11 Hereditary Angioedema Treatments: Berinert, Orladeyo, and More
Hereditary angioedema is a rare genetic disorder. Hereditary angioedema treatment may involve C1-INH replacement therapy and medications for related symptoms.
Cinryze
Used for Hereditary Angioedema
Used for Hereditary Angioedema
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.
What is Cinryze?
What is Cinryze used for?
- Prevention of hereditary angioedema (HAE) attacks in people 6 years of age and older
How Cinryze works
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.
Drug facts
| Common Brands | Cinryze |
|---|---|
| Drug Class | C1 esterase inhibitor replacement |
| Controlled Substance Classification | Not a controlled medication |
| Generic Status | No lower-cost generic available |
| Availability | Prescription only |
Side effects of Cinryze
The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.
Common Side Effects
- Headache (19%)
- Nausea (18%)
- Rash (10%)
- Vomiting (10%)
- Fever (5%)
Less Common Side Effects
- Pain at the injection site
- Redness
- Itching
- Dizziness
Cinryze serious side effects
Contact your healthcare provider immediately if you experience any of the following.
- Allergic reaction: hives, chest tightness, difficulty breathing, wheezing, fast heartbeat, swelling of the tongue or throat
- Blood clot: pain, swelling, warmth, or discoloration of the arm or leg, unexplained shortness of breath, chest pain or discomfort, unexplained fast heartbeat, numbness or weakness on one side of the body
The following Cinryze side effects have also been reported
Side effects that you should report to your care team as soon as possible:
- Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
- Blood clot—pain, swelling, or warmth in the leg, shortness of breath, chest pain
Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):
Pros and cons of Cinryze
Pros
- 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
Cons
- 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)
Pharmacist tips for Cinryze
- 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.
Risks and warnings for Cinryze
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.
Severe allergic reaction
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.
Blood clots
- 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.
Transmission of diseases
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.
Cinryze dosage
Typical dosage for Cinryze
-
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.
What are alternatives to Cinryze?
There are a number of medications that your doctor can prescribe in place of Cinryze. Compare a few possible alternatives below.
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