Hemophilia
-By Dr. Rima Jani PT
(MPT in Pediatric Science)
What is Hemophilia?

An incurable hereditary bleeding disorder due to absence or deficiency of
clotting factors in the blood.

Basically the process of hemostasis is impaired.

Means the process of stopping the flow of blood is impaired.

Repeated Spontaneous bleeds inside the joint and muscles of the patient, if
not treated promptly lead to permanent disability.
TYPES

Hemophilia A
• Most common type
• Caused by lack of clotting factor VIII

Hemophilia B
• Caused by lack of clotting factor IX

Hemophilia C
• Caused by lack of clotting factor XI
Genetics
Causes

Hemophilia (Inherited)
o
Hemophilia A, B and C

Hemophilia (Acquired)
o
Liver Failure (Liver synthesis I, II, V, VII, VIII, I, X, XI, XIII)
o
Vitamin K deficiency (Liver needs Vit. K to synthesis and release factors II, VII,IX and X)
o
Autoimmunity against a clotting factor.
o
Disseminated Intravascular coagulation which consumes clotting factors.
Classification based on severity

Mild
• Level is 5-50%
• Only occasional bleeding usually related to significant Trauma or
Surgery.

Moderate
• Level is 1-5%
• Spontaneous bleeding is uncommon but occur after minor Trauma
or Surgery.

Severe
• Level is <1% • Spontaneous joint and soft tissue bleeding several
times several months.
Signs and Symptoms

Easy bruising (Ecchymosis)

Hematomas (collection of blood outside of blood vessels often in muscles)

Prolonged bleeding after cut or surgery

Oozing after tooth extraction

Gastrointestinal bleeding

Hematuria (Blood in urine)

Severe nose bleeds

Hemarthrosis (Bleeding in joint spaces)

Severe complication can occur because of bleeding into brain (Stroke or
Increased Intracranial pressure)
Common areas affected

Joint bleeds are most common
• Knees • Ankles • Elbows
• Joints of the hands are not usually affected except after injury.

Muscles affected by Hemophilic bleeding
• Biceps brachii
• Forearm flexors
• Iliopsoas (hip flexor)
• Quadriceps
• Hamstrings
• Calf muscles
Clinical Features

Early
 Late
•Difficulty in movement
•Tingling sensation
•Pain with movement
•Feeling of warmth
•Tightness or swelling
•Numbness or Tingling feeling
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Diagnosis

Lab Test

Platelets (Normal)

Prothrombin Time (normal)
Tests Extrinsic and Common pathways (VII,X,V,II,I)

Partial Thromboplastic Time (Increases)
Tests Intrinsic and common pathways (XII,XI, IX,VIII, X, V, II, I)

Confirmatory Tests

Tests for special factor activities and

Mutation testing
Treatment

Replacing the missing clotting factor in the blood.

Whole blood
• Plasma • Cryoprecipitate • Factor concentrate

Physiotherapy
• Therapeutic exercise • Electro therapy
Goals of Physiotherapy

Pain Relief

Reduce Muscle spasm

Soften contracted tissue

To increase or maintain muscle power

To increase proprioception
In Subacute Stage

Isometric exercise

Active assisted

Active free exercise as pain allows
In Chronic Stage

Mobilization exercises according to ROM

Strengthening exercises

Self stretching

Ultrasound Therapy

Hydrotherapy
Lifestyle modification

Exercise regularly prescribed by the Physical Therapist.

Balanced diet to maintain weight and prevent obesity.

Practice good dental hygiene.

Parent counselling:
o
Protect your child from injuries that could cause bleeding.
o
Do not participate in contact sports.

Avoid certain medication—
o
o
Some drugs can aggravate bleeding such as “Aspirin”, “Ibuprofen.”
Some drugs prevent blood from clotting such as “Heparin”, ”Warfarin.”
THANK YOU