Protein C, Human (Intravenous)

Human Protein C aids in the normal blood coagulation processes in humans for patients diagnosed with severe congenital protein C deficiency.

Overview

Protein C is a naturally occurring protein in the human body. Some individuals, however, are born with protein C deficiency. In severe cases, this could result in life-threatening blood clots forming in the blood vessels or underneath the skin. These conditions are otherwise known as venous thrombosis and purpura fulminans, respectively. This deficiency could moreover cause fatal disseminated intravascular coagulation (DIC) if left untreated.

Human Protein C treatments are intended for children and adults with extreme cases of congenital protein C deficiency.

However, unlike other protein or vitamin treatments that are sourced from animals or nature, for instance, Human Protein C treatments are exclusively sourced from human volunteers who donate blood.

This treatment is administered intravenously by trained medical workers and is only available with a written order from a certified health specialist. In the United States, the treatment goes by the trade name Ceprotin.

The Importance of Human Protein C

Severe Congenital Protein C Deficiency affects one in half a million live births. There are at least three subtypes of the deficiency reported, each of which can manifest in different ways.

For example, in cases where the deficiency kicks coagulation processes into overdrive, the resulting diagnosis is thrombosis. People who are diagnosed with Protein C deficiency are at a higher risk of developing thrombosis, compared to the general population. In fact, roughly 50% of this subgroup have reportedly experienced a thrombotic event by the age of 40.

In order to prevent or treat thrombosis, Human Protein C can be initiated. The treatment has a high success rate as it works in roughly 94% of patients who use it, based on medical research.

In order to determine the level of deficiency in patients, a full pharmacokinetic profile is completed. This aids in determining the correct dosage amounts to prescribe. To investigate if the deficiency is congenital, healthcare workers test family members also.

Conditions Treated

  • Venous Thrombosis
  • Purpura Fulminans
  • Prophylaxis

Type of Medicine

  • Blood coagulation factor
  • Autoprothrombin medicine
  • Non-activated Protein C replacement therapy

Side Effects

Human Protein C may cause adverse reactions to occur in patients receiving this treatment.

The Most Common Side Effects of Human Protein C Include:

  • Chest tightness
  • Edema (swelling), particularly in the eye area
  • Feeling faint or dizzy
  • Labored or short breathing
  • Skin irritation, including rashes and itching

Rare Side Effects

Additionally, there have been some other less common side effects reported with Human Protein C use. These include:

  • Feeling restless or confused
  • Lethargy or sleepiness
  • Perspiration
  • Spikes in temperature
  • Vision changes

Dosage

Ceprotin or Human Protein C, as it's otherwise known as, is exclusively administered in an inpatient or hospital setting. It is supplied intravenously (in the veins).

During use, a physician who is well-versed in providing therapy with blood coagulation factors should be present at all times.

As with most prescription medications, healthcare specialists usually weigh a number of variables to determine the safest dosages. Before prescribing Human Protein C, some of the factors that are assessed include:

  • The age of the patient
  • The scale of Protein C deficiency
  • Pre-existing conditions
  • The overall health of the patient

Protein Plasma C panels are performed in all cases prior to treatment in order to determine the level of deficiency. The dosage amounts below reflect the standard amounts provided in most cases. However, it does not apply to all patient cases:

First Treatment

The first administration typically involves a single dose of 100-120 kg of Human Protein C.

Follow-Up Treatments

Subsequent treatments of Human Protein C are generally 60-80 kg every 6 hours. At least three follow up treatments are suggested.

Max Dose

The maximum amount of injection dosage recommended for all groups of patients is 2 mL | per minute. In patients who weigh less than 10 kg, the max dosage is 0.2 mL | per kg | per minute.

Note: Long-term treatments may be necessary for some patients.

Other Standard Dosages (Prophylaxis)

  • Short-term prophylaxis dosage: 45-60 IU/kg | Every 6 hours
  • Long-term prophylaxis dosage: 45-60 IU/kg | Every 12 hours

Reconstitution of Human Protein C (Ceprotin)

To reconstitute the solution, the following instructions are suggested:

  • 500 IU Vial: Mix with 5 ml of sterile water
  • 1000 IU Vial: Mix with 10 ml of sterile water

After reconstitution, the solution can be stored at room temperature for no more than three hours. Check for any discolorations or a lack of homogeneity. If any of these are observed, discard the solution.

Interactions

Some negative interactions with Ceprotin have been reported. These include:

Drug Interactions

For the most part, medical specialists are advised not to prescribe Human Protein C when patients are currently undergoing treatment with Defibrotide as serious contraindications have been observed in these cases. Additionally, the following medicines are not generally suggested for concomitant use with Human Protein C:

  • Bromfenac
  • Prasugrel
  • Etofenamate
  • Dexketoprofen
  • Lumiracoxib
  • Epoprostenol
  • Ibuprofen
  • Felbinac
  • Diflunisal
  • Streptokinase
  • Collagenase, Clostridium histolyticum
  • Droxicam
  • Phenylbutazone
  • Urokinase
  • Diclofenac
  • Nepafenac
  • Aceclofenac
  • Tenoxicam
  • Choline Salicylate
  • Tenecteplase
  • Vilazodone
  • Fenofibrate
  • Nimesulide Beta Cyclodextrin
  • Oxyphenbutazone
  • Ketoprofen
  • Flurbiprofen
  • Propyphenazone
  • Mefenamic Acid
  • Floctafenine
  • Loxoprofen
  • Selexipag
  • Meloxicam
  • Fepradinol
  • Flufenamic Acid
  • Etodolac
  • Dexibuprofen
  • Rofecoxib
  • Apixaban
  • Proquazone
  • Amtolmetin Guacil
  • Pentosan Polysulfate Sodium
  • Nintedanib
  • Alteplase, Recombinant
  • Parecoxib
  • Anagrelide
  • Tirofiban
  • Valdecoxib
  • Cilostazol
  • Tiaprofenic Acid
  • Rivaroxaban
  • Dabigatran Etexilate
  • Nimesulide
  • Ketorolac
  • Fenofibric Acid
  • Acemetacin
  • Sulfinpyrazone
  • Proglumetacin
  • Eptifibatide
  • Treprostinil
  • Vorapaxar
  • Piketoprofen
  • Iloprost
  • Clopidogrel
  • Abciximab
  • Fenoprofen
  • Morniflumate
  • Nabumetone
  • Ticlopidine
  • Clonixin
  • Tolmetin
  • Levomilnacipran
  • Tolfenamic Acid
  • Orlistat
  • Aspirin
  • Niflumic Acid
  • Lornoxicam
  • Piracetam
  • Meclofenamate
  • Naproxen
  • Reteplase, Recombinant
  • Oxaprozin
  • Feprazone
  • Anistreplase
  • Dipyridamole
  • Celecoxib
  • Fluoxetine
  • Alipogene Tiparvovec
  • Venlafaxine
  • Pranoprofen
  • Dipyrone
  • Cangrelor
  • Indomethacin
  • Etoricoxib
  • Bufexamac
  • Piroxicam
  • Edoxaban
  • Sulindac
  • Vortioxetine

Note: In the event that one or both medicines are necessary, the primary physician may decide on altering the dosage for one or both.

Warnings

For patients' safety, human blood donations are usually screened for a select group of viruses. There are still some risks associated with getting Human Protein C treatments, however, including the potential to pass on:

  • Infectious Diseases and Viruses
  • Creutzfeldt-Jakob disease (CJD) Agent

Underlying Medical Conditions

The presence of pre-existing conditions is a major concern for healthcare professionals prescribing any type of medicine. In cases where Human Protein C is intended for patients, doctors will generally weigh the benefits against risks, particularly when patients are diagnosed with one or more of the following health conditions:

  • Hypertension
  • Kidney Disease
  • Renal Impairment

Bleeding Disorders

If you have a blood or bleeding disorder, tell your doctor before commencing treatment with Intravenous Human Protein C. This treatment could cause the condition to become worse, especially in patients who take Heparin.

Sodium

Ceprotin contains more than 200 mg of sodium. As a result, patients on a low-sodium diet or individuals with a renal impairment should be closely monitored while taking Human Protein C treatments. Adjusted doses may also be necessary.

Hypersensitivities

Though this medicine is derived from human blood, there is still a risk of developing severe allergies. Ceprotin, for example, may contain small hints of mouse protein or heparin.

Patients are generally informed to disclose any incidences of past allergies, particularly any hypersensitivities to foods, dyes, or animal products.

Also, look out for the warning signs of a severe allergic reaction. Some examples include:

  • Difficulty breathing
  • Hives
  • Nonstop itching
  • Swelling

If these symptoms surface during or post-treatment, inform your health worker right away.

Warnings for Medical Workers

To reduce the risk of transmission of infectious diseases, medical workers on duty should adhere to the strict safety processes in place. Some examples include wearing a glove and other protective gear during dosing and administration.

For the patients' safety, needles are intended for single-use only. Following administration, the needles should be disposed of in a biohazard container.

When reconstituting the solution, aseptic techniques should be followed at all times. Read the insert label for additional instructions and confirm with a physician on duty regarding the prescribed dosing amounts.

Follow-Up Visits

To verify that the medicine is working as it should, a number of follow-ups are generally scheduled to gauge performance.

Storage

Ceprotin should always be stored at the following temperature: 2°C-8°C (36°F-46°F).

Care should be taken to not place the vials in the light. To preserve its efficacy, Human Protein C should never be frozen.

Summary

Human Protein C or Ceprotin is a replacement therapy for patients with severe forms of congenital protein C deficiency. When left untreated, this could result in a number of life-threatening and potentially fatal diagnoses, including

In essence, Human Protein C is a coagulation factor that helps to facilitate normal blood coagulating processes and reduce the risks of blood clots.

Due to the fact that Human Protein C originates from human plasma, there are some risks of acquiring viruses. Speak with your doctor to learn more about the screening protocols and any other concerns you may have.

Ceprotin is found to be effective in roughly 94% of patients based on medical research.

 

Resources
Last Reviewed:
January 31, 2018
Last Updated:
January 27, 2018
Content Source: