Pronunciation:

rav-ue-LIZ-ue-mab - cwvz

Brand Names:

• Ultomiris

Dosage Forms:

• Solution

Classifications:

Therapeutic—

Blood Modifier Agent

Pharmacologic—

Ravulizumab

Uses of This Medicine:

Ravulizumab-cwvz injection is used to treat a type of blood disease called paroxysmal nocturnal hemoglobinuria (PNH). This medicine helps reduce red blood cell destruction or breakdown (hemolysis) in patients with PNH.

Ravulizumab-cwvz injection is also used to treat atypical hemolytic uremic syndrome (aHUS) in adults and children.

Ravulizumab-cwvz injection is also used to treat a nerve and muscle problem called generalized myasthenia gravis (gMG) in patients who are anti-acetylcholine receptor (AChR) antibody positive.

Ravulizumab-cwvz injection is a monoclonal antibody that works on the immune system.

This medicine is available only under a restricted distribution program called Ultomiris® REMS (Risk Evaluation and Mitigation Strategy) Program.

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 ravulizumab-cwvz injection to treat to treat paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), given as a needle placed into one of the veins in children 1 month of age and older. However, safety and efficacy have not been established to treat paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), given as a needle placed into one of the veins in children younger than 1 month of age, to treat generalized myasthenia gravis (gMG), given as a needle placed into one of the veins in children.

Appropriate studies have not been performed on the relationship of age to the effects of ravulizumab-cwvz injection to treat to treat paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), given as a shot under the skin in children . Safety and efficacy have not been established.

Older adults—

Appropriate studies performed to date have not demonstrated geriatric-specific problems that would limit the usefulness of ravulizumab-cwvz 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 receiving 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.

• Immune Globulin

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:

• Infection (eg, gonorrhea, influenza, pneumonia)—Use with caution. This medicine may decrease your body’s ability to fight infection.

• Meningococcal infection—Should not be given to patients with this condition.

• No current vaccination against meningitis infection—Should not be given to patients with this condition unless the doctor decides that urgent treatment is needed.

Proper Use of This Medicine:

A nurse or other trained health professional will give you or your child this medicine in a medical facility. It is given through a needle placed into one of your veins. It must be injected slowly, so your IV tube will need to stay in place for at least 2 hours.

You may also be taught how to give your medicine at home. It is usually given as a shot under the skin of your stomach, thigh, or upper arm. Make sure you understand all instructions before you give yourself an injection. Do not use more medicine or use it more often than your doctor tells you to.

Your doctor will monitor you for at least 1 hour for any unwanted effects after the infusion.

It is very important that you understand the requirements of the Ultomiris® REMS program, and become familiar with the Ultomiris® Medication Guide and patient instructions. Read and follow these instructions carefully. Ask your doctor if you have any questions. Ask your pharmacist for the medication guide if you do not have one.

If you are changing treatment from Soliris® to Ultomiris™, you should start receiving Ultomiris™ 2 weeks after your last dose of Soliris®.

This medicine is available in two forms: a vial (glass container) or a prefilled cartridge with on-body injector. The prefilled cartridge with on-body injector is the dosage form you can use at home.

Allow the prefilled cartridge with on-body injector to warm to room temperature for 45 minutes, without removing the medicine from the carton, before you use it. Do not warm the medicine in any other way or return to the refrigerator.

To use the prefilled cartridge with on-body injector:

• 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 areas where the skin is tender, bruised, red, hard, or in areas with scars, stretch marks, moles, or excess hair.
• Check the liquid in the cartridge. It should be clear and colorless to slightly yellow. Do not use the medicine if it is cloudy, discolored, or has flakes or large particles in it. Do not shake.
• Use a new needle and cartridge each time you inject your medicine.
• Do not use the cartridge if it is cracked, broken, or if there are missing pieces. Do not remove the needle cover until you are ready to use it.

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 injectable dosage form (prefilled cartridge with on-body injector):
  • For paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS):
    • From intravenous (a needle placed into one of your veins) to subcutaneous (injected under the skin):
      • Adults weighing 40 kilograms (kg) or more—490 milligrams (mg) prefilled cartridge with on-body injector injected under your skin at different body areas once a week 8 weeks after your last dose.
      • Children—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 in the refrigerator. Do not freeze.

Keep the medicine in its original carton until you are ready to use it. Protect from light. If removed from the refrigerator, you may store it at room temperature for up to 3 days. Do not return to the refrigerator. Throw away unused cartridge after 3 days.

Throw away used needles in a hard, closed container that the needles cannot poke through. Keep this container away from children and pets.

Precautions While Using This Medicine:

It is very important that your doctor check your or your child's progress at regular visits to make sure that this medicine is working properly. Blood tests may be needed to check for unwanted effects.

Ravulizumab-cwvz may increase your chance of having serious infections, including a meningococcal infection. Avoid people who are sick or have infections. Tell your doctor right away if you or your child develop headaches, nausea, vomiting, fever, a stiff neck or back, rash, confusion, muscle aches, or if your eyes have become sensitive to light. Make sure you have received a vaccine to prevent meningococcus infections at least 2 weeks before you receive this medicine. You may also be given antibiotic medicines for 2 weeks to prevent infections if you are to use this medicine right away. If you have already received the meningococcal vaccine in the past, your doctor will decide if you need another dose.

Ask your doctor for a patient safety card. This card will list the symptoms of meningococcus infections and what to do if you have them. Carry the card with you at all times during treatment and for 8 months after your last dose. You will need to show the card to any doctor who treats you.

You could develop hemolysis (breakdown of red blood cells) when you stop receiving this medicine for PNH. Your doctor will monitor you closely for at least 16 weeks after the last dose of this medicine. Be sure to keep all appointments.

Ravulizumab-cwvz may cause infusion-related reactions, which can be life-threatening and require immediate medical attention. Tell your doctor right away if you or your child start to have a fever, chills or shaking, dizziness, trouble breathing, itching or rash, lightheadedness or fainting after receiving this medicine.

You could also develop a condition called thrombotic microangiopathy (TMA) when you or your child stop receiving this medicine for aHUS. Tell your doctor right away if you or your child have confusion, seizures, chest pain, trouble breathing, blood clots, or stroke.

Tell your doctor if you are also receiving other procedures (eg, plasma exchange, plasmapheresis) or medicines (eg, efgartigimod, immunoglobulin injection) for myasthenia gravis.

The on-body injector contains acrylic adhesive, which may cause allergic reactions in people who are sensitive to the adhesive.

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:

More common

Bladder pain

bloating or swelling of the face, arms, hands, lower legs, or feet

bloody or cloudy urine

blurred vision

body aches or pain

chills

confusion

cough

decreased urine

difficult, burning, or painful urination

difficulty with breathing

dizziness

dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position

dry mouth

ear congestion

extremely high fever or body temperature

fast, shallow breathing

fast, weak heartbeat

fever

frequent urge to urinate

headache

increased thirst

irregular heartbeat

loss of appetite

loss of voice

lower back or side pain

mood changes

muscle pain or cramps

nausea or vomiting

nervousness

numbness or tingling in hands, feet, or lips

pale, clammy skin

pounding in the ears

rapid weight gain

runny or stuffy nose

sneezing

slow or fast heartbeat

sore throat

sweating

swollen glands

thirst

tingling of the hands or feet

trouble breathing

unusual bleeding or bruising

unusual tiredness or weakness

unusual weight gain or loss

Incidence not known

Chest tightness

difficulty swallowing

hives, itching, or skin rash

puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue

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

Back pain

bleeding, blistering, burning, coldness, discoloration of the 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

diarrhea

difficulty having a bowel movement (stool)

difficulty with moving

fear

hair loss or thinning of the hair

joint pain

muscle pain, stiffness ,or spasms

nausea or vomiting

pain in the arms or legs

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.