CYSTADANE acts as a methyl donor, providing an alternate remethylation pathway to convert excess homocysteine to methionine in patients with homocystinuria.1,2,3,4,5
*No formal statistical analyses were performed.
Range of percent decrease in plasma concentrations1
*Homocystine is formed nonenzymatically from two molecules of homocysteine1
CYSTADANE is generally well tolerated.1
Hypermethioninemia and cerebral edema in patients with CBS deficiency were the most serious adverse reactions reported. To keep plasma methionine levels below 1,000 μmol/L, some patients may require dietary modification and, if necessary, a reduction in CYSTADANE dose.1
To determine additional adverse reactions, data was collected through a retrospective survey from 41 physicians who had treated 111 patients with CYSTADANE. Adverse events were retrospectively recalled and were not collected systematically.1
Most common adverse reactions (>2%) were nausea and gastrointestinal distress.1
In patients with low plasma methionine concentrations due to MTHFR deficiency or cbl defect, CYSTADANE has been demonstrated to increase plasma methionine and S-adenosylmethionine (SAM) concentrations.
Patients have taken CYSTADANE for many years without evidence of tolerance. There has been no demonstrated correlation between CYSTADANE concentrations and homocysteine concentrations.
Recommended dosage: 6 grams per day in divided doses of 3 grams twice daily1
Recommended starting dosage: 100 mg/kg/day divided in twice daily doses, and then increased weekly by 50 mg/kg increments1
Therapy with CYSTADANE should be directed by physicians knowledgeable in the management of patients with genetic disorders that cause homocystinuria1
CYSTADANE is available in plastic bottles containing 180 grams of betaine anhydrous. Each bottle comes with a plastic child-resistant cap and a polypropylene measuring scoop. One level 1.7 mL scoop equals 1 gram of betaine anhydrous powder.1
CYSTADANE should only be taken as directed.1
Measure the number of scoops for the patient’s dose with the scoop provided.
One level scoop is equivalent to 1 gram of betaine anhydrous powder.
Mix powder with 4 to 6 ounces of water, juice, milk, or formula until completely dissolved, or mix with food, then ingest mixture immediately.
Cystadane is a methylating agent indicated in pediatric and adult patients for the treatment of homocystinuria to decrease elevated homocysteine blood concentrations. Included within the category of homocystinuria are: