CABLIVI contains the active ingredient caplacizumab-yhdp, a von Willebrand factor-directed antibody fragment. It is presented as a sterile, white, lyophilized powder for injection, provided in single-dose vials with a dosage strength of 11 mg per vial. Key inactive ingredients include anhydrous citric acid, polysorbate 80, sucrose, and trisodium citrate dihydrate.

How should I use this medication?

This medication is injected into a vein or under the skin. It is usually given by a care team in a hospital or clinic setting. It may also be given at home.

If you get this medication at home, you will be taught how to prepare and give it. Use exactly as directed. Take it as directed on the prescription label at the same time every day. Keep taking it unless your care team tells you to stop.

It is important that you put your used needles and syringes in a special sharps container. Do not put them in a trash can. If you do not have a sharps container, call your pharmacist or care team to get one.

Talk to your care team about the use of this medication in children. Special care may be needed.

Overdosage: If you think you have taken too much of this medicine contact a poison control center or emergency room at once.

NOTE: This medicine is only for you. Do not share this medicine with others.

What if I miss a dose?

If you miss a dose, take it as soon as you can unless it is more than 12 hours late. If it is more than 12 hours late, skip the missed dose. Take the next dose at the normal time.

Where should I keep my medication?

Keep out of the reach of children and pets.

Store in a refrigerator or at room temperature up to 30 degrees C (86 degrees F). Keep this medication in the original carton. Protect from light.

Refrigeration: Store in the refrigerator. Do not freeze. Get rid of any unused medication after the expiration date.

Room Temperature: This medication may be stored at room temperature for up to 2 months. If it is stored at room temperature, get rid any unused medication after 2 months or after it expires, whichever is first. Do not return it to the refrigerator after it has been stored at room temperature.

To get rid of medications that are no longer needed or have expired:

• Take the medication to a medication take-back program. Check with your pharmacy or law enforcement to find a location.
• If you cannot return the medication, ask your pharmacist or care team how to get rid of this medication safely.

NOTE: This sheet is a summary. It may not cover all possible information. If you have questions about this medicine, talk to your doctor, pharmacist, or health care provider.

What side effects may I notice from receiving this medication?

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
• Bleeding—bloody or black, tar-like stools, vomiting blood or brown material that looks like coffee grounds, red or dark brown urine, small red or purple spots on skin, unusual bruising or bleeding

Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):

• Back pain
• Bleeding from the nose or gums that won't stop
• Fatigue
• Headache
• Muscle pain

This list may not describe all possible side effects. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

What should I tell my care team before I take this medication?

They need to know if you have any of these conditions:

• Bleeding disorder
• Having surgery
• Liver disease
• An unusual reaction to caplacizumab, other medications, foods, dyes, or preservatives
• Pregnant or trying to get pregnant
• Breast-feeding

What may interact with this medication?

• Certain medications that treat or prevent blood clots, such as warfarin, enoxaparin, dalteparin, apixaban, dabigatran, rivaroxaban

This list may not describe all possible interactions. Give your health care provider a list of all the medicines, herbs, non-prescription drugs, or dietary supplements you use. Also tell them if you smoke, drink alcohol, or use illegal drugs. Some items may interact with your medicine.

What should I watch for while using this medication?

Visit your care team for regular checks on your progress. Tell your care team if your symptoms do not start to get better or if they get worse.

This medication may increase your risk to bruise or bleed. Call your care team if you notice any unusual bleeding.

If you are going to need surgery or other procedure, tell your care team that you are using this medication.

Tell your dentist and dental surgeon that you are taking this medication. You should not have major dental surgery while on this medication. See your dentist to have a dental exam and fix any dental problems before starting this medication. Take good care of your teeth while on this medication. Make sure you see your dentist for regular follow-up appointments.