Drugs List

Therapeutic Indications

Uses

Treatment of acute attacks of hepatic porphyria

Administration

For intravenous infusion.

Due to the hypertonicity of the infusion, to prevent vein irritation, the diluted solution should be infused into a large antebrachial or central vein over at least 30 minutes.

After the infusion, the vein should be rinsed with 100 ml of sodium chloride infusion 0.9%. The vein should be flushed initially with 3 to 4 bolus injections of 10 ml sodium chloride injection 0.9% followed by the remaining solution which should be infused over 10 to 15 minutes.

Contraindications

Neonates under 1 month

Precautions and Warnings

Children 1 month to 18 years
Alcoholism
Brain damage
Brain disorder
Breastfeeding
Epileptic disorder
Hepatic disorder
Pregnancy

Confirm diagnosis of acute porphyria attack before initiating treatment
Contains alcohol
Contains propylene glycol: may cause irritation
Derived from human blood - transmission of infectious agents possible
Record name and batch number of administered product
Monitor cardiovascular function
Monitor neurological function
Monitor serum ferritin levels
May impart an unusual colouring to plasma

Before initiating treatment, an acute attack of porphyria must be confirmed by clinical and biological criteria including:
A suggestive family or personal history
Suggestive clinical signs
Determination of urinary delta-amino-laevulinic acid and porphobilinogen.

If the intravenous cannula is in place for too long, due to mechanical irritation and also due to irritation by the injection fluid, vascular damage may occur which may lead to extravasation. The cannula should be tested before infusing and also regularly during the infusion. In case of extravasation, skin discolouration may occur.

Repeated infusions may lead to increased serum ferritin concentrations. Any changes may require further investigation and appropriate treatment.

Hemin human should not be used as a preventive treatment as data available is limited and long term administration of regular infusions can cause iron overload.

Ensure there is a sufficient supply of carbohydrates in addition to treatment with hemin human.

Hemin human infusion contains 1 g of ethanol per 10 ml of concentrate.

Hemin human infusion contains 4 g of propylene glycol per 10 ml of concentrate.

Pregnancy and Lactation

Pregnancy

Hemin human infusion should be used with caution in pregnancy.

The manufacturer advises avoid hemin human infusion during pregnancy unless essential.

The risk of administering hemin human during pregnancy have not been established. However, adverse effects in the neonate have not been observed following the administration of this product during pregnancy.

Briggs concludes that the primary embryo or fetal risk from hemin appears to be from the transmission of viruses or other agents from a hemin-induced maternal infection or from a hemin-induced maternal adverse reaction (Briggs 2015).

Hemin human infusion contains 1 g of ethanol per 10 ml of concentrate. This may be harmful to pregnant patients.

The use of all medication in pregnancy should be avoided whenever possible; particularly in the first trimester. Non-drug treatments should also be considered. When essential, a medication with the best safety record over time should be chosen, employing the lowest effective dose for the shortest possible time. Polypharmacy should be avoided. Teratogens taken in the pre-embryonic period, often quoted as lasting until 14 to 17 days post-conception, are believed to have an all-or-nothing effect. Where drugs have a short half-life, and when the date of conception is certain, this may allow women to be reassured where drug exposure has occurred within this time frame. Further advice may be available from the UK National Teratology Information Service (NTIS) and through ToxBase, available via password on the internet ( www.toxbase.org ) or if this is unavailable at the backup site ( www.toxbasebackup.org ).

Lactation

Hemin human infusion should be used with caution in breastfeeding.

Studies of hemin human administration during breastfeeding have not been located. The manufacturer advises avoid hemin human infusion during breastfeeding unless essential.

Briggs concludes however, that it is doubtful if hemin is excreted in milk but even if small amounts were excreted, they would be digested in the infant's gut (Briggs 2015) .

Neonates, infants born prematurely, those with low birth weight, those with an unstable gastrointestinal function or who have serious illnesses may require special consideration. For any infant, if a drug is prescribed to the nursing mother, it should be at the lowest practical dose and for the shortest time. When drug administration is unavoidable and breastfeeding is to continue, minimisation of exposure of the infant to the drug may sometimes be achieved by timing the maternal doses to just after a feeding episode. Infants exposed to drugs via breast milk should be monitored for unusual signs or symptoms. Interactions between the drug received by the infant from the mother's milk and medication prescribed for the infant should also be considered, for example, when the drug given to the infant may prevent metabolism of the drug received via breast milk.
Specialist advice is available from the UK Drugs in Lactation Advisory Service at https://www.midlandsmedicines.nhs.uk/content.asp?section=6&subsection=17&pageIdx=1

Side Effects

Anaphylactoid reaction
Dermatitis
Erythema at injection site
Extravasation
Headache
Hypersensitivity reactions
Increased serum ferritin
Itching (injection site)
Local pain (injection site)
Necrosis (injection site)
Phlebitis (injection site)
Pyrexia
Serum creatinine increased
Skin discolouration
Swelling (injection site)
Thrombosis (injection site)
Tongue oedema
Venous thrombosis

Presentation

Concentrate for solution for infusion containing human hemin

Drugs List

Therapeutic Indications

Uses

Treatment of acute attacks of hepatic porphyria

Dosage

Adults

Elderly

Children

Administration

For intravenous infusion.

Due to the hypertonicity of the infusion, to prevent vein irritation, the diluted solution should be infused into a large antebrachial or central vein over at least 30 minutes.

After the infusion, the vein should be rinsed with 100 ml of sodium chloride infusion 0.9%. The vein should be flushed initially with 3 to 4 bolus injections of 10 ml sodium chloride injection 0.9% followed by the remaining solution which should be infused over 10 to 15 minutes.

Contraindications

Neonates under 1 month

Precautions and Warnings

Children 1 month to 18 years
Alcoholism
Brain damage
Brain disorder
Breastfeeding
Epileptic disorder
Hepatic disorder
Pregnancy

Confirm diagnosis of acute porphyria attack before initiating treatment
Contains alcohol
Contains propylene glycol: may cause irritation
Derived from human blood - transmission of infectious agents possible
Record name and batch number of administered product
Monitor cardiovascular function
Monitor neurological function
Monitor serum ferritin levels
May impart an unusual colouring to plasma

Before initiating treatment, an acute attack of porphyria must be confirmed by clinical and biological criteria including:
A suggestive family or personal history
Suggestive clinical signs
Determination of urinary delta-amino-laevulinic acid and porphobilinogen.

If the intravenous cannula is in place for too long, due to mechanical irritation and also due to irritation by the injection fluid, vascular damage may occur which may lead to extravasation. The cannula should be tested before infusing and also regularly during the infusion. In case of extravasation, skin discolouration may occur.

Repeated infusions may lead to increased serum ferritin concentrations. Any changes may require further investigation and appropriate treatment.

Hemin human should not be used as a preventive treatment as data available is limited and long term administration of regular infusions can cause iron overload.

Ensure there is a sufficient supply of carbohydrates in addition to treatment with hemin human.

Hemin human infusion contains 1 g of ethanol per 10 ml of concentrate.

Hemin human infusion contains 4 g of propylene glycol per 10 ml of concentrate.

Pregnancy and Lactation

Pregnancy

Hemin human infusion should be used with caution in pregnancy.

The manufacturer advises avoid hemin human infusion during pregnancy unless essential.

The risk of administering hemin human during pregnancy have not been established. However, adverse effects in the neonate have not been observed following the administration of this product during pregnancy.

Briggs concludes that the primary embryo or fetal risk from hemin appears to be from the transmission of viruses or other agents from a hemin-induced maternal infection or from a hemin-induced maternal adverse reaction (Briggs 2015).

Hemin human infusion contains 1 g of ethanol per 10 ml of concentrate. This may be harmful to pregnant patients.

The use of all medication in pregnancy should be avoided whenever possible; particularly in the first trimester. Non-drug treatments should also be considered. When essential, a medication with the best safety record over time should be chosen, employing the lowest effective dose for the shortest possible time. Polypharmacy should be avoided. Teratogens taken in the pre-embryonic period, often quoted as lasting until 14 to 17 days post-conception, are believed to have an all-or-nothing effect. Where drugs have a short half-life, and when the date of conception is certain, this may allow women to be reassured where drug exposure has occurred within this time frame. Further advice may be available from the UK National Teratology Information Service (NTIS) and through ToxBase, available via password on the internet ( www.toxbase.org ) or if this is unavailable at the backup site ( www.toxbasebackup.org ).

Lactation

Hemin human infusion should be used with caution in breastfeeding.

Studies of hemin human administration during breastfeeding have not been located. The manufacturer advises avoid hemin human infusion during breastfeeding unless essential.

Briggs concludes however, that it is doubtful if hemin is excreted in milk but even if small amounts were excreted, they would be digested in the infant's gut (Briggs 2015) .

Neonates, infants born prematurely, those with low birth weight, those with an unstable gastrointestinal function or who have serious illnesses may require special consideration. For any infant, if a drug is prescribed to the nursing mother, it should be at the lowest practical dose and for the shortest time. When drug administration is unavoidable and breastfeeding is to continue, minimisation of exposure of the infant to the drug may sometimes be achieved by timing the maternal doses to just after a feeding episode. Infants exposed to drugs via breast milk should be monitored for unusual signs or symptoms. Interactions between the drug received by the infant from the mother's milk and medication prescribed for the infant should also be considered, for example, when the drug given to the infant may prevent metabolism of the drug received via breast milk.
Specialist advice is available from the UK Drugs in Lactation Advisory Service at https://www.midlandsmedicines.nhs.uk/content.asp?section=6&subsection=17&pageIdx=1

Side Effects

Anaphylactoid reaction
Dermatitis
Erythema at injection site
Extravasation
Headache
Hypersensitivity reactions
Increased serum ferritin
Itching (injection site)
Local pain (injection site)
Necrosis (injection site)
Phlebitis (injection site)
Pyrexia
Serum creatinine increased
Skin discolouration
Swelling (injection site)
Thrombosis (injection site)
Tongue oedema
Venous thrombosis

Overdosage

It is strongly recommended that the UK National Poisons Information Service be consulted on cases of suspected or actual overdose where there is doubt over the degree of risk or about appropriate management.

The following number will direct the caller to the relevant local centre (0844) 892 0111

Information may be obtained if you have access to ToxBase the primary clinical toxicology database of the National Poisons Information Service. This is available via password on the internet ( www.toxbase.org ) or if this is unavailable at the backup site ( www.toxbasebackup.org ).

Further Information

Last Full Review Date: June 2016

Reference Sources

Drugs in Pregnancy and Lactation: A Reference Guide to Fetal and Neonatal Risk, 10th edition (2015) ed. Briggs, G., Freeman, R. Wolters Kluwer Health, Philadelphia.

Joint Formulary Committee. British National Formulary (online) London: BMJ Group and Pharmaceutical Press Accessed on 24 June 2016.

Paediatric Formulary Committee. BNF for Children (online) London: BMJ Group, Pharmaceutical Press, and RCPCH Publications Accessed on 24 June 2016.

Summary of Product Characteristics: Normosang. Orphan Europe (UK) Ltd. Revised November 2015.