Cinryze is a C1 esterase inhibitor. It's used to help prevent swelling and painful attacks in people 6 years of age or older with hereditary angioedema (HAE). Cinryze is infused into the vein, typically every 3 to 4 days, by a healthcare provider. But if appropriate, your provider can train you to do the infusion at home. Common side effects of this medication include headache, nausea, rash and vomiting. Cinryze is only available at certain pharmacies.
Prevention of hereditary angioedema (HAE) attacks in people 6 years of age and older
Cinryze is a C1 esterase inhibitor replacement. It contains C1 esterase inhibitor, a protein in your blood that helps relieve swelling (inflammation). Hereditary angioedema (HAE) is a swelling condition caused by low levels of C1 esterase inhibitor. By raising and restoring the levels of C1 esterase inhibitor in the blood, Cinryze helps prevent swelling attacks from happening in people with HAE.
Source: DailyMed
Side effects that you should report to your care team as soon as possible:
Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):
Can be given to adults and children 6 years or older
Given every 3 to 4 days
Can be refrigerated or stored at room temperature
Has to be given intravenously
Can't be prepared more than 3 hours in advance
Can't be used to treat HAE attacks (can only prevent them)
Don't use Cinryze until you or your caregiver has been trained by a healthcare provider. Review the instructions on how to prepare and give Cinryze properly.
Do your best to keep everything clean and free of germs when reconstituting (or mixing) Cinryze. Always wash your hands before preparing. Always finish preparing the medication as soon as possible after opening the vials. This will help lower the risk of contamination and infections.
Before giving yourself a dose, allow Cinryze and the sterile water for injection to slowly reach room temperature. Don't microwave them or boil them because this might damage the medication.
Make sure Cinryze is free of particles and looks colorless to slightly blue. If it looks different, don't use it and contact your provider.
Cinryze is injected into a vein, most commonly in your arm on the inside of your elbow. Follow your provider's instructions on how to do this safely.
Make sure that you or your caregiver know how to use epinephrine (EpiPen) in case of an allergic reaction from Cinryze. Though rare, it's possible to have a serious allergic reaction to this medication. Symptoms can include hives, tightness in the chest, and trouble breathing.
Talk to your provider if you're confused about the symptoms of a HAE attacks compared to those of an allergic reaction to this medication. Signs of an HAE attack are different in each person, but can commonly cause swelling. An allergic reaction can also cause swelling, but might also include a rash, hives, trouble breathing, chest tightness, or fast heartbeat.
Each vial of Cinryze should only be used once. If you don't use all of the medication after one dose, you should throw it away. This is because Cinryze is free of preservatives, so germs can grow in it once it's opened.
Place all of the needles in a sharps container after using Cinryze. This will help prevent needlestick injuries.
Stop using Cinryze and let your provider know right away if you experience an allergic reaction or signs of a blood clot.
Before traveling, make sure you talk to your provider to get enough medication to last you through your trip.
Cinryze can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
Cinryze can cause severe allergic reactions in some people, so make sure your provider also prescribes epinephrine (EpiPen) for you to have during your injection, just in case. Get emergency medical help if you experience hives, chest tightness, difficulty breathing, wheezing, lightheadedness, fast heartbeat, or swelling of the tongue or throat.
Risk factors: Current catheter or access device | History of blood clots | Buildup of fats in arteries | Taking birth control or androgens | Identified as obese | Not mobile
Blood clots have occurred in people who take Cinryze, even at the recommended dose. Let your provider know if you have any of the risk factors mentioned above, and they will determine if Cinryze is right for you. Get emergency medical help if you experience any signs of blood clots, such as pain, swelling, warmth, or discoloration of the arm or leg, unexplained shortness of breath, chest pain or discomfort, unexplained fast heartbeat, or numbness or weakness on one side of the body.
Although rare, Cinryze has a risk of transmitting (spreading) certain viruses and diseases to you because this medication is made from human blood. Before Cinryze is made, blood donors are screened, the blood is tested for infections, and the blood product is processed to remove or inactivate certain viruses. Even though all these steps are taken to lower this risk, there's still a small risk of transmitting diseases. If you have concerns, speak to your provider.
Adults and children 12 years and older: The typical dose is 1,000 international units (IU) injected intravenously (into the vein) at a speed of 1 mL/minute (over about 10 minutes) every three or four days. The maximum dose is 2,000 IU, but not more than 80 IU/kg of body weight, every three or four days.
Children 6 to 11 years old: The typical dose is 500 IU injected intravenously at a speed of 1 mL/minute (over about 5 minutes) every three or four days. The maximum dose is 1,000 IU every three or four days.
Prevention of hereditary angioedema (HAE) attacks in people 6 years of age and older
Prevention of hereditary angioedema (HAE) attacks in people 6 years and older
Prevention of hereditary angioedema (HAE) attacks in people 12 years and older
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Caliezi, C., et al. (2000). C1-esterase inhibitor: An anti-inflammatory agent and its potential use in the treatment of diseases other than hereditary angioedema. Pharmacological Reviews.
National Organization for Rare Disorders. (2021). Hereditary angioedema.
Takeda Pharmaceuticals America, Inc. (2023). Cinryze- human c1-esterase inhibitor injection, powder, lyophilized, for solution; human c1-esterase inhibitor kit [package insert]. DailyMed.
Takeda Pharmaceuticals U.S.A. Inc. (2023). Preparation and self‑administration instructions.
US Hereditary Angioedema Association. (n.d.). About hereditary angioedema.
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