VPRIV – Velaglucerase alfa uses, dose and side effects


400 units of powder for infusion solution,
velaglucerase alfa

What VPRIV is and what it is used for

VPRIV is a long-term enzyme replacement therapy (ERT) for patients with type 1 Gaucher disease.

Gaucher disease is a genetic disorder caused by the absence or defect of an enzyme called glucocerebrosidase. When this enzyme is missing or not working properly, a substance called glucocerebroside accumulates inside the body’s cells. The accumulation of this substance causes the signs and symptoms that occur in Gaucher disease.

VPRIV contains a substance called velaglucerase alfa that is designed to replace the missing or defective enzyme, glucocerebrosidase, in patients with Gaucher disease.

What you need to know before using VPRIV

Do not use VPRIV

  • if you are severely allergic to velaglucerase alfa or any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions

Talk to your doctor before using VPRIV.

  • If you are treated with VPRIV, you may experience side effects during or after the infusion (see section 4, Possible side effects ). These are called infusion-related reactions and can manifest as a hypersensitivity reaction with symptoms such as nausea, rash, difficulty breathing, back pain, chest discomfort (chest pressure), hives, joint pain, or headache.
  • In addition to symptoms of hypersensitivity reaction, infusion-related reactions can manifest as dizziness, high blood pressure, fatigue, fever, itching, blurred vision, or vomiting.
  • If you experience any of these symptoms, you must tell your doctor immediately.
  • You may be given additional medicines for treatment or to prevent future reactions. These drugs may include antihistamines, antipyretics, and corticosteroids.
  • If the reaction is severe, your doctor will immediately stop the intravenous infusion and start giving you appropriate medical treatment.
  • If the reactions are severe and/or the medicine loses its effect, your doctor will take a blood sample to check if there are antibodies that could affect the results of your treatment.
  • The doctor or nurse may decide to continue administering VPRIV even if you experience infusion-related reactions. Your condition will be closely monitored.

Tell your doctor if you have previously had any infusion-related reaction with other ERT for Gaucher disease.


Do not use in children younger than 4 years as there is no experience of using the medicine in this age group.

Other medicines and VPRIV

Tell your doctor if you are taking, have recently taken, or might take any other medicines.


Gaucher disease can become more active in a woman during pregnancy and a few weeks after giving birth. Women with Gaucher disease who are pregnant or planning to become pregnant should talk to their doctor before using this medicine.


It is not known whether VPRIV passes into breast milk. If you are breastfeeding or plan to breastfeed, talk to your doctor before using this medicine. The doctor will then help you decide whether to stop breastfeeding or whether to stop using VPRIV, taking into account the benefit of breastfeeding for the baby and the benefit of VPRIV for the mother.

Driving ability and use of machinery

VPRIV has no or negligible effect on the ability to drive or use machines.

VPRIV contains sodium

This medicine contains 12.15 mg of sodium (the main ingredient in common/table salt) per vial. This corresponds to 0.6% of the maximum recommended daily intake of sodium for adults.

How to use VPRIV

This medicine should only be used under appropriate medical supervision by a physician experienced in the treatment of Gaucher’s disease. It is given by a doctor or nurse by intravenous infusion.


The recommended dose is 60 units/kg given every two weeks.

If you are currently being treated for Gaucher disease with another ERT and your doctor wants you to switch to VPRIV, you can initially receive VPRIV at the same dose and frequency as you have received the other ERT.

Use for children and adolescents

VPRIV can be given to children and adolescents (aged 4 to 17 years) at the same dose and frequency as in adults.


VPRIV can be given to the elderly (over 65 years of age) at the same dose and frequency as in other adults.

Treatment response

Your doctor will monitor your response to treatment and may change your dose (up or down) over time.

If you tolerate your infusions well at the clinic, your doctor or nurse can give you your infusions at home.


VPRIV is supplied in a vial as a packed powder that is mixed with sterile water and then further diluted in sodium chloride 9 mg/ml (0.9%) solution for infusion before intravenous infusion.

After preparation, your doctor or nurse gives you the medicine as a drip into a vein (by intravenous infusion ) over 60 minutes.

If you have any further questions about this medicine, ask your doctor or pharmacist.

Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.

Commonly (may affect up to 1 in 10 people) patients experienced a serious allergic reaction with difficulty breathing, chest discomfort (chest pressure), nausea, swelling of the face, lips, tongue, or throat (anaphylactic/anaphylactoid reactions), commonly also an allergic skin reaction such as hives, severe rash or itching. If any of these occur, tell your doctor immediately.

Most side effects, including allergic reactions, occurred during or shortly after the infusion. These are called infusion-related reactions. Other infusion-related reactions that occurred very commonly (may affect more than 1 in 10 people) include headache, dizziness, fever/increased body temperature, back pain, joint pain and tiredness, as well as high blood pressure (commonly reported), blurred vision and vomiting (less commonly reported). . If any of these occur, tell your doctor immediately.

Other side effects include:

Very common side effects (may affect more than 1 in 10 people):

  • bone pain
  • weakness/powerlessness
  • stomach pain

Common side effects (may affect up to 1 in 10 people):

  • prolongation of the time it takes for a cut to stop bleeding which may lead to easy/spontaneous bleeding/easy bruising
  • redness
  • rapid heartbeat
  • development of antibodies against VPRIV (see section 2)
  • lowered blood pressure

How to store VPRIV

Keep this medicine out of the sight and reach of children.

Use before the expiry date stated on the carton and label after “EXP”. The expiration date is the last day of the specified month.

Store in a refrigerator (2 ºC–8 ºC).

Do not freeze.

Store the vial in the outer carton. Light sensitive.

Prepared and diluted solution for infusion:

Use immediately. Do not leave for longer than 24 hours at 2–8 °C.

Do not use VPRIV if the solution is discolored or foreign particles are present.

Medicines must not be thrown into the drain or among the household waste. Ask the pharmacist how to dispose of medicines that are no longer used. These measures will help to protect the environment.

Contents of the packaging and other information

Contents declaration

  • The active substance is velaglucase alfa. Each vial contains 400 units of velaglucase alfa.
    After preparation, one ml of solution contains 100 units of velaglucase alfa.
  • The other ingredients are sucrose, sodium citrate dihydrate, citric acid monohydrate, and polysorbate 20 (see section 2, “VPRIV contains sodium”).

Appearance and package sizes of the medicine

20 ml glass vial containing a white to off-white powder for infusion solution, solution.

Packs of 1, 5, or 25 vials.

Not all pack sizes may be marketed.

Marketing authorization holder and manufacturer

Marketing authorization holder and manufacturer

Marketing Authorisation Holder

Takeda Pharmaceuticals International AG Ireland Branch

Block 2Miesian Plaza

50-58 Baggot Street Lower

Dublin 2



Takeda Pharmaceuticals International AG Ireland Branch
Block 2 Miesian Plaza
50-58 Baggot Street Lower
Dublin 2
D02 HW68 Ireland

Shire Pharmaceuticals Ireland Limited

Block 2 & 3 Miesian Plaza

50 – 58 Baggot Street Lower

Dublin 2


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