DISORDERS OF COAGULATION
Hemophilia
Etiology/Pathophysiology
Hemophilia is a hereditary bleeding disorder caused by defec-tive or deficient coagulation
factors. The two major forms of hemophilia which can occur is mild to severe forms are
hemophilia A (Classic hemophilia, factor VIII deficiency) and hemophilia B (Christmas disease,
factor IX deficiency). The disorder termed “von Willebrand’sdisease” is a related disorder
involving a congenitally acquired deficiency of von Willebrand compilation proteins. Factor VIII
is synthesized in the liver and circulates complexed to von Willebrand’s protein (VWP). And one
more hemophilia C (Factor XI deficiency). Hemophilias are inherited as sex-linked recessive
disorders and are therefore almost exclusively limited to males. The incidence of hemophilia A
(1:10,000) hemophilia B (1:100,000) of the male population.
Hemophilia C is rare with incidence 2 to 3 percent.
The inherent pattern of these hemophilion are as follows:
• Hemophilia A: Recessive sex-linked (transmitted by female carriers, displayed almost
exclusively in men).
• Hemophilia B: Recessive sex-linked (transmitted by female carriers, displayed almost
exclusively in men).
• von Willebrand disease: (VWP dysfunction) Autosomal dominant, seen in both sexes,
recessive (in severe form of the disease).
Clinical Manifestation
Clinical manifestation and complications related to hemophilia include:
• Slow, persistent, prolonged bleeding from minor trauma and small cuts.
• Delayed bleeding after minor injuries (the delay may be for several hours or days).
• Uncontrollable hemorrhage after dental extractions or irritation of gingiva with a hard-bristle
tooth brush.
• Epistaxis, especially after blow to the face.
• GI bleeding from ulcers and gastritis.
• Hematuria from GU trauma and splenic rupture resulting from fall or abdominal trauma.
• Ecchymoses and subcutaneous hematomas (common).
• Neurologic signs, such as pain, anesthesia and paralysis which may develop from nerve
compression caused by hematoma formation and
• Hemarthrosis (bleeding from joints) which may lead to joint deformity severe enough to cause
unresolvable crippling (most commonly in knees, elbows, shoulders, hips and ankles).
Life-threatening bleeding involves retroperitoneal, intracranial soft tissue hemorrhages.
Management
A diagnosis of haemophila is made by specific assays for factors VIII, IX and XI. The partial
thromboplastotime (PTT) which reflects the intrinsic pathway of coagulation, is prolonged in
hemophilia A, hemophilia B and hemophilia C. The platelet count and prothrombic time is
normal.
Treatment
Treatment is replacement of the deficient coagulation factor. When bleeding episodes do not
respond to local treatment, i.e., ice bags, manual pressure or dressings, immobilization, elevation,
or topical coagulate such as fibrin foam and thrombin.
Fresh frozen plasma once commonly used for replacement therapy is rarely used now.
Cryoprecipitate which primarily contains factor VIII and fibrinogen is prepared for plasma,
frozen rapidly and kept frozen until used. Before administration, the cryoprecipitate is thawed
slowly.
The standard therapeutic products, i.e. concentrate factor used in treating hemophilia today are:
1. Factor VIII
• Plasma derived products: Monoclate, Hemofil, Profilate, Huma
• Recombination products: Recombinates, Kogenate,
2. Factor IX
• Plasma-derived products: Alpha-Nine, Mononine, Ronyne, Profilinine.
• Recombination products: Bebulin, autoplex, FEIBA and Hyate.
Nursing intervention
Nursing interventions are related primarily to controlling bleeding and include the following.
• Stop the topical bleeding as quickly as possible by applying direct pressure or ice packing the
area with Gelfoam or fibrin foam, and applying topical hemostatic agents such as thrombin.
• Administer the specific coagulation factor concentrate ordered to raise the patient’s level of the
deficient coagulation factor.
• When joint bleeding occurs it is important to totally rest the involved joint, in addition to
administering antihemophilitic factors to help prevent crippling deformities from hemarthrosis.
The joint may be packed in ice, analgesics are given to reduce joint pain. However, aspirin and
aspirin containing compounds should never be used.
As soon as bleeding ceases, it is important to ROM exercises and physical therapy. Actual
weight
bearing is avoided until all swellings has resolved and muscle strength has returned.
• Manage any life-threatening complication that may develop as a result of hemorrhage. Example
includes nursing intervention to prevent or treat airway obstruction from hemorrhage into the
neck and pharynic, as well as early assessment and treatment of intracranial bleeding.
In addition, the nurse must provide ongoing assessment of the patient’s adaptation to the illness.
Psychosocial support and assistance should be readily available as needed. Most of the long
terms are related to patient education. The patients with hemophilia must be taught to recognize
disease-related problems and to learn which problem can be resolved or borne and which require
hospitalization.
Immediate medical attention is required for severe pain or swelling of a muscle or joint that
restricts movement or inhibits sleep and for a head injury, a swelling in the neck or mouth,
abdominal pain, hematuria, melena and skin wound needed for suturing. Daily oral hygiene must
be performed without causing trauma. There are many potential sources of trauma. The patient
can learn to prevent trauma by using gloves whenever needed to prevent cuts or abrasion from
knives, hammer and other tools.
The patient should wear a medic alert tag to ensure that health care providers know about the
hemophilia in case of an accident.
Since hemophilia is hereditary, genetic counselling is necessary as preventive measures if
needed.
