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Definition:
- from the Greek haima means
'blood' and philia 'love'
- a sex-linked trait bleeding
disorder (deficiency of one or
more clotting factors) affecting
sons and transmitted by symptom
free mothers.
- is a group of hereditary genetic
disorders that impair the body's
ability to control blood clotting
or coagulation, which is used to
stop bleeding when a blood
vessel is broken - Haemophilia A or classic
- Factor VIII (FVIII) is an hemophilia is a recessive X-linked genetic
essential blood clotting factor disorder involving a lack of functional
also known as anti-hemophilic clotting Factor VIII and
factor (AHF). represents 80% of haemophilia cases.
Etiology:
- Haemophilia B or Christmas
Hemphilia is an X-linked recessive disease is a recessive X-linked genetic
disorder transmitted by females and found disorder involving a lack of functional
predominantly in males. It may also caused clotting Factor IX. It comprises
by gene mutation. approximately 20% of haemophilia cases.
Clinical Manifestations:
Laboratory and diagnostic study findings 2. Concentrates of the clotting factor are
infused, or injected, directly into the
a. Coagulation studies will reveal bloodstream. The specific factors used to
normal prothrombin and bleeding times, treat hemophilia are: Factor VIII for
normal fibrinogen levels, low factor VIII in hemophilia A – Factor IX for hemophilia B
hemophilia A, low factor IX in hemophilia
B, and a prolonged partial thromboplastin 3. Replacement therapy can be used:
time.
a. To prevent bleeding (prophylactic
b. CBC will reveal a normal plate or preventive therapy)
count.
b. To stop bleeding when it occurs,
c. DNA testing for hemophilia A will on an as-needed basis (demand therapy)
detect carriers of the disease
4. The type of treatment you receive depends
d. Amniocentesis will diagnose on several things, including whether you
hemophilia prenatally. have mild, moderate or severe hemophilia.
Nursing Management: b. Recommend using a soft
toothbrush and point out the need for regular
1. Assess for acute or chronic bleeding dental checkups:
a. Assess home safety and teach c. Refer to the child and family to
about injury prevention. Consider the child’s support groups such as the national
developmental level to ask specific safety hemophilia Foundation.
questions.
6. Provide the child and family teaching.
a. Explain how to care for, (vasoconstriction), epistaxis: sit
administer, store and reconstitute the up and lean slightly forward and
replacement factor. systematic measure:
administration of anthemophillic
b. Inform the child and family that factor.
superficial injuries are treated with ice and
pressure. 3. Prevent Joint Degeneration
Nursing Goals: