Ceprotin 500unit Inj, 1 Vial
It is used to treat or prevent blood clots and blood spots, bruising, and change in skin color in people with protein C deficiency.
Drug Class
Anticoagulant, Protein C replacement
Pharmacologic Class
Plasma-derived protein, Antithrombotic agent
Pregnancy Category
Category C
FDA Approved
Feb 1998
DEA Schedule
Not Controlled
Overview
What is this medicine?
Ceprotin is a medicine that replaces a missing protein in your blood called Protein C. This protein helps prevent your blood from clotting too much. It is used in people who are born with a severe lack of Protein C, which can cause dangerous blood clots.
How to Use This Medicine
Proper Use of This Medication
To use this medication correctly, follow your doctor's instructions and read all the information provided. This medication is administered into a vein over a period of time. If you will be self-administering the injection, your doctor or nurse will instruct you on the proper technique. Before and after handling the medication, wash your hands thoroughly.
Preparation and Administration
Before use, this medication must be mixed according to the instructions provided by your doctor. Allow the medication to reach room temperature before mixing, but do not heat it. Gently mix the solution without shaking it. After mixing, do not refrigerate the solution. Use the medication within 3 hours of preparation.
Inspecting the Solution
Before administering the medication, inspect the solution for any visible particles, cloudiness, or leakage. The solution should be colorless to faint yellow. If the solution has changed color, do not use it.
Single-Use Vial and Disposal
Each vial is intended for single use only. Discard any unused portion after administering the dose. Dispose of needles and other sharp objects in a designated needle/sharp disposal box. Do not reuse needles or other items. When the disposal box is full, follow local regulations for proper disposal. If you have any questions, consult your doctor or pharmacist.
Storage and Disposal
Store this medication in a refrigerator at a temperature between 2°C and 8°C (36°F and 46°F). Do not freeze the medication.
Missed Dose
If you miss a dose, contact your doctor to determine the best course of action.
To use this medication correctly, follow your doctor's instructions and read all the information provided. This medication is administered into a vein over a period of time. If you will be self-administering the injection, your doctor or nurse will instruct you on the proper technique. Before and after handling the medication, wash your hands thoroughly.
Preparation and Administration
Before use, this medication must be mixed according to the instructions provided by your doctor. Allow the medication to reach room temperature before mixing, but do not heat it. Gently mix the solution without shaking it. After mixing, do not refrigerate the solution. Use the medication within 3 hours of preparation.
Inspecting the Solution
Before administering the medication, inspect the solution for any visible particles, cloudiness, or leakage. The solution should be colorless to faint yellow. If the solution has changed color, do not use it.
Single-Use Vial and Disposal
Each vial is intended for single use only. Discard any unused portion after administering the dose. Dispose of needles and other sharp objects in a designated needle/sharp disposal box. Do not reuse needles or other items. When the disposal box is full, follow local regulations for proper disposal. If you have any questions, consult your doctor or pharmacist.
Storage and Disposal
Store this medication in a refrigerator at a temperature between 2°C and 8°C (36°F and 46°F). Do not freeze the medication.
Missed Dose
If you miss a dose, contact your doctor to determine the best course of action.
Lifestyle & Tips
- Adhere strictly to the prescribed dosing schedule and administration instructions.
- Report any signs of bleeding (e.g., unusual bruising, nosebleeds, blood in urine/stools) or new blood clots (e.g., pain/swelling in legs, chest pain, shortness of breath) to your doctor immediately.
- Inform all healthcare providers, including dentists, that you are receiving Protein C Concentrate.
- Carry identification indicating your condition (severe congenital protein C deficiency) and treatment.
Available Forms & Alternatives
Available Strengths:
Dosing & Administration
Adult Dosing
Standard Dose:
Initial: 100-150 IU/kg IV. Maintenance: 60-80 IU/kg IV every 6-12 hours, adjusted to maintain protein C activity levels.
Dose Range:
60 - 150 mg
Condition-Specific Dosing:
severe congenital protein C deficiency:
Dosing is highly individualized based on clinical response and protein C activity levels. Target protein C activity levels are typically >0.25 IU/mL (or >25 IU/dL).
Pediatric Dosing
Neonatal:
Dosing is individualized based on clinical response and protein C activity levels, similar to adult dosing (e.g., 100-150 IU/kg initial, then 60-80 IU/kg every 6-12 hours).
Infant:
Dosing is individualized based on clinical response and protein C activity levels, similar to adult dosing.
Child:
Dosing is individualized based on clinical response and protein C activity levels, similar to adult dosing.
Adolescent:
Dosing is individualized based on clinical response and protein C activity levels, similar to adult dosing.
Dose Adjustments
Renal Impairment:
Mild:
No specific dose adjustment recommended, but monitor clinical response and protein C levels.
Moderate:
No specific dose adjustment recommended, but monitor clinical response and protein C levels.
Severe:
No specific dose adjustment recommended, but monitor clinical response and protein C levels.
Dialysis:
Not applicable as protein C is a large protein and not significantly cleared by dialysis. Monitor clinical response and protein C levels.
Hepatic Impairment:
Mild:
No specific dose adjustment recommended, but monitor clinical response and protein C levels.
Moderate:
No specific dose adjustment recommended, but monitor clinical response and protein C levels.
Severe:
No specific dose adjustment recommended, but monitor clinical response and protein C levels.
Pharmacology
Mechanism of Action
Protein C Concentrate (Human) is a highly purified, plasma-derived concentrate of human protein C. Protein C is a vitamin K-dependent glycoprotein that plays a crucial role in the regulation of coagulation. When activated by thrombin (in complex with thrombomodulin), activated protein C (APC) inactivates coagulation factors Va and VIIIa, thereby limiting thrombin generation and promoting fibrinolysis. This action helps to prevent and control thrombotic complications in patients with severe congenital protein C deficiency.
Pharmacokinetics
Absorption:
Bioavailability:
100%
Tmax:
Not applicable (IV administration, immediate effect)
FoodEffect:
Not applicable
Distribution:
Vd:
Approximately 0.1 L/kg (similar to plasma volume)
ProteinBinding:
Not applicable (it is a protein itself)
CnssPenetration:
Limited
Elimination:
HalfLife:
Approximately 8-10 hours (range 6-14 hours)
Clearance:
Not precisely quantified, but related to protein catabolism.
ExcretionRoute:
Metabolized and excreted via cellular pathways; not renally excreted as intact protein.
Unchanged:
Not applicable (metabolized)
Pharmacodynamics
OnsetOfAction:
Immediate (upon IV administration, protein C activity levels rise rapidly)
PeakEffect:
Within minutes of infusion completion
DurationOfAction:
Approximately 6-12 hours, depending on half-life and clinical need for sustained levels.
Safety & Warnings
Side Effects
Urgent Side Effects: Seek Medical Help Right Away
Although rare, this medication can cause severe and potentially life-threatening side effects. If you experience any of the following symptoms, contact your doctor or seek medical attention immediately:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Signs of bleeding, including:
+ Vomiting or coughing up blood
+ Vomit that resembles coffee grounds
+ Blood in the urine
+ Black, red, or tarry stools
+ Bleeding from the gums
+ Abnormal vaginal bleeding
+ Unexplained bruises or bruises that increase in size
+ Uncontrollable bleeding
Dizziness or fainting
Shortness of breath
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. If you have side effects that bother you or persist, contact your doctor for guidance.
Reporting Side Effects
If you have questions or concerns about side effects, you can reach out to your doctor or report them to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Although rare, this medication can cause severe and potentially life-threatening side effects. If you experience any of the following symptoms, contact your doctor or seek medical attention immediately:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Signs of bleeding, including:
+ Vomiting or coughing up blood
+ Vomit that resembles coffee grounds
+ Blood in the urine
+ Black, red, or tarry stools
+ Bleeding from the gums
+ Abnormal vaginal bleeding
+ Unexplained bruises or bruises that increase in size
+ Uncontrollable bleeding
Dizziness or fainting
Shortness of breath
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. If you have side effects that bother you or persist, contact your doctor for guidance.
Reporting Side Effects
If you have questions or concerns about side effects, you can reach out to your doctor or report them to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Seek Immediate Medical Attention If You Experience:
- Signs of bleeding: unusual bruising, prolonged bleeding from cuts, nosebleeds, blood in urine or stools (black or tarry stools), severe headache, dizziness.
- Signs of new blood clots: sudden pain or swelling in an arm or leg, chest pain, shortness of breath, sudden vision changes, slurred speech.
- Signs of allergic reaction: rash, hives, itching, difficulty breathing, wheezing, dizziness, fainting, swelling of the face, lips, tongue, or throat.
Before Using This Medicine
Before Taking This Medication: Important Information to Share with Your Doctor
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, any of its components, or other substances, such as foods or drugs. Be sure to describe the allergic reaction you experienced, including any symptoms that occurred.
If you are allergic to heparin or mouse proteins, discuss this with your doctor to determine the best course of action.
To ensure safe treatment, it is crucial to disclose all of your medications, including:
Prescription and over-the-counter (OTC) drugs
Natural products
Vitamins
Additionally, inform your doctor about any existing health problems. This information will help your doctor and pharmacist assess potential interactions between this medication and other substances or health conditions.
Remember to always consult with your doctor before:
Starting any new medication
Stopping or discontinuing a medication
Changing the dosage of any medication
This will help ensure your safety and the effectiveness of your treatment plan.
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, any of its components, or other substances, such as foods or drugs. Be sure to describe the allergic reaction you experienced, including any symptoms that occurred.
If you are allergic to heparin or mouse proteins, discuss this with your doctor to determine the best course of action.
To ensure safe treatment, it is crucial to disclose all of your medications, including:
Prescription and over-the-counter (OTC) drugs
Natural products
Vitamins
Additionally, inform your doctor about any existing health problems. This information will help your doctor and pharmacist assess potential interactions between this medication and other substances or health conditions.
Remember to always consult with your doctor before:
Starting any new medication
Stopping or discontinuing a medication
Changing the dosage of any medication
This will help ensure your safety and the effectiveness of your treatment plan.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication. Regular blood tests will be necessary, as directed by your doctor, to monitor your condition. If you have any questions or concerns, be sure to discuss them with your doctor.
If you are following a low-sodium or sodium-free diet, consult with your doctor before taking this medication, as some products may contain sodium.
This medication contains small amounts of heparin, which may increase the risk of developing heparin-induced thrombocytopenia (HIT), a potentially life-threatening blood disorder. If you develop HIT, you may experience easier bleeding. It is crucial to discuss this risk with your doctor.
As this medication is derived from human plasma, a component of blood, there is a theoretical risk of transmitting viruses that can cause disease. However, the medication undergoes rigorous screening, testing, and treatment to minimize this risk. Your doctor can provide more information on this topic.
If you are pregnant, planning to become pregnant, or breastfeeding, it is essential to discuss the potential benefits and risks of this medication with your doctor to ensure the best possible outcome for you and your baby.
If you are following a low-sodium or sodium-free diet, consult with your doctor before taking this medication, as some products may contain sodium.
This medication contains small amounts of heparin, which may increase the risk of developing heparin-induced thrombocytopenia (HIT), a potentially life-threatening blood disorder. If you develop HIT, you may experience easier bleeding. It is crucial to discuss this risk with your doctor.
As this medication is derived from human plasma, a component of blood, there is a theoretical risk of transmitting viruses that can cause disease. However, the medication undergoes rigorous screening, testing, and treatment to minimize this risk. Your doctor can provide more information on this topic.
If you are pregnant, planning to become pregnant, or breastfeeding, it is essential to discuss the potential benefits and risks of this medication with your doctor to ensure the best possible outcome for you and your baby.
Overdose Information
Overdose Symptoms:
- Increased risk of bleeding (e.g., excessive bruising, prolonged bleeding, hematoma formation)
What to Do:
In case of suspected overdose, seek immediate medical attention. Management typically involves close monitoring for bleeding and supportive care. If bleeding is severe, consider temporary discontinuation of Ceprotin and administration of procoagulant agents if necessary. Call 1-800-222-1222 (Poison Control Center).
Drug Interactions
Moderate Interactions
- Anticoagulants (e.g., heparin, warfarin)
Monitoring
Baseline Monitoring
Protein C activity levels
Rationale: To establish baseline deficiency and guide initial dosing.
Timing: Prior to initiation of therapy
Coagulation parameters (PT, aPTT, fibrinogen)
Rationale: To assess overall coagulation status and identify any pre-existing coagulopathy.
Timing: Prior to initiation of therapy
Clinical assessment for signs of thrombosis or bleeding
Rationale: To establish baseline clinical status.
Timing: Prior to initiation of therapy
Routine Monitoring
Protein C activity levels
Frequency: Initially every 6-12 hours, then as clinically indicated to maintain target levels (e.g., >0.25 IU/mL).
Target: >0.25 IU/mL (or >25 IU/dL)
Action Threshold: If levels fall below target or clinical signs of thrombosis recur, consider increasing dose or frequency.
Clinical assessment for signs of thrombosis (e.g., pain, swelling, redness, shortness of breath)
Frequency: Daily or as clinically indicated
Target: Absence of new thrombotic events
Action Threshold: New or worsening thrombotic symptoms require immediate medical evaluation and potential dose adjustment.
Clinical assessment for signs of bleeding (e.g., bruising, petechiae, hematuria, melena)
Frequency: Daily or as clinically indicated
Target: Absence of significant bleeding
Action Threshold: Any significant bleeding requires immediate medical evaluation and potential dose reduction or temporary discontinuation.
Signs of hypersensitivity/allergic reaction (e.g., rash, urticaria, dyspnea, hypotension)
Frequency: During and immediately after infusion, then as clinically indicated
Target: Absence of allergic symptoms
Action Threshold: Any signs of allergic reaction require immediate discontinuation and appropriate medical management.
Symptom Monitoring
- Signs of thrombosis (e.g., limb pain/swelling, chest pain, shortness of breath, sudden vision changes)
- Signs of bleeding (e.g., unusual bruising, prolonged bleeding from cuts, nosebleeds, blood in urine/stools, severe headache)
- Signs of allergic reaction (e.g., rash, hives, itching, difficulty breathing, wheezing, dizziness, fainting, swelling of face/lips/tongue/throat)
Special Patient Groups
Pregnancy
Category C. Use during pregnancy only if the potential benefit justifies the potential risk to the fetus. There are no adequate and well-controlled studies in pregnant women. Protein C is a normal component of human plasma, but the effects of exogenous administration on fetal development are unknown.
Trimester-Specific Risks:
First Trimester:
Potential risk cannot be ruled out; use only if clearly needed.
Second Trimester:
Potential risk cannot be ruled out; use only if clearly needed.
Third Trimester:
Potential risk cannot be ruled out; use only if clearly needed.
Lactation
L3 (Moderately Safe). It is not known whether Protein C Concentrate is excreted in human milk. However, human protein C is a normal component of human plasma. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for Ceprotin and any potential adverse effects on the breastfed infant from Ceprotin or from the underlying maternal condition. Consider monitoring the infant for any unusual bleeding or allergic reactions.
Infant Risk:
Low to moderate risk; large protein unlikely to be absorbed intact by infant, but theoretical risk of allergic reaction or altered coagulation.
Pediatric Use
Ceprotin is indicated for pediatric patients with severe congenital protein C deficiency. Dosing is individualized based on weight, clinical response, and protein C activity levels, similar to adult dosing. Safety and efficacy have been established in pediatric patients.
Geriatric Use
Clinical studies of Ceprotin did not include sufficient numbers of subjects aged 65 and over to determine whether they respond differently from younger subjects. In general, dose selection for an elderly patient should be cautious, usually starting at the low end of the dosing range, reflecting the greater frequency of decreased hepatic, renal, or cardiac function, and of concomitant disease or other drug therapy. Monitor closely for adverse effects.
Clinical Information
Clinical Pearls
- Ceprotin is an orphan drug indicated for patients with severe congenital protein C deficiency for the prevention and treatment of venous thrombosis and purpura fulminans.
- Dosing is highly individualized and requires careful monitoring of protein C activity levels and clinical response.
- Patients should be monitored for signs of both thrombotic events and bleeding, as well as hypersensitivity reactions.
- Administer via intravenous infusion. Do not administer by subcutaneous or intramuscular route.
- Reconstitution should be done with sterile water for injection, and the solution should be administered within 3 hours after reconstitution.
Alternative Therapies
- There are no direct therapeutic alternatives that replace protein C in patients with severe congenital protein C deficiency. Other anticoagulants (e.g., heparin, warfarin) may be used to manage acute thrombotic events but do not address the underlying protein C deficiency.
Cost & Coverage
Average Cost:
Highly variable, typically several thousand dollars per vial depending on unit strength and supplier. per vial
Insurance Coverage:
Specialty Tier (requires prior authorization, often covered for approved indications by major medical insurance plans)
General Drug Facts
If your symptoms or health issues persist or worsen, it's essential to contact your doctor for further guidance. To ensure your safety, never share your medication with others or take someone else's medication. Some medications may come with an additional patient information leaflet, so be sure to check with your pharmacist for more information. If you have any questions or concerns about your medication, don't hesitate to reach out to your doctor, nurse, pharmacist, or other healthcare provider. In the event of a suspected overdose, immediately call your local poison control center or seek emergency medical attention. When seeking help, be prepared to provide details about the medication taken, the amount, and the time it happened.