Question

Rubric Category Etiology Pathophysiology Signs and Symptoms Treatment Nursing Management APA and Grammar

Answer 1

Bleeding disorders are those disease in which there is excessive bleeding due to various causes like decreased production of platelets , increased destruction of platelets , missing of clotting factors .

1. Hemophilia:

A group of hereditary bleeding disorders that result from deficiencies of specific clotting disorders

Hemophilia is:

LIfelong, hereditary blood disorder, no cure, but can be treated and managed, until recently transfusion of replacement factors to prevent or stop bleeding is only treatment, transfusions increased risk of HIV

Hemophilia etiology:

1. X=linked autosomal recessive - 80%

2. Carrier females passed it on to affected male children

3. Males are affected

4. May silently carry gene for generations

5. Typically, there is a family hx of hemophilia. Up to 1/3 of cases have no known family hx, in these cases, diseases is caused by a new mutation

Types of Hemophilia: A

Classic hemophilia, deficiency of factor 8, accounts of 80% of cases in hemophilia, occurrence: 1 in 6000 males

Types of Hemophilia: B

Also known as Christmas disease, caused by deficiency of factor 9, accounts for 15% -20% of cases of hemophilia

Pathophysiology of hemophilia:

Normal sequence of blood clotting requires more than 10 factors. The two missing factors in hemophilia: Factor 8 or antihemophilic factor, factor 9 or plasma thromboplastic component. Normal levels of factor 8 and 9 are 50% to 150%.

Treatment

- replacement of blood products

- avoiding the causes

- administration of deficient clotting factor

Nursing Care: Management of bleeding in the hospital

■ Avoid taking temperature rectally.

■ Avoid unnecessary skin punctures and use surgical aseptic technique.

■ Apply pressure for 5 min after injections, venipuncture, or needle sticks.

■ Monitor urine, stool, and nasogastric fluid for occult blood.

2. Von willebrand disease

Hereditary bleeding disorder characterized by prolonged bleeding time, moderate deficiency of clotting factor VIII (antihemophilic factor), and impaired platelet function

Also known as angiohemophilia, pseudohemophilia, and vascular hemophilia

Inherited or acquired; several different types (see Classification of von Willebrand disease )

Pathophysiology :-

Mutations lead to impaired synthesis or action of von Willebrand factor (vWF).

A deficiency of vWF, a carrier that helps stabilize factor VIII and prevent it from degrading, leads to low levels of factor VIII.

A mild to moderate deficiency of factor VIII and defective platelet adhesion prolong coagulation time.

Defective platelet function is characterized by decreased agglutination and adhesion at the bleeding site, reduced platelet retention when filtered through a column of packed glass beads, and diminished ristocetin-induced platelet aggregation.

Etiology :-

Acquired form: Cancer (such as multiple myeloma, non-Hodgkin's lymphoma, chronic myelogenous leukemia, and Wilms' tumor), drugs (such as ciprofloxacin and valproic acid) and immune disorders (such as systemic lupus erythematosus, acquired form)

Inherited form (genetic): Gene located on chromosome 12 is inherited as an autosomal dominant trait (types 1 and 2) or autosomal recessive trait (type 3)

Treatment :-

Depends on the symptoms and underlying type of disease

Decreasing bleeding time by local measures

Avoidance of aspirin

Alternation of activities and rest periods if the patient is fatigued after a bleeding episode

No restrictions with mild disease; avoidance of contact sports with type 3 disease

Nursing Care: Management of bleeding in the hospital

■ Avoid taking temperature rectally.

■ Avoid unnecessary skin punctures and use surgical aseptic technique.

■ Apply pressure for 5 min after injections, venipuncture, or needle sticks.

■ Monitor urine, stool, and nasogastric fluid for occult blood.