Procysbi Delayed Release
Procysbi Delayed Release Uses, Dosage, Side Effects, Mechanism of Action, How Long Does it Take to Work? Procysbi Delayed Release is a brand name. Its generic name is Cysteamine.
Procysbi Delayed Release
Procysbi Delayed Release is an amino acid derivative and is a medication used primarily in the treatment of Cystinosis, a rare inborn disorder of the lysosomal metabolism. It is available in a variety of forms including oral capsule, chewable tablets, and delayed-release capsules.
Uses
Procysbi Delayed Release is used in the treatment of nephropathic cystinosis, an inherited metabolic disorder, in children and adults. It works by breaking down deposits of cystine in cells to an inactive form. Cystinosis causes damage to the cells of the body by the accumulation of cystine which is an amino acid.
Mechanism of Action
Procysbi Delayed Release works by reducing the accumulation of cystine in cells that are caused by lysosomal storage. It catalyzes the reaction of lysine to form cystathionine, which is then converted to cysteine and its derivatives. The cysteine produced is then released from the cell and used as an energy source. The result is that the cystine accumulation decreases, and the cells of the body are able to function properly.
How Long Does It Take to Work?
The effects of cysteamine usually start to be seen within a few weeks or months of treatment. However, the full effect of cysteamine treatment may take up to a year to be seen, depending on the severity of the cystinosis.
Absorption
Procysbi Delayed Release is rapidly absorbed after oral administration. It is extensively bound to proteins in the blood, but is almost completely absorbed from the gastrointestinal tract.
Route of Elimination
The elimination of cysteamine from the body is mainly through the kidneys. It is primarily eliminated as cysteamine metabolites and is slowly excreted through the urine.
Dosage
The recommended dosage of cysteamine is 0.1-0.2 mg/kg body weight twice daily. This dose may be adjusted according to the individual response. The tablets or capsules should be taken with or without food.
Administration
Procysbi Delayed Release is available in oral capsule, chewable tablets, and delayed-release capsules. It should be taken as prescribed by the physician. It is usually pediatric patients who take chewable tablets and adult patients usually take the capsules.
Side Effect
The most common side effects of cysteamine are gastrointestinal (GI) distress, including nausea, vomiting, abdominal pain, and diarrhea. Other side effects may include loss of appetite, dizziness, headache, fatigue, and rash. It is important to contact your doctor if any of these side effects become severe or persist.
Toxicity
Procysbi Delayed Release is considered to have low toxicity. However, high doses may cause adverse effects, such as nausea, vomiting, abdominal pain, and diarrhea. If experienced, contact your doctor immediately.
Precaution
It is important to take cysteamine as prescribed by the doctor. Your doctor will monitor your blood levels and adjust the dosage as needed. It is also important to monitor signs and symptoms of dehydration and drink plenty of fluids throughout the day.
Interaction
Procysbi Delayed Release may interact with certain medications, such as aminoglycoside antibiotics, chemotherapeutic drugs, anticonvulsants, and immunosuppressants. It is important to tell your doctor about any medications you are taking prior to starting cysteamine.
Disease Interaction
Procysbi Delayed Release may interact with certain medical conditions, such as kidney disease, liver disease, and genetic disorders that affect protein metabolism. It is important to tell your doctor about any medical condition you have prior to starting cysteamine.
Drug Interaction
Procysbi Delayed Release may interact with certain drugs, such as aminoglycosides, anticonvulsants, chemotherapeutics, and immunosuppressants. It is important to tell your doctor about any medications you are taking prior to starting cysteamine.
Food Interactions
Certain foods may interact with cysteamine and increase or decrease its effectiveness. Examples of such foods include fortified grains and grains with added cysteine. It is important to tell your doctor about any dietary changes that you plan to make prior to starting cysteamine.
Pregnancy Use
It is not recommended to take cysteamine during the first trimester of pregnancy since it may cause harm to the fetus. It is best to consult with your doctor before taking this medication during pregnancy.
Lactation Use
Procysbi Delayed Release is not recommended during lactation. If the medication is necessary, it is best to consult with your doctor before taking cysteamine while breastfeeding.
Acute Overdose
An acute overdose of cysteamine can cause nausea, vomiting, and abdominal pain. If an overdose is suspected, contact your doctor immediately or call a poison control center for further treatment.
Contraindication
Procysbi Delayed Release is contraindicated in patients with a known sensitivity to it, or in certain conditions, such as kidney or liver disease, or genetic disorders that affect protein metabolism. It is important to talk to your doctor before taking this medication.
Use Direction
It is important to take cysteamine exactly as prescribed by your doctor. Do not take a double dose if you miss a dose. This medication should be taken with or without food.
Storage Condition
Procysbi Delayed Release should be stored at room temperature in a dry place, away from light and heat. It should be kept out of reach of children and pets.
Volume of Distribution
Procysbi Delayed Release has a volume of distribution of 1.014 to 2.13 L/kg.
Half-Life
Procysbi Delayed Release has a half-life of 3-8 hours.
Clearance
Procysbi Delayed Release has an estimated renal clearance of 1.26 to 3.74 mL/min/kg.
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