C1 Esterase Inhibitor Subcutaneous (Human)
Freeze-Dried Powder for Reconstitution
This leaflet summarizes important information about
HAEGARDA. Please read it carefully before using HAEGARDA and each time you get
a refill. There may be new information provided. This information does not take
the place of talking with your healthcare provider, and it does not include all
of the important information about HAEGARDA. If you have any questions after
reading this, ask your healthcare provider.
Do not attempt to self-administer unless you have been
taught how by your healthcare provider.
What is HAEGARDA?
HAEGARDA is an injectable medicine used to prevent
swelling and/or painful attacks in adults and adolescents with Hereditary
Angioedema (HAE). HAE is caused by the poor functioning or lack of a protein
called C1 that is present in your blood and helps control inflammation (swelling)
and parts of the immune system. HAEGARDA contains C1 esterase inhibitor (C1-INH),
a protein that helps control C1.
HAEGARDA should not be used to treat an acute HAE attack.
In case of an acute HAE attack, initiate individualized treatment as discussed
with your prescribing health care professional.
Who should not use HAEGARDA?
You should not use HAEGARDA if you have experienced
life-threatening immediate hypersensitivity reactions, including anaphylaxis,
to the product.
What should I tell my healthcare provider before using
Tell your healthcare provider about all of your medical
conditions, including if you:
- Are pregnant or planning to become pregnant. It is not
known if HAEGARDA can harm your unborn baby.
- Are breastfeeding or plan to breastfeed. It is not known
if HAEGARDA passes into your milk and if it can harm your baby.
- Have a history of blood clotting problems. Blood clots
have occurred in patients receiving HAEGARDA. Very high doses of C1-INH could
increase the risk of blood clots. Tell your healthcare provider if you have a
history of heart or blood vessel disease, stroke, blood clots, or have thick
blood, an indwelling catheter/access device in one of your veins, or have been immobile
for some time. These things may increase your risk of having a blood clot after
using HAEGARDA. Also, tell your healthcare provider what drugs you are using,
as some drugs, such as birth control pills or certain androgens, may increase
your risk of developing a blood clot.
Tell your healthcare provider and pharmacist about all of
the medicines you take, including all prescription and non-prescription
medicines such as over-the-counter medicines, supplements, or herbal remedies.
What are the possible side effects of HAEGARDA?
Allergic reactions may occur with HAEGARDA. Call your
healthcare provider or seek emergency support services right away if you have
any of the following symptoms after using HAEGARDA:
- difficulty breathing
- chest tightness
- turning blue (look at lips and gums)
- fast heartbeat
- swelling of the face
- rash or hives
Signs of a blood clot include:
- pain and/or swelling of an arm or leg with warmth over
the affected area
- discoloration of an arm or leg
- unexplained shortness of breath
- chest pain or discomfort that worsens on deep breathing
- unexplained rapid pulse
- numbness or weakness on one side of the body
Because HAEGARDA is made from human blood, it may carry a
risk of transmitting infectious agents, e.g., viruses, the variant
Creutzfeldt-Jakob disease (vCJD) agent and, theoretically, the Creutzfeldt-Jakob
disease (CJD) agent.
The most common side effects with HAEGARDA are injection
site reactions (pain, redness, swelling), hypersensitivity (itching and rash),
nasopharyngitis (runny or stuffy nose, sneezing, watery eyes) and dizziness.
These are not all the possible side effects of HAEGARDA.
Tell your healthcare provider about any side effect that
bothers you or that does not go away. You can also report side effects to the
FDA at 1-800-FDA-1088.
How should I store HAEGARDA?
- Keep the non-reconstituted HAEGARDA in its original
carton to protect from light until ready to use.
- When stored at temperatures of 2-30°C (36-86°F), HAEGARDA
is stable for the period indicated by the expiration date on the carton and
- Do not freeze.
What else should I know about HAEGARDA?
Medicines are sometimes prescribed for purposes other
than those listed here. Do not use HAEGARDA for a condition for which it is not
prescribed. Do not share HAEGARDA with other people, even if they have the same
symptoms that you have.
This leaflet summarizes the most important information
about HAEGARDA. If you would like more information, talk to your healthcare
provider. You can ask your healthcare provider or pharmacist for information
about HAEGARDA that was written for healthcare professionals. For more
information, go to www.HAEGARDA.com or call 1-877-236-4423.
What should I know about self-administration?
- You should prepare the prescribed dose of HAEGARDA for
self-administration as directed by your healthcare provider.
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 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
- Prepare and administer using aseptic techniques.
- Each vial of HAEGARDA is for single-use 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
The procedures below are provided as general guidelines
for the reconstitution of HAEGARDA.
Table 1. 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.
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).
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).
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.
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.
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).
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).
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.
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-Administration (subcutaneous administration)
Your healthcare provider will teach you how to safely
administer HAEGARDA. Once you learn how to self-administer, follow the
instructions provided below.
Table 2: HAEGARDA Self-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 your healthcare provider)
Step 2: Clean surface
- Thoroughly clean a table or other flat surface using
Step 3: Wash hands
- Thoroughly wash and dry your hands.
- If you have been told to wear gloves when preparing your infusion,
put the gloves on.
Step 4: Prepare injection site
- Select an area on your abdomen (stomach) or another site
for the injection as discussed with your doctor (Figure 9).
- Use a different place from your 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 yourself an injection in areas where the skin
is itchy, swollen, painful, bruised, or red.
- Avoid giving yourself 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).
Step 5: Injection in the abdominal area
As instructed by your healthcare provider:
- Attach a hypodermic needle or S.C. infusion set
(butterfly) as instructed by your 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).
Injection by S.C Infusion Set:
- Insert the needle into the fold of skin (Figure 12).
Step 6: Clean up
- After injecting the entire amount of HAEGARDA, remove the
- 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 your
treatment diary or log book with the date and time of infusion every time you
Resources at CSL Behring available to the patient:
For Adverse Reaction Reporting contact:
CSL Behring Pharmacovigilance Department at
Contact CSL Behring to receive more product
Customer Support 1-800-683-1288
For more information, visit www.HAEGARDA.com.