Maapliv – Package Leaflet: InfoCons – Consumer Protection presents, in an accessible format, the official package leaflet of the medicine Maapliv, as authorised in the European Union and published in the Union Register of medicinal products.
Before anything else
Read all of this leaflet carefully before you start using this medicine because it contains important information for you.
- Keep this leaflet. You may need to read it again.
- If you have any further questions, ask your doctor, pharmacist or nurse.
- 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.
ℹ️ 1. What Maapliv is and what it is used for
Maapliv contains amino acids, which belong to a group of medicines called amino acids for parenteral
use. It contains the following amino acids
L-alanine, L-arginine , L-aspartic acid, L -cysteine hydrochloride monodhydrate , L-glutamic acid, Glycine , L-histidine , L-lysine m onohydrate, L -tethionine , L-phenylalanine , L-proline , L-serine, Taurine, L-threonine , L-tryptophan , L-tyrosine .
Maapliv is used to treat maple syrup urine disease (MSUD) in patients from birth. It is used when patients have a sudden worsening of MSUD and cannot take a special diet free of certain amino acids.
MSUD is a rare genetic disorder where the body can’t break down certain amino acids found in protein. This leads to a buildup of harmful substances in the blood and urine, which can cause serious health problems.
⚠️ 2. What you need to know before you use Maapliv
Do not use Maapliv
- if you are allergic to one of the amino acids or any of the other ingredients of this medicine (listed in section 6) .
Warnings and precautions
Inform your doctor if you have :
- heart failure
- reduced kidney function
- reduced liver function
- predisposition to tromboembolism Before you start treatment your doctor will monitor you and do tests, especially at the beginning of the infusion (drip) . These include:
- checking fluid and electrolyte levels;
- serum osmolarity (measure of water and dissolved substances, such as glucose, urea nitrogen and sodium, in the blood);
- acid-base balance (having the right amount s of acid s and bases in the blood and other body fluids);
- blood glucose;
- blood ammonia (a normal waste product in your body);
- blood proteins;
- liver and kidney function.
If you have severe water and elec trolyte imbalances, too much fluid in the body or a severe metabolic disorder, these will be corrected before you are given the infusion.
Sudden, severe anaphylactic reactions can occur with amino acid solutions, such as Maapliv, that are used for intravenous nutrition. If you develop any signs or symptoms of an allergic reaction during treatment tell your doctor or nurse immediately. These include:
- hives
- rash
- itching
- dizziness
- wheezing
- difficulty breathing
- swelling of the tongue Patients receiving intravenous nutrition can develop blockages in the blood vessels of the lungs. If you develop any signs or symptoms of a blockage during treatment, tell your doctor or nurse immediately.
These include:
- shortness of breath
- difficulty breathing
- cough
- chest discomfort
- fast heart rate
- bluish colour around the mouth, lips or fingernails due to low blood oxygen levels.
In patient s with severe or critical acute decompensations, with very high levels of leucine in the blood, Maapliv intravenous might need to be administered in concomitance with haemodyalisis or haemofiltration.
The clinical studies focused primarily on leucine levels. The impact on symptom improvement or other aspects of clinical status is unknown.
There is limited information on the repeated administration of the intravenous BCAA – free formula.
Other medicines and Maapliv
Tell your doctor if you are taking, have recently taken or might take any other medicines . Amino acid solutions may precipitate acute folate deficiency and folic acid should be given daily .
Pregnancy, breast-feeding and fertility
If you are pregnant or breast -feeding, think you may be pregnant or are planning to have a baby, ask your doctor for advice before you are given this medicine.
There are no adequate data concerning the use of Maapliv in pregnant or breast -feeding women.
If you are pregnant, you will receive this medicine only if your doctor considers it absolutely necessary for your recovery. Maapliv should only be given to pregnant women after careful consideration.
At therapeutic doses of Maapliv no effects on the breastfed newborns/infants are anticipated. Nevertheless, Maapliv should only be given to breast-feeding women after careful consideration .
Driving and using machines
Maapliv does not affect your ability to drive or use machines.
💊 3. H ow to use Maapliv
Maapliv will be given as an infusion (drip) into a vein.
The dose of Maapliv will be adjusted according t o your ability to process amino acids, age, weight and nutritional needs.
The recommended dose is between 3 9 and 58 mL/kg/24h for newborn, infant up to 2 years old and between 1 9 to 39 mL/kg/24h for children , adolescent s and adults.
Keep the bottle protected from light during administration. If you use more Maapliv than you should It is very unlikely that you will receive more of this medicine in your infusion than you should, as a doctor or nurse will monitor your treatment .
The effects of an overdose may include symptoms of fluid overload/retention, electrolytic disturbances and acute pulmunary oedema; symptoms of amino acid overdose with shivering, vomiting, headache, nausea, hyperammonaemia (too much ammonia in your blood) and increased loss of amino acid s in the kidneys .
In case of overdose, infusion should be stopped immediately and may be resumed with a reduced dose or slower infusion rate.
If you have any further questions on the use of this medicine, ask your doctor or pharmacist .
❗ 4. Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them . The frequency of the se adverse reaction s is “uncommon” ( may affect up to 1 in 100 people ).
Serious side effects
- Allergic (anaphylactic, anaphylactoid) reactions. Hypersensitivity. S ymptoms may include fever or chills, shivering, excessive sweating, skin rashes, oedema of the face or the eyelids, breathing difficulties, abnormally high heart rate, abnormally low or high blood pressure .
If such signs of an allergic reaction occur, t he infusion should be stopped immediately .
- Metabolic/nutritional disorders with consequential hyperammonaemia.
If hyeperammonaemia occur s, infusion should be stopped immediately with specific treatment to be initiated by the treating physician.
- Renal and urinary disorders with consequential azotaemia.
- Formation of blood clots that block blood vessels in the lungs (pulmonary thromboembolism) or veins (venous thromboembolism). This can cause chest pain, breathlessness and fainting.
If such signs occur, the infusion should be stopped, and medical evaluation initiated.
Other side effects
- Abnormal blood test for liver function impairment. Blood bilirubin and hepatic enzymes might be increased; and cholecystitis and cholelithiasis might occur.
- Inflammation of the gall bladder, gallstones and problems with bile flow .
- Vein inflammation at infusion site, venous irritation, pain, warmth, swelling and vein duct induration , might occur If you notice any changes in the way you feel during or after the treatment, tell your healthcare professional or another member of your medical team immediately.
Reporting of side effects
If you get any side effects, talk to your doctor, pharmacist or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the national reporting system listed in Appendix V . By reporting side effects, you can help provide more information on the safety of this medicine.
🌡️ 5. H ow to store Maapliv
Keep this medicine out of the sight and reach of children.
Store in the original package in order to protect from light .
Do not refrigerate or freeze.
Do not use this medicine after the expi ry date , which is stated on the carton and the bottle after EXP .
The expiry date refers to the last day of that month.
Do not use this medicine if the bottle is leaking or if you notice particles in the solution.
Do not throw away any medicines via wastewater. Ask your pharmacist how to throw away medicines you no longer use. These measures wil l help protect the environment.
📦 6. Contents of the pack and other information
What Maapliv contains
- The active substances are L-alanine, L-arginine , L-aspartic acid, L -cysteine hydrochloride monohydrate , L-glutamic acid, Glycine , L-histidine , L-lysine monohydrate , L-methionine , L-phenylalanine, L-proline , L-serine, Taurine, L-threonine , L-tryptophan, L-tyrosine .
- The other ingredients are acetic acid or sodium hydroxide (for pH adjustment) and water for injections.
What Maapliv looks like and contents of the pack
Maapli v is a solution for infusion a vailable in 500 ml colourless glass bottles. Each bottle is packed in a carton.
Clear solution free of particles.
Marketing Authorisation Holder
RECORDATI RARE DISEASES Tour Hekla 52 avenue du Général De Gaulle 92800 Puteaux France
Manufacturer
RECORDATI RARE DISEASES Eco River Parc 30 rue des Peupliers 92000 Nanterre France RECORDATI RARE DISEASES Tour Hekla 52 avenue du Général De Gaulle 92800 Puteaux France Industria Farmaceutica Galenica Senese S.r.l.
Via Cassia Nord, 351, 53014 Monteroni d’Arbia (SI) Italy For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder:
Belgique/België/Belgien Recordati bv Tél/Tel: +32 2 46101 36 Lietuva Recordati AB.
Tel: + 46 8 545 80 230 Švedija България RECORDATI RARE DISEASES Teл.: +33 (0)1 47 73 64 58 Франция Luxembourg/Luxemburg Recordati bv Tél/Tel: +32 2 46101 36 Belgique/Belgien Česká republika RECORDATI RARE DISEASES Tel: +33 (0)1 47 73 64 58 Francie Magyarország RECORDATI RARE DISEASES Tel: +33 (0)1 47 73 64 58 Franciaország Danmark Recordati AB.
Tlf : +46 8 545 80 230 Sverige Malta RECORDATI RARE DISEASES Tel: +33 1 47 73 64 58 Franza Deutschland RECORDATI RARE DISEASES Germany GmbH Tel: +49 731 140 554 0 Nederland Recordati bv Tel: +32 2 46101 36 België Eesti Recordati AB.
Tel: + 46 8 545 80 230 Rootsi Norge Recordati AB.
Tlf : +46 8 545 80 230 Sverige Ελλάδα Recordati Hellas Τηλ: +30 210 6773822 Österreich RECORDATI RARE DISEASES Germany GmbH Tel: +49 731 140 554 0 Deutschland España RECORDATI RARE DISEASES Spain S.L.U.
Tel: + 34 91 659 28 90 Polska RECORDATI RARE DISEASES Tel: +33 (0)1 47 73 64 58 Francja France RECORDATI RARE DISEASES Tél: +33 (0)1 47 73 64 58 Portugal Jaba Recordati S.A.
Tel: +351 21 432 95 00 Hrvatska Recordati Rare Diseases Tél: +33 (0)1 47 73 64 58 Francuska România RECORDATI RARE DISEASES Tel: +33 (0)1 47 73 64 58 Franţa Ireland RECORDATI RARE DISEASES Tel: +33 (0)1 47 73 64 58 France Slovenija RECORDATI RARE DISEASES Tel: +33 (0)1 47 73 64 58 Francija Ísland Recordati AB.
Simi:+46 8 545 80 230 Svíþjóð Slovenská republika RECORDATI RARE DISEASES Tel: +33 (0)1 47 73 64 58 Francúzsko Italia RECORDATI RARE DISEASES Italy Srl Tel: +39 02 487 87 173 Suomi/Finland Recordati AB.
Puh/Tel : +46 8 545 80 230 Sverige Κύπρος RECORDATI RARE DISEASES Τηλ : +33 1 47 73 64 58 Γαλλία Sverige Recordati AB.
Tel : +46 8 545 80 230 Latvija Recordati AB.
Tel: + 46 8 545 80 230 Zviedrija This leaflet was last revised i n.
This medicine has been authorised under ‘exceptional circumstances’.
This means that because of the rarity of the disease it has been im possible to get complete information on this medicine .
The European Medicines Agency will review any new information on this medicine every year and this leaflet will be updated as necessary.
Other sources of information Detailed information on this medicine is available on the European Medicines Agency website: https://www.ema. europa.eu . There are also links to other websites about rare diseases and treatments.
This leaflet is available in all EU /EEA languages on the European Medicines Agency website.
📄 Package Leaflet – official source
This article reproduces the information in the official package leaflet, as authorised in the European Union. You can consult the original document here: Product information Maapliv (PDF, ec.europa.eu).
You can check this medicine at any time in the Union Register of medicinal products (EU number EU/1/25/1955).
⚠️ Important: This article is for information purposes only and does not replace medical advice or the package leaflet inside the medicine box. Read the leaflet carefully before use. For any questions about your treatment, talk to your doctor or pharmacist. If you experience side effects, tell your doctor or pharmacist and report them through your national reporting system.