Hemophilia

 Definition of Hemophilia

Hemophilia is a group of x-linked recessive disorder that result in deficiency in one of the coagulation factors in the blood.

X-linked recessive disorders are transmitted by carrier mothers to their son, so usually only males are affected by hemophilia.

Coagulation factors in the blood are essential for clot formation either from spontaneously or from injury, when factor are absent bleeding will be difficult to stop.

Types of Hemophilia

there are several types of hemophilia, including:

1- Factor VIII deficiency (hemophilia A).

2- Factor IX deficiency or Christmas disease (hemophilia B).

3- Factor XI deficiency (hemophilia C).

 Most common type is hemophilia A, occurs when there is a deficiency of factor VIII in an individual.

Factor VIII is essential in activation of factor x, which required for the conversion of prothrombin into thrombin resulting inability of the platelets to be used in clot formation.

Hemophilia classified to the severity of disease (mild to sever) as picture.

 Clinical manifestation

 Prolong bleeding any where from the body.

Subcutaneous or intramuscular hemorrhage is common.

hemarthrosis (bleeding into joint cavities especially knee, ankle, elbow) as picture.

Bleeding into tissue serious if occur into neck, mouth, or thorax. 

Hemorrhage any where in GI tract, can lead to obstruction.

 Epistaxis (bleeding from the nose).

 Diagnosis

- History of bleeding.

- Chromosomal studies.

- Blood test, bleeding time, clothing time, PTT etc.

Complications

Complications may be both directly from the disease or from its treatment:

- Deep internal bleeding, e.g. deep-muscle bleeding, leading to swelling, numbness or pain of a limb.

- Joint damage from hemoartharosis.

- Transfusion transmitted infection from blood transfusions that are given as treatment.

- Adverse reactions to clotting factor treatment.

- Intracranial hemorrhage is a serious medical emergency caused by the buildup of pressure inside the skull. It can cause disorientation, nausea, loss of consciousness, brain damage, and death.

Therapeutic and nursing management

 1- Primary therapy is preventing spontaneous bleeding by replacement of missing factor. Cryoprecipitate (concentrated from of AHF anti hemophilia factor).

2- If bleeding in the joint :

- Apply ice on the area.

- Immobilize in normal.

- Anatomical position.

- Teaching family and patient how to administer I.V cryoprecipitate.

 3. If bleeding occurs from a wound:

- apply pressure for 10 – 15 minute.

- Immobilize and elevate the area above the level of the heart.

- Apply cold compresses to promote vasoconstriction.

- RICE (rest, ice, compression and elevation) to control bleeding.

4. If bleeding doesn't stop in 15 minutes factor must be replace I.V.

-  Preventing bleeding by decrease risk of injury

- Safe environment at home.

- Dental care as soft toothbrush with water irrigation for mouth.

- Diet to prevent excessive weight.

- Use of electrical shaving machine instead of blades.

- Use small needle for IM injection and put direct pressure over site for 5 minute.

- Aspirin for sedation is contraindicate.