Hemophilia B / Christmas Disease Facts

Hemophilia B / Christmas Disease Facts

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Hemophilia B is a rare single gene disorder. It is also known as Factor IX deficiency or Christmas disease. This disorder tends to run in families for generations as it only affects males because it is X- linked disorder.

Due to the deficiency of an important clotting factor called factor IX, blood clot formation followed by an injury may not be proper, and so following an accident, hemophiliacs can suffer from excessive bleeding, as it takes longer to heal, in comparison to normal individuals.

Facts

  1. Hemophilia B is thought to be named after Stephen Christmas, who was first diagnosed with this disease in 1952.
  2. Hemophilia B is inherited as an X-linked recessive disorder, where the males are only affected, and females act as carriers of the disease.
  3. Worldwide, Hemophilia
    B is considered to be seven times less common than Hemophilia A which occurs in about 1 in 25,000 and 1 in 30,000–60,000 boys in US and India.
  4. It is estimated that currently, around 20,000 men in the United States are suffering from this disorder.
  5. In the United States, it is estimated that Hemophilia A is four times more common than Hemophilia B and almost half of those who are affected have a severe form of this disorder.
  6. Hemophilia can affect people from all racial and ethnic groups.
  7. Hemophilia B is sometimes also referred to as “The royal disease,” because it has affected the royal families of England, Germany, Russia and Spain in the 19th and 20th centuries.
  8. Queen Victoria who ruled England between 1837-1901, is said to have been a carrier of Hemophilia B.
  9. The global incidence of Hemophilia is estimated to be more than 400,000, and it is estimated that around 75% of people with hemophilia still don’t have access to adequate treatment.
  10. There is no cure for the disease, and the standard treatment of factor IX concentrates can be effective to overcome the deficiency.
References:
  1. Factor IX deficiency (Christmas disease) - (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3862613/)
  2. Hemophilia - (https://www.medindia.net/patients/patientinfo/hemophilia.htm)
  3. Hemophilia facts - (https://www.cdc.gov/ncbddd/hemophilia/facts.html)
  4. History of Bleeding Disorders - (https://www.hemophilia.org/Bleeding-Disorders/History-of- Bleeding-Disorders)




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