Zemaira contain the protein alpha 1-proteinase, also called alpha 1-antitrypsin. This protein occurs naturally in the body and is important for preventing the breakdown of tissues in the lungs.

In people who lack the alpha 1-antitrypsin protein, breakdown of lung tissues can lead to emphysema (damage to the air sacs in the lungs).

Zemaira is used to treat alpha 1-antitrypsin deficiency in people who have symptoms of emphysema.

Alpha 1-antitrypsin deficiency is a genetic (inherited) disorder and Zemaira will not cure this condition.

You should not use Zemaira if you have ever had an allergic reaction to alpha 1-proteinase inhibitor, or if you have an IgA (immunoglobulin A) deficiency or antibody against IgA.

Zemaira must be mixed with a liquid (diluent) before given as an injection. If you are using the injections at home, be sure you understand how to properly prepare and store your medicine.

Call your doctor at once if you have a serious side effect such as fever, chills, body aches, flu symptoms, mouth sores, pain or burning when you urinate, wheezing, chest pain or tightness, trouble breathing, or vision changes. Zemaira is made from human plasma (part of the blood) which may contain viruses and other infectious agents. Donated plasma is tested and treated to reduce the risk of it containing infectious agents, but there is still a small possibility it could transmit disease. Talk with your doctor about the risks and benefits of using this medication.

Before taking this medicine

You should not use Zemaira if:

• you've had a severe allergic reaction to an alpha 1-proteinase inhibitor; or

• you have an IgA (immunoglobulin A) deficiency or antibody against IgA.

To make sure Zemaira is safe for you, tell your doctor if you have:

• liver disease; or

• chronic obstructive pulmonary disease (COPD), or other breathing disorder.

Tell your doctor if you are pregnant or breastfeeding.

Zemaira in not approved for use by anyone younger than 18 years old.

Zemaira is made from donated human plasma and may contain viruses or other infectious agents. Donated plasma is tested and treated to reduce the risk of contamination, but there is still a small possibility it could transmit disease. Ask your doctor about any possible risk.

Take Zemaira exactly as prescribed by your doctor. Follow all directions on your prescription label and read all medication guides or instruction sheets.

Zemaira is injected into a vein, usually once per week.

Your first dose may be given in a medical setting where any serious side effects can be quickly treated.

Ask your doctor or pharmacist if you don't understand how to use an injection.

Be sure you understand how to properly mix the powder form of Zemaira with a liquid (diluent) and how to store the mixture.

Prepare an injection only when you are ready to give it. Call your pharmacist if the medicine looks cloudy, has changed colors, or has particles in it.

Do not reuse a needle or syringe. Place them in a puncture-proof "sharps" container and dispose of it following state or local laws. Keep out of the reach of children and pets.

Doses are based on weight. Your dose may change if you gain or lose weight.

Store Zemaira at cool room temperature, away from moisture and heat. Use within 3 hours after mixing.

Do not freeze Zemaira, and do not use it past the expiration date on the medicine label.

Throw away a vial (bottle) after one use, even if there is still medicine left inside.

Usual Adult Dose for Alpha-1 Proteinase Inhibitor Deficiency:

60 mg/kg intravenously once a week

Comments:
-Dose ranging studies using efficacy endpoints have not been performed.

Use: Chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to hereditary deficiency of Alpha 1-Proteinase Inhibitor (alpha 1-antitrypsin deficiency)

You should not use Zemaira if:

• you've had a severe allergic reaction to an alpha 1-proteinase inhibitor; or

• you have an IgA (immunoglobulin A) deficiency or antibody against IgA.

To make sure Zemaira is safe for you, tell your doctor if you have:

• liver disease; or

• chronic obstructive pulmonary disease (COPD), or other breathing disorder.

Tell your doctor if you are pregnant or breastfeeding.

Zemaira in not approved for use by anyone younger than 18 years old.

Zemaira is made from donated human plasma and may contain viruses or other infectious agents. Donated plasma is tested and treated to reduce the risk of contamination, but there is still a small possibility it could transmit disease. Ask your doctor about any possible risk.

Call your doctor for instructions if you miss a dose.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Get emergency medical help if you have signs of an allergic reaction to Zemaira: hives, rash; confusion, feeling light-headed; fast heartbeats, chest tightness, difficult breathing; swelling of your face, lips, tongue, or throat.

Your doctor may also prescribe epinephrine (Epi-Pen) to keep with you in case you ever have an allergic reaction to Zemaira.

Stop using Zemaira and call your doctor at once if you have:

• wheezing, chest pain, trouble breathing;

• dizziness, severe headache;

• a seizure; or

• sudden numbness or weakness, slurred speech, problems with vision or balance.

You may feel faint during the injection. You may need to rest for a short time afterward.

Common Zemaira side effects may include:

• cold symptoms such as runny or stuffy nose, sneezing, cough, sore throat;

• headache;

• muscle or joint pain;

• weakness;

• flushing (sudden warmth, redness, or tingly feeling);

• nausea; or

• bruising or bleeding where the medicine was injected.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.