Benefix ^*

BeneFIX  (recombinant coagulation factor IX) is a self-injectable medication prescribed for:

• On-demand treatment and control of bleeding episodes in adults with hemophilia B
• Perioperative management of bleeding
• Routine prophylaxis to reduce the frequency of bleeding episodes in patients 16 years of age and older with hemophilia B.

BeneFIX is not used to treat hemophilia A. Hemophilia B is also known as congenital factor IX deficiency, alternatively Christmas disease (named for the first patient identified with this disease, Stephen Christmas), or Factor IX deficiency. Hemophilia B is an inherited (recessive) bleeding disorder caused by a deficiency of clotting factor IX (FIX). It’s caused by a mutation in the F9 gene on the X chromosome and usually affects mainly males, although females can be carriers. Symptoms are bleeding, easy bruising, and joint pain. The severity of bleeding can vary. Factor IX replacement therapy, such as BeneFIX, is the standard treatment.

Dosage

The prescribing doctor will set out a precise regimen for using the drug, and you should not change from it in any way without consulting with a physician, nurse practitioner, or pharmacist. Read the instructions that come with each refill of BeneFIX in case there have been changes since the last time it was purchased. In most cases, the first use of BeneFIX will be under the supervision of a trained healthcare provider in case there are allergic reactions. Subsequent use at home must follow the steps laid out in the Patient Leaflet that comes with each refill.

Storage

The BeneFIX kit can be stored in a refrigerator but not frozen, or at room temperature in the original packaging, away from moisture and direct sunlight. For more information, read our article on drug storage.

Questions people ask about BeneFIX.

Why is hemophilia B called the royal disease?

Hemophilia B was also known as the “royal disease” because it was passed down through several European royal families. It is believed that English Queen Victoria carried a gene for hemophilia, which was likely caused by a spontaneous mutation. This gene was passed from Queen Victoria to the ruling families of Russia, Spain, and Germany.

Does hemophilia B happen in any other species?

Yes, there is a form of canine hemophilia that can affect dogs.

Is it possible to live with hemophilia?

Once a person has been diagnosed with the defective gene, proper treatment and care can allow a person to live a relatively ordinary life. However, the life expectancy of males with hemophilia is still 10 years shorter than that of the general male population.