Human hemin orphan europe

Package leaflet: Information for the user

HUMAN HEMIN ORPHAN EUROPE 25 mg/ml, concentrate for solution for infusion.
Human heme
Please read all of this leaflet carefully before using this medicine because it contains important information for you.
Keep this leaflet as you may need to read it again.

• If you have any further questions, please ask your doctor.
• If you experience any side effects, including any not listed in this leaflet, tell your doctor, pharmacist, or nurse. See section 4.

Contents of the leaflet:

1. What Human Hemin Orphan Europe is and what it is 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 pack and other information

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

Human Hemin Orphan Europe contains human heme, a substance derived from human blood.
Human Hemin Orphan Europe is used for the treatment of acute attacks in patients with 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 these substances leads to the occurrence of disease symptoms, including pain (mainly in the abdomen, back, and legs), nausea, vomiting, and constipation.

2. Important information before using Human Hemin Orphan Europe

When not to use Human Hemin Orphan Europe

• if the patient is allergic to the active substance or to any of the other components of this medicine (listed in section 6).

Warnings and precautions

• Before starting treatment with Human Hemin Orphan Europe, the treating physician should confirm an acute attack of hepatic porphyria based on several clinical and biological criteria: suggestive family history and patient medical history; clear clinical symptoms; quantitative measurement of urinary delta-aminolevulinic acid and porphobilinogen (specific disease markers).
• The earlier Human Hemin Orphan Europe is administered after the onset of an attack, the greater its therapeutic effectiveness.

Following intravenous infusion of Human Hemin Orphan Europe, abdominal pain and other gastrointestinal symptoms usually resolve within 2–4 days. The effect of treatment on neurological complications (paralysis and psychiatric disturbances) is less pronounced.
During the full course of treatment, the physician will monitor the patient's condition, as attacks of porphyria are often accompanied by various effects on the heart and circulatory system, as well as on the nervous system.

• Patients should avoid:
• making sudden changes to their daily diet, particularly prolonged fasting;
• taking medications or substances such as estrogens (e.g. oral contraceptives), barbiturates (sleeping pills, also sometimes used in epilepsy treatment), or steroids (derivatives of natural hormones); such substances may trigger or worsen an attack. Always consult a doctor or pharmacist for advice regarding medications and substances that should not be used (now or in the future).
• To minimize the risk of vein irritation, the solution should be administered into large veins in the arm or into chest veins over a period of at least 30 minutes. After infusion, the vein should be flushed with physiological saline solution.

Blood clot (a condition known as venous thrombosis) may block the vein into which the medicine has been infused.

• If the intravenous catheter remains in the vein for too long, vascular damage may occur, potentially leading to unintended leakage of Human Hemin Orphan Europe outside the vein (extravasation). Such leakage may cause skin discoloration.

To reduce the risk of extravasation, the nurse/physician should check the catheter before infusion and monitor it regularly during infusion.
The infused solution may give the patient's blood a different color.
To minimize the risk of iron accumulation, Human Hemin Orphan Europe should not be used as a preventive treatment for acute attacks.
Human heme contains iron. It may happen that after several years of treatment involving repeated infusions of Human Hemin Orphan Europe, iron accumulates in the body.
The treating physician may occasionally perform blood tests to monitor iron levels in the patient's body.

• Standard measures to prevent infections associated with products derived from human blood or plasma include careful selection of donors, testing of each collected donation for specific infection markers, and strict adherence to manufacturing procedures designed to inactivate or remove viruses. Nevertheless, the risk of transmitting infectious agents cannot be completely ruled out when medicines prepared from plasma and human blood are administered. This also applies to unknown or newly emerging viruses and other pathogens.
• The methods used are considered effective against enveloped viruses such as HIV, HBV, and HCV. It is important that each time Human Hemin Orphan Europe is administered to a patient, the name and batch number of the product are recorded to allow subsequent identification of the product given to that patient.

Human Hemin Orphan Europe and other medicines
Do not take medications or substances such as estrogens (e.g. oral contraceptives), barbiturates (sleeping pills, also sometimes used in epilepsy treatment), or steroids (hormone-like drugs), as they may trigger an attack or worsen its course.
Inform your doctor about all medicines currently or recently used, as well as any medicines planned for future use.

Pregnancy and breastfeeding
It is not known whether the use of Human Hemin Orphan Europe during pregnancy involves risks.
However, it is known that mothers treated with Human Hemin Orphan Europe have given birth to healthy children.
Always consult your doctor before administering Human Hemin Orphan Europe if you are pregnant.
The doctor will prescribe the medicine only if absolutely necessary.
Human Hemin Orphan Europe has not been studied in breastfeeding women. However, since many drugs pass into breast milk, always inform your doctor if you are breastfeeding before starting treatment with Human Hemin Orphan Europe. The doctor will prescribe the medicine only if absolutely necessary or may recommend discontinuing breastfeeding.
Human Hemin Orphan Europe contains ethanol (alcohol). This should be taken into account during pregnancy or breastfeeding. See section “Important information about certain components of Human Hemin Orphan Europe”.

Driving and operating machinery
This medicine is not expected to affect the ability to drive or operate machinery.

Important information about certain components of Human Hemin Orphan Europe
This medicine contains 11.78% v/v ethanol (alcohol), i.e. up to 1000 mg per dose (one vial), equivalent to 23.6 ml of beer or 9.8 ml of wine per dose. Harmful for individuals with alcohol-related disorders. This should be considered when administering the medicine to pregnant or breastfeeding women, children, and individuals in high-risk groups such as patients with liver disease or epilepsy.
If any of the above conditions apply, the patient should consult a doctor before using Human Hemin Orphan Europe.

3. How to use Human Hemin Orphan Europe

This medicine may only be administered in a hospital setting by qualified medical personnel.
The dose to be administered will be calculated based on the patient's body weight; the dose is approximately 3 mg per kilogram of body weight daily, but not more than 250 mg (1 vial) per day. The calculated amount of medicine will be diluted in physiological saline (0.9% sodium chloride solution) in a glass bottle, forming a darkly coloured solution.
The solution will be administered as an infusion (intravenous drip) into a large vein (blood vessel) of the forearm or into a vein located within the chest area, over a period of at least 30 minutes. The infused solution may give blood an unnatural colour.
After completion of the infusion, the vein will be flushed with physiological saline.
Typically, the patient receives one infusion daily for four days.
If symptoms do not resolve during the first treatment cycle, the physician may exceptionally decide to initiate a second treatment cycle.
Overdose of Human Hemin Orphan Europe
If a higher than recommended dose of Human Hemin Orphan Europe has been administered, the physician will initiate treatment aimed at preventing harmful effects.

4. Possible adverse reactions

Like all medicines, this medicine can cause adverse reactions, although not everyone will experience them.

Rare (may affect up to 1 in 1,000 people ):

• Fever and severe allergic reactions (rash, swelling of the tongue), including anaphylactoid reactions, may rarely occur.

Anaphylactoid reactions are sudden and potentially life-threatening reactions that occur rarely. If the patient experiences symptoms such as facial swelling, difficulty breathing, chest tightness, rapid heartbeat, low blood pressure, urticaria, or sudden loss of consciousness (caused by insufficient blood flow to the brain), the infusion must be stopped immediately and medical help must be sought without delay.

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

• After repeated infusions, access to the vein in the arm may become difficult, necessitating infusion into a vein in the chest area.

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

• Administering the medicine via infusion into too small a vein may lead to inflammation and pain.

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

• Repeated treatment over several years may lead to increased levels of an iron compound (called ferritin) in the blood. To reduce the risk of iron accumulation, Human Hemin Orphan Europe should not be used as a preventive treatment for acute attacks.

Frequency not known (frequency cannot be estimated from available data ):

• Headache.
• Venous thrombosis (formation of blood clots in peripheral or major veins), including thrombosis at the injection site.
• Leakage of infused fluid into surrounding tissues (extravasation).
• Skin damage (necrosis).
• Redness of the skin at the injection site (erythema at injection site).
• Itching at the injection site (pruritus at injection site).
• Increased blood creatinine levels (a substance excreted by the kidneys).
• Skin discoloration.

Reporting of adverse reactions
If any adverse reactions occur, including any not listed in this leaflet, inform your doctor, pharmacist, or nurse. Adverse reactions can also be reported directly to the Department of Monitoring Adverse Drug Reactions, Office for Registration of Medicinal Products, Medical Devices and Biocidal Products {current address, telephone and fax numbers of the Department}. E-mail: [email protected]. Reporting adverse reactions helps provide more information on the safety of this medicine.

5. How to store Human Hemin Orphan Europe

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

6. Contents of the pack and other information

What Human Hemin Orphan Europe contains
The active substance in this medicine is human heme (25 mg/ml). A 10 ml vial contains 250 mg of human heme.
After diluting one 10 ml vial in 100 ml of 0.9% NaCl solution, the diluted solution contains 2273 micrograms of human heme per ml.

• Other components 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 vials – pack of 4 vials). It is a darkly coloured solution, even after dilution of the concentrate for solution for infusion.

Marketing Authorisation 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 authorised in the European Economic Area countries 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