"The National Institutes of Health has launched DS-Connect, a Web-based health registry that will serve as a national health resource for people with Down syndrome and their families, researchers, and health care providers.
Cystadane® (betaine anhydrous for oral solution) is indicated for the treatment of homocystinuria to decrease elevated homocysteine blood levels. Included within the category of homocystinuria are:
- Cystathionine beta-synthase (CBS) deficiency
- 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency
- Cobalamin cofactor metabolism (cbl) defect
DOSAGE AND ADMINISTRATION
The usual dosage in adult and pediatric patients is 6 grams per day administered orally in divided doses of 3 grams twice daily. In pediatric patients less than 3 years of age, dosage may be started at 100 mg/kg/day divided in twice daily doses, and then increased weekly by 50 mg/kg increments.
Therapy with Cystadane (betaine anhydrous) should be directed by physicians knowledgeable in the management of patients with homocystinuria. Patient response to Cystadane (betaine anhydrous) can be monitored by homocysteine plasma levels. Dosage in all patients can be gradually increased until plasma total homocysteine is undetectable or present only in small amounts. Response (by homocysteine plasma levels) usually occurs within several days and steady state within a month. Plasma methionine concentrations should be monitored in patients with CBS deficiency [See WARNINGS AND PRECAUTIONS].
Dosages of up to 20 grams per day have been necessary to control homocysteine levels in some patients. However, one pharmacokinetic and pharmacodynamic in vitro simulation study indicated minimal benefit from exceeding a twice-daily dosing schedule and a 150 mg/kg/day dosage for Cystadane (betaine anhydrous) .
The prescribed amount of Cystadane (betaine anhydrous) should be measured with the measuring scoop provided (one level 1.7 mL scoop is equal to 1 gram of betaine anhydrous powder) and then dissolved in 4 to 6 ounces (120 to 180 mL) of water, juice, milk, or formula, or mixed with food for immediate ingestion.
Dosage Forms And Strengths
Cystadane (betaine anhydrous) is a white, granular, hygroscopic powder for oral solution available in bottles containing 180 grams of betaine anhydrous.
Storage And Handling
Cystadane (betaine anhydrous) is available in plastic bottles containing 180 grams of betaine anhydrous. Each bottle is equipped with a plastic child-resistant cap and is supplied with a polystyrene measuring scoop. One level scoop (1.7 mL) is equal to 1 gram of betaine anhydrous powder.
NDC 66621-4000-1 180 g/bottle
Cystadane (betaine anhydrous) can be ordered by calling Accredo Health Group, Inc., Customer service at 1-888-454-8860
Store at room temperature, 15 – 30 °C (59 – 86 °F). Protect from moisture.
Distributed in the U.S. by: Rare Disease Therapeutics, Inc. 2550 Meridian Blvd., Suite 150 Franklin, TN 37067.This monograph has been modified to include the generic and brand name in many instances.
Last reviewed on RxList: 12/5/2016
Additional Cystadane Information
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