Cysteamine Bitartrate helps preserve kidney function and manage kidney damage and other
issues in patients with an inherited disorder, caused by a build-up of cysteine in the body called
nephropathic cystinosis. Excessive Cystine build-up causes problems like kidney problems,
slower growth, weaker bones, and eye problems. Cysteamine works by getting the body rid of
Cystine. Cysteamine Bitartrate is a salt of the ammonium derivative including the hydrochloride,
phosphocysteamine, and bitartrate of Cysteamine. The first use of it and the recorded
therapeutic effect dates back to the 1950s. Cysteamine is biosynthesized in the body, by
degrading the coenzyme A. Cysteamine and pantothenic acid are a result of breaking of the
intermediate pantetheine. It is a biosynthetic precursor to the neurotransmitter hypotaurine and
a stable organic compound with amine and thiol functional groups.
Cysteamine, sold under the brand name Cystagon, procysbi among others.
Helene Curtis Inc filed for the patent in 1993 and was first approved as a drug for cystinosis in
the US in 1994. Later in 2013, the USFDA also approved an extended-release form. After the
expiry of its initial patent protection, it is now made and sold as a generic drug by several
manufacturers around the world.
Dosage and Administration
Cysteamine Bitartrate is administered orally as a capsule and extended-release capsule and
also sold as eye drops. Cysteamine Bitartrate has to be taken exactly as directed and
prescribed. It is usually taken 4 times a day or as directed by the doctor. It should be taken with
food or just after a meal to reduce stomach upset. For children under 6 years, the ingredients of
the capsule are sprinkled over soft food like mashed potatoes and consumed immediately, and
the patient can take a dose in the morning for use in the evening or later, it has to be consumed
immediately. The dose is set by the doctor based on the weight, medical condition, and
response to therapy. The patient can start off on a lower dose and can be increased based on
the response to the initial dose and adjusted as the patients feel the benefits.
For managing nephropathic cystinosis, the initial dose is ¼ to 1/6 of the maintenance dose and
then be raised gradually over four to six weeks to avoid intolerance. The recommended
maintenance dose for kids up to age 12 years is 1.30 grams per m2 per day of the free base
and administered in four divided doses. For a patient older than 12 or more than 110 in pounds
weight, the standard dose is 2.0 grams per day, given four times a day. If the patient misses a
dose, they have to take it within 2 hours or else skip the next dose with the next meal but should
not double the dose to make up for the missed dose.
The price of Cysteamine Bitartrate
In the USA, the oral delayed-release capsule of 25 mg strength, costs around $6,356 for a
supply of 60 capsules. Whereas in India, a box of 100 tablets of 150mg costs around USD 9.
How does it work?
Cysteamine bitartrate lowers the cystine content of cells in patients with nephropathic cystinosis,
an inherited defect of lysosomal transport. Cysteamine is an aminothiol, in that, it can participate
within lysosomes in a thiol-disulfide interchange reaction. This converts cystine into cysteine
and cysteine¬cysteamine mixed disulfide. These two can exit the lysosome in patients with
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