Aspaveli 1080 mg solucion para perfusion

Product Information for the User

ASPAVELI 1 080 mg Infusion Solution

pegcetacoplan

This medicine is subject to additional monitoring, which will facilitate the detection of new information about its safety. You can contribute by reporting any adverse effects you may experience. The final part of section 4 includes information on how to report these adverse effects.

Read this entire product information carefully before starting to use this medicine, as it contains important information for you.

• Keep this product information, as you may need to refer to it again.
• If you have any questions, consult your doctor, pharmacist, or nurse.
• This medicine has been prescribed only for you, and you should not give it to others even if they have the same symptoms as you, as it may harm them.
• If you experience any adverse effects, consult your doctor, pharmacist, or nurse,even if they are not listed in this product information. See section 4.

1.What ASPAVELI is and for what it is used

2.What you need to know before starting to use ASPAVELI

3.How to use ASPAVELI

4.Possible adverse effects

5.Storage of ASPAVELI

6.Contents of the package and additional information

What is ASPAVELI

ASPAVELI is a medication that contains the active ingredient pegcetacoplan. Pegcetacoplan has been designed to bind to the C3 protein of the complement system, which is part of the body's defense system. Pegcetacoplan prevents the body's immune system from destroying red blood cells.

What is ASPAVELI used for

ASPAVELI is used to treat adult patients with a disease called paroxysmal nocturnal hemoglobinuria (PNH) who have anemia as a result of this disease.

In patients with PNH, the complement system is overactive and attacks their red blood cells, which can cause low blood counts (anemia), fatigue, difficulty functioning, pain, abdominal pain, dark urine, shortness of breath, difficulty swallowing, erectile dysfunction, and blood clots. By binding to the C3 protein and blocking it, this medication can prevent the complement system from attacking red blood cells, thus controlling the symptoms of the disease. It has been shown that this medication increases the number of red blood cells (reduces anemia), which can improve these symptoms.

Do not use ASPAVELI

• if you are allergic to pegcetacoplan or any of the other components of this medication (listed in section 6).
• if you have an infection caused by encapsulated bacteria.
• if you are not vaccinated againstNeisseria meningitidis,Streptococcus pneumoniaeandHaemophilus influenzae.

Warnings and precautions

Consult your doctor, pharmacist or nurse before starting to use ASPAVELI.

Symptoms of infections

Inform your doctor before starting to use ASPAVELI if you have any infection.

Since the medication targets the complement system, which is part of the body's defenses against infections, the use of this medication increases the risk of developing infections, including those caused by encapsulated bacteria, such asStreptococcus pneumoniae,Neisseria meningitidisandHaemophilus influenzae. These are serious infections that affect the nose, throat and lungs or the tissue that covers the brain and can spread through the blood and body.

Consult your doctor before starting to use ASPAVELI to ensure you receive vaccinations againstStreptococcus pneumoniae,Neisseria meningitidisandHaemophilus influenzaeif you have not received these vaccinations before..If you have received these vaccinations in the past, you may still need additional vaccinations before starting this medication. These vaccinations should be administered at least 2 weeks before starting treatment. If you cannot be vaccinated 2 weeks in advance, your doctor will prescribe antibiotics to reduce the risk of infection during 2 weeks after being vaccinated. After vaccination, your doctor may closely monitor you for symptoms of infection.

Symptoms of infection

If you experience any of the following symptoms, inform your doctor immediately:

• headache and fever
• fever and rash
• fever with or without chills or shivering
• shortness of breath
• rapid pulse
• sweaty skin
• headache with neck or back stiffness
• headache with nausea (feeling like vomiting) or vomiting
• light-sensitive eyes
• muscle pain with flu-like symptoms
• confusion
• intense pain or discomfort

Make sure to keep your vaccinations up to date. You should also be aware that vaccinations reduce the risk of developing serious infections, but do not prevent all serious infections. According to national recommendations, your doctor may consider that you need additional measures, such as antibacterial medications, to prevent infections.

Allergic reactions

Some patients may experience allergic reactions. In case of severe allergic reaction, discontinue ASPAVELI infusion and seek immediate medical assistance. Severe allergic reaction may manifest as difficulty breathing, chest pain or tightness and/or dizziness/dizziness, intense itching or raised skin bumps, facial, lip, tongue and/or throat swelling, which may cause difficulty swallowing or syncope.

Reactions at the injection site

Reactions at the injection site have been observed with the use of ASPAVELI. Before self-administration, you should receive proper training on the correct injection technique.

Analytical controls

During treatment with ASPAVELI, your doctor will perform regular checks, including blood lactate dehydrogenase (LDH) concentration analysis and renal function analysis, and may adjust the dose as needed.

Effects on analytical tests

Use of silica reagents in coagulation tests should be avoided, as it may cause artificial prolongation of the partial thromboplastin time (PTT).

Children and adolescents

Do not administer this medication to children under 18 years of age, as there is no data on its safety and efficacy in this age group.

Other medications and ASPAVELI

Inform your doctor or pharmacist if you are using or have used recently or may need to use any other medication.

Pregnancy, breastfeeding and fertility

Women of childbearing age

The effects of the medication on the fetus are unknown. It is recommended to use effective contraceptive methods during treatment and for 8 weeks after treatment in women who may become pregnant. Consult your doctor before using this medication.

Pregnancy/breastfeeding

ASPAVELI is not recommended during pregnancy and breastfeeding. If you are pregnant or breastfeeding, or think you may be pregnant, or plan to become pregnant, consult your doctor before using this medication.

Driving and operating machinery

The influence of this medication on the ability to drive and operate machinery is negligible or insignificant.

ASPAVELI contains sorbitol

Sorbitol is a source of fructose. If your doctor has told you that you have an intolerance to certain sugars, or you have been diagnosed with hereditary fructose intolerance (HFI), a rare genetic disease in which the patient cannot break down fructose, consult your doctor before taking this medication.

ASPAVELI contains sodium

This medication contains less than 1 mmol of sodium (23 mg) per dose; it is essentially "sodium-free".

Follow exactly the administration instructions of this medication as indicated by your doctor. In case of doubt, consult your doctor again.

At least 2 weeks before starting treatment with this medication, your doctor will review your medical history and may administer one or more vaccines. If you cannot be vaccinated at least 2 weeks before starting treatment with ASPAVELI, to reduce the risk of infection, your doctor will prescribe antibiotics for 2 weeks after you have been vaccinated.

Dose

The recommended initial dose for adults with HPN is 1,080 mg twice a week. You should take the dose twice a week, on Day 1 and Day 4 of each week of treatment.

If you are replacing another type of medication for HPN, called a C5 inhibitor, with ASPAVELI, you should take ASPAVELI in addition to your current dose of the C5 inhibitor as prescribed for 4 weeks. After 4 weeks, you should stop taking the C5 inhibitor.

The dose or dosing interval should not be modified without consulting your doctor. Your doctor may adjust your dose to 1,080 mg every 3 days (e.g., Day 1, Day 4, Day 7, Day 10, Day 13, and so on) if necessary. If you think you have forgotten a dose, speak with your doctor as soon as possible.

Form and route of administration

ASPAVELI is intended to be administered in the form of a subcutaneous infusion (needle) using a syringe pump. The first doses of the medication will be administered by a healthcare professional in a clinic or treatment center. If treatment is going well, your doctor may discuss the possibility of administering the medication at home. If this is suitable, a healthcare professional will teach you or a caregiver how to administer the infusion.

Infusion rates

The usual infusion time is about 30 minutes if two infusion sites are used or about 60 minutes if one site is used. The infusion should be started without delay (and completed within 2 hours after the syringe is prepared) after loading this medication into the syringe.

Instructions for use

If you forgot to use ASPAVELI

If you forget a dose, you should receive it as soon as possible; then receive the next dose at the scheduled time.

If you interrupt treatment with ASPAVELI

HPN is a lifelong disease, so it is expected that you will use this medication for a long time. If you want to stop using the medication, consult your doctor first. If you interrupt treatment abruptly, you may be at risk of worsening symptoms.

If your doctor decides to stop treatment with this medication, follow their instructions on how to stop it. Your doctor will closely monitor you for at least 8 weeks after stopping treatment to detect any signs of red blood cell destruction (hemolysis) due to HPN. Symptoms or problems that may occur due to red blood cell destruction include:

-fatigue

-shortness of breath

-blood in the urine

-abdominal pain

-decrease in red blood cell count

-blood clots in the blood (thrombosis)

-difficulty swallowing

-erectile dysfunction in men

Contact your doctor if you experience any of these signs and symptoms.

Like all medicines, this medicine can cause side effects, although not everyone will experience them.

Your doctor will discuss possible side effects with you and explain the risks and benefits of ASPAVELI before treatment.

The most serious side effect is a severe infection.

If you experience any of the symptoms of infection (see section 2 “Symptoms of infection"), you must inform your doctor immediately.

In case of doubt about what the following side effects are, ask your doctor to explain them to you.

Very common(may affect more than 1 in 10 people):

• Reactions at the injection site: these include redness (erythema), swelling, itching (pruritus), rashes, and pain. These reactions usually disappear in a few days
• Upper respiratory tract infection (infection of the nose, throat, or airways)
• Diarrhea
• Red blood cell destruction (hemolysis)
• Abdominal pain (dyspepsia)
• Headache
• Fatigue (fatigue)
• Fever or elevated temperature (pyrexia)
• Cough
• Urinary tract infection
• Complications related to mandatory vaccinations
• Pain in the arms and legs (limb pain)
• Dizziness
• Joint pain (arthralgia)
• Back pain

Common(may affect up to 1 in 10 people):

• Reaction at the injection site, such as redness or skin thickening
• Ear, mouth, or skin infection
• Sore throat
• Decreased platelet count in the blood (thrombocytopenia), which may cause bleeding or the formation of rashes more easily than normal
• Nausea (nausea)
• Decreased potassium levels in the blood (hypokalemia)
• Nasal bleeding (epistaxis)
• Redness of the skin (erythema)
• Muscle pain (myalgia)
• Gastrointestinal infection, which may cause mild to severe symptoms of nausea, vomiting, abdominal cramps, diarrhea (gastrointestinal infection)
• Elevated liver function tests
• Dyspnea (shortness of breath)
• Decreased white blood cell count (neutropenia)
• Renal function deterioration
• Urine of a different color
• High blood pressure
• Muscle spasms
• Stuffy nose (nasal congestion)
• Rash
• Blood infection (sepsis)
• Viral infection
• Fungal infection
• Respiratory tract infection
• Eye infection
• COVID-19
• Bacterial infection
• Vaginal infection

Uncommon(may affect up to 1 in 100 people):

• Cervical inflammation
• Groin infection
• Nasal abscess
• Pneumonia
• Tuberculosis
• Candida esophageal infection
• Anal abscess
• Rash

Reporting of side effects

If you experience any type of side effect, consult your doctor, pharmacist, or nurse, even if it is a possible side effect that does not appear in this leaflet. You can also report them directly through thenational notification system included in theAppendix V.By reporting side effects, you can contribute to providing more information about the safety of this medicine.

• Keep this medication out of the sight and reach of children.
• Do not use this medication after the expiration date that appears on the box after “CAD”. The expiration date is the last day of the month indicated.
• Store in refrigerator (between 2 °C and 8 °C).
• Store the vial in the original box to protect it from light.
• Medicines should not be disposed of through drains. Ask your pharmacist how to dispose of the containers and medications that you no longer need. In this way, you will help protect the environment.

Composition of ASPAVELI

The active principle is pegcetacoplan 1,080 mg (54 mg/ml in a 20 ml vial).

The other components are sorbitol (E 420) (see section 2 “ASPAVELI contains sorbitol”), glacial acetic acid, sodium acetate trihydrate (see section 2 “ASPAVELI contains sodium”), sodium hydroxide (see section 2 “ASPAVELI contains sodium”) and water for injection preparations.

Appearance of the product and contents of the packaging

ASPAVELI is a transparent, colorless to slightly yellowish subcutaneous infusion solution (54 mg/ml in each 20 ml vial). Turbid solutions or those with particles or color changes should not be used.

Packaging sizes

ASPAVELI is available in a 1 vial pack or a multi-pack of 1 x 8 vials.

Please note that the packaging does not contain alcohol swabs, needles, or other supplies or equipment.

Only some packaging sizes may be commercially available.

Marketing authorization holder

Swedish Orphan Biovitrum AB (publ)

SE-112 76 Stockholm

Sweden

Responsible manufacturer

Swedish Orphan Biovitrum AB (publ)

Norra Stationsgatan93

11364Stockholm

Sweden

Last review date of this leaflet: 05/2024.

Other sources of information

Detailed information on this medicine is available on the European Medicines Agency website:http://www.ema.europa.eu.There are also links to other websites on rare diseases and orphan medicines.