Fabrazyme

Fabrazyme is a drug of international importance. Fabrazyme is used in dermatology to treat Fabry disease. We suggest you consider the features of the drug, dosage form, effectiveness in treatment and impact on the body.

Fabrazyme has an international nonproprietary name - Agalzide beta. The drug dosage form is a concentrate for solution preparation. The drug is used for infusions, i.e. for intravenous and intra-arterial administration. Fabrazyme contains such active substances as agalzide beta, mannitol, sodium hydrogen phosphate heptahydrate and sodium dihydrogen phosphate monohydrate.

The preparation is a dense white powder. After dissolution, the preparation becomes colorless, the solution should be without foreign inclusions.

Indications Fabrazyme

The main indications for the use of Fabrazyme are Fabry disease. Also, the drug is prescribed for long-term enzyme replacement therapy for patients with the disease described above.

The drug is prescribed for patients who have a deficiency of α-galactosidase A in their body. The drug can be taken by both adults and children over the age of eight.

Release form

The drug is available in 5 and 35 mg vials. The vials are made of colorless glass and sealed with rubber stoppers with a snap-on lid. Each package of the drug contains one vial with instructions for use in the state and Russian languages.

The Fabrazyme preparation also comes with a lyophilisate preparation for the preparation of a concentrate. That is, this preparation is used to prepare a solution for infusions from Fabrazyme. This preparation is released in 10 vials in one package.

Pharmacodynamics

Pharmacodynamics of Fabrazyme allows to find out the biochemical effects and impact of the drug on the body. The drug Fabrazyme is prescribed for patients with Farbi disease. In this case, Fabrazyme is a drug for the treatment of a polysystemic and heterogeneous disease. When the drug is administered, the body receives the missing amount of α-galactosidase - lysosomal hydralase. This substance catalyzes the hydrolysis of glycosphingolipids.

The drug is perfectly absorbed into the blood and quickly achieves the desired effect. But, despite the high efficiency of Fabrizim, there are a number of precautions for the use of this drug.

Pharmacokinetics

Pharmacokinetics of Fabrazyme is the work of the drug in the body, that is, the process of absorption, metabolism and elimination. After the administration of a dose of the drug, Fabrazyme is concentrated in the blood plasma and thus functions in the body.

Fabrazyme is a protein, so the process of drug elimination is metabolic destruction by peptide hydrolysis. But such an elimination process can cause kidney dysfunction, which has a negative effect on the pharmacokinetics of Fabrazyme. Excretion of the drug through the kidneys is considered an insignificant and safe way to eliminate Fabrazyme from the blood.

Dosing and administration

The method of administration and dosage of the drug are prescribed by a doctor. Since the drug is prescribed for the treatment of Fabry disease, the drug is taken under the supervision of a doctor. This allows you to track the body's metabolic reactions to the drug.

The recommended dose of Fabrazyme is 1 mg per kg of body weight. The drug is administered to the patient every two weeks as an infusion or intravenous injection. In this case, the initial rate of infusion should not exceed 0.25 mg/min. After some time, the rate of administration of the drug can be increased, but gradually.

If the drug is prescribed to patients with renal failure, then if side effects occur, the dose of the drug is not subject to adjustment. The drug is very rarely prescribed to children, since the study of the effect of Fabrazyme on children has not been conducted, therefore, the drug is taken after the doctor's permission and under his supervision.

Use Fabrazyme during pregnancy

The use of Fabrazyme during pregnancy is undesirable, although the effect of the drug on the female body has not been reliably studied. Despite the fact that the potential risk is unknown, the drug should not be used by pregnant women unless absolutely necessary. As a rule, the use of the drug is provided only for clear indications and an analysis of the risk-benefit ratio for the mother and the future baby.

The drug is prohibited to use during lactation, as fabrazyme is excreted in milk. If it is impossible to stop treatment, then breastfeeding should be stopped so that the drug does not get to the baby.

Contraindications

The main contraindications for the use of Fabrazyme are caused by individual intolerance to the drug and the active substances that are part of the drug.

Some patients may experience dizziness, fainting, and drowsiness when taking the drug for the first time. That is why it is recommended to follow the inpatient treatment regimen and refrain from driving a car or operating automatic equipment when taking the drug.

Side effects Fabrazyme

The main side effects of Fabrazyme are caused by individual sensitivity to the components of the drug. Common side effects:

• Back and lower back pain.
• Dermatological reactions (discoloration, redness of the skin, rash or hives).
• Vision problems, lacrimation, swelling, severe itching.
• Swelling of the extremities, throat, face and other parts of the body.
• Muscle pain, joint pain, muscle spasms, musculoskeletal stiffness.
• Cardiovascular problems (bradycardia and tachycardia).
• Diarrhea, stomach pain, abdominal pain.
• Chest pain when breathing, inflammation of the nasopharynx, cough, nasal congestion, tinnitus.
• Elevated temperature, fainting, decreased sensitivity to pain.
• Chills, nausea, vomiting, tingling in the extremities.

[ 1 ]

Overdose

Overdose of the drug is extremely rare. Overdose can be caused by large doses of the drug and the use of Fabrazyme not as prescribed by the doctor. In case of overdose, you should stop using Fabrazyme and seek medical help.

Very often, in case of an overdose of the drug, a gastric lavage procedure is performed and infusions are given to remove the drug from the blood and body.

Interactions with other drugs

Interaction of Fabrazyme with other drugs is permitted if complex treatment is prescribed. However, there have been no studies in pharmacology and medicine that would confirm the possibility of interaction of Fabrazyme with other drugs.

Fabrazyme is not recommended to be taken simultaneously with such drugs as: amiodarone, chloroquine, gentamicin, benoquine. Since there is a high risk of reducing the activity of the active substance of Fabrazyme - agalsidase beta. Particular attention should be paid to the fact that the drug is strictly prohibited to be mixed with other drugs in one infusion.

[ 2 ], [ 3 ]

Storage conditions

Storage conditions for Fabrazyme include storing the drug in a cool place at a temperature of 2–8 °C (in the refrigerator). The drug should be kept away from sunlight and children.

If the storage rules for the drug are not followed, Fabrazyme must be disposed of. Since the storage conditions ensured the deterioration of the drug, and therefore reduced its medicinal functions to zero.

Shelf life

The shelf life of Fabrazyme is 36 months, i.e. three years from the production date indicated on the package. After the expiration date, the drug must be thrown away. It is strictly forbidden to use the drug after the expiration date, as this can cause serious side effects of an irreversible nature.