1,000 mg, 4,000 mg and 5,000 mg powder and liquid for infusion , solution
human alpha-1-proteinase inhibitor
What Respreeza is and what it is used for
What Respreeza is
This medicine contains the active substance human alpha-1-proteinase inhibitor, which is a normal component of the blood and is found in the lungs. 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 harm if its effect is not controlled (for example, if you are deficient in alpha-1 proteinase inhibitors).
What Respreeza is used for
This medicine is used in adults with known, severe alpha-1-proteinase inhibitor deficiency (a hereditary disease also known as the alpha-1-antitrypsin deficiency) who have developed a lung disease called emphysema.
Emphysema develops when the lack of alpha-1-proteinase inhibitors leads to a condition where neutrophil elastase is not sufficiently controlled, which damages the small air bubbles in the lung through which oxygen passes into the body. Due to this injury, the lungs do not function normally.
Regular use of this drug increases blood and lung levels of alpha-1-proteinase inhibitors and thus slows the development of emphysema.
What you need to know before using Respreeza
Ta NOT Respreeza
- if you are allergic to human alpha-1 proteinase inhibitors or any of the other ingredients of this medicine (listed in section 6).
- if you are deficient in certain blood proteins called type A immunoglobulin ( IgA ) and have developed antibodies against them.
Warnings and cautions
- Talk to your doctor or healthcare professional before using Respreeza.
Information on allergic reactions: when to infuse one must be reduced or discontinued?
You may be allergic to human alpha-1-proteinase inhibitors even if you have previously received human alpha-1-proteinase inhibitors and tolerated them well. In some cases, severe allergic reactions may occur. Your doctor will tell you about signs of allergic reactions (eg chills, redness, faster heartbeat, drop in blood pressure, dizziness, rash, urticaria, itching, difficulty breathing or swallowing as well as swelling of the hands, face or mouth) (see also section 4).
- Tell your doctor or healthcare professional immediately if you notice any such reactions during the infusion of this medicine. Depending on the type and severity of the reaction, your doctor or healthcare professional may decide to slow down or stop the infusion completely and initiate appropriate treatment.
- In case of self-administration / treatment at home, the infusion should be stopped immediately and a doctor or healthcare professional should be contacted.
Safety information regarding your infection
Respreeza is made from human blood plasma (this is the liquid part of the blood with the blood cells removed).
Because blood can contain the infection, special measures are taken when drugs are made from human blood or plasma, to prevent them from being present in the drug and transferred to patients. These measures include:
- a careful selection of blood and plasma donors to ensure that people at risk of being infected are excluded;
- testing of samples of donated blood and plasma to try to avoid the use of materials with signs of virus/infection.
- introduction of steps in the preparation of blood or plasma that can inactivate or remove viruses.
The measures taken are considered effective against viruses such as human immunodeficiency virus ( HIV ), hepatitis A virus, hepatitis B virus, hepatitis C virus, and parvovirus B19.
Despite these measures, however, the risk of transmission of infection can not be completely ruled out when drugs made from human blood or plasma are given. This also applies to new, hitherto unknown viruses or other types of infection.
Your doctor may suggest hepatitis A and B vaccination if you are regularly / repeatedly treated with human plasma-derived protein inhibitors.
- It is strongly recommended that each time you receive a dose of Respreeza, the product name and batch number of the medicine should be registered to maintain a list of used kits.
Smoking
As smoking is an important risk factor for the development and progression of emphysema, it is strongly recommended that you stop smoking and avoid passive smoking.
Children and young people
This medicine should not be used by children or adolescents under 18 years of age.
Other medicines and Respreeza
- Tell your doctor or healthcare professional if you are taking, have recently taken, or might take any other medicines.
Pregnancy, breastfeeding, and fertility
- If you are pregnant or breastfeeding, think you may be pregnant, or are planning to have a baby, ask your doctor or healthcare professional for advice before taking this medicine.
Because alpha-1 proteinase inhibitors are normally present in human blood, the recommended dose of this medicine is not expected to cause harm to the growing fetus. However, as there is no safety information available when using Respreeza during pregnancy, this medicine should only be given to you with caution if you are pregnant.
It is unknown whether Respreeza passes into human breast milk. If you are breastfeeding, your doctor will discuss the risks and benefits of taking this medicine.
There are no data on the effect on fertility, but as alpha-1-proteinase inhibitors are a normal component of human blood, no adverse effects on fertility are expected when using Respreeza at the recommended dose.
Driving and using machines
Dizziness may occur after the administration of this medicine. If you become dizzy, do not drive or use machines until the dizziness has passed (see section 4).
Respreeza contains sodium
This medicine contains approximately 37 mg sodium per vial of Respreeza 1,000 mg, 149 mg sodium per vial of Respreeza 4,000 mg, and 186 mg of sodium per vial of Respreeza 5,000 mg. This corresponds to 1.9%, 7.4%, and 9.3% respectively of the WHO maximum recommended daily intake of 2 g sodium for adults. Your doctor or healthcare professional will take this into account if you have been prescribed a low-salt diet.
How to use Respreeza
After reconstitution, Respreeza is given as an infusion into a friend. Healthcare professionals experienced in the treatment of alpha-1-proteinase inhibitor deficiency will monitor the first infusions.
Treatment at home / self-administration
After the first infusions, you or your healthcare provider can also administer Respreeza, but only after receiving adequate training. If your doctor decides that you are suitable for such treatment at home / self-administration, he or she will instruct you on:
- how to prepare and administer this medicine (see the illustrated instructions at the end of this leaflet in “Tasks for healthcare professionals and for patients considered suitable for home treatment / self-administration”),
- how to keep the product sterile (aseptic infusion techniques),
- how to keep a treatment diary,
- how to identify side effects, including signs of allergic reactions, and measures to be taken if such side effects occur (see also section 2 and section 4).
Your doctor or healthcare professional will regularly review your or your healthcare provider’s infusion technology to ensure that continued handling is appropriate.
Dosage
The amount of Respreeza you receive is based on your body weight. The recommended dose is 60 mg per kg body weight and should be administered once a week. The infusion solution is normally given for about 15 minutes (about 0.08 ml solution per kg body weight every minute). Your doctor will determine the appropriate infusion rate for you by taking into account your weight and how you tolerate the infusion.
If you use more Respreeza than you should
The consequences of an overdose are not known.
- Tell your doctor or healthcare professional if you think you have used too much Respreeza. He or she will take appropriate action.
If you forget to use Respreeza
- Continue with the next dose immediately and continue at regular intervals recommended by your doctor or healthcare professional.
- Do not take a double dose to make up for a forgotten dose.
If you stop using Respreeza
- Do not stop using this medicine without consulting your doctor or healthcare professional. If treatment with Respreeza is stopped, your condition may worsen.
Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them. Such side effects may occur even if you have previously received human alpha-1 proteinase inhibitors and have tolerated them well.
Some side effects can be serious:
In less common cases (which may affect up to 1 in 100 people) allergic reactions have been observed. In some very rare cases (which may affect up to 1 in 10,000 people) they may develop into severe allergic reactions even if you have not shown any signs of allergy during previous infusions.
- Tell your doctor or healthcare professional immediately if you notice signs of allergic reactions (eg chills, redness, faster heartbeat, drop in blood pressure, feeling unsteady, skin rash, hives, itching, difficulty breathing or swallowing, swelling of hands, face or mouth) under the administration of Respreeza. Depending on the type and severity of the reaction, your doctor or healthcare professional may decide to slow down or stop the administration altogether and provide appropriate treatment for the reaction. In case of self-administration / treatment at home, stop the infusion immediately and contact your doctor or healthcare professional.
Other side effects can be:
Common (may affect up to 1 in 10 people)
Dizziness, headache, shortness of breath ( dyspnoea ), nausea.
Uncommon (may affect up to 1 in 100 people)
Altered sensations such as burning sensation, tingling or numbness in the hands, arms, legs, or feet ( paresthesia ), redness, hives ( urticaria ), scaly rash and rash all over the body, physical weakness ( asthenia ), reactions to the infusion site (such as burning, tingling, pain, swelling or redness at the infusion site (hematoma)).
Very rare (may affect up to 1 in 10,000 people)
Decreased sensations such as burning sensation, tingling, or feeling of numbness in hands, arms, legs, or feet ( hypoesthesia ), excessive sweating ( hyperhidrosis ), itching, chest pain, chills, fever ( pyrexia ).
No known frequency (frequency can not be calculated from available data)
Pain in the lymph nodes (oval tissue nodules that are distributed over the body and may be palpated (feel with light pressure) in, for example, armpits, groin, or neck), swelling of the face, swelling of the eyes and lips.
How to store Respreeza
Keep this medicine out of the sight and reach of children.
Do not use this medicine after the expiry date which is stated on the carton and bottle label after EXP. The expiration date is the last day of the specified month.
Do not store above 25 ° C. Do not freeze.
After reconstitution, the solution should be used immediately. If this is not possible, the solution can be stored for up to 3 hours at room temperature (up to 25 ° C). The prepared solution must not be frozen.
Contents of the pack and other information
Content declaration
The active substance is a human alpha-1 proteinase inhibitor. One vial contains approximately 1,000 mg, 4,000 mg, or 5,000 mg of human alpha-1 proteinase inhibitors.
The other ingredients are sodium chloride, sodium dihydrogen phosphate monohydrate, and mannitol (see section 2).
Liquid: Water for injections.
What the medicine looks like and the contents of the pack
This medicine is a white to off-white powder.
After reconstitution with water for injections, the solution should be clear, colorless to pale yellow, and free from visible particles.
Packaging
A package contains:
Respreeza 1,000 mg powder and solvent for solution for infusion :
- 1 single-use powder vial
- 1 vial with 20 ml water for injections
- 1 transfer set 20/20 (Mix2Vial set) for preparation
Respreeza 4,000 mg powder and solvent for solution for infusion :
- 1 single-use powder vial
- 1 vial of 76 ml water for injections
- 1 transfer set 20/20 (Mix2Vial set) for preparation
Administration set (inner packaging):
- 1 iv infusion set
- 1 set of a butterfly needle
- 3 alcohol dryers
Respreeza 5 000 mg powder and solvent for solution for infusion :
- 1 single-use powder vial
- 1 vial of 95 ml water for injections
- 1 transfer set 20/20 (Mix2Vial set) for preparation
Administration set (inner packaging):
- 1 iv infusion set
- 1 set of a butterfly needle
- 3 alcohol dryers
Not all pack sizes may be marketed.
Marketing Authorization Holder and Manufacturer
CSL Behring GmbH
Emil-von-Behring-Strasse 76
D-35041 Marburg
Germany