The active ingredient of Procysbi is listed as cysteamine in the United States but mercaptamine in the United Kingdom. Mercaptamine is the International Non-proprietary Name of the product while cysteamine is the name used in the United States. The same chemical has 2 names as cysteamine and mercaptamine.
Prescription Procysbi contains the active ingredient cysteamine (also known as mercaptamine) used by adults and children in the treatment of nephropathic cystinosis. In this disease, the amino acid cystine accumulates in the body causing kidney damage along with damage to other organs. Procysbi lowers the levels of cystine within cells. Procysbi treatment must be initiated immediately once diagnosed with nephropathic cystinosis to get the most benefit from the medicine.
Avoid taking Procysbi with high fat or high protein food as well as anything that will reduce the acidity of the stomach (like milk or yoghurt). Rather take Procysbi with an acidic drink like orange or apple juice or even water. Take Procysbi exactly as prescribed at a dose usually based on age and weight.
Procysbi is taken every 12 hours (twice per day) in a consistent way with every dose. The duration of Procysbi treatment is usually life-long as directed by your physician. Your physician will order regular blood tests to measure the amount of cystine inside your blood or white blood cells.
If you miss a dose of Procysbi, take as soon as you remember. If it is within 4 hours to your next dose, skip the missed dose and take the next dose at the regular scheduled time. Do not take double the dose of Procysbi at any time.
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Information about Procysbi (Cysteamine or Mercaptamine)
Procysbi (also known as cysteamine bitartrate) is a prescription medication primarily used to treat nephropathic cystinosis, a rare genetic disorder characterized by the accumulation of cystine in various tissues, particularly the kidneys. Procysbi helps reduce cystine buildup by breaking it down, which can prevent damage to organs and tissues.
Product Highlights
- Procysbi is mainly prescribed for treating nephropathic cystinosis, a genetic disorder that leads to the accumulation of cystine in the kidneys, eyes, and other tissues.
- By reducing cystine buildup, it helps protect kidney function and prevent damage to other organs.
Key Ingredient
Key Benefits
- Helps lower cystine levels in tissues, reducing the risk of organ damage, particularly to the kidneys.
- By preventing cystine accumulation, Procysbi helps to protect kidney function in individuals with nephropathic cystinosis.
- Cysteamine also helps prevent damage to other organs, such as the eyes, muscles, and liver, which can be affected by cystine buildup.
- The extended-release formulation makes dosing more convenient, helping patients follow their treatment plan more easily.
Directions for Use
- The typical dose for Procysbi varies based on the patient’s age, weight, and clinical condition. Dosing generally starts at 1.3 grams per day, divided into two doses, and may be adjusted based on the individual’s response.
- The capsules should be taken twice daily with food, and the contents should not be crushed or chewed. For children unable to swallow capsules, the contents can be mixed with a small amount of food or liquid.
- It is important to take Procysbi consistently at the prescribed times and follow your doctor’s recommendations regarding dosage adjustments.
Safety Concerns
- Common side effects may include nausea, vomiting, diarrhea, stomach pain, and headaches.
- Prolonged use of cysteamine may have an impact on bone growth, and monitoring is important to ensure proper growth and development, especially in children.
- Although the medication helps to prevent damage from cystine buildup, it’s important to monitor kidney function regularly, as nephropathic cystinosis can still cause progressive kidney damage despite treatment.
- Cysteamine may cause eye irritation or other vision-related issues in some individuals.
- Since cysteamine is processed through the liver, patients with liver conditions should be monitored during treatment.
Avoid Procysbi (Cysteamine or Mercaptamine) If
- Avoid using Procysbi if you are allergic to cysteamine or any of its ingredients.
- Avoid use in patients with severe liver problems, as the medication can place additional strain on liver function.
- Cysteamine may increase the risk of stomach ulcers or bleeding, so it should be avoided in patients with active peptic ulcers.
- Procysbi should be used during pregnancy only if the potential benefits justify the risks, and breastfeeding mothers should exercise caution. Always consult with a healthcare provider before starting treatment.
This product requires special packaging to maintain its integrity during the shipping process. DO NOT USE THIS MEDICATION if the attached temperature indicator shows that the medication was exposed to temperatures below 2 degrees or above 8 degrees Celsius, and contact the pharmacy immediately.
