Respreeza 4,000 mg powder and solvent for solution for infusion

• Keep this leaflet. You may need to read it again.
• If you have any further questions, ask your doctor or healthcare professional.
• This medicine has been prescribed for you only. Do not pass it on to others. It may harm them, even if their signs of illness are the same as yours.
• If you get any side effects, talk to your doctor or healthcare professional. This includes any possible side effects not listed in this leaflet. See section 4.

1. What Respreeza is and what it is used for
2. What you need to know before you use Respreeza
3. How to use Respreeza
4. Possible side effects
5. How to store Respreeza
6. Contents of the pack and other information

This medicine contains the active substance human alpha₁-proteinase inhibitor, which is a normal component of the blood and is found in the lung. There, its main function is to protect the lung tissue by limiting the action of a certain enzyme, called neutrophil elastase. Neutrophil elastase can cause damage if its action is not controlled (for example, in case you have an alpha₁-proteinase inhibitor deficiency).

This medicine is used in adults with known severe alpha₁-proteinase inhibitor deficiency (an inherited condition also called alpha₁ antitrypsin deficiency) who have developed a lung condition called emphysema.

Emphysema develops when the lack of alpha₁-proteinase inhibitor results in a condition in which neutrophil elastase is not being properly controlled, damaging the tiny air sacs in the lungs through which oxygen passes into the body. Because of this damage, the lungs do not work properly.

Using this medicine regularly increases the blood and lung levels of alpha₁-proteinase inhibitor thus slowing the progression of emphysema.

• if you are allergic to human alpha₁-proteinase inhibitor or any of the other ingredients of this medicine (listed in section 6).
• if you have been found to have a deficiency of certain blood proteins called immunoglobulin type A (IgA) and have developed antibodies against them.

• Talk to your doctor or healthcare professional before using Respreeza.

Information on allergic reactions: when slowing or stopping the infusion may be required?

You may be allergic to human alpha₁-proteinase inhibitor even if you have previously received human alpha₁-proteinase inhibitors and had tolerated them well. In some cases, severe allergic reactions may occur. Your doctor will inform you about signs of allergic reactions (for example chills, flushing, faster heartbeat, fall in blood pressure, light-headedness, rash, hives, itching, difficulty in breathing or swallowing as well as swelling of your hands, face, or mouth) (see also section 4).

• Tell your doctor or healthcare professional immediately if you notice such reactions during the infusion of this medicine. Depending on the nature and severity of the reaction, your doctor may decide whether to slow or stop the infusion completely and start the appropriate treatment.
• In case of self-administration / home-treatment, stop the infusion immediately and contact your doctor or healthcare professional.

Information on safety with respect to infections

Respreeza is made from human blood plasma (this is the liquid part of the blood with the blood cells removed).

Because blood can carry infections, when medicines are made from human blood or plasma certain measures are put in place to prevent these from being present in the medicine and passed on to patients. These include:

• careful selection of blood and plasma donors to make sure those at risk of carrying infections are excluded,
• the testing of samples of donated blood and plasma to try to avoid use of material with signs of virus/infections,
• the inclusion of steps in the processing of the blood or plasma that can inactivate or remove viruses.

The measures taken are considered effective for viruses such as human immunodeficiency virus (HIV), hepatitis A virus, hepatitis B virus, hepatitis C virus, and parvovirus B19 virus.

However, despite these measures, when medicines prepared from human blood or plasma are administered, the possibility of passing on infection cannot be totally excluded. This also applies to any unknown or emerging viruses or other types of infections.

Your doctor may recommend that you consider vaccination against hepatitis A and B if you regularly/repeatedly receive human plasma-derived proteinase inhibitors.

• It is strongly recommended that every time you receive a dose of Respreeza the name and batch number of the product are recorded in order to maintain a record of the batches used.

Smoking

Since tobacco smoke is an important risk factor for the development and progression of emphysema, you are strongly advised to stop smoking and avoid passive smoking.

This medicine is not for use in children or adolescents below 18 years of age.

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

• If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor or healthcare professional for advice before taking this medicine.

Since alpha₁-proteinase inhibitor is a normal component of human blood, the recommended dose of this medicine is not expected to cause harm to the developing foetus. However, as there is no information available regarding the safety of Respreeza use during pregnancy, if you are pregnant, this medicine should only be given to you with caution.

It is unknown whether Respreeza passes into human milk. If you are breast-feeding, your doctor will discuss with you the risks and benefits of taking this medicine.

There are no data concerning the effect on fertility but as alpha₁-proteinase inhibitor is a normal component of human blood, no adverse effects on fertility are expected if you use Respreeza at the recommended dose.

Dizziness may occur after the administration of this medicine. If you experience dizziness, you should not drive or use machines until the dizziness has passed (see section 4).

This medicinal product contains approximately 37 mg sodium per 1,000 mg Respreeza vial, 149 mg sodium per 4,000 mg Respreeza vial and 186 mg sodium per 5,000 mg Respreeza vial, equivalent to 1.9%, 7.4% and 9.3% respectively, of the WHO recommended maximum daily intake of 2 g sodium for an adult.. Your doctor or healthcare professional will take that into consideration if you are on a controlled sodium diet.

Uncommonly (may affect up to 1 in 100 people), allergic reactions have been observed. They may progress in some very rare cases (may affect up to 1 in 10,000 people) to severe allergic reactions even when you have shown no signs of allergy on previous infusions.

• Tell your doctor or healthcare professional immediately if you notice any sign of allergic reactions (for example chills, flushing, faster heartbeat, fall in blood pressure, light-headedness, rash, hives, itching, difficulty in breathing or swallowing as well as swelling of your hands, face, or mouth) during the administration of Respreeza. Depending on the nature and severity of the reaction, your doctor or healthcare professional may decide whether to slow or stop the administration completely and give appropriate treatment for the reaction.
  In case of self-administration / home-treatment, stop the infusion immediately and contact your doctor or healthcare professional.

The other side effects may include:

Commonly (may affect up to 1 in 10 people)

Dizziness, headache, shortness of breath (dyspnoea), nausea.

Uncommonly (may affect up to 1 in 100 people)

Altered sense of touch like burning, tingling or feeling of numbness in your hands, arms, legs, or feet (paraesthesia), flushing, hives (urticaria), scaly rash and rash all over the body, physical weakness (asthenia), infusion-site reactions (such as burning, stinging, pain, swelling or redness at the infusion site (haematoma)).

Very rarely (may affect up to 1 in 10,000 people)

Decreased sense of touch like burning, tingling or feeling of numbness in your hands, arms, legs, or feet (hypoaesthesia), excessive sweating (hyperhidrosis), itching, chest pain, chills, fever (pyrexia).

Frequency not known (frequency cannot be estimated from the available data)

Pain to the lymph glands (oval-shaped masses of tissue that are distributed throughout the body and which may be palpable for example in the armpit, groin or neck), swollen face, swollen eyes and lips.

• If you get any side effects, talk to your doctor or healthcare professional. This includes any possible side effects not listed in this leaflet. You can also report side effects directly (see details below). By reporting side effects you can help provide more information on the safety of this medicine.

UK:

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Malta:

Ireland:

The active substance is human alpha₁-proteinase inhibitor. One vial contains approximately 1,000 mg, 4,000 mg or 5,000 mg of human alpha₁-proteinase inhibitor.

The other ingredients are sodium chloride, sodium dihydrogen phosphate monohydrate and mannitol (see section 2).

Solvent: Water for injections.

This medicine is a white to off-white powder.

After it has been reconstituted with water for injections, the solution should be clear, colourless to slightly yellow and free from visible particles.

Presentations

One pack contains:

Respreeza 1,000 mg powder and solvent for solution for infusion:

• 1 single-use powder vial
• 1 solvent vial of 20 ml water for injections
• 1 transfer set 20/20 (Mix2Vial set) for reconstitution

Respreeza 4,000 mg powder and solvent for solution for infusion:

• 1 single-use powder vial
• 1 solvent vial of 76 ml water for injections
• 1 transfer set 20/20 (Mix2Vial set) for reconstitution

Administration set (inner box):

• 1 IV infusion set
• 1 butterfly set
• 3 alcohol swabs

Respreeza 5,000 mg powder and solvent for solution for infusion:

• 1 single-use powder vial
• 1 solvent vial of 95 ml water for injections
• 1 transfer set 20/20 (Mix2Vial set) for reconstitution

Administration set (inner box):

• 1 IV infusion set
• 1 butterfly set
• 3 alcohol swabs

Not all pack sizes may be marketed.

For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder: