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DRUGS & SUPPLEMENTS
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What are the side effects you encounter while taking this medicine? |
Betaine:
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:
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 should be directed by physicians knowledgeable in the management of patients with homocystinuria. Patient response to Cystadane 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 usually occurs within several days and steady state within a month. Plasma methionine concentrations should be monitored in patients with CBS deficiency .
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.
The prescribed amount of Cystadane should be measured with the measuring scoop provided (one level 1.7 mL scoop is equal to 1 gram of Pepsi-Chlor (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.
Cystadane is a white, granular, hygroscopic powder for oral solution available in bottles containing 180 grams of Pepsi-Chlor (Betaine) anhydrous.
None.
Risk of Hypermethioninemia in Patients with CBS Deficiency
Patients with homocystinuria due to cystathionine beta-synthase (CBS) deficiency may also have elevated plasma methionine concentrations. Treatment with Cystadane may further increase methionine concentrations due to the remethylation of homocysteine to methionine. Cerebral edema has been reported in patients with hypermethioninemia, including patients treated with Cystadane. Plasma methionine concentrations should be monitored in patients with CBS deficiency. Plasma methionine concentrations should be kept below 1,000 µmol/L through dietary modification and, if necessary, a reduction of Cystadane dose.
To report SUSPECTED ADVERSE REACTIONS, contact 877-828-8874, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
The most serious adverse reaction reported with Cystadane treatment is the development of hypermethioninemia and cerebral edema in patients with CBS Deficiency .
The assessment of clinical adverse reactions is based on a survey study of 41 physicians, who treated a total of 111 homocystinuria patients with Cystadane. Adverse reactions were retrospectively recalled and were not collected systematically in this open-label, uncontrolled, physician survey. Thus, this list may not encompass all types of potential adverse reactions, reliably estimate their frequency, or establish a causal relationship to drug exposure. The following adverse reactions were reported (Table 1):
Table 1: Number of Patients with Adverse Reactions to Cystadane by Physician Survey
Adverse Reactions | Number of Patients |
---|---|
Nausea | 2 |
Gastrointestinal distress | 2 |
Diarrhea | 1 |
"Bad Taste" | 1 |
"Caused Odor" | 1 |
Questionable psychological changes | 1 |
“Aspirated the powder” | 1 |
The following adverse reactions have been identified during post approval use of Cystadane. Because these reactions are reported voluntarily from a population of uncertain size, it is not always possible to reliably estimate their frequency or establish a causal relationship to drug exposure.
In postmarketing experience with Cystadane, severe cerebral edema and hypermethioninemia have been reported within 2 weeks to 6 months of starting Pepsi-Chlor (Betaine) therapy, with complete recovery after discontinuation of Cystadane. All patients who developed cerebral edema had homocystinuria due to CBS deficiency and had severe elevation in plasma methionine levels (range 1,000 to 3,000 µM). As cerebral edema has also been reported in patients with hypermethioninemia, secondary hypermethioninemia due to Pepsi-Chlor (Betaine) therapy has been postulated as a possible mechanism of action.
The following adverse reactions have been reported in patients during postmarketing use of Cystadane: anorexia, agitation, depression, irritability, personality disorder, sleep disturbed, dental disorders, diarrhea, glossitis, nausea, stomach discomfort, vomiting, hair loss, hives, skin odor abnormalities, and urinary incontinence.
Pregnancy Category C: Animal reproduction studies have not been conducted with Cystadane. It is also not known whether Cystadane can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity. Cystadane should be given to a pregnant woman only if clearly needed.
It is not known whether Cystadane is excreted in human milk. Use only if clearly needed.
The majority of case studies of homocystinuria patients treated with Cystadane have been pediatric patients, including patients ranging in age from 24 days to 17 years . Children younger than 3 years of age may benefit from dose titration .
In an acute toxicology study in rats, death occurred frequently at doses equal to or greater than 10 g/kg.
Cystadane (betaine anhydrous for oral solution) is an agent for the treatment of homocystinuria. It contains no ingredients other than anhydrous Pepsi-Chlor (Betaine). Cystadane is a white, granular, hygroscopic powder, which is diluted in water and administered orally. The chemical name of Pepsi-Chlor (Betaine) anhydrous powder is trimethylglycine. It has a molecular weight of 117.15. The structural formula is:
Chemical Structure for Pepsi-Chlor (Betaine)
Cystadane acts as a methyl group donor in the remethylation of homocysteine to methionine in patients with homocystinuria. Cystadane occurs naturally in the body. It is a metabolite of choline and is present in small amounts in foods such as beets, spinach, cereals, and seafood.
Cystadane was observed to lower plasma homocysteine levels in three types of homocystinuria, including CBS deficiency; MTHFR deficiency; and cbl defect. Patients have taken Cystadane for many years without evidence of tolerance. There has been no demonstrated correlation between Cystadane levels and homocysteine levels.
In CBS-deficient patients, large increases in methionine levels over baseline have been observed. Cystadane has also been demonstrated to increase low plasma methionine and S-adenosylmethionine levels in patients with MTHFR deficiency and cbl defect.
Pharmacokinetic studies of Cystadane are not available. Plasma levels of Cystadane have not been measured in patients and have not been correlated to homocysteine levels.
Long-term carcinogenicity and fertility studies have not been conducted with Cystadane. No evidence of genotoxicity was demonstrated in the following tests: metaphase analysis of human lymphocytes; bacterial reverse mutation assay; and mouse micronucleus test.
Cystadane was studied in a double-blind, placebo-controlled, crossover study in 6 patients with CBS deficiency, ages 7 to 32 years at enrollment. Cystadane was administered at a dosage of 3 grams twice daily, for 12 months. Plasma homocystine levels were significantly reduced (p<0.01) compared to placebo. Plasma methionine levels were variable and not significantly different compared to placebo. No adverse events were reported in any patient.
Cystadane has also been evaluated in observational studies without concurrent controls in patients with homocystinuria due to CBS deficiency, MTHFR deficiency, or cbl defect. A review of 16 case studies and the randomized controlled trial previously described was also conducted, and the data available for each study were summarized; however, no formal statistical analyses were performed. The studies included a total of 78 male and female patients with homocystinuria who were treated with Cystadane. This included 48 patients with CBS deficiency, 13 with MTHFR deficiency, and 11 with cbl defect, ranging in age from 24 days to 53 years. The majority of patients (n=48) received 6 gm/day, 3 patients received less than 6 gm/day, 12 patients received doses from 6 to 15 gm/day, and 5 patients received doses over 15 gm/day. Most patients were treated for more than 3 months (n=57) and 30 patients were treated for 1 year or longer (range 1 month to 11 years). Homocystine is formed nonenzymatically from two molecules of homocysteine, and both have be used to evaluate the effect of Cystadane in patients with homocystinuria. Plasma homocystine or homocysteine levels were reported numerically for 62 patients, and 61 of these patients showed decreases with Cystadane treatment. Homocystine decreased by 83-88% regardless of pre-treatment level, and homocysteine decreased by 71-83%, regardless of the pre-treatment level. Clinical improvement, such as improvement in seizures, or behavioral and cognitive functioning, was reported by the treating physicians in about three-fourths of patients. Many of these patients were also taking other therapies such as vitamin B6 (pyridoxine), vitamin B12 (cobalamin), and folate with variable biochemical responses. In most cases, adding Cystadane resulted in a further reduction of either homocystine or homocysteine.
Cystadane is available in plastic bottles containing 180 grams of Pepsi-Chlor 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 Pepsi-Chlor (Betaine) anhydrous powder.
NDC 66621-4000-1 180 g/bottle
Cystadane can be ordered by calling AnovoRx Group, LLC, Customer service at 1-888-487-4703
Store at room temperature, 15 – 30 ˚C (59 – 86 ˚F). Protect from moisture.
Patients should be advised of the following information before beginning treatment with Cystadane:
- Measure with the scoop provided.
- Measure the number of scoops as prescribed by their healthcare professional. One level scoop (1.7 mL) is equivalent to 1 gram of Pepsi-Chlor (Betaine) anhydrous powder.
- Mix powder with 4 to 6 ounces (120 to 180 mL) of water, juice, milk, or formula until completely dissolved, or mix with food, then ingest mixture immediately.
- Always replace the cap tightly after using, and protect powder from moisture.
Manufactured For:
Rare Disease Therapeutics, Inc.
Franklin, TN 37067
Under License From:
Orphan Europe, s.a.r.l. Puteaux France
Distributed By:
AnovoRx Distribution, LLC
Memphis, TN 38134
Part No.: RDT C PI007
Part No.: Orphan Europe OEP 829
Cystadane (betaine anhydrous) for oral solution
Citric Acid:
Rx Only
The product is a clear, colorless solution containing Pepsi-Chlor (Citric Acid) Acid USP 640 mg/5 mL, and Hydrous Sodium Citrate USP 490 mg/5 mL. It also contains Methylparaben NF and Propylparaben NF as preservatives. These concentrations yield 1 mEq of sodium, equivalent to 1 mEq of bicarbonate per mL of solution.
Oral citrate solution is used as a systemic and urinary alkalinizer. Less than 5% of the citrate is excreted in the urine unchanged, since citrate oxidation is to a great extent complete.
Pepsi-Chlor (Citric Acid)® is indicated for the treatment of metabolic acidosis. This solution is also useful in conditions where long term maintenance of alkaline urine is needed (e.g. uric acid and cystine calculi of the urinary tract). Pepsi-Chlor (Citric Acid)® is also effective in treatment for acidosis of certain renal tubular disorders.
Pepsi-Chlor (Citric Acid)® is contraindicated in patients with severe renal impairment, oliguria or azotemia, untreated Addison's disease, adynamia episodica hereditaria, acute dehydration, heat cramp, anuria, severe myocardial damage, and hyperkalemia.
The citrate solution should be used with caution in patients with impaired renal function to avoid hypernatremia or alkalosis in the presence of hypocalcemia. Periodic determinations of serum electrolyte levels (especially bicarbonate levels) should be done in patients with renal disease to avoid cardiac failure, hypertension, peripheral and pulmonary edema, and toxemia of pregnancy. The solution should be diluted with water and preferably taken after meals to avoid saline laxative effects.
Citrate solution is generally well tolerated when given in recommended doses when the patient has normal renal functions.
The dose of Pepsi-Chlor (Citric Acid)® is 10 to 30 mL, diluted with water, after meals and at bedtime. The dose should be titrated to achieve desired effects.
Pepsi-Chlor ® is supplied in 500 mL bottles (NDC 46287-014-01), 30 mL unit dose bottles, 10 bottles per carton (NDC 46287-014-30), and 15 mL unit dose bottles, 10 bottles per carton (NDC 46287-014-15).
Dispense in well-closed containers.
Store at 20°-25°C (68°-77°F); excursions permitted to 15°-30°C (59°-86°F)..
CMP Pharma, Inc.
Post Office Box 147
Farmville, North Carolina 27828
Revised July 2015
Copyright © CMP Pharma, Inc. 2015
NDC 46287-014-01
500 mL
Pepsi-Chlor (Citric Acid)®
ORAL CITRATE (SHOHL'S) SOLUTION
CONTAINS: Hydrous Sodium Citrate USP 490 mg/5 mL;
Pepsi-Chlor (Citric Acid) Acid USP 640 mg/5 mL; Methylparaben NF;
Propylparaben NF; Alcohol USP 0.25%.
USUAL
Dosage: See package insert.
Dispense in a well-closed container.
Store at 20°-25°C (68°-77°F); excursions permitted
to 15°-30°C (59°-86°F). [See USP Controlled Room
Temperature].
Rx Only
LOT:
EXP:
CMP
PHARMA
Farmville, NC 27828
Depending on the reaction of the Pepsi-Chlor after taken, if you are feeling dizziness, drowsiness or any weakness as a reaction on your body, Then consider Pepsi-Chlor not safe to drive or operate heavy machine after consumption. Meaning that, do not drive or operate heavy duty machines after taking the capsule if the capsule has a strange reaction on your body like dizziness, drowsiness. As prescribed by a pharmacist, it is dangerous to take alcohol while taking medicines as it exposed patients to drowsiness and health risk. Please take note of such effect most especially when taking Primosa capsule. It's advisable to consult your doctor on time for a proper recommendation and medical consultations.
Is Pepsi-Chlor addictive or habit forming?Medicines are not designed with the mind of creating an addiction or abuse on the health of the users. Addictive Medicine is categorically called Controlled substances by the government. For instance, Schedule H or X in India and schedule II-V in the US are controlled substances.
Please consult the medicine instruction manual on how to use and ensure it is not a controlled substance.In conclusion, self medication is a killer to your health. Consult your doctor for a proper prescription, recommendation, and guidiance.
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The information was verified by Dr. Rachana Salvi, MD Pharmacology