25 mg/ml concentrate for solution for infusion Human
hemin
What Normosang is and what it is used for
Formosan contains human hemin, which is a substance derived from human blood.
Formosan is used to treat acute seizures in patients suffering from acute hepatic porphyria, a disease characterized by the accumulation of certain substances in the liver (including porphyrins and their toxic precursors). There are three types of hepatic porphyria. The medical names are acute intermittent porphyria, porphyria variegata, and hereditary coproporphyria. This accumulation causes disease symptoms such as pain (including pain in the abdomen, back, and thighs), nausea, vomiting, and constipation.
What you need to know before using Normosang
Do not use Normosang
– if you are allergic to human hemin or any of the other ingredients of this medicine (listed in section 6).
Warnings and cautions
- During treatment with Normosang, your doctor should be able to confirm that you have a seizure of hepatic porphyria using a number of clinical and biological criteria:
- family background or personal background indicating this
- clinical signs indicating this
- quantitative determination of delta-aminolevulinic acid and porphobilinogen (special markers of disease) in the urine.
- The faster treatment with Normosang is started after the onset of the attack, the greater the effect.
- Abdominal pain and other gastrointestinal symptoms usually resolve within 2-4 days as a result of infusion of Normosang. Neurological complications (paralysis and psychological disorders) are less affected by the treatment.
- You will be monitored throughout the treatment period as porphyria attacks are often associated with various effects on the heart and blood circulation as well as on the nervous system.
- Avoid:
- Sudden changes in your normal diet, especially long periods of fasting.
- Taking medicines or substances that contain estrogen (eg oral contraceptives), barbiturates (sleeping pills and medicines that are sometimes used to treat epilepsy ) or steroids (body hormone-like medicines) as these can trigger or aggravate an attack.
Ask your doctor or pharmacist for advice on which medicines or substances you should not use (now and in the future)
- To prevent irritation in the vein, the solution is administered as an infusion into a large vein (blood vessel) in your arm or in a chest vein for a period of at least 30 minutes. After infusion one, the vein should be flushed with saline solution.
- A blood clot (so-called “venous thrombosis”) can clog the vein used for infusion .
- Vascular damage can occur if your cannula is left for a long time and this can lead to an unintentional migration of Normosang outside the vein (extravasation). This migration can cause discoloration of the skin.
- To reduce the risk of extravasation, the nurse / physician will test your needle before infusion one and then check it regularly during infusion one.
- The infused solution may give your blood an unusual color.
- To minimize the risk of iron buildup, Normosang should not be given as a preventative treatment for acute seizures.
- Human hemin contains iron. Iron may accumulate in the body after several years of treatment with repeated infusions of Normosang. Your doctor will therefore take blood samples at regular intervals to check the iron levels in your body.
- Standard precautions to prevent infection as a result of the use of medicinal products prepared from human blood or
plasma include the selection of donors, the examination of individual donations for specific markers of infections and the use of effective manufacturing steps to inactivate / remove viruses . Nevertheless, the risk of transmission of infectious substances can not be completely ruled out when administering medicinal products prepared from human blood or plasma . This also applies to unknown or new viruses and other pathogens . - The precautions taken are considered effective against enveloped viruses such as HIV , HBV and HCV . It is strongly recommended that the name and batch number of the product be registered each time Normosang is administered to a patient in order to maintain a link between the patient and the product set.
Other medicines and Normosang
Do not use medicines or substances such as estrogen (eg oral contraceptives), barbiturates (sleeping pills and medicines that are sometimes used to treat epilepsy ), or steroids (body hormone-like medicines) as these may cause or aggravate a seizure.
Tell your doctor if you are taking, have recently taken, or might take any other medicines.
Pregnancy and breastfeeding
It is not known if Normosang poses a risk during pregnancy. However, mothers who have been treated with Normosang have given birth to normal children. Ask your doctor if you are pregnant before receiving Normosang. Your doctor will only prescribe the medicine if necessary.
Formosan has not been studied during breastfeeding. However, as some medicines are excreted in breast milk, you should inform and consult your doctor when breastfeeding before receiving Normosang. Your doctor will only prescribe treatment with Normosang when necessary or recommend that you stop breastfeeding.
Normosang contains ethanol (alcohol). This should be considered if you are pregnant or breastfeeding. See the heading “Normosang contains alcohol”.
Driving and using machines
There is no evidence that your medicine affects your ability to drive and use machines.
Normosang contains alcohol
Normosang contains 11.78 vol. % ethanol (alcohol), i.e. up to 1000 mg per daily dose (one ampoule ), equivalent to 23.6 ml of beer or 9.8 ml of wine per daily dose.
This can be harmful to those who suffer from alcoholism. It should also be considered during pregnancy and lactation, children, and high-risk groups such as patients with liver disease or epilepsy.
Therefore, consult your doctor or pharmacist when receiving Normosang if you suffer from any of the above conditions.
How to use Normosang
The medicine will only be administered by qualified hospital staff.
The dose you receive is calculated based on your body weight and is approximately 3 mg per kg body weight per day, but not more than 250 mg (1 ampoule ) per day. The calculated amount is diluted in a saline solution (0.9% sodium chloride) in a glass bottle and it gives a dark-colored solution.
The solution is administered by infusion into a large vein (blood vessel) in your arm or a central vein for at least 30 minutes. The infused solution may give your blood an unusual color.
After infusion one, the vein should be flushed with saline solution.
You will usually receive one infusion per day for four days.
If the symptoms do not improve after the first round of treatment, the doctor may in exceptional cases decide to start a new round of treatment.
If you take more Normosang than you should
If you have been given too much Normosang, your doctor will treat you to prevent harmful effects.
Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them.
Rare (may affect up to 1 in 1,000 people) :
- Fever and severe allergic reactions (rash, swollen tongue) including anaphylactic reactions may occur in rare cases.
Anaphylactic reactions are sudden and life-threatening reactions that can occur in rare cases. If you get such symptoms as swelling of the face, difficulty breathing ( dyspnoea ), constriction in the chest, rapid heartbeat ( tachycardia ), low blood pressure, hives, spontaneous unconsciousness (due to insufficient blood supply to the brain) then infusion must be stopped and a doctor must be called immediately.
Very common (may affect more than 1 in 10 people) :
- Repeated treatments can make it difficult to access a vessel in your arms, which may require the use of a central vein.
Common (may affect up to 1 in 10 people) :
- If the drug is administered in a vessel that is too small , it can cause pain and inflammation .
Uncommon (may affect up to 1 in 100 people) :
- The amount of an iron compound (called ferritin ) can increase in the blood during repeated treatments for several years. To limit the risk of increased iron concentration, Normosang should not be used for the prophylaxis of acute seizures.
No known frequency (can not be calculated from the available data) :
- Headache.
- Venous thrombosis (clots form in the peripheral or central veins) including thrombosis at the injection site.
- Leakage of the infusion fluid to nearby tissue (extravasation).
- Skin damage ( necrosis ).
- Skin redness at the injection site ( erythema at the injection site).
- Skin itching at the injection site ( pruritus at the injection site).
- Increase in creatinine concentration in the blood (a substance secreted by the kidneys).
- Discoloration of the skin.
How to store Normosang
Keep this medicine out of the sight and reach of children.
Do not use this medicine after the expiry date which is stated on the ampoule label and after EXP. The expiration date is the last day of the specified month.
Store in a refrigerator (2 ° C-8 ° C).
Store ampoule one in the outer carton – Sensitive to light.
The solution should be used within one hour after dilution.
Medicines should not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of medicines no longer required. These measures will help to protect the environment.
Contents of the pack and other information
Content declaration
- The active substance is human hemin (25 mg / ml). A 10 ml ampoule contains 250 mg of human hemin. After diluting a 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 the medicine looks like and contents of the pack
Normosang is a concentrate solution for infusion (10 ml ampoules – pack size of 4 ampoules ). Normosang is a dark-colored solution even after dilution of the concentrate for solution for infusion.
Marketing Authorisation Holder
Record Rare Diseases
“Le Wilson” property
70 avenue du Général de Gaulle
F-92800 Puteaux
France
Manufacturer
Record Rare Diseases
Real Estate “Le Wilson”
70, avenue du Général de Gaulle
F-92800 Puteaux
France
or
Record Rare Diseases
Eco River Parc
30, rue des Peupliers
F-92000 Nanterre
France
This medicinal product is authorized under the European Economic Area under the names:
Normosang – Belgium / Cyprus / Denmark / Estonia / Finland / France / Great Britain / Greece / Ireland / Iceland / Italy / Latvia / Lithuania / Luxembourg / Malta / Netherlands / Norway / Austria / Portugal / Slovakia / Slovenia / Spain / Sweden / Czech Republic / Germany / Hungary
Human Hemin Orphan Europe – Poland