Hemophilia

 Hemophilia

Haemophilia is inherited blood clotting disorder, caused by deficiency of coagulation factor Vlll Antihemophilic factor (AHF) or factor IX (Christmas factor).

Blood disorder in which blood doesn't clot normally because it lacks sufficient blood clotting protein (clotting factors). 

Types of Hemophilia

1. Haemophilia A or Classic haemophilia

Due to deficiency of clotting factor Vlll.

2. Hemophilia B or Christmas disease

Due to deficiency of clotting factor lX.

3. Hemophilia C

Occurs due to lack of factor XI.

4. Hageman's disease

Due to deficiency of factor XIl (Hageman factor).

5. Von Willebrand's factor

Low level of clotting protein in blood.

(Reduce level of von Willebrand's factor).

6. Acquired hemophilia

Autoimmune disorder.

Pathophysiology

Due to etiological factor

Inherited deficiency of factor Vlll or IX alters activation of intrinsic clothing pathway.

Show manifestation of prolonged bleeding or delay in clotting.

Children with hemophilia A or B.

Severity of manifestation is depend of factor deficiency.

Normal level of factor VIll; 60-140% and or factor lX; 60-145%.

Mild hemophilia; level of factor Vlll or lX; 6-30%.

Moderate hemophilia; level of factor Vlll or IX; 2-5%.

Severe hemophilia; level of factor Vlll or IX; 1% or less.

Normal clotting mechanism

1. Extrinsic factor

2. Intrinsic factor

Clinical features

1. Frequent bleeding episodes

2. Bleeding gums

3. Nose bleed (epistaxis)

4. Frequent bruises

5. Hematuria

6. Joint bleeding

7. Prolonged bleeding clothing time

8. Bleeding after injections or minor procedure

9. Spontaneous bleeding into joint cavities (hemarthrosis); elbow, knee, ankle joint 

Diagnostic Evaluation

1. History and physical examination 

2. Genetic history 

3. Laboratory test

a. aPTT test; Activated Partial Thromboplastin Clotting Time

b. CT and BT 

c. Normal PT time

4. CVS (chronic villus sampling/test)

5. Coagulation assay test

Identification of coagulation factor.

Management

No cure for Hemophilia.

Treatment done to stop or prevent bleeding.

1. Replacement therapy

Factor Vlll and IX

Not routinely used, used to prevent hemophilics from bleeding during invasive procedures.

2. Fresh frozen plasma (FFP)

To correct factor deficiency

3. Desmopressin

Drug used to treat hemophilia; increasing amount of clotting factor Vlll in child blood.

Nursing Management

1. Prevention of bleeding

Finger nail keep short, avoid scratching.

Provide soft toy to play child.

Given soft tooth brush to child.

Soft food given to child, to prevent gum bleeding.

2. Control of bleeding

First aid given during bleeding episodes.

R; Rest; make child lie down calmly untill bleeding stops.

I; Ice; apply ice pack on bleeding area.

C; Compression; apply pressure over bleeding site. 

Bleeding joint wrapped with elastic compression bandage.

E; Elevate; Position the child and elevate the bleeding area above the level of chest.

3. Administration of replacement therapy

Care of IV lines.

Use aseptic technique, prevent from infection.

Place cannual at Peripheral vein access.

If annual not working then Central venous line placed.

4. Education to patient and family

1. Education the parent regarding disease conditions.

2. Prevent the child from injury.

3. Provide safe environment for child and guide how to prevent bleeding episodes in children.

4. Child wear "Medic Alert Identification Card" when go outside, includes child's identity, blood group, and name of physician.

5. Genetic counseling

6. Screening test