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Overview: C1 esterase inhibitor (human) is commonly used for a rare condition called hereditary angioedema (HAE). People with this condition have too little of a substance called C1 esterase inhibitor. C1 esterase inhibitor (human) replaces this substance, which helps prevent swelling attacks. Common side effects include discomfort near the injection site, headache, nausea or vomiting, serious allergic reactions, infections in the nose or throat, and dizziness. Some C1 esterase inhibitor (human) products are given as an injection into the vein (IV) and others are given as an injection under the skin.

Uses

What is C1 esterase inhibitor (human) used for?

C1 esterase inhibitor (human) is commonly used for a rare, inherited condition called hereditary angioedema (HAE).

C1 esterase inhibitor (human) may also be used for other conditions as determined by your health care provider.

See also: Ruconest (C1 esterase inhibitor [recombinant]) and Berinert (C1 esterase inhibitor [human])

How does C1 esterase inhibitor (human) work (mechanism of action)?

People with hereditary angioedema have too little of a substance called C1 esterase inhibitor. This substance helps to block the process that causes swelling attacks. C1 esterase inhibitor (human) replaces this substance, which helps to prevent swelling attacks in people with this condition.

How is C1 esterase inhibitor (human) supplied (dosage forms)?

Brands and other names

  • Cinryze
  • Haegarda

Dosage forms and strengths

  • 500 IU single-dose vials
  • 2000 IU or 3000 IU single-dose vials

How should I store C1 esterase inhibitor (human)?

Vials for Intravenous (IV) Injection. C1 esterase inhibitor (human) should be stored in the refrigerator or at room temperature, between 36 F and 77 F (2 C and 25 C). Store the medicine in its original package to protect it from light. Do not freeze.

Vials for Injection Under the Skin. C1 esterase inhibitor (human) should be stored at temperatures no higher than 86 F (30 C). Store the medicine in its original package to protect it from light. Do not freeze.

Side Effects

What are the most common side effects of C1 esterase inhibitor (human)?

The most common side effects of C1 esterase inhibitor (human) are listed below. These side effects may vary depending on the specific product you are using. Tell your health care provider if you have any of these side effects that bother you.

  • Headache
  • Nausea or vomiting
  • Fever or rash
  • Pain, itching, swelling, or redness near the injection site
  • Serious allergic reactions (see below)
  • Infection in the nose or throat, sore throat, or common cold symptoms
  • Dizziness

There may be other side effects of C1 esterase inhibitor (human) that are not listed here. Contact your health care provider if you think you are having a side effect of a medicine. In the U.S., you can report side effects to the FDA at www.fda.gov/medwatch or by calling 800-FDA-1088 (800-332-1088). In Canada, you can report side effects to Health Canada at www.health.gc.ca/medeffect or by calling 866-234-2345.

What are the serious side effects of C1 esterase inhibitor (human)?

While less common, the most serious side effects of C1 esterase inhibitor (human) are described below, along with what to do if they happen.

Severe Allergic Reactions. C1 esterase inhibitor (human) may cause allergic reactions, which can be serious. Use the rescue medicine (such as epinephrine) provided to you by your health care provider and get help right away if you have any of the following symptoms of a serious allergic reaction.

  • Breathing problems or wheezing
  • Racing heart
  • Fever or general ill feeling
  • Swollen lymph nodes
  • Swelling of the face, lips, mouth, tongue, or throat
  • Trouble swallowing or throat tightness
  • Itching, skin rash, or pale red bumps on the skin called hives
  • Nausea or vomiting
  • Dizziness, feeling lightheaded, or fainting
  • Stomach cramps
  • Joint pain

Blood Clots. C1 esterase inhibitor (human) may cause a blood clot in your arteries or veins and could cause a stroke, heart attack, or venous thromboembolism, including a blood clot in the lungs (pulmonary embolism) or deep vein thrombosis (DVT). Get emergency help if you have any of the following signs of blood clots.

  • Chest pain or pressure
  • Fast or abnormal heartbeat
  • Trouble breathing
  • Pain in your back, jaw, throat, or arm
  • Sweating, upset stomach, vomiting, or dizziness
  • Sudden weakness on one side of your body
  • Sudden and severe headache
  • Slurred speech or trouble understanding speech
  • Pain, swelling, or tenderness in your arm or leg
  • Vision loss
  • Bulging eyes
  • Double vision

Risk of Infection. C1 esterase inhibitor (human) is made from human blood, which means that it may contain microbes (such as bacteria or viruses) that can cause an infection. This risk is very low because the process used to make this medicine includes many steps to remove microbes. Talk to your health care provider to make sure that you understand this potential risk.

Warnings & Precautions

Who should not use C1 esterase inhibitor (human)?

Allergies to Ingredients. People who are allergic to any of the following should not use C1 esterase inhibitor (human).

  • C1 esterase inhibitor (human)
  • C1 esterase inhibitor (recombinant)
  • Cinryze
  • Haegarda
  • Any of the ingredients in the specific product dispensed

Your pharmacist can tell you all of the ingredients in the specific C1 esterase inhibitor (human) products they stock.

What should I know about C1 esterase inhibitor (human) before using it?

Do not use C1 esterase inhibitor (human) unless it has been prescribed to you by a health care provider. Use it as prescribed.

Do not share C1 esterase inhibitor (human) with other people, even if they have the same condition as you. It may harm them.

Keep C1 esterase inhibitor (human) out of the reach of children. Since this medicine may be kept in a refrigerator, take special precautions to keep it away from children who also use the refrigerator.

Depending on the specific product you are using, this medicine may be given either as an injection under the skin or as an infusion into the vein (IV). Make sure that you or a caregiver understands how to properly store, prepare, and use your C1 esterase inhibitor (human) product. If you have any questions, talk to your health care provider.

C1 esterase inhibitor (human) is used on a regular basis to help prevent symptoms (swelling attacks), but it does not treat swelling attacks when they occur. If you are having a swelling attack, seek medical help or use a different medicine that was prescribed by your health care provider to treat the attack.

C1 esterase inhibitor (human) can cause dizziness, which may affect your alertness or coordination. Do not drive or do other activities that require alertness or coordination until you know how this medicine affects you.

C1 esterase inhibitor (human) may cause serious allergic reactions in some people. Your health care provider will train you on how to treat these allergic reactions. You may need to have a rescue medicine called epinephrine available to treat an allergic reaction. Make sure that you and anyone else who may be able to help during an emergency know where your epinephrine is stored and how to use it.

It is important to bring C1 esterase inhibitor (human) with you while you are travelling. If you are planning a trip, work with your health care provider to make sure that you will have enough medicine for your trip.

What should I tell my health care provider before using C1 esterase inhibitor (human)?

Tell your health care provider about all of your health conditions and any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using. This will help them determine if C1 esterase inhibitor (human) is right for you.

In particular, make sure that you discuss any of the following.

Current and Past Health Conditions. Tell your health care provider if you have any of the following.

  • An indwelling catheter or line that allows access to a vein
  • History of blood clots
  • Heart or circulation problems
  • Stroke

Other Medicines and Supplements. C1 esterase inhibitor (human) may interact with other medicines and supplements. Before using C1 esterase inhibitor (human), tell your health care provider about any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using. See the Interactions section for more details.

Pregnancy. It is not known if or how C1 esterase inhibitor (human) could affect pregnancy or harm a fetus. Tell your health care provider if you are pregnant or plan to become pregnant.

Breastfeeding. It is not known if C1 esterase inhibitor (human) passes into breast milk. Tell your health care provider if you are breastfeeding or plan to breastfeed.

Interactions

Does C1 esterase inhibitor (human) interact with foods or drinks?

There are no known interactions between C1 esterase inhibitor (human) and foods or drinks.

It is unknown if drinking alcohol will affect C1 esterase inhibitor (human).

Does C1 esterase inhibitor (human) interact with other medicines (drug interactions)?

Always tell your health care provider about any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using.

In particular, make sure that you discuss if you are using any of the following before using C1 esterase inhibitor (human).

  • A birth control pill, patch, vaginal ring, implant, injection, or intrauterine device (IUD) that contains hormones
  • A male sex hormone (androgen), such as testosterone

This may not be a complete list of medicines that can interact with C1 esterase inhibitor (human). Always check with your health care provider.

Does interact with other drugs you are taking?

Enter your medication into the WebMD interaction checker

Overdose/Missed Dose

What should I do if I accidentally use too much C1 esterase inhibitor (human)?

If you or someone else has used too much C1 esterase inhibitor (human), get medical help right away, call 911, or contact a Poison Control center at 800-222-1222.

What should I do if I miss a dose of C1 esterase inhibitor (human)?

If you miss a dose, use it as soon as you remember. If it is almost time for your next dose, skip the missed dose and only use the next dose. Do not use double or extra doses.

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