Hemophilia Blood Disorder

Hemophilia

• A rare, genetic blood disorder where blood doesn't clot and bleeding doesn't slow down or stop due to low clotting factors.
• Three main types: Hemophilia A, Hemophilia B, and Hemophilia C, each caused by a deficiency of a specific clotting factor (factor VIII, factor IX, and factor XI, respectively).

Inheritance Patterns

• Hemophilia A and B are X-linked recessive diseases, where males are more affected because they only need one mutated gene to show symptoms.
• Hemophilia C is an autosomal recessive disease, where both males and females are equally affected and need two mutated genes to show symptoms.

Symptoms

• Prolonged bleeding, sudden bloody noses, bleeding of the mouth and gums, oozing after tooth extractions, bleeding in the urine or stool, easy bruising, joint bleeding (hemarthrosis), and bleeding into the brain.
• Severity of symptoms depends on the severity of the mutation.

Severity and Clotting Factor Levels

• Hemophilia can be categorized as mild, moderate, or severe based on the amount of clotting factors in the blood.
• Clotting factor levels:
  • Severe hemophilia: less than 1% of normal clotting factors.
  • Moderate hemophilia: 1% to 5% of normal clotting factors.
  • Mild hemophilia: 5% to 30% of normal clotting factors.

Diagnosis

• Healthcare provider will start with a complete history and physical examination.
• Tests may include:
  • Complete blood count (CBC).
  • Prothrombin time test (PT).
  • Specific clotting factor test.
  • Genetic or DNA testing.

Epidemiology

• Hemophilia affects patients worldwide, with varying prevalence rates in different regions.

Treatment

• Currently, there is no cure for hemophilia.
• Main treatment is Protein Replacement Therapy, which involves infusing clotting factor concentrates (factor VIII for Hemophilia A or factor IX for Hemophilia B) into a vein to replace missing or low clotting factors.
• Additional treatments may include fresh frozen plasma and medications to prevent clotting factor breakdown.

Oral Manifestations and Dental Management

• Symptoms in the oral cavity include excessive gingival bleeding, high rate of infections, joint bleeds, and orofacial pain.
• Dental management involves:
  • Obtaining medical history and communicating with hematologists.
  • Avoiding certain pain medications that can exacerbate bleeding.
  • Intensive treatments, such as deep cleaning and tartar removal, should be carried out in hospitals or community dental services.
  • Maintaining good oral health through regular dental visits, brushing, and interdental cleaning aids.