INTRODUCTION

Lets talk a little bit on the history of Haemophilia.
In the year 1802 the first case of Heamophila was discovered in Us, Later on in the year 1843 it was discovered that Queen Victoria of England had this disease and has transfered the mutated gene to her son and daughters, her son Leopald died at the age of 30 after excessive bleeding.
It was from here that this disease got its name as the Royal disease after spreading throughout the European royalties including the royal families in Germany, Russia and Spain

DEFINITION

SOURSE
The word was originally called "Haemohaphila" which means the WILLINGNESS/THE LOVE TO BREED, it is an inherited disorder due to the mutation of the genes that codes for clotting factors, this leads to excessive bleeding mainly in the male, the females are seen as the carrier of this abnormal trait which they pass on to the male, hence the female gender rarely bleeds due to haemophilia but the males suffers it the most since bleeding are more evident in them.

TYPES OF HAEMOPHILA

There are different types of Haemophilia depending on the deficient clotting factors, so let's get into it

1.HAEMOPHILIA TYPEA: This occurs as a result of deficient of a clotting factor called Factor VIII(8), it is the most common type of Haemophilia

2.HAEMOPHILIA TYPEB: It is as a result of deficiency in a clotting factor called Factor IX(9), this type is also called Christmas disease because it was named after the first patient presented with the disease Stephen Christmas

3.HAEMOPHILIA TYPEC: This type is mild and result from deficiency of factor XI(11)

DEMOGRAPHICS AND EPIDEMIOLOGY

Haemophilia A is known to exist in all ethnic group in the world and hence the most common Haemophilia it occurs in 1:4000 male birth, while Haemophilia B occurs in 1:30000 male births.

HOW HAEMOPHILIA PRESENTS CLINICALLY

There are different presentations of Haemophilia in the clinic so let us get into it

Hemathrosis

SOURCE
This involves bleeding into the joint, it is known that about 70% of Haemophilia occurs in the joint, this causes severe pain and if not treated on time can lead to joint destruction

Hematomas

SOURCE
In Hematomas, bleeding occurs due to injuries in the veins, arteries as a result of anuerysm, head injury or car accident.

Hematuria

SOURCE
This is seen as blood in the urine, it occurs when bacteria in the bladder multiplies leading to small leakage of blood from smaller blood vessels in the bladder. A patient suffering from this often experiences burning pain during urination.

TREATMENT OF HAEMOPHILIA

The best form of treatment for Haemophilia is Prophylaxis i.e the treatment given to prevent this disease. Some drug product like Concizumab, Fitusiran and Emicizumab are used in the treatment of Haemophilia.
Gene therapy has also been proven to be effective in the the treatment of Haemophilia.

CONCLUSION

The purpose of this write up is to enlighten us about Haemophilia which is a very serious condition, but when identified early can be managed properly. Thanks.

@whitestallion
@emmanuel-malume