Bleeding Disorders

A. Hemophilia – deficiency of one or more factors necessary for coagulation of

blood
a. Types of Hemophilia
i. Hemophilia A – Factor VIII deficiency (most common)
ii. Hemophilia B – Factor IX deficiency (Christmas disease)
iii. Von Willebrand disease – von Willebrand factor and factor VIII
deficiency – affects females & males
b. Transmission: X-linked recessive disorder
i. Affected father w/ carrier mother – 25% affected son or daughter,
25% carrier daughter, 25% normal son
ii. Affected father, normal mother – carrier daughters, normal sons
iii. Unaffected father, carrier mother – 25% carrier daughters, 25%
affected sons
c. Pathophysiology – Clotting factors are produced by the liver and are
necessary for blood clotting. Missing factors result in bleeding anywhere
in the body. Joints & muscles are most frequent areas of internal bleeding.
d. Diagnostic Evaluation:
i. History of bleeding episodes, family history
ii. Coagulation studies – PTT
iii. DNA testing, Clotting factor assays
e. Clinical Manifestations – page 997, box 26-6
f. Treatment:
i. Administer missing clotting factors
ii. Administer DDAVP (vasopressin)
iii. Blood transfusions for severe blood loss
iv. Corticosteroids for hematuria, hemarthrosis, chronic synovitis
v. NSAIDS for pain – use w/ caution as they inhibit platelet function
vi. Epsilon aminocaproic acid (amicar) – prevents clot destruction for
mouth or trauma surgery
vii. Activity – no contact sports, no active passive range of motion
viii. Prophylaxis for severe hemophilia – infusion of clotting factors on
a regular basis – 3 times a week
ix. When joint bleed occurs, infuse high dose of clotting factors
followed by 2 days of maintenance dose
x. Parents are taught to do venipuncture at home. Patient is taught to
do own venipuncture by 8 – 12 years of age.
g. Prognosis – no cure
i. Risk of HIV due to infusion of blood products during 1979 – 1985
h. Nursing Management:
i. Thorough assessment – esp headache, slurred speech, loss of
consciousness, black, tarry stools
ii. Teach how to prevent bleeding
1. Safe environment
2. Protective equipment
 3. Noncontact sports
4. Soft toothbrush
5. Medic-alert bracelet
6. Subc injections instead of IM injections
7. No ASA
iii. Recognize internal bleeding and to control external bleeding
1. R=rest
2. I = ice
3. C = compression
4. E = elevation
iv. Prevent crippling of jo9nts by immobilizing during bleeding phase.
Have patient exercise joints after bleeding stops
v. Sufficient diet and calories for normal growth
vi. Teach home care
vii. Support family and patient
viii. Encourage genetic counseling

B. Epistaxis – nosebleed
a. Causes –
i. Trauma – hit to nose, foreign body, picking nose
ii. Mucosal inflammation – allergy, URI
iii. Disease processes – deficient clotting factors, thrombocytopenia,
leukemia
iv. ASA use
b. Nursing Management:
i. Instruct child to sit up, lean forward and apply pressure to lower
part of nose
ii. For hemorrhage – pack nose w/ epinephrine-soaked gauze
1. Petroleum or water-soluble jelly to nose to prevent crusting
after bleeding stops
iii. Humidify child’s room
iv. Bleeding usually stops in 10 – 15 minutes. If lasts 30 minutes,
evaluate for bleeding disorder