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.
Cystagon 150 mg hard capsules
⚠ 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 Mercaptamine bitartrate 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
Cystinosis is a metabolic disease called 'nephropathic cystinosis' which is characterized by an abnormal accumulation of the amino acid cystine in various organs of the body such as the kidney, eye, muscle, pancreas, and brain. Cystine build up causes kidney damage and excretion of excess amounts of glucose, proteins and electrolytes. Different organs are affected at different ages. CYSTAGON is prescribed to manage this rare inherited disorder. CYSTAGON is a medicine that reacts with cystine to decrease its level within the cells. 2.
What you need to know before you take it
e CYSTAGON
Do not use CYSTAGON if you -or your child- are allergic to cysteamine bitartrate or penicillamine or any of the other ingredients of this medicine (listed in section 6). If you are pregnant, this is particularly relevant during the first trimester if you are breast-feeding. Warnings and precautions When your or your child's disorder has been confirmed by leucocyte cystine measurements, the therapy with CYSTAGON must be started as soon as possible. A few cases of skin lesions on elbows like little hard lumps have been reported in children treated with high doses of different cysteamine preparations. These lesions were associated with skin striae and bone lesions such as fracture and bone deformities, and with laxity of joints. Your doctor could require a regular physical and X-ray examination for the skin and the bones to control the effects of the medicinal product. Self examination of your or your child's skin is recommended. If any skin or bone abnormalities appear, please inform your doctor immediately. Your doctor could require controlling the blood cell count on a regular basis. 1
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CYSTAGON has not been shown to prevent cystine crystals accumulating in the eye. Where cysteamine ophthalmic solution has been used for that purpose, its usage should continue. In contrast to phosphocysteamine, another active substance close to cysteamine bitartrate, CYSTAGON does not contain phosphate. You may already be receiving phosphate supplements and the dose of these may need to be changed when CYSTAGON is substituted for phosphocysteamine. To avoid any risk of aspiration in the lungs, the capsules should not be given to children under the age of approximately 6 years. Do not swallow the desiccant canister found in the bottle.
Other medicines and CYSTAGON Tell your doctor or pharmacist if you are using, have recently used or might use any other medicines.. CYSTAGON with food and drink For children under approximately six years of age, the hard capsule may be opened and the contents sprinkled on food (e.g. milk, potatoes or starch based foods) or mixed in formula. Do not add to acidic drinks e.g. orange juice. Consult the doctor for complete directions. Pregnancy You should not use CYSTAGON if you are pregnant. Please consult your doctor if you plan to become pregnant. Breast-feeding CYSTAGON must not be used during breast-feeding. Driving and using machines CYSTAGON may cause some drowsiness. When starting therapy, you or your child should not engage in potentially hazardous activities until the effects of the drug are well known. 3.
How to take it
CYSTAGON
Always use this medicine exactly as your doctor or your child's doctor has told you. Check with your doctor if you are not sure. The dose of CYSTAGON prescribed for you or your child will depend on your or your child's age and weight. For children up to age 12 years, the dose will be based on the body size (surface area), the usual dose being 1.30 g/m2 of body surface area per day. For patients over age 12 and over 50 kg weight, the usual dose is 2g/day. In any case the usual dose should not exceed 1.95 g/m2/day. CYSTAGON should be taken or given only by mouth and exactly as your or your child's doctor directs. In order for CYSTAGON to work correctly, you must do the following: Follow your doctor's directions exactly. Do not increase or decrease the amount of medicine without your doctor's approval. Hard capsules should not be given to children under approximately six years of age because they may not be able to swallow them and they may choke. For children under approximately six years of age, the hard capsule may be opened and the contents sprinkled on food (e.g. milk, potatoes or starch based foods) or mixed in formula. Do not add to acidic drinks e.g. orange juice. Consult the doctor for complete directions. Your or your child's medical treatment may include, in addition to CYSTAGON, one or more supplements to replace important electrolytes lost through the kidneys. It is important to take or give these supplements exactly as instructed. If several doses of the supplements are missed or weakness or drowsiness develops, call the doctor for instructions. Regular blood tests to measure the amount of cystine inside white blood cells are necessary to help determine the correct dose of CYSTAGON. Your or your child's doctor will arrange for the blood tests to be done. Regular blood and urine tests to measure the levels of the body's 2
important electrolytes are also necessary to help your or your child's doctor correctly adjust the doses of these supplements. CYSTAGON should be taken 4 times a day, every 6 hours, preferably just after or with food. It is important to take the dose as close to every 6 hours as possible. Treatment with CYSTAGON should continue indefinitely, as instructed by your doctor. To easily open and close the Cystagon bottle, please follow the instructions below: Directions for opening and closing the container
To Open Place pen or similar object between the raised sections of the lid and turn in the direction shown (anticlockwise)
To Close Place pen or similar object between the raised sections of the lid and turn in the direction shown (clockwise)
If you use more CYSTAGON than you should: You should contact your or your child's doctor or the hospital emergency department immediately if more medicine has been taken than has been prescribed, drowsiness develops . If you forget to take CYSTAGON: If a dose of medicine is missed, it should be taken as soon as possible. However if it is within two hours of the next dose, skip the missed dose and go back to the regular dosing schedule. Do not take a double dose to make up for a forgotten dose. 4.
Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them. CYSTAGON may cause some people to become drowsy or less vigilant than they are normally. Make sure you know how you or your child reacts to this medicine before doing anything that could be dangerous if not alert. The following side effects were reported as follows: very common (occurring in at least one in 10 patients), common (occurring in at least one in 100 patients), uncommon (occurring in at least one in1,000 patients), rare (occurring in at least one in 10,000 patients), very rare (occurring in at least one in 100,000 patients). –
Very common: vomiting, nausea, diarrhoea, loss of appetite, fever and sensation of sleep
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Common: abdominal pain or discomfort, unpleasant breath and body odour, skin eruption, gastroenteritis, fatigue, headache, encephalopathy (brain disorder) and liver function test abnormalities.
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Uncommon: skin striae, skin lesion (little-hard lumps on elbows), joint laxity, leg pain, bone fracture, scoliosis (deviation of the vertebral column), bone deformity and fragility, hair discoloration, severe allergic reaction, somnolence, fits, nervousness, hallucination, decrease in white blood cells, gastrointestinal ulcer manifested by bleeding in the digestive tract and effect on the kidney manifested by swelling of the extremities and weight gain. Since some of these side effects are serious, ask your or your child's doctor to explain their warning signs. 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
CYSTAGON
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 label after EXP. The expiry date refers to the last day of that month. Do not store above 25°C and keep the container tightly closed in order to protect from light and moisture. 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 CYSTAGON contains The active substance is cysteamine bitartrate (mercaptamine bitartrate). Each hard capsule of CYSTAGON 50 mg contains 50 mg of cysteamine (as mercaptamine bitartrate) Each hard capsule of CYSTAGON 150 mg contains 150 mg of cysteamine (as mercaptamine bitartrate) The other ingredients are microcrystalline cellulose, starch, pregelatinized, magnesium stearate/sodium lauryl sulphate, colloidal silicon dioxide, croscarmellose sodium, capsule shells: gelatin, titanium dioxide, black ink on hard capsules (E172). What CYSTAGON looks like and contents of the pack Hard Capsules
- Cystagon 50 mg: white, opaque hard capsules with CYSTA 50 on the body and RECORDATI RARE DISEASES on the cap. Bottles of 100 or 500 hard capsules. All pack sizes may be not marketed.
- Cystagon 150 mg: white, opaque hard capsules with CYSTAGON 150 on the body and RECORDATI RARE DISEASES on the cap. Bottles of 100 or 500 hard capsules. All pack sizes may be not marketed. Marketing Authorisation Holder Recordati Rare Diseases Tour Hekla 4
52, Avenue du Général de Gaulle F-92800 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 12/2024
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Frequently asked questions about Cystagon 150 mg hard capsules
How do I take Cystagon 150 mg hard capsules?
Cystagon 150 mg hard capsules comes as capsule containing 150mg. 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 Cystagon 150 mg hard capsules?
The active substance in Cystagon 150 mg hard capsules is mercaptamine bitartrate.
Where does this information come from?
This leaflet reproduces the patient information leaflet approved for Cystagon 150 mg hard capsules, 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 Cystagon 150 mg hard capsules 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: mercaptamine bitartrate
- 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
CYSTAGON is indicated for the treatment of proven nephropathic cystinosis. Cysteamine reduces cystine accumulation in some cells (e.g. leukocytes, muscle and liver cells) of nephropathic cystinosis patients and, when treatment is started early, it delays the development of renal failure.
4.2. Posology and method of administration
CYSTAGON treatment should be initiated under the supervision of a physician experienced in the treatment of cystinosis.
The goal of therapy is to keep leucocyte cystine levels below 1 nmol hemicystine/mg protein. White blood cell (WBC) cystine levels should therefore be monitored to adjust the dose. The WBC levels should be measured 5 to 6 hours after dosing and should be checked frequently when initiating therapy (e.g. monthly) and every 3-4 months when on a stable dose.
• For children up to age 12 years, CYSTAGON dosing should be on the basis of body surface area (g/m2/day). The recommended dose is 1.30 g/m2/day of the free base divided four times daily.
• For patients over age 12 and over 50 kg weight, the recommended CYSTAGON dose is 2 g/day, divided four times daily.
Starting doses should be 1/4 to 1/6 of the expected maintenance dose, increased gradually over 4-6 weeks to avoid intolerance. The dose should be raised if there is adequate tolerance and the leucocyte cystine level remains >1 nmol hemicystine/mg protein. The maximum dose of CYSTAGON used in clinical trials was 1.95 g/m2/day.
The use of doses higher than 1.95 g/m2/day is not recommended (see section 4.4).
Digestive tolerance of cysteamine is improved when the medicinal product is taken just after or with food.
In children who are at risk of aspiration, aged approximately 6 years and under, the hard capsules should be opened and the content sprinkled on food. Experience suggests that foods such as milk, potatoes and other starch based products seem to be appropriate for mixing with the powder. However, acidic drinks, e.g. orange juice, should generally be avoided as the powder tends not to mix well and may precipitate out.
Patients on dialysis or post-transplantation:
Experience has occasionally shown that some forms of cysteamine are less well tolerated (i.e. leading to more adverse events) when patients are on dialysis. A closer monitoring of the leucocyte cystine levels is recommended in these patients.
Patients with hepatic insufficiency:
Dose adjustment is not normally required; however, leucocyte cystine levels should be monitored.
4.3. Contraindications
Hypersensitivity to the active substance or to any of the excipients listed in section 6.1 The use of CYSTAGON is contra-indicated during breast-feeding. CYSTAGON should not be used during pregnancy, particularly during the first trimester, unless clearly necessary (see section 4.6 and section 5.3), as it is teratogenic in animals.
CYSTAGON is contraindicated in patients who have developed hypersensitivity to penicillamine.
4.4. Special warnings and precautions for use
CYSTAGON therapy must be initiated promptly after confirmation of the diagnosis of nephropathic cystinosis to achieve maximum benefit.
Nephropathic cystinosis must have been diagnosed by both clinical signs and biochemical investigations (leucocyte cystine measurements).
Cases of Ehlers-Danlos like syndrome and vascular disorders on elbows have been reported in children treated with high doses of different cysteamine preparations (cysteamine chlorhydrate or cystamine or cysteamine bitartrate) mostly above the maximal dose 1.95 g/m2/day. These skin lesions were associated with vascular proliferation, skin striae and bone lesions.
It is therefore recommended to monitor regularly skin and to consider X-ray examinations of the bone as necessary. Self-examination of the skin by the patient or the parents should also be advised. If any similar skin or bone abnormalities appear, it is recommended to decrease the dose of CYSTAGON.
The use of doses higher than 1.95g/m2/day is not recommended (see sections 4.2 and 4.8).
Monitoring of blood cell count is recommended on a regular basis.
Oral cysteamine has not been shown to prevent eye deposition of cystine crystals. Therefore, where cysteamine ophthalmic solution is used for that purpose, its usage should continue.
In contrast to phosphocysteamine, CYSTAGON does not contain phosphate. Most patients will already be receiving phosphate supplements and the dose of these may need to be altered when CYSTAGON is substituted for phosphocysteamine.
Intact CYSTAGON hard capsules should not be administered to children under the age of approximately 6 years due to risk of aspiration (see section 4.2).
Do not swallow the desiccant canister found in the bottle.
4.5. Interaction with other medicinal products and other forms of interaction
No interaction studies have been performed
Interactions with other medicines have not been studied. CYSTAGON can be administered with electrolyte and mineral replacements necessary for management of the Fanconi syndrome as well as vitamin D and thyroid hormones. Indomethacin and CYSTAGON have been used simultaneously in some patients. In cases of patients with kidney transplants, anti-rejection treatments have been used with cysteamine.
4.6. Fertility, pregnancy and lactation
Pregnancy
There are no adequate data from the use of cysteamine bitartrate in pregnant women. Studies in animals have shown reproductive toxicity, including teratogenesis (see section 5.3). The potential risk for humans is unknown. The effect on pregnancy of untreated cystinosis is also unknown.
Therefore, CYSTAGON should not be used during pregnancy, particularly during the first trimester, unless clearly necessary.
If a pregnancy is diagnosed or planned, the treatment should be carefully reconsidered and the patient must be advised of the possible teratogenic risk of cysteamine.
Breast-feeding
CYSTAGON excretion in human's milk is unknown. However, due to the results of animal studies in breast-feeding mothers and neonates (see section 5.3), breast- feeding is contra-indicated in women taking CYSTAGON.
4.7. Effects on ability to drive and use machines
CYSTAGON has minor or moderate influence on the ability to drive ans use machines
CYSTAGON may cause drowsiness. When starting therapy, patients should not engage in potentially hazardous activities until the effects of the medicinal product on each individual are known.
4.8. Undesirable effects
Approximately 35% of patients can be expected to experience adverse reactions. These mainly involve the gastrointestinal and central nervous systems. When these effects appear at the initiation of cysteamine therapy, temporary suspension and gradual reintroduction of treatment may be effective in improving tolerance.
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) and uncommon (≥1/1,000 to <1/100).
Within each frequency grouping, undesirable effects are presented in order of decreasing seriouness
Investigations
Common: Liver function tests abnormal
Blood and lymphatic system disorders
Uncommon: Leukopenia
Nervous system disorders
Common: Headache, encephalopathy
Uncommon: Somnolence, convulsions
Gastrointestinal disorders
Very common: Vomiting, nausea, diarrhoea
Common: Abdominal pain, breath odour, dyspepsia, gastroenteritis
Uncommon: Gastrointestinal ulcer
Renal and urinary disorders
Uncommon: Nephrotic syndrome
Skin and subcutaneous tissue disorders
Common: Skin odour abnormal, rash
Uncommon: Hair colour changes, skin striae, skin fragility (molluscoid pseudotumor on elbows)
Musculoskeletal and connective tissue disorders
Uncommon: Joint hyperextension, leg pain, genu valgum, osteopenia, compression fracture, scoliosis.
Metabolism and nutrition disorders
Very common: Anorexia
General disorders and administration site conditions
Very common: Lethargy, pyrexia
Common: Asthenia
Immune system disorders
Uncommon: Anaphylactic reaction
Psychiatric disorders
Uncommon: Nervousness, hallucination
Cases of nephrotic syndrome have been reported within 6 months of starting therapy with progressive recovery after treatment discontinuation. In some cases, histology showed a membranous glomerulonephritis of the renal allograft and hypersensitivity interstitial nephritis.
Cases of Ehlers-Danlos like syndrome and vascular disorders on elbows have been reported in children chronically treated with high doses of different cysteamine preparations (cysteamine chlorhydrate or cystamine or cysteamine bitartrate) mostly above the maximal dose 1.95 g/m2/day.
In some cases, these skin lesions were associated with vascular proliferation, skin striae and bone lesions first seen during an X-ray examination. Bone disorders reported were genu valgum, leg pain and hyperextensive joints, osteopenia, compression fractures, and scoliosis.
In cases where histopathological examination of the skin was performed, the results suggested angioendotheliomatosis.
One patient subsequently died of acute cerebral ischemia with marked vasculopathy.
In some the patients, the skin lesions on elbows regressed after CYSTAGON dose reduction.
Cysteamine mechanism of action by interfering with the cross-linking of collagen fibers has been postulated (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
An overdose of cysteamine may cause progressive lethargy.
Should overdosage occur, the respiratory and cardiovascular systems should be supported appropriately. No specific antidote is known. It is not known if cysteamine is removed by haemodialysis.
🇷🇴 Known in Romania as
Medicines sold in Romania with the same active substance: Cunoscut în România ca
⚠ Not the same combination. This medicine contains Mercaptamine bitartrate. The products below do not contain exactly the same set of active substances — they are not direct substitutes.
- PROCYSBI 25 mg prescription partial — not the same combinationMERCAPTAMINUM · taken by mouth
- PROCYSBI 75 mg prescription partial — not the same combinationMERCAPTAMINUM · taken by mouth
Some of these do not contain exactly the same active substances — check each one. The strength, the form and whether you need a prescription can differ. Always ask a pharmacist before you switch. Romanian medicines in the UK →
🇵🇱 Known in Poland as
Medicines sold in Poland with the same active substance: W Polsce znany jako
⚠ Not the same combination. This medicine contains Mercaptamine bitartrate. The products below do not contain exactly the same set of active substances — they are not direct substitutes.
⚠ Same active substance, but a different pharmaceutical form (for example a gel instead of a tablet). Not interchangeable — ask a pharmacist.
- Cystadrops partial — not the same combinationMercaptamini hydrochloridum · eye / ear / nose
Some of these do not contain exactly the same active substances — check each one. The strength, the form and whether you need a prescription can differ. Always ask a pharmacist before you switch. Polish medicines in the UK →
💬 Ask about this leaflet
Ask anything about Cystagon 150 mg hard capsules. 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.