Hemophilia
Short Description:
“Mild, moderate, or severe bleeding in response to trauma due to
clotting factor deficiency which impairs the formation of stable fibrin
clots.”
- Hereditary bleeding disorder with 2 forms resulting from different
clotting factor deficiencies.
- Patient has abnormal bleeding in response to any trauma because
of a deficiency of the specific clotting factor
- Platelet plugs are formed at the bleeding site, but deficiency
impairs the formation of stable fibrin clots
- Excessive bleeding may be mild, moderate, or severe depending on
the degree of factor deficiency
Signs and Symptoms:
- Excessive bleeding from minor cuts, bruises, or abrasions
- Joint and muscle hemorrhages that lead to disabling long-term
problems (may require joint replacement), especially hips and
knees
- Limited or painful range of motion
- Blood in urine and/or stool
- Tendency to bruise easily
- Prolonged and potentially fatal hemorrhage after surgery
Lab Values:
- Prolonged activated partial thromboplastin time (aPTT)
- Normal bleeding time
- Normal platelet count
- Normal prothrombin time (PT)
- Factor VIII or IX concentration is < 0.4 IU/mL
o Mild 0.06-0.4 IU/mL
o Moderate 0.01-0.05 IU/mL
, o Severe undetectable at < 0.01 IU/mL
(Schub & Oji, 2018)
Diagnostic Tests:
- Head or body CT scan or MRI – to assess for spontaneous or
traumatic hemorrhage (Schub & Oji, 2018)
- X-rays of extremities – may identify evidence of hemophiliac
arthropathy in the joint space or synovia (Schub & Oji, 2018)
Interventions:
- Hemophilia A:
o Regularly scheduled infusions of synthetic factor VIII
Cost may be prohibitive for many individuals with
hemophilia
o Or infusion of substance only when injury or bleeding occurs
o During infusion, monitor for reaction to clotting factor
(hives, flushing, fever, headache, tingling, and/or wheezing
- Antihemophilic drugs derived from pooled human serum are no
longer recommended because of the risk for transfusion-related
infections
Desired Outcome:
- Controlling/preventing bleeding
- Relieving pain
- Preserving mobility
- Promoting coping and good quality of life (QOL)
- Improving patient’s self-management skills to prevent bleeding
Patient Teaching:
- Aspirin and ibuprofen are contraindicated (Schub & Oji, 2018)
Short Description:
“Mild, moderate, or severe bleeding in response to trauma due to
clotting factor deficiency which impairs the formation of stable fibrin
clots.”
- Hereditary bleeding disorder with 2 forms resulting from different
clotting factor deficiencies.
- Patient has abnormal bleeding in response to any trauma because
of a deficiency of the specific clotting factor
- Platelet plugs are formed at the bleeding site, but deficiency
impairs the formation of stable fibrin clots
- Excessive bleeding may be mild, moderate, or severe depending on
the degree of factor deficiency
Signs and Symptoms:
- Excessive bleeding from minor cuts, bruises, or abrasions
- Joint and muscle hemorrhages that lead to disabling long-term
problems (may require joint replacement), especially hips and
knees
- Limited or painful range of motion
- Blood in urine and/or stool
- Tendency to bruise easily
- Prolonged and potentially fatal hemorrhage after surgery
Lab Values:
- Prolonged activated partial thromboplastin time (aPTT)
- Normal bleeding time
- Normal platelet count
- Normal prothrombin time (PT)
- Factor VIII or IX concentration is < 0.4 IU/mL
o Mild 0.06-0.4 IU/mL
o Moderate 0.01-0.05 IU/mL
, o Severe undetectable at < 0.01 IU/mL
(Schub & Oji, 2018)
Diagnostic Tests:
- Head or body CT scan or MRI – to assess for spontaneous or
traumatic hemorrhage (Schub & Oji, 2018)
- X-rays of extremities – may identify evidence of hemophiliac
arthropathy in the joint space or synovia (Schub & Oji, 2018)
Interventions:
- Hemophilia A:
o Regularly scheduled infusions of synthetic factor VIII
Cost may be prohibitive for many individuals with
hemophilia
o Or infusion of substance only when injury or bleeding occurs
o During infusion, monitor for reaction to clotting factor
(hives, flushing, fever, headache, tingling, and/or wheezing
- Antihemophilic drugs derived from pooled human serum are no
longer recommended because of the risk for transfusion-related
infections
Desired Outcome:
- Controlling/preventing bleeding
- Relieving pain
- Preserving mobility
- Promoting coping and good quality of life (QOL)
- Improving patient’s self-management skills to prevent bleeding
Patient Teaching:
- Aspirin and ibuprofen are contraindicated (Schub & Oji, 2018)
