This medication is used to treat lung problems (emphysema) caused by a certain inherited disease (alpha-1-proteinase inhibitor deficiency). In people with this condition, lung damage is caused by elastase, a natural substance that the body needs to kill bacteria in the lungs. Normally, a protein (alpha-1-proteinase inhibitor) stops elastase from working when it is no longer needed. However, in people who do not make enough of this protein, elastase does not stop working and damages the lungs. This medication replaces the missing alpha-1-proteinase inhibitor and helps to prevent further lung damage.

This medication is given by injection into a vein as directed by your doctor, usually once a week.

If you are giving this medication at home, learn all preparation and usage instructions in the product package and from your health care professional. If any of the information is unclear, consult your doctor or pharmacist. If your brand of medication is stored in the refrigerator, allow the medication and mixing solution to warm to room temperature before mixing. Do not shake the mixed drug. Doing so may decrease the effectiveness of this medication.

Before using, check this product for particles or discoloration. Occasionally, a few small particles may remain in the vial. These particles will be removed by the filter provided with the medication. If the product is discolored, do not use the liquid.

Learn how to store and discard needles and medical supplies safely. Consult your pharmacist for details.

Dosage is based on your weight, medical condition and response to treatment.

Use this medication regularly to get the most benefit from it. To help you remember, use it on the same day each week. Mark your calendar with a reminder.

Tell your doctor if your condition gets worse after several weeks.

Pain at the injection site, fever, dizziness, headache, drowsiness, numb/tingling skin, and muscle aches may occur. If any of these effects last or get worse, tell your doctor or pharmacist promptly.

Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.

A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including:

• rash
• itching/swelling (especially of the face/tongue/throat)
• severe dizziness
• trouble breathing

This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.

In the US - Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.

In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

Before using alpha-1-proteinase inhibitor, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.

Before using this medication, tell your doctor or pharmacist your medical history, especially of:

• selective IgA deficiency with antibodies to IgA
• liver disease
• heart problems (such as heart failure)

This drug may make you dizzy or drowsy. Alcohol or marijuana (cannabis) can make you more dizzy or drowsy. Do not drive, use machinery, or do anything that needs alertness until you can do it safely. Limit alcoholic beverages. Talk to your doctor if you are using marijuana (cannabis).

This medication is made from human blood. Even though the blood is carefully tested, and this medication goes through a special manufacturing process, there is an extremely small chance that you may get infections from the medication (for example, viruses such as hepatitis). Consult your doctor or pharmacist for more information.

During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.

It is unknown if this drug passes into breast milk. Consult your doctor before breastfeeding.

Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.

If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call 1-800-222-1222. Canada residents can call 1-844-764-7669.

Do not share this medication with others.

Lab and/or medical tests (such as lung function) should be done while you are using this medication. Keep all medical and lab appointments. Consult your doctor for more details.

If you miss a dose, ask your doctor or pharmacist right away for a new dosing schedule.

Different brands of this medication have different storage requirements. Ask your pharmacist how to store your brand. Do not freeze. After mixing this drug, do not refrigerate. Use the mixed drug within 3 hours. Discard any unused portion. Keep all medications away from children and pets.

Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.