Categories: Haematology

World Hemophilia Day

If a person is bleeding periodically with the slightest bump, it can be a bleeding disorder. Haemophilia can be one of the causes of continuous bleeding. Let’s have a brief knowledge of what hemophilia is.

What is hemophilia?

Hemophilia is a genetically inherited bleeding disorder. A hemophilic person suffers from spontaneous or profuse bleeding after an injury or surgery. In hemophilia, blood would not clot properly because of a low level of blood clotting factors. A hemophilic person may have a low level of either factor 8 or 9.

The severity of the disease depends on the amount of blood clotting factor in the patient’s blood. The lower the quantity of the clotting factor, the more serious the hemophilic condition and person suffers from heavy blood loss.

The small cut does not cause any problem. But, if you have a lower level of blood clotting factors, then internal bleeding due to bumping or injury may occur. Continuous internal bleeding can cause organ damage and can be life-threatening.

What is the cause of hemophilia?

Hemophilia is a genetic condition. It occurs due to a mutation or alteration in a gene that provides information for blood clotting factor manufacturing. As this gene is present on the X chromosome, the male has a high prevalence of hemophilia. Females are a carrier of disease and rarely have hemophilia.

Although hemophilia is an inherited disease, some people may acquire it without a family history. The main reason for acquired hemophilia is our immune system’s attack on clotting factor 8 or 9 in blood.

How many types of hemophilia are there?

There are several types of hemophilia, but two are the most common:

  • Hemophilia A (classic hemophilia):

The person suffering from hemophilia A has a low level or absent factor 8 in their blood.

  • Hemophilia B (Christmas disease): The reason for hemophilia B is a lack or lower level of factor 9 in the patient’s blood.

What are the signs and symptoms of hemophilia?

Signs and symptoms mainly depend on the level of clotting factor in your blood. In mild cases, a person may live near-normal life with bleeding episodes only after trauma or surgery. But in severe clotting factor deficiency, bleeding is evident without reason. Some of the common signs and symptoms of hemophilia are:

  • Anonymous and excessive bleeding after injuries or surgery
  • Profuse bleeding during dental treatment
  • Large and deep bruises after a simple bump with a hard object
  • Hematoma (accumulation of blood in soft tissues and muscle) formation
  • Bleeding gums after harsh brushing
  • Blood in the stool and urine
  • Joint pain or swelling in injury-prone joints such as knees, ankles, and elbows
  • Frequent nosebleed

Which is the diagnostic test for hemophilia?

Mild hemophilia usually remains undiagnosed until adulthood and may get detected during any surgical procedure. The severe form came to notice during the first year of life.

Diagnosis of hemophilia includes:

  • A positive family history
  • Screening tests: It tests if the blood is clotting properly, and it includes:
  • Activated partial thromboplastin time (APTT) test
  • Prothrombin time (PT) test
  • Fibrinogen test
  • Clotting factor test or factor assays: This test shows the type and severity of hemophilia.

What is the treatment of hemophilia?

Treatment of hemophilia depends on the type and severity of the disorder. The purpose is to replace the missing blood clotting factor so that the blood can clot normally and prevent complications. The hemophilia treatment includes:

  • Clotting factor concentrate infusion: There are two types of clotting factor concentrates are available:
  • Plasma-derived factor concentrate: The human plasma undergoes several processes to separate the clotting factors 8 and 9.
  • Recombinant factor concentrate: factor 8 and 9 concentrates are genetically-engineered via DNA technology. Recombinant factor concentrate does not contain any plasma or blood protein.
  • DDAVP (Desmopressin Acetate) medication: It is hormonal replacement therapy. Desmopressin hormone controls the release of factor 8 in our body. DDAVP medicine activate factor 8 release in patients’ body naturally. So, they do not need a factor 8 concentrate replacement to stop bleeding.
  • Cryoprecipitate: It is rich in factor 8 and used in severe bleeding.
  • Antifibrinolytic: These are clot-preserving medication that helps in stopping the clot from breaking down.
  • Physiotherapy: It can ease the discomfort and pain in joints that suffered damage from internal bleeding.

If you or your child has hemophilia, you need regular guidance from your doctor to manage the condition. But, if you notice any uncontrollable bleeding or unexpected large bruises, immediately contact your doctor.

On this Hemophilia Awareness Day, let’s raise awareness of hemophilia and increase equitable access to care and treatment.

Dr. Sarita Rani Jaiswal | Program Director – Haploidentical BMT & Consultant – BMT and Haematology | Dharamshila Narayana Superspeciality Hospital, Delhi

Narayana Health

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