Eculizumab

What it is used for

BKEMV is a complement inhibitor indicated for: The treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. The treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. Limitation of Use BKEMV is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS).

The treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AChR) antibody positive. The treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive. 1.1 Paroxysmal Nocturnal Hemoglobinuria (PNH) BKEMV is indicated for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis.

1.2 Atypical Hemolytic Uremic Syndrome (aHUS) BKEMV is indicated for the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. Limitation of Use BKEMV is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS).

1.3 Generalized Myasthenia Gravis (gMG) BKEMV is indicated for the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AChR) antibody positive. 1.4 Neuromyelitis Optica Spectrum Disorder (NMOSD) BKEMV is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.

The most important thing to know

SOLIRIS is a medicine that affects your immune system. SOLIRIS may lower the ability of your immune system to fight infections. SOLIRIS increases your chance of getting serious meningococcal infections caused by Neisseria meningitidis bacteria. Meningococcal infections may quickly become life-threatening or cause death if not recognized and treated early.

You must complete or update your meningococcal vaccine(s) at least 2 weeks before your first dose of SOLIRIS. If you have not completed your meningococcal vaccines and SOLIRIS must be started right away, you should receive the required vaccine(s) as soon as possible. If you have not been vaccinated and SOLIRIS must be started right away, you should also receive antibiotics to take for as long as your healthcare provider tells you.

If you had a meningococcal vaccine in the past, you might need additional vaccines before starting SOLIRIS. Your healthcare provider will decide if you need additional meningococcal vaccines. Meningococcal vaccines do not prevent all meningococcal infections. Call your healthcare provider or get emergency medical care right away if you get any of these signs and symptoms of a serious meningococcal infection: fever fever with high heart rate headache and fever confusion muscle aches with flu-like symptoms fever and a rash headache with nausea or vomiting headache with a stiff neck or stiff back eyes sensitive to light Your healthcare provider will give you a Patient Safety Card about the risk of serious meningococcal infection.

Carry it with you at all times during treatment and for 3 months after your last dose of SOLIRIS. Your risk of meningococcal infection may continue for several weeks after your last dose of SOLIRIS. It is important to show this card to any healthcare provider who treats you. This will help them diagnose and treat you quickly.

SOLIRIS is only available through a program called the ULTOMIRIS and SOLIRIS Risk Evaluation and Mitigation Strategy (REMS). Before you can receive SOLIRIS, your healthcare provider must: enroll in the ULTOMIRIS and SOLIRIS REMS program counsel you about the risk of serious meningococcal infections give you information about the signs and symptoms of serious meningococcal infection make sure that you are vaccinated against serious infections caused by meningococcal bacteria and that you receive antibiotics if you need to start SOLIRIS right away and you are not up to date on your vaccines give you a Patient Safety Card about your risk of meningococcal infection, as discussed above SOLIRIS may also increase the risk of other types of serious infections caused by encapsulated bacteria, including Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria gonorrhoeae.

If your child is treated with SOLIRIS, your child should receive vaccines against Streptococcus pneumoniae and Haemophilus influenzae type b (Hib). Certain people may be at risk of serious infections with gonorrhea. Talk to your healthcare provider about whether you are at risk for gonorrhea infection, about gonorrhea prevention, and regular testing.

Certain fungal infections (aspergillus) may also happen if you take SOLIRIS and have a weak immune system or a low white blood cell count. For more information about side effects, see "

Side effects, and when to stop

" What is SOLIRIS? SOLIRIS is a prescription medicine used to treat: people with paroxysmal nocturnal hemoglobinuria (PNH). people with atypical hemolytic uremic syndrome (aHUS). SOLIRIS is not for use in treating people with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). people 6 years of age and older with generalized myasthenia gravis (gMG) who are anti-acetylcholine receptor (AChR) antibody positive.

adults with neuromyelitis optica spectrum disorder (NMOSD) who are anti-aquaporin-4 (AQP4) antibody positive. It is not known if SOLIRIS is safe and effective in children with PNH or NMOSD. It is not known if SOLIRIS is safe and effective in children with gMG below 6 years of age. Who should not receive SOLIRIS? Do not receive SOLIRIS if you have a serious meningococcal infection when you are starting SOLIRIS treatment.

Before you receive SOLIRIS, tell your healthcare provider about all of your medical conditions, including if you: have an infection or fever. are pregnant or plan to become pregnant. It is not known if SOLIRIS will harm your unborn baby. are breastfeeding or plan to breastfeed. It is not known if SOLIRIS passes into your breast milk.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements. SOLIRIS and other medicines can affect each other causing side effects. Know the medications you take and the vaccines you receive. Keep a list of them to show your healthcare provider and pharmacist when you get a new medicine.

How should I receive SOLIRIS? Your healthcare provider will give you SOLIRIS into your vein through an intravenous (IV) line usually over 35 minutes in adults and 1 to 4 hours in children. Adults will usually receive a SOLIRIS infusion: weekly for 5 weeks, then every 2 weeks. Children less than 18 years of age, your healthcare provider will decide how often you will receive SOLIRIS depending on your age and body weight.

After each infusion, you should be monitored for at least 1 hour for infusion-related reactions. See " What are the possible side effects of SOLIRIS? " If you have an infusion-related reaction during your SOLIRIS infusion, your healthcare provider may decide to give SOLIRIS more slowly or stop your infusion. If you miss a SOLIRIS infusion, call your healthcare provider right away.

If you have PNH, your healthcare provider will need to monitor you closely for at least 8 weeks after stopping SOLIRIS. Stopping treatment with SOLIRIS may cause breakdown of your red blood cells due to PNH. Symptoms or problems that can happen due to red blood cell breakdown include: drop in the number of your red blood cell count kidney problems drop in your platelet counts blood clots confusion difficulty breathing chest pain If you have aHUS, your healthcare provider will need to monitor you closely for at least 12 weeks after stopping SOLIRIS for signs of worsening aHUS symptoms or problems related to abnormal clotting (thrombotic microangiopathy).

Symptoms or problems that can happen with abnormal clotting may include: stroke difficulty breathing confusion kidney problems seizure swelling in arms or legs chest pain (angina) a drop in your platelet count What are the possible side effects of SOLIRIS? SOLIRIS can cause serious side effects including: Serious infusion-related reactions.

Serious infusion-related reactions can happen during your SOLIRIS infusion. Tell your healthcare provider or nurse right away if you get any of these symptoms during your SOLIRIS infusion: chest pain trouble breathing or shortness of breath swelling of your face, tongue, or throat feel faint or pass out If you have an infusion-related reaction to SOLIRIS, your healthcare provider may need to infuse SOLIRIS more slowly or stop SOLIRIS.

See " How should I receive SOLIRIS? " The most common side effects in people with PNH treated with SOLI