BEKEMV 300 mg concentrate for solution for infusion

• Keep this leaflet. You may need to read it again.
• If you have any further questions, ask your doctor, pharmacist or nurse.
• This medicine has been prescribed for you only. Do not pass it on to others. It may harm them, even if their signs of illness are the same as yours.
• If you get any side effects, talk to your doctor, pharmacist or nurse. This includes any possible side effects not listed in this leaflet. See section 4.

1. What BEKEMV is and what it is used for
2. What you need to know before you use BEKEMV
3. How to use BEKEMV
4. Possible side effects
5. How to store BEKEMV
6. Contents of the pack and other information

BEKEMV contains the active substance eculizumab and it belongs to a class of medicines called monoclonal antibodies. Eculizumab binds to and inhibits a specific protein in the body that causes inflammation and so prevents your body’s systems from attacking and destroying vulnerable blood cells or kidneys.

Paroxysmal Nocturnal Haemoglobinuria

BEKEMV is used to treat adults and children with a certain type of disease affecting the blood system called Paroxysmal Nocturnal Haemoglobinuria (PNH). In patients with PNH, their red blood cells can be destroyed which can lead to low blood counts (anaemia), tiredness, difficulty in functioning, pain, dark urine, shortness of breath, and blood clots. Eculizumab can block the body’s inflammatory response, and its ability to attack and destroy its own vulnerable PNH blood cells.

Atypical Haemolytic Uraemic Syndrome

BEKEMV is also used to treat adults and children with a certain type of disease affecting the blood system and kidney called atypical Haemolytic Uraemic Syndrome (aHUS). In patients with aHUS, their kidney and blood cells, including platelets, can be inflamed which can lead to low blood counts (thrombocytopenia and anaemia), reduced or lost kidney function, blood clots, tiredness and difficulty in functioning. Eculizumab can block the body’s inflammatory response, and its ability to attack and destroy its own vulnerable blood and kidney cells.

• If you are allergic to eculizumab or any of the other ingredients of this medicine (listed in section 6).
• In babies and young children below 2 years of age hereditary fructose intolerance (HFI) may not yet be diagnosed and may be fatal, thus, they must not receive this medicine (see “BEKEMV contains sorbitol”).
• If you have not been vaccinated against meningococcal infection unless you take antibiotics to reduce the risk of infection until 2 weeks after you have been vaccinated.
• If you have a meningococcal infection.

Meningococcal and other Neisseria infections alert

BEKEMV treatment may reduce your natural resistance to infections, especially against certain organisms that cause meningococcal infection (severe infection of the linings of the brain and sepsis) and other Neisseria infections including disseminated gonorrhoea.

Consult your doctor before you take BEKEMV to be sure that you receive vaccination against Neisseria meningitidis, an organism that causes meningococcal infection, at least 2 weeks before beginning therapy, or that you take antibiotics to reduce the risk of infection until 2 weeks after you have been vaccinated.

Ensure that your current meningococcal vaccination is up to date. You should also be aware that vaccination may not prevent this type of infection. In accordance with national recommendations, your doctor might consider that you need supplementary measures to prevent infection.

If you are at risk of gonorrhoea, ask your doctor or pharmacist for advice before using this medicine.

Meningococcal infection symptoms

Because of the importance of rapidly identifying and treating certain types of infection in patients who receive BEKEMV, you will be provided a card to carry with you, listing specific trigger symptoms. This card is named: “Patient Safety Card”.

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

• headache with nausea or vomiting
• headache with a stiff neck or back
• fever
• rash
• confusion
• severe muscle aches combined with flu-like symptoms
• sensitivity to light

Treatment for meningococcal infection while travelling

If you are travelling in a remote region where you are unable to contact your doctor or in which you find yourself temporarily unable to receive medical treatment, your doctor can make arrangements to issue, as a preventive measure, a prescription for an antibiotic to counter Neisseria meningitidis that you keep with you. If you experience any of the symptoms amongst those cited above, you should take the antibiotics as prescribed. You should bear in mind that you should see a doctor as soon as possible, even if you feel better after having taken the antibiotics.

Before starting BEKEMV, inform your doctor if you have any infections.

BEKEMV contains a protein and proteins can cause allergic reactions in some people.

Patients less than 18 years of age must be vaccinated against Haemophilus influenzae and pneumococcal infections.

There are no special precautions needed for the treatment of patients aged from 65 years and over.

Tell your doctor or pharmacist if you are using or have recently used or might use any other medicines.

If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before using this medicine.

Women of childbearing potential

The use of effective contraception during treatment and up to 5 months after treatment should be considered in women who are able to get pregnant.

BEKEMV has no or negligible influence on the ability to drive and use machines.

This medicine contains 50 mg sorbitol in each mL.

Sorbitol is a source of fructose. If you (or your child) have hereditary fructose intolerance (HFI), a rare genetic disorder, you (or your child) must not receive this medicine. Patients with HFI cannot break down fructose, which may cause serious side effects.

You must tell your doctor before receiving this medicine if you (or your child) have HFI or if your child can no longer take sweet foods or drinks because they feel sick, vomit or get unpleasant effects such as bloating, stomach cramps or diarrhoea.

This medicine contains less than 1 mmol of sodium (23 mg) per dose, that is to say essentially “sodium free”.

The treatment will be given by your doctor or other health care provider by infusing a dilution of the BEKEMV vial from a drip bag through a tube directly into one of your veins. It is recommended that the beginning of your treatments, called the initial phase, will extend over 4 weeks, followed by a maintenance phase.

If you use this medicine to treat PNH

For adults:

• Initial phase:

Every week for the first four weeks, your doctor will administer an intravenous infusion of diluted BEKEMV. Each infusion will consist of a dose of 600 mg (2 vials of 30 mL) and will take 25 - 45 minutes (35 minutes ± 10 minutes).

• Maintenance phase:
  • In the fifth week, your doctor will administer an intravenous infusion of diluted BEKEMV at a dose of 900 mg (3 vials of 30 mL) over a 25 – 45 minute (35 minutes ± 10 minutes) period.
  • After the fifth week, your doctor will administer 900 mg of diluted BEKEMV every two weeks as a long-term treatment.

If you use this medicine to treat aHUS

For adults:

• Initial phase:

Every week for the first four weeks, your doctor will administer an intravenous infusion of diluted BEKEMV. Each infusion will consist of a dose of 900 mg (3 vials of 30 mL) and will take 25 – 45 minutes (35 minutes ± 10 minutes).

• Maintenance phase:
  • In the fifth week, your doctor will administer an intravenous infusion of diluted BEKEMV at a dose of 1,200 mg (4 vials of 30 mL) over a 25 – 45 minute (35 minutes ± 10 minutes) period.
  • After the fifth week, your doctor will administer 1,200 mg of diluted BEKEMV every two weeks as a long-term treatment.

For children and adolescents:

Children and adolescents with PNH or aHUS and who are 40 kg weight and over are treated with the adult dosing.

Children and adolescents with PNH or aHUS and who are under 40 kg weight require a lower dose based on how much they weigh. Your doctor will calculate this.

For children and adolescents with PNH or aHUS above 2 years of age and with body weight below 40 kg:

Patient body weight – 30 to < 40 kg

Initial phase – 600 mg weekly for the first 2 weeks

Maintenance phase – 900 mg at week 3; then 900 mg every 2 weeks

Patient body weight – 20 to < 30 kg

Initial phase – 600 mg weekly for the first 2 weeks

Maintenance phase – 600 mg at week 3; then 600 mg every 2 weeks

Patient body weight – 10 to < 20 kg

Initial phase – 600 mg weekly single dose at week 1

Maintenance phase – 300 mg at week 2; then 300 mg every 2 weeks

Patient body weight – 5 to < 10 kg

Initial phase – 300 mg weekly single dose at week 1

Maintenance phase – 300 mg at week 2; then 300 mg every 3 weeks

Subjects who undergo plasma exchange may receive additional doses of BEKEMV.

Following each infusion, you will be monitored for about one hour. Your doctor’s instructions should be carefully observed.

If you suspect that you have been accidentally administered a higher dose of BEKEMV than prescribed, please contact your doctor for advice.

If you forget an appointment, please contact your doctor immediately for advice and see section below “If you stop using BEKEMV”.

Interrupting or ending treatment with BEKEMV may cause your PNH symptoms to come back more severely soon. Your doctor will discuss the possible side effects with you and explain the risks. Your doctor will want to monitor you closely for at least 8 weeks.

The risks of stopping BEKEMV include an increase in the destruction of your red blood cells, which may cause:

• A significant fall in your red blood cell counts (anaemia),
• Confusion or change in how alert you are,
• Chest pain, or angina,
• An increase in your serum creatinine level (problems with your kidneys), or
• Thrombosis (blood clotting).

If you have any of these symptoms, contact your doctor.

Interrupting or ending treatment with BEKEMV may cause your aHUS symptoms to come back. Your doctor will discuss the possible side effects with you and explain the risks. Your doctor will want to monitor you closely.

The risks of stopping BEKEMV include an increase in the inflammation of your platelets, which may cause:

• A significant fall in your platelets (thrombocytopenia),
• A significant rise in destruction of your red blood cells,
• Decreased urination (problems with your kidneys),
• An increase in your serum creatinine level (problems with your kidneys),
• Confusion or change in how alert you are,
• Chest pain, or angina,
• Shortness of breath, or
• Thrombosis (blood clotting).

If you have any of these symptoms, contact your doctor.

If you get any side effects, talk to your doctor or pharmacist or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly (see details below). By reporting side effects you can help provide more information on the safety of this medicine.

or search for MHRA Yellow Card in the Google Play or Apple App Store

• The active substance is eculizumab (300 mg/30 mL in a vial corresponding to 10 mg/mL).
• The other ingredients are:
  • acetic acid,
  • sodium hydroxide,
  • disodium edetate (EDTA),
  • sorbitol (E420),
  • polysorbate 80,
  • water for injections.

BEKEMV is presented as a concentrate for solution for infusion (30 mL in a vial – pack size of 1).

BEKEMV is a clear to slightly opalescent, colourless to slightly yellow solution.