Pronunciation:

em-i-SIZ-ue-mab - kxwh

Brand Names:

• Hemlibra

Dosage Forms:

• Solution

Warnings:

Warning: Thrombotic Microangiopathy and ThromboembolismCases of thrombotic microangiopathy and thrombotic events were reported when on average a cumulative amount of greater than 100 units/kg/24 hours of activated prothrombin complex concentrate was administered for 24 hours or more to patients receiving emicizumab-kxwh prophylaxis. Monitor for the development of thrombotic microangiopathy and thrombotic events if aPCC is administered. Discontinue aPCC and suspend dosing of emicizumab-kxwh if symptoms occur .

Classifications:

Therapeutic—

Antihemophilic Agent

Pharmacologic—

Emicizumab

Uses of This Medicine:

Emicizumab-kxwh injection is used to prevent or decrease the frequency of bleeding episodes in patients with hemophilia A (congenital Factor VIII deficiency).

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 performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of emicizumab-kxwh injection in children. Safety and efficacy have been established.

Older adults—

Appropriate studies performed to date have not demonstrated geriatric-specific problems that would limit the usefulness of emicizumab-kxwh injection in the elderly.

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. When you are taking this medicine, it is especially important that your healthcare professional know if you are taking any of the medicines listed below. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following medicines is usually not recommended, but may be required in some cases. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.

• Anti-Inhibitor Coagulant Complex

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:

• Bleeding problem or
• Blood vessel problem—Use with caution. May make these conditions worse.

Proper Use of This Medicine:

A nurse or other trained health professional will give you this medicine. It is given as a shot under your skin, usually in the upper outer arms, thighs, or stomach. This medicine may also be given at home to patients who do not need to be in a hospital or clinic. You or your caregiver may be trained to prepare and inject this medicine at home. Be sure that you understand how to use the medicine.

This medicine should come with a Medication Guide and patient instructions. Read and follow these instructions carefully. Ask your doctor if you have any questions.

If you use this medicine at home, you will be shown the body areas where this shot can be given. Use a different body area each time you give yourself a shot. Keep track of where you give each shot to make sure you rotate body areas. This will help prevent skin problems from the injections. Do not inject into skin areas that are tender, red, bruised, hard, or not intact, or into areas with moles or scars.

Check the liquid in the vial. It should be colorless or slightly yellow. Do not use if it is cloudy, hazy, discolored, or has particles in it. Do not shake.

You might not use all of the medicine in each vial. Use each vial only one time. Do not save an open vial.

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 (solution):
  • For prevention of bleeding episodes:
    • Adults and children—Dose is based on body weight and must be determined by your doctor. The starting dose is usually 3 milligrams (mg) per kilogram (kg) injected under the skin once a week for the first 4 weeks followed by a maintenance dose of 1.5 mg/kg once a week, or 3 mg/kg once every 2 weeks, or 6 mg/kg once every 4 weeks.

Missed dose—

If you miss a dose of this medicine, take it as soon as possible. However, if it is almost time for your next dose, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

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 in the refrigerator. Do not freeze.

Store it in its original carton. You may store the unopened vial at room temperature if needed, and then return to the refrigerator. It should not be stored out of the refrigerator for more than 7 days. Throw away any unused medicine left in the vial.

Precautions While Using This Medicine:

It is very important that your doctor check your progress closely at regular visits while you or your child are receiving this medicine to make sure it is working properly. Blood tests will be needed to check for unwanted effects.

Tell your doctor if you are pregnant or plan to become pregnant. Women should use an effective form of birth control to keep from getting pregnant during treatment with this medicine. If you think you have become pregnant while using this medicine, tell your doctor right away.

Thrombotic microangiopathy (damage in the smallest blood vessels) may occur while you are using this medicine. Tell your doctor right away if you have fever, tiredness, confusion, loss of vision, or seizures.

This medicine may increase your chance of having blood clotting problems. Tell your doctor right away if you have a sudden or severe headache, problems with vision or speech, chest pain, shortness of breath, or numbness or weakness while you are receiving this medicine.

Tell your doctor if this medicine does not prevent or stop bleeding as expected.

Make sure any doctor or dentist who treats you knows that you are using this medicine. You may need to stop using this medicine several days or months before you have surgery or medical tests.

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 immediately if any of the following side effects occur:

Incidence not known

Blue-green to black skin discoloration

changes in skin color, pain, tenderness, or swelling of the foot or leg

large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or genitals

pain, redness, or sloughing of the skin at the injection site

sudden weakness in the arms or legs

sudden, severe chest pain

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

Bleeding, blistering, burning, coldness, discoloration of skin, feeling of pressure, hives, infection, inflammation, itching, lumps, numbness, pain, rash, redness, scarring, soreness, stinging, swelling, tenderness, tingling, ulceration, or warmth at the injection site

headache

diarrhea

difficulty in moving

fever

joint pain or swelling

muscle aches, cramps, pain, or stiffness

muscle pain or stiffness

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.