Coagulation Factor IX Recombinant (Intravenous)

Coagulation Factor IX Recombinant is a prescription only medication used to control or prevent bleeding in patients that have a Factor IX protein deficiency.

Overview

Coagulation Factor IX Recombinant is provided as a solution that can be diluted and administered via the intravenous route.

Factor IX deficiency happens when a patient has a condition called haemophilia B (sometimes referred to as Christmas disease). Factor IX is a natural protein that is produced by the body. It is a clotting agent that helps the blood to clot and prevent bleeding. Without this protein, it is difficult for wounds to heal, and therefore patients may bleed for longer and lose more blood. They may also often experience unusual or sudden bleeding that happens without an obvious cause. An occasion where people have the potential to lose a lot of blood is after surgery; those with Factor IX deficiency are therefore at high risk due to loss of blood and the inability to heal the wound. Coagulation Factor IX Recombinant is then typically prescribed to reduce this bleeding. The drug is a man-made version of Factor IX, therefore replicating the natural protein and creating the same effect.

Coagulation Factor IX Recombinant should not be used to replace any other of the body's natural proteins, such as factors II, VII, VIII, or X. It should also not be used as a blood thickener (e.g. reversing the impact of blood thinning medications)

This medication is also available under three brand names in the United States. These each contain the same active ingredients and are each used for the same purpose:

  • Benefix
  • Ixinity
  • Rixubis

Type of medication

  • Solution for dilution

Conditions treated

  • IX protein deficiency

Side effects

As with any medication, there are potential side effects of taking a protein replacement. Many of them are not serious, and will not require medical attention. However, if you experience any of the below side effects, you should seek medical advice from your doctor or nurse straight away, as they may be a sign of a serious condition:

  • Unusual sensations in the skin, such as itching, burning, prickling, numbness, pins and needles or tingling
  • Anxiety, confusion or restlessness
  • Blurry vision
  • Persistent cough or wheezing
  • Difficulty breathing, or catching a full breath
  • Pain in your chest
  • Difficulty swallowing
  • Change in your heartbeat, such as increase in the pace, pounding or irregularities
  • Fainting, faintness, dizziness, light-headedness, particularly when getting up from lying or
  • sitting down
  • Skin rash or hives
  • Swelling, particularly to areas such as the eyelids, face, tongue, lips, hands, feet, legs, groin
  • Nausea or vomiting
  • Severe headaches
  • Pain in the legs, particularly the calves
  • Lack of coordination or balance
  • Shortness of breath for no reason, or after only mild physical exertion
  • Loss of speech or slurred speech
  • Excessive sweating
  • Skin discoloration
  • Veins becoming more prominent or protruding
  • Excessive tiredness
  • Feeling of weakness in the body, particularly the muscles
  • Drowsiness
  • Loss of interest in doing things you once enjoyed
  • Tight feeling in the chest

While you may need medical attention if you are experiencing any of the above, there are other side effects that you may feel that should not be a cause for concern unless they persist. They are listed below:

  • Losing your sense of taste
  • Change in your taste
  • Mild headaches
  • Nausea
  • Slight pain in your legs or arms
  • Reaction at the site of injection (e.g. pain, swelling, bruising, redness or itchiness)
  • Drowsiness or tiredness
  • Chills

This replacement Factor IX medication contains human blood to replicate the substance. Therefore, there is a very slim chance of catching an infection from this blood. However, all blood donors will have been screened meticulously to minimize this risk. If you notice any signs of an infection, such as fever, sore throat that won't go away, persistent tiredness, nausea or vomiting, jaundice (yellowing of the eyes and skin), report it to your doctor immediately.

Dosage

The dosage patients receive of this medication will depend on their individual body weight and medical history, as well as their age and severity of the deficiency. As a general guideline, one unit of Coagulation Factor IX Recombinant per kilogram of body weight is expected to increase the level of Factor IX in the patient's blood by 0.7 international units / dL of plasma. The formula that is used for calculating dosage is the same for all adult patients:

  • Initial Dose = body weight (kg) x desired factor IX increase (% of normal or IU/dL) x reciprocal of observed recovery (IU/kg per IU/dL)
  • For children under the age of 12, this dosage will be altered according to the following formula:
  • Dose (international units) = body weight (kg) x desired factor IX increase (% of normal or IU/dL) x 1.4 dL/kg
  • The dosage given may be adjusted after the initial dose, depending on the body's reaction and success rate.

Major drug interactions

A drug interaction occurs when two or more substances react with each other, causing unwanted or unknown side effects. These interactions can vary in severity but are grouped into three categories minor, moderate and major. This categorization helps doctors to establish whether or not it is safe to take certain drug combinations together. Those with minor reactions can often be taken at the same time; however, the dosage may be adjusted for either medication. Major interactions should always be avoided as the risk to health will be greater than any benefit.

The only drugs that have been found to have a major interaction with this one are carfilzomib

and its branded version Kyprolis. Tell your doctor if you are taking this medication. Taking the two drugs together could cause potentially dangerous blood clots. This risk will be significantly higher in patients that are older, smoke cigarettes, have high cholesterol levels or high blood pressure. This could then lead to severe conditions such as heart attacks, strokes or a blockage of lung arteries.

You should also tell your doctor about any other medications that you are taking, so that he or she can determine the most effective and safe treatment method for you.

Warnings

Pregnancy

There is not currently any data that suggests Coagulation Factor Ix Recombinant can cause harm to pregnant women or fetuses, nor any data available from animal studies. However, the drug has been classed as a category C in terms of risk for use during pregnancy. Therefore, it should only be used if your doctor has assessed the benefit of taking it to be greater than the risk.

Breastfeeding

There is no data to suggest that Coagulation Factor IX Recombinant passes into human breast milk. However, there is the potential to cause serious adverse reactions in nursing babies; therefore, the drug is not recommended for administration into breastfeeding mothers. If the benefit has been seen to outweigh the risk, the mother and baby should be closely monitored throughout the treatment cycle.

Children

Studies and data show that there is a 22 percent reduction in effectiveness of this drug when used in patients under the age of 12 years. Therefore, more medication may be needed to have the same impact, or a different treatment may need to be sought if the medication is not successful.

Infections

Coagulation Factor IX Recombinant is made from human DNA and blood. Therefore, there is a risk of the patient contracting an infection. Patients should report any signs of infection to a doctor immediately, such as fever, sore throat, vomiting or diarrhea.

Allergic reactions

There is a high risk of allergic reactions to this medication (hypersensitivity reactions). This can include conditions such as anaphylaxis, pruritus, rash, swelling, dizziness, urticaria, hives, hypotension and tightness in the chest. Injections should be performed in a medical environment where the patient can be monitored closely for allergic reactions.

Storage

This medication is supplied in powder form, and therefore should be kept at room temperature (between 15 and 25 degrees centigrade). Always keep the powder and dilutant in the box with which they were provided if you have been given the medication to self-administer and keep it out of the reach of children.

When ready for use, the powder should be diluted with the liquid provided and then used immediately. However, the mixture can be refrigerated for up to four hours. It should not be used after this time dispose of any solution that has been left longer than this.

Summary

Under medical supervision, Coagulation Factor IX Recombinant has been found to be very effective at replacing the natural protein, Factor IX, and therefore preventing or reducing bleeding in patients with a deficiency. It is intended as a short-term treatment to help the patient get through traumas such as surgery without an excessive loss of blood. However, the medication is not a cure for Factor IX deficiency and should therefore not be used as a long-term replacement.

This is a relatively safe medication, with little evidence found from medical studies that there are any groups of patients to which it should not be applied. However, medical professionals may wish to proceed with extra caution in administering to patients that are pregnant, elderly or under the age of 12. These patients may need to stay in a medical environment while treatment is taking place. Due to the reduction in the success rate of the drug in children, there may be other treatments that could be more effective. Medical professionals should be trained in dealing with haemophilia B specifically when administering to high-risk groups in order to detect and potential adverse reactions.

As Coagulation Factor IX Recombinant is a man-made protein, manufactured using human DNA and blood, the risk of an infection is possible. Although it is very rare, the possible consequences can be dangerous, so doctors should fully explain to patients about how to detect an infection before it becomes serious. There is also a higher risk of developing blood clots, particularly if taken with carfilzomib. This can be serious even life-threatening so treatment should stop immediately upon signs of clotting.