be used simultaneously with TNF antagonists. Named patient supply It is not recommended to use ORENCIA together with other biological rheumatoid arthritis (RA) treatments (such as anakinra).