Cystagon contains cysteamine bitartrate salt with inactive ingredients such as microcrystalline cellulose, starch, pregelatinized, magnesium stearate or sodium lauryl sulphate, colloidal silicon dioxide, croscarmellose sodium, capsule shells. Cysteamine is an aminothiol that plays its part within lysosomes in a thiol-disulfide interchange reaction that converts cystine into cysteine and cysteine-cysteamine mixed disulfide, both of which can exit the lysosome in patients with cystinosis, an inherited defect of lysosomal transport.
It helps in the treatment of nephropathic cystinosis. It is a lysosomal storage disease identified by the abnormal accumulation of cystine. Nephropathic cystinosis is a genetic disorder that follows an autosomal recessive inheritance pattern. It is a rare autosomal recessive disorder resulting from the accumulation of free cysteine in lysosomes, resulting in intracellular crystal formation through the whole body.
The standard dose strengths are 50 mg and 150 mg, in the form of capsules for oral administration only.
Start treatment with Cysteamine 50 mg immediately after diagnosis of nephropathic cystinosis. Start Cystagon at a fraction of the maintenance dosage.
The only aim to start Cystagon dosage immediately after diagnosis is to keep leukocyte cystine levels below 1 nmol hemicystine per mg protein. Monitor the White blood cell (WBC) cystine levels to adjust the dose. Measure and frequently check the WBC levels 5-6 hours after dosing and when initiating therapy, for example monthly, and every 3-4 months when on a stable dose.
Initial doses should be 1/4 to 1/6 of the regular maintenance dose; increase the dosage slowly over 4- 6 weeks to avoid intolerance. Increase the dose amount if there is adequate tolerance and the leukocyte cystine level remains >1 nmol hemicystine per mg protein. The maximum dose of the Cystagon used in clinical trials was 1.95 g/day. Professionals do not recommend the use of doses higher than 1.95 g/m2 /day.
To improve the digestive tolerance of cysteamine, take medicine just after or with food.
According to experience, it is observed that some forms of cysteamine lead to more adverse events when patients are on dialysis. Healthcare professionals recommend closer monitoring of the leukocyte cystine levels in these patients.
There is no necessity to adjust the dose generally; however, monitor the leukocyte cystine levels in such patients.
An overdose of cysteamine can cause progressive lethargy. If an overdose occurs, then one should support the respiratory and cardiovascular systems appropriately. No specific antidote is known. Removal of cysteamine is not known by hemodialysis.
Every medicine has some side effects, but everyone doesn’t need to experience them. Before taking medication, the patient should consult a doctor to prescribe doses according to the medical history of the patient. Doctors should acknowledge the patient of possible side effects of the drug.
Vomiting, nausea, diarrhea, loss of appetite, fever, and sensation of sleep are very common side effects.
Stomach and abdominal ache or discomfort, unpleasant breath, body odor, skin eruption, gastroenteritis, fatigue, headache, brain disorder, and liver function test abnormalities are common side effects.
Stretch marks, skin lesion (little-hard lumps on elbows), joint laxity, leg pain, bone fracture, scoliosis, bone deformity and fragility, hair discoloration, severe allergic reaction, drowsiness, fits, nervousness, hallucination, decrease in white blood cells, gastrointestinal ulcer indicated by bleeding in the digestive tract and effect on the kidney characterized by weight gain and
swelling of the extremities.
Cystagon contains cysteamine bitartrate salt. It is used to treat nephropathic cystinosis and comes in the form of capsules for oral administration.
Cystagon contains cysteamine bitartrate salt with inactive ingredients as microcrystalline cellulose, starch, pregelatinized, magnesium stearate/sodium lauryl sulphate, colloidal silicon dioxide, croscarmellose sodium, and capsule shells.
Swallow the capsules or granules without chewing with food or just after the meal. Don’t take this medicine with any acidic drinks as the powder tends not to mix well and precipitate out.
Ikris Pharma Network is an authorized partner of “Recordati,” who can help access this medicine in India for the patient through legal procedure. To know more, contact Ikris at toll-free no. 18008891064 or write at firstname.lastname@example.org.
In India, you can buy Cystagon via Ikris Pharma Network, an authorized partner of “Recordati” for Cystagon medicine.
There is no known antidote to Cystagon medicine. If the patient takes an overdose, consult the doctor for possible symptoms and monitor any worsening signs.
Do not store the medicine above 25°C and keep the container tightly closed to protect the drug from light and moisture. Do not consume the medication after the expiry date mentioned on the pack. Keep the medicine out of reach and sight of children.