FABRAZYME 5MG

Storage/Handling:

Store in a refrigerator in 2C - 8C

Uses of FABRAZYME 5MG:

Used as enzyme replacement therapy for people with Fabry disease, which is a genetic disorder where the levels of alpha-galactosidase (an enzyme that breaks down fats) is low or absent.

Product Introduction:

Fabrazyme 5mg is a medication classified under enzyme replacement therapy. It contains agalsidase beta, a recombinant form of human α-galactosidase A, produced using recombinant DNA technology from Chinese Hamster Ovary (CHO) cell culture. The amino acid and nucleotide sequence of the recombinant form are identical to the natural form of α-galactosidase A.

This medication is used for the treatment of Fabry disease, a genetic disorder where the levels of alpha-galactosidase, an enzyme that breaks down fats, are low or absent. The disease causes the accumulation of globotriaosylceramide (GL-3), a fat substance, in the walls of blood vessels and organs, leading to the progression of the disease in the kidney, heart, and brain. Agalsidase beta reduces GL-3 levels in the body, slowing down the progression of Fabry disease.

Fabrazyme 5mg comes in the form of a powder for concentrate for solution for infusion. Each vial contains 5mg of agalsidase beta, which is reconstituted with 1.1 ml of water for injections to obtain a concentration of 5 mg/ml. The reconstituted solution must be further diluted before use.

It is important to note that this medication may be available under different brand names and in various forms. Some forms may not be suitable for all conditions. If you are unsure about why you have been prescribed this medication or have not discussed it with your doctor, please consult them before use. Do not stop using this medication without consulting your doctor and do not give it to anyone else without a prescription.

Administration & Precautions For FABRAZYME 5MG:

To ensure that the medication is administered safely, it is advisable to use an in-line low protein-binding 0.2 µm filter to remove any protein particles from the diluted solution. This will not affect the activity of agalsidase beta. It is important to start the infusion at a slow rate of no more than 0.25 mg/min (15 mg/hour) to reduce the risk of infusion-associated reactions. Once the patient tolerates the medication well, the infusion rate may be gradually increased during subsequent treatments.

Infusion-associated reactions can be mild to severe and may include fever, chills, headache, rash, difficulty breathing, or low blood pressure. These reactions can occur during or after the infusion and may require medical attention. The use of pre-medication such as antipyretics, antihistamines, or corticosteroids may be considered to reduce the risk of infusion-associated reactions.

It is essential to closely monitor the patient during the infusion and for an appropriate period after the infusion to identify any adverse reactions. In case of a severe reaction, the infusion should be stopped immediately, and the patient should receive appropriate medical treatment. The healthcare provider should also be informed of any adverse reactions or concerns regarding the medication.

Side Effects of FABRAZYME 5MG:

The following side effects have been reported by at least 1% of people taking this medication. Many of these side effects can be managed, and some may go away on their own over time.
1. anxiety
2. back pain
3. decreased hearing
4. depression
5. difficulty sleeping
6. Nausea
7. pain, including muscle and neck pain
8. symptoms of an infection (cough, runny nose, sore throat)
9. Toothache
10. stomach discomfort

Check your doctor immediately if following symptoms occur-
1. Chest & throat tightness
2. dizziness or lightheadedness
3. fast heartbeat
4. signs of depression
5. symptoms of anemia

Drug Interactions:

Drug-Drug Interactions: Fabrazyme 5mg may have an interaction with amiodarone, chloroquine, gentamicin and hydroxychloroquine. It is advisable to speak to your doctor before consuming.

Drug-Food Interactions: No interactions were found.

Drug-Disease Interactions: Consult your doctor regarding any restrictions to follow for consumption of certain foods, beverages

How FABRAZYME 5MG Works:

Fabrazyme is an enzyme replacement therapy (ERT) that works by replacing or supplying the enzyme missing or deficient in people with Fabry disease.

How To Use FABRAZYME 5MG:

The medication should not be self-administered. It is advisable to consume the medication under the recommendation of the doctor. The medication should be consumed immediately after prepared for an infusion.

Safety Instructions For FABRAZYME 5MG:

ALCOHOL Consult your Doctor Fabrazyme 5mg may not interact with alcohol. However, to avoid possible side effects it is advicable to avoid consumption of alcohol. PREGNANCY Consult your Doctor There have been no such effects listed but it is better to inform your doctor if you are pregnant or planning pregnancy before receiving Fabrazyme 5mg.  BREAST FEEDING Consult your Doctor The effects are not known yet. Let the doctor assess the benefits and the risks before prescribing Fabrazyme 5mg if you are breastfeeding before receiving. KIDNEY Safe No changes in dose are necessary for patients with kidney disease. Still a doctor’s consultation is adviced. Driving Caution There is a possibility of feeling dizziness, somnolence,vertigo so do not drive or operate heavy machinery on the day of administration. CHILDREN Unsafe Fabrazyme 5mg Powder should be kept out of the reach of children.

Habit Forming

No

Diet & Lifestyle Advices:

1. Follow a healthy diet.
2. It is advisable to get enough sleep.
3. Reduce intake of sugar-rich foods or drinks.
4. Drink more water,Stay hydrated.
5. Practice good hygiene.
6. Manage your stress levels.

Remember:

1. The powder for concentrate for solution for infusion has to be reconstituted with water for injections, diluted with 0.9% sodium chloride solution for injection and then administered by intravenous infusion. Aseptic technique should be used.

2. The number of vials should be determined to be reconstituted based on the individual patient's weight and the required vials should be removed from the refrigerator in order to allow them to reach room temperature (in approximately 30 minutes). Each vial of Fabrazyme is intended for single use only.

3. Each vial of Fabrazyme 5 mg has to be reconstituted with 1.1 ml water for injections. Forceful impact of the water for injections on the powder and foaming should be avoided. This is done by slow drop-wise addition of the water for injection down the inside of the vial and not directly onto the lyophilisate. Each vial should be rolled and tilted gently. The vial should not be inverted, swirled or shaken.

4. The reconstituted solution contains 5 mg agalsidase beta per ml, and appears as a clear colourless solution. The pH of the reconstituted solution is approximately 7.0. Before further dilution, the reconstituted solution in each vial should be visually inspected for particulate matter and discolouration. The solution should not be used if foreign particles are observed or if the solution is discoloured.

5. The initial infusion rate should be no more than 0.25 mg/min (15 mg/hour) to minimise the potential occurrence of infusion-associated reactions. After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.

FAQs:

1. What is the use of Fabrazyme 5 mg?

Fabrazyme 5mg is used as enzyme replacement therapy for people suffering from Fabry Disease.

2. How should Fabrazyme 5 mg be handled?

From a microbiological point of view, the medicinal product should be used immediately. If not used immediately, in-use storage and conditions of the medicinal product prior to use are the responsibility of the user. The reconstituted solution cannot be stored and should be promptly diluted; only the diluted solution can be held for up to 24 hours at 2°C-8°C.

3. What precautions should I take after receiving Fabrazyme 5 mg?

It is adviced to take doctor’s recommendation for intaking certain type of foods,beverages etc. Avoid doing any activity like driving or using machinery because of the side effects.

4. How long does the effect of Fabrazyme 5mg last?

Fabrazyme infusions are given by a healthcare professional, and they typically last from 1.5 hours to several hours. You'll be monitored during and after your infusion for symptoms of a reaction. Symptoms may occur at any time during an infusion.

5. How frequently should Fabrazyme 5mg be consumed?

The recommended dose of Fabrazyme for adults is 1 mg/kg body weight, once every 2 weeks and the dose for children and adolescents 8 – 16 years is 1 mg/kg body weight, once every 2 weeks.

6. What organ is affected by Fabry disease?

Fabry disease affects the heart, kidneys, brain, central nervous system and skin. It is an inherited condition passed from parent to child. It's sometimes called Anderson-Fabry disease.