HEMOPHILIA

1. Mr. HIREN GEHLOTH
JG COLLEGE OF NURSING

5. • Hemophilia is usually an inherited bleeding disorder in which the blood
does not clot properly. This can lead to spontaneous bleeding as well as
bleeding following injuries or surgery. Blood contains many proteins called
clotting factors that can help to stop bleeding.
• People with hemophilia have low levels of either factor VIII (8) or factor IX
(9). The severity of hemophilia that a person has is determined by the
amount of factor in the blood. The lower the amount of the factor, the more
likely it is that bleeding will occur which can lead to serious health
problems.

6. • In rare cases, a person can develop hemophilia later in life.
• The majority of cases involve middle-aged or elderly people, or young
women who have recently given birth or are in the later stages of
pregnancy.
• This condition often resolves with appropriate treatment.

7. • Hemophilia is caused by a mutation or change, in one of the genes, that provides
instructions for making the clotting factor proteins needed to form a blood clot.
This change or mutation can prevent the clotting protein from working properly or
to be missing altogether. These genes are located on the X chromosome.
• Males have one X and one Y chromosome (XY) and females have two X
chromosomes (XX). Males inherit the X chromosome from their mothers and the
Y chromosome from their fathers. Females inherit one X chromosome from each
parent.
CAUSES:

9. • There are two main types of inherited hemophilia:
• Type A, the most common type, is caused by a deficiency of factor
VIII, one of the proteins that helps blood to form clots. This type is
known as classic hemophilia.
• Type B hemophilia is caused by a deficiency of factor IX. This type is
also called Christmas disease.
TYPES:

10. • Although hemophilia is usually diagnosed at birth, the disorder
can also be acquired later in life if the body begins to produce
antibodies that attack and destroy clotting factors.
• However, this acquired type of hemophilia is very rare.
• Acquired hemophilia is also called autoimmune hemophilia, or
acquired hemophilia A (AHA).

11. Clinical manifestation
• Hemorrhage occurs into various body parts (large, spreading bruises and
bleeding into muscles, joints, and soft tissues) after even minimal trauma.
• Most bleeding occurs in joints (most often in knees, elbows, ankles,
shoulders, wrists, and hips); pain in joints may occur before swelling and
limitation of motion are apparent.
• Chronic pain or ankylosis (fixation) of the
joint may occur with recurrent hemorrhage;
many patients are crippled
by joint damage before
adulthood.

12. • Spontaneous hematuria and gastrointestinal
bleeding can occur. Hematomas within the
muscle can cause peripheral nerve compression
with decreased sensation, weakness, and
atrophy of the area.
• Surgical procedures typically result in
excessive bleeding at the surgical site; bleeding
is most commonly associated with
dental extraction.

13. ASSESSMENT AND DIAGNOSTIC
EVALUATION
Laboratory tests include clotting factor measurement and
CBC count.

14. MEDICAL MANGEMENT
• Factors VIII and IX concentrates are given when active bleeding occurs or
as a preventive measure before traumatic procedures (eg, lumbar puncture,
dental extraction, surgery).
• Plasmapheresis or concurrent immunosuppressive therapy may be required
for patients who develop antibodies (inhibitors) to factor concentrates.
• Aminocaproic acid may slow the dissolution of blood clots; desmopressin
acetate (DDAVP) induces transient increase in factor VIII.
• Desmopressin is useful for patients with mild forms of hemophilia A.

15. • Assist family and patient in coping with the condition because it is chronic,
places restrictions on their lives, and is an inherited disorder that can be passed
to future generations.
• From childhood, help patients to cope with the disease and to identify the
positive aspects of their lives.
• Encourage patients to be self-sufficient and to maintain independence by
preventing unnecessary trauma.
• Patients with mild factor deficiency that were not diagnosed until adulthood need
extensive teaching about activity restrictions and self-care measures to diminish
the chance of hemorrhage and complications of bleeding; emphasize safety at
home and in the workplace.
NURSING MANGEMENT

16. • Instruct patients to avoid any agents that interfere with platelet aggregation,
such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), herbs,
nutritional supplements, and alcohol. (Also applies to over-the-counter
medications such as cold remedies.)
• Promote good dental hygiene as a preventive measure because dental
extractions are hazardous.
• Instruct patient that applying pressure to a minor wound may be sufficient to
control bleeding if the factor deficiency is not severe; avoid nasal packing.
• Splints and other orthopedic devices may be useful in patients with joint or
muscle hemorrhages.
• Avoid all injections; minimize invasive procedures (eg,endoscopy, lumbar
puncture) or perform after administration of appropriate factor replacement.

17. • Carefully assess bleeding during hemorrhagic episodes; patients at risk for
significant compromise (eg, bleeding into the respiratory tract or brain) warrant close
observation and systematic assessment for emergent complications (eg, respiratory
distress, altered LOC).
• If patient has had recent surgery, frequently and carefully assess the surgical site for
bleeding; frequent monitoring of vital signs is needed until the nurse is certain that
there is no excessive postoperative bleeding.
• Administer analgesics as required; allow warm baths but avoid during bleeding
episodes.
• Patients who have been exposed to infections (eg, HIV infection, hepatitis) through
previous transfusions may need assistance in coping with the diagnosis and
consequences.

18. • Recommend genetic testing and counseling to female carriers so that
they can make informed decisions regarding having children and
managing pregnancy.