Emicizumab (Hemlibra)

These instructions are for Children's Hospital of Philadelphia (CHOP) patients who are prescribed emicizumab.

Important information about emicizumab:

Emicizumab, also known by the brand name Hemlibra®, is a medicine for patients with hemophilia A (factor VIII deficiency). This is an inherited bleeding disorder in which the body does not make enough of the clotting factor, factor VIII. The medicine helps to prevent or decrease the number of bleeding episodes. Emicizumab is approved for use in all ages and is given as a subcutaneous injection. Learn more about emicizumab.

The medicine is an antibody that substitutes for factor VIII to prevent bleeding. It connects factor X with factor IX and helps the body form a clot, without the need for factor VIII and without causing development of inhibitors to factor VIII. Emicizumab works for patients with hemophilia A (factor VIII deficiency) with or without inhibitors.

It is important to understand that emicizumab cannot be used to treat a bleed. Bleeding must be treated with your regular factor.

Instructions for taking emicizumab:

• Emicizumab is an injection given into the layer of fat under the skin (subcutaneous injection). It feels similar to receiving a vaccine. It is easier and faster than receiving treatment that is infused through a vein.

• It is important to rotate the site where you give the injection with each dose. This helps prevent skin irritation.

• The medicine is given once each week for the first four doses. After that, the treatment can sometimes be spread out to be given every two or four weeks.

• If you forget to give a dose of emicizumab, give the dose as soon as you remember and resume normal dosing times. Do not give 2 doses on the same day. Contact the hematology team if you are unsure what to do about a missed dose.

• Do not use Feiba® (activated prothrombin complex concentrate, aPCC) to prevent or treat breakthrough bleeds once you start taking emicizumab. Serious side effects related to blood clots were reported in patients taking emicizumab and certain doses of Feiba.

• The Hemostasis and Thrombosis Center will make a plan to treat bleeding if you have an inhibitor. Your team will review your plan with you in detail and provide a written care plan for you to keep.

Monitoring

Patients taking emicizumab require special tests to monitor their factor VII levels and inhibitors. Routine testing is not accurate and cannot be used. Your team will tell you what tests you will need and how often.

What should I do for a bleed?

• Since emicizumab works very well and bleeding should be rare, it is important to call the Hemostasis and Thrombosis Center for each bleed to discuss a treatment plan.

• Always keep two emergency doses of factor at home and call your hematology team for advice.

General safety tips:

• You and your child should know the names of all the medicines they are taking. Share this information with anyone involved in your child's care.

• Before giving the first dose, read the label. Be sure it is what was prescribed. After a refill, if the medicine looks different to you, ask your pharmacist about it before giving it.

• Always make sure you have enough medicine on hand. Each time you refill your prescription, check to see how many refills are left.

• Always check the label and expiration date before giving any medicine. Do not give expired medicines. Ask your pharmacy what to do with expired or unused medicines. If there is no take-back program, empty them into the trash.

• Store all medicines in their original container away from direct sunlight or heat. Do not store in humid places such as the bathroom. Keep them out of children's reach, locked up if possible.

• If too much or the wrong kind of medicine is taken, call the Poison Control Center at 1-800-222-1222.

Contact your CHOP hematology team with questions, concerns or if you:

• Have any bleeding

• Notice any new symptoms

Call 911 if your child has signs of an allergic reaction such as swelling of the face, lips, tongue or throat; trouble breathing, wheezing or chest tightness. If you are concerned the reaction is related to emicizumab, call your healthcare team after symptoms resolve to discuss a plan moving forward.

Division of Hematology

215-590-3535

Hemostasis and Thrombosis Center

For non-urgent issues, send your team a message in the MyCHOP portal.

Reviewed on July 1, 2023, by Marylou Macdermott, MSN, CRNP; Rebecca Kendsersky, PharmD, BCPPS