Pharmacy Guide
Patient leaflets and SmPCs, drug interaction checker, official dosages and NHS pharmacy opening hours — all in one place.
Pharmacy Guide
Patient leaflets and SmPCs, drug interaction checker, official dosages and NHS pharmacy opening hours — all in one place.
Cystadane 1 g oral powder
⚠ This medicine appears to have been discontinued
The electronic medicines compendium (emc) no longer publishes a Summary of Product Characteristics for this product, which usually means it is no longer marketed in the UK. The patient leaflet below is kept for reference, but the product may not be available.
If you were prescribed this medicine, other products containing Betaine anhydrous may still be available. Do not stop your treatment — ask your pharmacist or GP what to use instead.
What it is and what it is used for
for
Cystadane contains betaine anhydrous which is intended to be an adjunctive treatment of homocystinuria, an inherited (genetic) disease where the amino acid methionine cannot be broken down completely by the body. Methionine is present in regular food protein (e.g. meat, fish, milk, cheese, eggs). It is converted into homocysteine which is then normally converted into cysteine during digestion. Homocystinuria is a disease caused by the accumulation of homocysteine which is not converted to cysteine and is characterised by formation of clots in the veins, bone weakness, and skeletal and crystalline lens abnormalities. The use of Cystadane together with other treatments such as vitamin B6, vitamin B12, folate and a specific diet aims to reduce the elevated homocysteine levels in your body. 2.
What you need to know before you take it
e Cystadane
Do not take Cystadane If you are allergic to betaine anhydrous or any of the other ingredients of this medicine (listed in section 6). Warnings and precautions Talk to your doctor or pharmacist before taking Cystadane. If you notice side effects like headaches, vomiting or a change in your vision and you are of the homocystinuria subtype called CBS (cystathionine beta-synthase deficiency), please contact your doctor immediately, they could be signs of a swelling in the brain (cerebral oedema). In that case your doctor will monitor your methionine level in your body and may review your diet. Your treatment with Cystadane may need to be interrupted. If you are treated with Cystadane and with an amino-acid mixture and if you need to take other medicines at the same time, leave 30 minutes between the intake (see "section "Other medicines and Cystadane").
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Other medicines and Cystadane Tell your doctor if you are taking, have recently taken or might take any other medicines. If you are taking amino-acid mixture or medicines such as vigabatrin or Gaba analogues (medicine used to treat epilepsy), please tell your doctor as they might interact with your treatment with Cystadane. Pregnancy and breast-feeding 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 taking this medicine. Your doctor will decide if you can use this medicine during pregnancy and breast-feeding. Driving and using machines Cystadane has no or negligible influence on the ability to drive and use machines. 3.
How to take it
Cystadane
The use of this medicine will be supervised by a doctor experienced in the treatment of patients with homocystinuria. Always take this medicine exactly as your doctor or pharmacist has told you. Check with your doctor or pharmacist if you are not sure. The recommended dose in children and adults is 100mg/kg/day divided in 2 dosesper day. In some patients doses above 200 mg/ kg/day were needed to reach therapeutic goals. Your doctor may adapt the dose depending on your laboratory values. You will therefore need regular blood tests to determine the correct daily dose. You should take Cystadane orally (by mouth). To measure the dose: • shake the bottle lightly before opening • take the correct measuring spoon: • the small green spoon measures 100 mg of betaine anhydrous powder; • the middle size blue spoon measures 150 mg of betaine anhydrous powder ; • the large pink spoon measures 1 g of betaine anhydrous powder. • take a heaped spoonful of powder out of the bottle • pass the flat back of a knife over the top of the spoon • the powder left in the spoon is one spoonful • take the correct number of spoonfuls of powder from the bottle Mix the measured dose of powder with water, juice, milk, formula or food until completely dissolved and ingest immediately after mixing. If you take more Cystadane than you should If you accidentally take too much Cystadane, talk to a doctor or pharmacist immediately. If you forget to take Cystadane Do not take a double dose to make up for forgotten doses. If you forget to take a dose take it as soon as you remember it and continue the next dose as planned. If you stop taking Cystadane Do not stop the treatment without consulting your doctor. Contact your doctor or pharmacist before stopping.
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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 most commonly side effect when taking Cystadane which may affect more than 1 in 10 people (frequency very common) is elevated levels of methionine in the blood. Methionine level can be related to swelling in the brain (cerebral swelling), which may affect up to 1 in 100 people (frequency uncommon). If you experience morning headaches with vomiting and/or visual changes, contact immediately your doctor (they could be signs of a swelling in the brain). Gastrointestinal disorders like diarrhoea, nausea, vomiting, stomach discomfort and inflammation of the tongue may occur uncommonly (may affect up to 1 in 100 people). Other uncommon side effects (may affect up to 1 in 100 people) may include decreased appetite (anorexia), agitation, irritability, hair loss, hives, skin odour abnormal, lack of control over passing urine (urinary incontinence). Reporting of side effects If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the national reporting system : Yellow Card Scheme, Website: www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store. By reporting side effects you can help provide more information on the safety of this medicine. 5.
How to store it
Cystadane
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 bottle label and the carton after EXP. The expiry date refers to the last day of that month. Do not store above 25°C. Keep the bottle tightly closed in order to protect from moisture. After the first opening of the bottle, the medicine should be used within 3 months. Do not throw away any medicines via wastewater or household waste. Ask your pharmacist how to throw away medicines you no longer use. These measures will help protect the environment. 6.
Contents of the pack and other information
What Cystadane contains
- The active substance is betaine anhydrous. 1 g of oral powder contains 1 g of betaine anhydrous.
- There is no other ingredient. What Cystadane looks like and contents of the pack Cystadane is a white crystalline free flowing powder. It is presented in bottles with child resistant closures. Each bottle contains 180 g of powder. Each carton contains one bottle and three measuring spoons.
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Marketing Authorisation Holder Recordati Rare Diseases Tour Hekla 52, avenue du General de Gaulle F-92 800 Puteaux France Manufacturer Recordati Rare Diseases Tour Hekla 52, avenue du Général de Gaulle F-92800 Puteaux France or Recordati Rare Diseases Eco River Parc 30, rue des Peupliers F-92000 Nanterre France For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder. United Kingdom Recordati Rare Diseases UK Ltd. Tel: +44 (0)1491 414333 This leaflet was last revised in December 2024
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Frequently asked questions about Cystadane 1 g oral powder
How do I take Cystadane 1 g oral powder?
Cystadane 1 g oral powder comes as powder containing 1g. Always follow the dose your doctor or pharmacist has given you, and read the leaflet that comes with the medicine.
What is the active substance in Cystadane 1 g oral powder?
The active substance in Cystadane 1 g oral powder is betaine anhydrous.
Are there equivalent medicines to Cystadane 1 g oral powder?
Medicines with the same active substance, strength and form include: Amversio 1 g oral powder. They are interchangeable only if your prescriber or pharmacist says so.
Where does this information come from?
This leaflet reproduces the patient information leaflet approved for Cystadane 1 g oral powder, as published on the electronic medicines compendium (emc). The version printed inside your medicine’s packaging is the one that applies to you.
Can I get Cystadane 1 g oral powder without a prescription?
Whether a medicine is available over the counter or on prescription only depends on its licence. Check the leaflet, or ask your pharmacist — they can tell you straight away.
About this leaflet
The text above reproduces the patient information leaflet approved for this medicine, restructured for easier reading.
- Source: electronic medicines compendium (emc), Datapharm
- Active substance: betaine anhydrous
- Official document: view the original leaflet on emc →
⚕For healthcare professionals — Summary of Product Characteristics (SmPC)Full SmPC: dosage, interactions, contraindications, warnings+
4.1. Therapeutic indications
Adjunctive treatment of homocystinuria, involving deficiencies or defects in:
• cystathionine beta-synthase (CBS),
• 5,10-methylene-tetrahydrofolate reductase (MTHFR),
• cobalamin cofactor metabolism (cbl).
Cystadane should be used as supplement to other therapies such as vitamin B6 (pyridoxine), vitamin B12 (cobalamin), folate and a specific diet.
4.2. Posology and method of administration
Cystadane treatment should be supervised by a physician experienced in the treatment of patients with homocystinuria.
Posology
Children and Adult
The recommended total daily dose is 100 mg/kg/day given in 2 doses daily. However, the dose should be individually titrated according to plasma levels of homocysteine and methionine. In some patients doses above 200 mg/ kg/day were needed to reach therapeutic goals. Caution should be exercised with up-titrating doses for patients with CBS deficiency due to the risk for hypermethioninaemia. Methionine levels should be closely monitored in these patients.
Special populations
Hepatic or renal impairment
Experience with betaine anhydrous therapy in patients with renal insufficiency or non-alcoholic hepatic steatosis has demonstrated no need to adapt the dose regimen of Cystadane.
Method of administration
The bottle should be lightly shaken before opening. Three measuring spoons are provided which dispense either 100 mg, 150 mg or 1 g of betaine anhydrous. It is recommended that a heaped measuring spoon is removed from the bottle and a flat surface e.g. base of a knife is drawn across the top of the measure. This will give the following doses: small measure 100 mg, middle size measure 150 mg and large measure 1 g of betaine anhydrous.
The powder should be mixed with water, juice, milk, formula or food until completely dissolved and ingested immediately after mixing.
Therapeutic monitoring
The aim of treatment is to keep plasma levels of total homocysteine below 15 µM or as low as possible. The steady-state response usually occurs within a month.
4.3. Contraindications
Hypersensitivity to the active substance.
4.4. Special warnings and precautions for use
Uncommon cases of severe cerebral oedema associated with hypermethioninemia were reported with betaine anhydrous therapy in patients with CBS deficiency (see section 4.8). Complete recovery was seen after treatment discontinuation:
- The plasma methionine concentrations should be kept below 1000 µM. It is recommended to measure plasma methionine level at start of treatment and about annually or biannually thereafter. If methionine increases particularly above the first safety threshold of 700 µmol/L, patient should be monitored more frequently and compliance with diet should be checked. In order to reduce methionine levels, modification of diet as well as dose reduction of Cystadane or temporal interruption of Cystadane treatment should be considered.
- If any symptoms of cerebral oedema like morning headaches with vomiting and/or visual changes appear, plasma methionine level and compliance to the diet should be checked and treatment with Cystadane interrupted.
- If symptoms of cerebral oedema recur after re-introduction of treatment then betaine anhydrous therapy should be discontinued indefinitely.
To minimise the risk of potential drug interactions, it is advisable to leave 30 minutes between the intake of betaine anhydrous and amino acids mixtures and/or medicinal products containing vigabatrin and GABA analogues (see section 4.5).
4.5. Interaction with other medicinal products and other forms of interaction
No interaction studies have been performed.
Based on in vitro data, betaine anhydrous might interact with amino acids mixtures and medicinal products containing vigabatrin and GABA analogues.
4.6. Fertility, pregnancy and lactation
Pregnancy
Data on a limited number of exposed pregnancies indicate no adverse event of betaine anhydrous on pregnancy or on the health of the foetus/newborn child. To date, no other relevant epidemiologic data are available. Animal reproduction studies have not been conducted. During pregnancy, administering betaine anhydrous in addition to pyridoxine, folate, anticoagulant and diet under close monitoring of plasma homocysteine would be compatible with good maternal and foetal outcomes. However, Cystadane should not be used during pregnancy unless clearly necessary.
Breast-feeding
It is not known whether betaine anhydrous is excreted in human milk (although its metabolic precursor, choline, occurs at high levels in human milk). Because of lack of data, caution should be exercised when prescribing Cystadane to breast-feeding women.
Fertility
No data is available.
4.7. Effects on ability to drive and use machines
Cystadane has no or negligible influence on the ability to drive and use machines.
4.8. Undesirable effects
Summary of the safety profile
In general, adverse reactions seen with betaine anhydrous therapy appeared to be not serious and are mainly related to the gastrointestinal system. Gastrointestinal disorders like diarrhoea, glossitis, nausea, stomach discomfort, vomiting and dental disorders may occur uncommonly.
The most commonly reported adverse reaction during treatment is blood methionine increased. Complete recovery was seen after treatment discontinuation (see section 4.4).
Tabulated list of adverse reactions
Reported adverse reactions are listed below, by system organ class and by frequency.
Frequencies are defined as: very common (≥ 1/10), common (≥ 1/100 to < 1/10), uncommon (≥ 1/1,000 to < 1/100), rare (≥ 1/10,000 to < 1/1,000), very rare (< 1/10,000). Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.
Metabolism and nutrition disorders
Uncommon: anorexia
Psychiatric disorders
Uncommon: agitation, irritability
Nervous system disorders
Uncommon: brain oedema*
Gastrointestinal disorders
Uncommon: diarrhoea, glossitis, nausea, stomach discomfort, vomiting
Skin and subcutaneous tissue disorders
Uncommon: hair loss, hives, skin odour abnormal
Renal and urinary disorders
Uncommon: urinary incontinence
Investigations
Very common: blood methionine increased*
Description of selected adverse reactions
*Uncommon cases of severe cerebral oedema and hypermethioninemia were reported within 2 weeks to 6 months of starting betaine anhydrous therapy in patients with CBS deficiency, with complete recovery after treatment discontinuation.
Symptoms of cerebral oedema include morning headaches with vomiting and/or visual changes
High increases in plasma methionine levels in a range from 1,000 to 3,000 µM were noted in these patients. As cerebral oedema has also been reported in patients with hypermethioninemia, secondary hypermethioninemia due to betaine anhydrous therapy has been postulated as a possible mechanism of action.
For specific recommendations, see section 4.4.
Reporting of suspected adverse reactions
Reporting suspected adverse reactions after authorisation of the medicinal product is important. It allows continued monitoring of the benefit/risk balance of the medicinal product. Healthcare professionals are asked to report any suspected adverse reactions via the national reporting system: Yellow Card Scheme, Website: www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store.
4.9. Overdose
No case of overdose has been reported.
💬 Ask about this leaflet
Ask anything about Cystadane 1 g oral powder. The assistant answers only from this leaflet — if the leaflet does not cover it, it will say so. It does not give medical advice.
Answers come from the patient leaflet published on the electronic medicines compendium (emc). They are not medical advice. Ask a pharmacist or your GP if you are unsure. For urgent help call NHS 111, or 999 in an emergency.