Hemophilia is a bleeding disorder caused by deficiency and hereditary factors essential for blood coagulation (Wong, 2003).
Hemophilia is a congenital blood clotting disease caused by deficiency of blood clotting factors, ie factor VIII and factor IX. Factor VIII and factor IX is a plasma protein that is a component needed for blood clotting, these factors are required for fibrin clot formation in the area of trauma. (Hidayat, 2006).
Hemophilia is a congenital coagulation disorders the most frequent and serious. The disorder is associated with a deficiency of factor VIII, IX or XI is determined genetically (Nelson, 1999).
Hemophilia is a hereditary or acquired coagulation disorders are most common, manifest as intermittent episodes of bleeding (Price & Wilson, 2005)
Hemophilia there are 3 kinds:
- Hemophilia A: Disorders of the factor VIII (Anti - hemophilic factor)
- Hemophilia B: Disorders of the factor IX (Christmas factor)
- Van Willebrand disease
1. Infant (for diagnosis)
- Prolonged bleeding after circumcision
- Subcutaneous ecchymoses over the bumps of bone (at the age of 3-4 months)
- Large hematoma after infection
- Bleeding from the oral mucosa
- Soft tissue bleeding
- Early symptoms, including pain
- After the pain, the swelling, warmth, and decreased mobility
- Prolonged bleeding in the muscle can cause nerve compression and muscle fibrosis.
Pathophysiology of Hemophilia
Bleeding due to clotting disorder usually occurs in such a network that is located in muscles, joints, and other disorders because they can occur in the first, second and third, here the only disturbance will be discussed at the first stage, wherein the first stage is exactly what is the mechanism of interference freezing found in hemophilia A and B. Easy bleeding occurs in hemophilia, due to clotting disorder, at the start when a person is ± 3 months old or moments will begin to crawl the initial bleeding will occur due to minor injuries, followed by subsequent complaints.
Hemophilia can also cause cerebral hemorrhage, and fatal. Rationale is that when bleeding, there is a vascular injury (ie a channel where blood flows through the body) → blood out of the vessel. Blood vessels to shrink / shrank → Platelet (platelets) will close the wound on the vessel → Lack of a specific amount of blood clotting factors, resulting in wound closure webbing is not fully formed blood → did not stop flowing out → bleeding vessels (normal: blood clotting factors work to make webbing (fibrin strands) which will close the wound so that the blood stops flowing vessels).
Assessment and Physical Examination for Hemophilia
1. Assessment
- Family history of bleeding disorder
- Ask an unusual bleeding (bleeding that is difficult to stop a long time)
- Spontaneous bleeding (hemorrhage without trauma)
a. Activity
Symptoms: Muscle weakness
Symptoms: fatigue, malaise, inability to perform activities.
b. Circulation
Symptoms: skin, mucous membranes pale, cerebral nerve deficit / signs of cerebral hemorrhage
Symptoms: Palpitations
c. Elimination
Symptoms: Hematuria
d. Ego integrity
Symptoms: Depression, withdrawal, anxiety, anger.
Symptoms: Feelings of hopelessness and helplessness.
e. Nutrition
Symptoms: Anorexia, weight loss.
f. Painful
Mark:. Cautious behavior, anxiety, irritability.
Symptoms: Pain in the bones, joints, central tenderness, muscle cramps
g. Security
Signs: hematoma
Symptoms: mild trauma history.
- There was spontaneous bleeding in joints and muscles over and over accompanied by pain and swelling occurs.
- Recurrent joint bleeding caused by hemophilia Atropati give rise to joint space, bone crest and limited joint movement.
- Usually found in the Gastrointestinal bleeding also, excessive hematuria, and brain hemorrhage.
- There was hematoma at the extremities.
- Limitations and joint pain continued to hemorrhage
3. Psychology
- Assess the patient's self-concept à body image, roles, etc.
- Assess the patient and family understanding about the condition and action
- Assess the impact on lifestyle lung condition
Nursing Care Plan for Hemophilia
Nursing Management for Hemophilia
People with hemophilia should be aware of circumstances that can cause bleeding. They should really pay attention to teeth care to not have to undergo a tooth extraction. Rest of the body where there are injuries. When the leg is bleeding, use a tool such as a cane. Compressed injured body part and the surrounding area with ice or other soft material and frozen / cold. Press and tie, so the bleeding body part can not be moving (immobilization). Use an elastic bandage but keep in mind, do not press too hard and tie. Put these body parts in a higher position than the position of the chest and place it on a soft object like a pillow.
