Hemophilia seminar presentation in some point

Hemophilia is a rare inherited bleeding disorder in which the blood doesn’t clot properly due to the absence or dysfunction of certain clotting factors. This condition leads to prolonged bleeding after an injury, surgery, or even spontaneously, without a clear cause.

Types of Hemophilia

1. Hemophilia A: Caused by a deficiency in clotting factor VIII. It is the most common form.
2. Hemophilia B: Caused by a deficiency in clotting factor IX, also known as Christmas disease.
3. Hemophilia C: Caused by a deficiency in clotting factor XI. This type is less common and tends to have milder symptoms.

Genetics

• Hemophilia A and B are X-linked recessive disorders, meaning they predominantly affect males. Females can be carriers and might have mild symptoms, but rarely experience the severe bleeding typical of males.
• Hemophilia C is inherited differently and can affect both males and females.

Symptoms

• Prolonged bleeding: After injury, surgery, or dental procedures.
• Spontaneous bleeding: Without a clear cause, especially in joints (hemarthrosis), muscles, or soft tissues.
• Easy bruising: Even from minor bumps or falls.
• Joint pain and swelling: Repeated bleeding into joints can cause chronic joint damage.
• Internal bleeding: Can be life-threatening if it occurs in organs like the brain.

Diagnosis

• Blood tests: To measure the levels of clotting factors (factor VIII or IX) and assess clotting function.
• Family history: A key factor in identifying individuals at risk, especially for X-linked types.

Treatment

• Replacement therapy: Involves intravenous infusion of the missing clotting factor (either factor VIII for hemophilia A or factor IX for hemophilia B). This can be done on-demand (in response to bleeding) or prophylactically (to prevent bleeding episodes).
• Desmopressin (DDAVP): For mild hemophilia A, it can stimulate the release of stored factor VIII from the body.
• Gene therapy: A promising area of research, aiming to correct the underlying genetic defect to provide a long-term solution.
• Antifibrinolytics: Medications that help prevent clots from breaking down too quickly, used alongside other treatments.

Complications

• Inhibitors: Some patients develop antibodies (inhibitors) against the replacement clotting factors, making treatment less effective.
• Joint damage: Recurrent bleeding into joints can cause arthritis and reduced mobility.
• Internal bleeding: Serious cases can lead to damage to vital organs and require immediate medical attention.

Prognosis

With modern treatment, people with hemophilia can lead relatively normal lives. However, without proper management, severe cases can result in disability or life-threatening complications.

Lifestyle Considerations

• Patients with hemophilia are encouraged to avoid contact sports or activities that increase the risk of injury.
• Maintaining a healthy weight and regular exercise, especially low-impact activities, can help reduce joint strain and bleed

1. Introduction of hemophilia: Hemophilia is a common hereditary coagulation blood disorder due to deficiency or reduced activity of clotting factor V III or clotting factor IX. Hemophilia is a bleeding disorder that slow down the blood clotting process. People who have hemophilia often have longer bleeding time after some sort of contact to injury. People who have severe hemophilia start to have spontaneous bleeding in the joints and muscles all around their bodies. Hemophilia is more common in males than females.

2. Types of hemophilia : Hemophilia A : also called factor VIII deficiency or classic hemophilia is a genetic disorder caused by missing or defective factor VIII ( Antihemophilic factor) Hemophilia B : also called factor IX deficiency or (Christmas disease) is a genetic disorder caused by missing or defective factor IX Hemophilia c : genetic bleeding disorder caused by mutations in the factor XI (plasma thromboplastin antecedent)

3. Laboratory tests required: Complete blood count (CBC) This test measures the amount of hemoglobin (the red pigment inside red blood cells that carries oxygen), the size and number of red blood cells, and numbers of different types . (( hemoglobin normal range 12 -15 gm/dl (( Platelets count normal range from 150,000 to 450,000 )) Activated partial thromboplastin time (APTT) test : This test measures how long it takes for blood to clot. It measures the clotting ability of factors VIII (8), IX (9), XI (11), and XII (12).  (( Normal range 21 – 35 seconds )) Prothrombin time (PT) test : This test also measures the time it takes for blood to clot. It measures primarily the clotting ability of factors I (1), II (2), V (5), VII (7), and X (10). (( Normal range 10 - 13 second ))

4. The bleeding tendency can be aggravated by NSAIDS ,safer alternatives for pain control are acetaminophen ,codeine and cox-2 inhibitors. Infiltration, intraligamentary , intraosseuos or intrapulpal injections techniques are safer and preferred. Local anesthetic regional blocks , lingual infiltrations or injections into the the floor of the mouth must not be used in absence of factor VIII replacement because of the risk of hemorrhage hazarding the airway and being life-threatening. Anesthesia and pain management:

5. Dental Management of hemophilic patient: Patient past medical and family history should be taken Any complications during previous dental procedures such as prolonged healing and long bleeding periods should be taken into consideration Consult a dental surgeon and the patient’s haematologist before proceeding with any procedures In most cases of extractions and surgeries, procedures will have to be carried out in a hospital environment to allow the administration of appropriate cover and immediate post operative care

6. In case of post extraction hemorrhage : Contact the hemophilia unit. Inspect the site of bleeding , instruct the patient to sit up and bite on a damp gauze pack for at least 10 minutes. Use 10% tranexamic acid to dampen the swab or as a mouthwash if the bleeding is difficult to stop. Monitor the patient’s blood pressure as it may increase due to worry and pain.

7. Conclusion: Early dental care is of prime importance in such patients to avoid invasive procedures at a future date. A thorough understanding of the condition helps the dental professional to perform a systematic evaluation and anticipate potential hazards and critical changes mid-procedure. A protocol should be followed while treating these cases to continue with specialty dental procedures without major risks.

8. Thank you