Pronunciation:

C1 ES-ter-ase in-HIB-i-ter ree-KOM-bi-nant

Brand Names:

• Ruconest

Dosage Forms:

• Powder for Solution

Classifications:

Therapeutic—

Immune Modulator

Uses of This Medicine:

C1 esterase inhibitor recombinant is used to treat hereditary angioedema (HAE) in adults and teenagers. HAE is a rare disease that causes swelling of the face, hands, feet, throat, stomach, bowels, or sexual organs. People who have HAE have low levels of C1 esterase inhibitor in their body, and this medicine increases the amount of C1 esterase inhibitor in the body.

This medicine is available only with your doctor's prescription.

Before Using This Medicine:

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

Allergies—

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Children—

Appropriate studies have not been performed on the relationship of age to the effects of C1 esterase inhibitor recombinant in children 12 years of age and younger. Safety and efficacy have not been established.

Older adults—

Appropriate studies have not been performed on the relationship of age to the effects of C1 esterase inhibitor recombinant in the geriatric population. Safety and efficacy have not been established.

Breast-feeding—

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Other medicines—

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

Other interactions—

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

Other medical problems—

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

• Allergy to rabbits or rabbit-derived products—Should not be used in patients with this condition.

• Atherosclerosis or
• Blood clots, history of or
• Obesity or
• Prolonged immobilization—May increase risk for serious blood clots.

Proper Use of This Medicine:

A nurse or other trained health professional will give you this medicine. This medicine is given through a needle placed in one of your veins. The medicine must be injected slowly, so your IV tube will need to stay in place for 5 minutes.

This medicine may sometimes be given at home to patients who do not need to be in the hospital or clinic. If you are using this medicine at home, your doctor or nurse will teach you how to prepare and inject the medicine. Be sure that you understand how to use the medicine.

Make sure family members or other people you are with know how to inject the medicine in case you are unable to do it yourself during an HAE attack.

Use a new needle and syringe each time you inject your medicine.

Check the injection kits regularly to make sure that the powder or liquid has not changed its color. Do not use this medicine if the powder or liquid has changed its color, or if there are solids in the mixed liquid.

Carry this medicine with you at all times for emergency use in case you have an HAE attack.

This medicine comes with a patient information leaflet. Read and follow the instructions carefully. Ask your doctor if you have any questions.

Dosing—

The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

• For injection dosage form:
  • For hereditary angioedema:
    • Adults and teenagers—Dose is based on body weight and must be determined by a doctor. The usual dose is 50 international units per kilogram (kg) of body weight injected into a vein. Your doctor may increase your dose as needed. However, the dose is usually not more than 4200 international units per day.
    • Children 12 years of age and younger—Use and dose must be determined by your doctor.

Missed dose—

Call your doctor or pharmacist for instructions.

Storage—

Keep out of the reach of children.

Do not keep outdated medicine or medicine no longer needed.

Ask your healthcare professional how you should dispose of any medicine you do not use.

Store the powder vial in the refrigerator or at room temperature. Keep the medicine in the original carton until you are ready to use it. Do not freeze.

Use the mixed medicine immediately, or you may store in the refrigerator within 8 hours of mixing it.

Throw away the vial after you have used it, even if there is medicine left in it.

Precautions While Using This Medicine:

It is very important that your doctor check your progress at regular visits. This will allow your doctor to see if the medicine is working properly and to decide if you should continue to use it.

HAE attacks are life-threatening and require immediate medical attention. Check with your doctor right away, or go to an emergency room as soon as possible, even if you feel better after using this medicine.

This medicine may cause serious allergic reactions. These reactions can be life-threatening and require immediate medical attention. Call your doctor or nurse right away if you have a rash, itching, hives, hoarseness, chest tightness, lightheadedness, dizziness or fainting, shortness of breath, trouble breathing, trouble swallowing, or any swelling of your hands, face, or mouth after you receive this medicine.

This medicine may increase your risk of developing blood clots. Check with your doctor right away if you have swelling and pain in your arms, legs, or stomach, chest pain, shortness of breath, loss of sensation, confusion, or problems with muscle control or speech.

Side Effects of This Medicine:

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor or nurse immediately if any of the following side effects occur:

Less common

Large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs

Incidence not known

Cough

difficulty swallowing

dizziness

fast heartbeat

hives, itching, or skin rash

pains in the chest, groin, or legs, especially calves of the legs

severe headaches of sudden onset

shortness of breath

sudden loss of coordination

sudden onset of shortness of breath for no apparent reason

sudden onset of slurred speech

sudden vision changes

tightness in the chest

unusual tiredness or weakness

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common

Headache

Less common

Back pain

burning sensation on the skin

diarrhea

feeling of constant movement of self or surroundings

nausea

sensation of spinning

sneezing

Incidence not known

Abdominal or stomach pain

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.