Human Hemin Orphan Europe

Leaflet attached to the packaging: information for the user

HUMAN HEMIN ORPHAN EUROPE 25 mg/ml, concentrate for solution for infusion.

Human hemin

Read the leaflet carefully before using the medicine, as it contains important information for the patient.

Keep this leaflet, you may need to read it again.

• In case of any doubts, consult a doctor.
• If the patient experiences any side effects, including any side effects not listed in this leaflet, tell the doctor, pharmacist, or nurse. See section 4.

Table of contents of the leaflet:

• 1. What is Human Hemin Orphan Europe and what is it used for
• 2. Important information before using Human Hemin Orphan Europe
• 3. How to use Human Hemin Orphan Europe
• 4. Possible side effects
• 5. How to store Human Hemin Orphan Europe
• 6. Contents of the packaging and other information

1. What is Human Hemin Orphan Europe and what is it used for

Human Hemin Orphan Europe contains human hemin, a substance derived from human blood.
Human Hemin Orphan Europe is used to treat acute attacks in patients suffering from acute hepatic porphyria. This disease is characterized by the accumulation of certain chemical compounds in the liver (including porphyrins and their toxic precursors). There are three types of hepatic porphyria with the following medical names: acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria.
The accumulation of substances leads to the occurrence of disease symptoms, including pain (mainly abdominal, back, and limb), nausea, vomiting, and constipation.

2. Important information before using Human Hemin Orphan Europe

When not to use Human Hemin Orphan Europe

Warnings and precautions

• Before starting treatment with Human Hemin Orphan Europe, the doctor should confirm the diagnosis of acute hepatic porphyria based on a series of clinical and biological criteria: significant family and medical history; clear clinical symptoms; quantitative determination of delta-aminolevulinic acid and porphobilinogen (i.e., specific disease markers) in urine
• The sooner the treatment with Human Hemin Orphan Europe is started after the onset of the attack, the more effective the treatment will be.

As a result of administering Human Hemin Orphan Europe by infusion, abdominal pain and other gastrointestinal symptoms usually disappear within 2-4 days. The treatment has a smaller effect on neurological complications (paralysis and mental disorders).
During the full treatment cycle, the doctor will monitor the patient's condition, as acute porphyria attacks are often accompanied by various effects on the heart and circulatory system, as well as the nervous system.

• Do not:
• make sudden changes to your daily diet, in particular, do not make long breaks in eating;
• take medications or substances such as estrogens (e.g., oral contraceptives), barbiturates (sleeping pills, also used to treat epilepsy), or steroids (derivatives of natural hormones); their use may cause an attack or worsen its course. Always consult a doctor or pharmacist for advice on medications and substances that should not be used (now and in the future).
• To prevent vein irritation, the solution should be administered into large veins in the arm or chest for at least 30 minutes. After the infusion, the vein should be flushed with a physiological saline solution.

Blood clots (a condition called venous thrombosis) can block the vein into which the medicine was administered by infusion.

• If the intravenous cannula is left in the vein for too long, it may cause vessel damage, which can lead to unintended leakage of Human Hemin Orphan Europe outside the vein (extravasation). This leakage can cause skin discoloration.

To minimize the risk of extravasation, the nurse/doctor will check the cannula before infusion and will regularly check it during infusion.
The solution administered by infusion may give the patient's blood a different color.
To limit the risk of iron accumulation, Human Hemin Orphan Europe should not be used as a preventive treatment for acute attacks.
Human hemin contains iron. It may happen that after several years of treatment involving repeated administration of Human Hemin Orphan Europe by infusion, iron will accumulate in the body.
The doctor may from time to time perform blood tests to check the iron content in the patient's body.

• Standard measures to protect against infections resulting from the use of products derived from human blood or plasma involve proper selection of donors, testing each donated unit for specific infection markers, and strict adherence to the manufacturing process stages aimed at inactivating or removing viruses. Despite this, the risk of transmitting infectious agents cannot be excluded if the medicines are prepared from plasma and human blood. This also applies to unknown or newly emerging viruses and other pathogens.
• The methods used are considered effective for enveloped viruses, such as HIV, HBV, and HCV. It is necessary to record the name and batch number of the Human Hemin Orphan Europe preparation each time it is administered to the patient, in order to identify the preparation administered later.

Human Hemin Orphan Europe and other medicines

Do not take medications or substances such as estrogens (e.g., oral contraceptives), barbiturates (sleeping pills, also used to treat epilepsy), or steroids (medicines similar to hormones found in the body), as they may trigger an attack or worsen its course.
Tell the doctor about all medicines currently or recently used by the patient, as well as about medicines that the patient plans to use.

Pregnancy and breastfeeding

It is not known whether the use of Human Hemin Orphan Europe during pregnancy is associated with a risk.
However, it is known that mothers treated with Human Hemin Orphan Europe have given birth to normal children.
Always, when pregnant, before administering Human Hemin Orphan Europe, consult a doctor.
The doctor will prescribe the medicine only if it is absolutely necessary..
No studies have been conducted on Human Hemin Orphan Europe in breastfeeding women. However, since many medicines pass into breast milk, always inform the doctor about breastfeeding before starting to take Human Hemin Orphan Europe. The doctor will prescribe the medicine only if it is absolutely necessary, or may recommend stopping breastfeeding.
Human Hemin Orphan Europe contains ethanol (alcohol). This should be taken into account in case of pregnancy or breastfeeding. See the section "Important information about some ingredients of Human Hemin Orphan Europe".

Driving and using machines

The medicine should not affect the ability to drive and use machines.

Important information about some ingredients of Human Hemin Orphan Europe

The medicine contains 11.78% v/v ethanol (alcohol), i.e., up to 1000 mg per dose (one ampoule), which corresponds to 23.6 ml of beer or 9.8 ml of wine per dose. Harmful to people with alcoholism. This should be taken into account when used in pregnant or breastfeeding women, children, and in people at high risk, such as patients with liver disease or epilepsy.
In case of any of the above conditions, before using Human Hemin Orphan Europe, the patient should consult a doctor.

3. How to use Human Hemin Orphan Europe

This medicine can only be administered in a hospital setting by qualified medical personnel.
The prescribed dose will be calculated based on the patient's body weight; this dose is approximately 3 mg per kilogram of body weight per day, but not more than 250 mg (1 ampoule) per day. The calculated amount of the medicine will be diluted with physiological saline solution (0.9% sodium chloride solution) in a glass bottle, creating a dark-colored solution.
The solution will be administered by infusion (infusion) into a large vein (blood vessel) in the forearm or in the chest, over a period of at least 30 minutes. The administered solution may give the blood an unnatural color.
After the infusion is completed, the vein will be flushed with physiological saline solution.
Usually, the patient receives one infusion per day for four days.
If the symptoms do not disappear during the first treatment cycle, the doctor may decide to start a second treatment cycle.

Overdose of Human Hemin Orphan Europe

If a higher dose of Human Hemin Orphan Europe is administered than recommended, the doctor will undertake treatment aimed at preventing harmful effects.

4. Possible side effects

Like all medicines, Human Hemin Orphan Europe can cause side effects, although not everybody gets them.
Rare (may occur in up to 1 in 1000 people):

• -Rarely, fever and severe allergic reactions (rash, tongue swelling), including anaphylactoid reactions, may occur.

Anaphylactoid reactions are sudden and potentially life-threatening reactions that occur rarely. If the patient experiences symptoms such as facial swelling, shortness of breath, chest tightness, rapid heartbeat, low blood pressure, hives, sudden loss of consciousness (caused by insufficient blood flow to the brain), the infusion should be stopped and the doctor should be contacted immediately.
Very common(may occur in more than 1 in 10 people):

• -After repeated infusions, access to the arm vein may be difficult, which means that infusion into the chest vein may be necessary. Common(may occur in up to 1 in 10 people):
• Administering the medicine by infusion into too small a vein may lead to inflammation and pain.

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

• -As a result of repeated treatment over several years, the amount of iron compound (called ferritin) in the blood may increase. To minimize the risk of increasing the iron compound, Human Hemin Orphan Europe should not be used as a preventive treatment for acute attacks. Frequency not known(frequency cannot be estimated from the available data):
• Headache.
• Thrombosis (formation of blood clots in peripheral or central veins), including thrombosis at the injection site.
• Extravasation of the infusion into surrounding tissues.
• Skin damage (necrosis).
• Redness of the skin at the injection site (injection site erythema).
• Itching of the skin at the injection site (injection site pruritus).
• Increased creatinine levels (a substance excreted by the kidneys) in the blood.
• Discoloration of the skin.

Reporting side effects

If any side effects occur, including any side effects not listed in the leaflet, tell the doctor, pharmacist, or nurse. Side effects can be reported directly to the Department of Monitoring of Adverse Reactions to Medicinal Products, Office for Registration of Medicinal Products, Medical Devices, and Biocidal Products {current address, phone number, and fax of the aforementioned Department} e-mail: ndl@urpl.gov.pl. By reporting side effects, more information can be collected on the safety of the medicine.

5. How to store Human Hemin Orphan Europe

Keep the medicine out of sight and reach of children.
Do not use this medicine after the expiry date stated on the ampoule label and the carton. The expiry date refers to the last day of the month.
Store in a refrigerator (2°C – 8°C).
Keep the ampoule in the outer carton in order to protect from light.
The diluted solution should be used within one hour of dilution.
Medicines should not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of medicines no longer required. This will help protect the environment.

6. Contents of the packaging and other information

What Human Hemin Orphan Europe contains

The active substance of the medicine is human hemin (25 mg/ml). One 10 ml ampoule contains 250 mg of human hemin. After dilution of one 10 ml ampoule in 100 ml of 0.9% NaCl solution, the diluted solution contains 2273 micrograms of human hemin per ml.

• The other ingredients are: arginine, ethanol 96%, propylene glycol, and water for injections.

What Human Hemin Orphan Europe looks like and contents of the pack

Human Hemin Orphan Europe is a concentrate for solution for infusion (10 ml ampoule – pack of 4 ampoules). It is a dark-colored solution, even after dilution of the concentrate for solution for infusion.

Marketing authorization holder

Recordati Rare Diseases
Immeuble « Le Wilson »
70, avenue du Général de Gaulle
F-92800 Puteaux
France

Manufacturer

Recordati Rare Diseases
Immeuble « Le Wilson »
70, avenue du Général de Gaulle
F-92800 Puteaux
France
or
Recordati Rare Diseases
Eco River Parc
30, rue des Peupliers
F-92000 Nanterre
France

This medicinal product is authorized in the Member States of the European Economic Area under the following names:

Normosang – Austria / France / Belgium / Cyprus / Czech Republic / Denmark / Estonia / Finland / Greece / Spain / Netherlands / Ireland / Iceland / Latvia / Lithuania / Luxembourg / Malta / Germany / Norway / Portugal / Slovakia / Slovenia / Sweden / Hungary / United Kingdom / Italy
Human Hemin Orphan Europe – Poland

Date of last revision of the leaflet 12/2019