Instructions for Use

Do not attempt to self-administer unless you have been taught how by your healthcare provider.
See the step-by-step instructions for injecting HAEGARDA at the end of this leaflet. You should always follow the specific instructions given by your healthcare provider. The steps listed below are general guidelines for using HAEGARDA. If you are unsure of the steps, please contact your healthcare provider or pharmacist before using.
Your healthcare provider will prescribe the dose that you should administer, which is based on your body weight.
Call your healthcare provider if you miss a dose of HAEGARDA.
Talk to your healthcare provider before traveling to make sure you have an adequate supply of HAEGARDA.
Use a new needle for each HAEGARDA injection. Do not reuse or share your needles with other people. You may give other people a serious infection, or get a serious infection from them.

Reconstitution and Administration

The 2000 IU HAEGARDA vial contains C1-INH as a lyophilized concentrate for reconstitution with 4 mL of Sterile Water for Injection, USP provided; or, the 3000 IU HAEGARDA vial contains C1-INH as a lyophilized concentrate for reconstitution with 5.6 mL of Sterile Water for Injection, USP provided.
Check the expiration date on the product vial label. Do not use beyond the expiration date.
Work on a clean surface and wash hands before performing the following procedures.
Use either the Mix2Vial transfer set provided with HAEGARDA or a commercially available double-ended needle and vented filter spike.
Prepare and administer using aseptic techniques.
Each vial of HAEGARDA is for single-dose only. Promptly use the reconstituted solution. The solution must be used within 8 hours. Discard partially used vials. HAEGARDA contains no preservative.
After reconstitution and prior to administration inspect HAEGARDA. The reconstituted solution should be colorless, clear, and free from visible particles. Do not use if the solution is cloudy, discolored, or contains particulates.

Reconstitution

The procedures below are provided as general guidelines for the reconstitution of HAEGARDA.

HAEGARDA Reconstitution Instructions

1.: Ensure that the HAEGARDA vial and Sterile Water for Injection (diluent) vial are at room temperature.

2.: Place the HAEGARDA vial, diluent vial and Mix2Vial transfer set on a flat surface.

3.: Remove flip caps from the HAEGARDA and diluent vials.

4.: Wipe the stoppers with an alcohol swab and allow to dry prior to opening the Mix2Vial transfer set package.

5.: Open the Mix2Vial transfer set package by peeling away the lid (Figure 1). Do not remove the device from the package.

Figure 1

6.: Place the diluent vial on a flat surface and hold the vial tightly. Grip the Mix2Vial transfer set together with the clear package and push the plastic spike at the blue end of the Mix2Vial transfer set firmly through the center of the stopper of the diluent vial (Figure 2).

Figure 2

7.: Carefully remove the clear package from the Mix2Vial transfer set. Do not remove the Mix2Vial transfer set or touch the exposed end of the device (Figure 3).

Figure 3

8.: With the HAEGARDA vial placed firmly on a flat surface, invert the diluent vial with the Mix2Vial transfer set attached and push the plastic spike of the transparent adapter firmly through the center of the stopper of the HAEGARDA vial (Figure 4). The diluent will automatically transfer into the HAEGARDA vial.

Figure 4

9.: With the diluent and HAEGARDA vial still attached to the Mix2Vial transfer set, gently swirl the HAEGARDA vial to ensure that the powder is fully dissolved (Figure 5). Do not shake the vial.

Figure 5

10.: With one hand, grasp the HAEGARDA side of the Mix2Vial transfer set and with the other hand grasp the blue diluent side of the Mix2Vial transfer set, and unscrew the set into two pieces (Figure 6).

Figure 6

11.: Draw air into an empty, sterile syringe. Use a silicone-free syringe. While the HAEGARDA vial is upright, screw the syringe to the Mix2Vial transfer set. Inject air into the HAEGARDA vial.

12.: While keeping the syringe plunger pressed, invert the system upside down and draw the concentrate into the syringe by pulling the plunger back slowly (Figure 7).

Figure 7

13.: Disconnect the filled syringe by unscrewing it from the Mix2Vial transfer set (Figure 8). The reconstituted solution should be colorless, clear, and free from visible particles. Do not use if particles or discoloration are observed.

Figure 8

14.: Use immediately or within 8 hours of reconstitution. Store reconstituted solution at room temperature. Do not refrigerate.

15.: If the dose requires more than one vial, use a separate, unused Mix2Vial transfer set and diluent vial for each product vial. Repeat steps 10-12 to pool the contents of the vials into one syringe.

Self (or caregiver)-Administration (subcutaneous administration)

Your healthcare provider will teach you (or caregiver) how to safely administer HAEGARDA. Once you (or caregiver) learn how to administer, follow the instructions provided below.

HAEGARDA administration instructions apply to patients, 6 years of age and older.

HAEGARDA Self (or caregiver)-Administration Instructions

Step 1: Assemble supplies

Gather the HAEGARDA syringe, the following disposable supplies (not provided with HAEGARDA), and other items (sharps or other container, treatment diary or log book):

Hypodermic needle or S.C. infusion set
Sterile syringe (Use a silicone-free syringe)
Alcohol wipes
Gloves (if recommended by the healthcare provider)

Step 2: Clean surface

Thoroughly clean a table or other flat surface using alcohol wipes.

Step 3: Wash hands

Thoroughly wash and dry your hands.
If you have been told to wear gloves when preparing the infusion, put the gloves on.

Step 4: Prepare injection site

Select an area on the abdomen (stomach) or another site for the injection as discussed with your doctor (Figure 9).

Figure 9

Use a different place from the last injection; you should rotate the places where you are injecting.
New injection sites should be at least 2 inches (5 centimeters) away from the place where you gave yourself an injection before.
Never give an injection in areas where the skin is itchy, swollen, painful, bruised, or red.
Avoid giving injections in places where you have scars or stretch marks.
Clean the skin at the injection site with an alcohol swab and let the skin dry (Figure 10).

Figure 10

Step 5: Injection in the abdominal area

As instructed by the healthcare provider:

Attach a hypodermic needle or S.C. infusion set (butterfly) as instructed by the healthcare provider. Prime the needle or tubing as required and instructed.

Injection with Hypodermic Needle:

Insert the needle into the fold of skin (Figure 11).

Figure 11

Injection by S.C Infusion Set:

Insert the needle into the fold of skin (Figure 12).

Figure 12

Step 6: Clean up

After injecting the entire amount of HAEGARDA, remove the needle.
Discard any unused solution and all administration equipment in an appropriate manner as per local requirements.

Step 7: Record treatment

Record the lot number from the HAEGARDA vial label in the treatment diary or log book with the date and time of infusion every time you use HAEGARDA.

Resources at CSL Behring available to the patient:
For Adverse Reaction Reporting contact:
CSL Behring Pharmacovigilance Department at 1-866-915-6958

Contact CSL Behring to receive more product information:
Customer Support 1-800-683-1288

For more information, visit www.HAEGARDA.com.

Manufactured by:
CSL Behring GmbH
35041 Marburg, Germany
US License No. 1765

Distributed by:
CSL Behring LLC
Kankakee, IL 60901 USA

Mix2Vial® is a registered trademark of West Pharma. Services IL, Ltd., a subsidiary of West Pharmaceuticals Services, Inc.