Hemophilia
Definition
Hemophilia is an inherited bleeding disorder.
Hemophilia A & Hemophilia B : clinically indistinguishable.
Aetiology
Genetic defect inherited resulting in deficient or defective
Factor VIII - Type A
Factor IX - Type B (also called Christmas Disease
Both are X-linked
Almost all are males
Females are carriers - asymptomatic
Disease is recognized in early childhood
Clinical Manifestations
Haemorrhages into various parts of the body.
Even minimal trauma causes bleeding the severity of bleeding depending on the degree of deficiency.
Hemarthroses most common especially knee, elbow, ankle, shoulder, wrist, hip. Pain felt before swelling and limitation of movement.
Ankylosis may set in.
Hematomas superficial or deep - subcutaneous or intramuscular
Hematomas can compress nerves - leading to decreased sensation, weakness and atrophy of the area involved.
Hematuria
GIT bleeding
Epistaxis
Intra cranial or extra cranial bleeding.
Clot formation is poor
Wound healing poor.
Most common during dental extraction.
Medical Management
Factor VIII or Factor IX concentrates - used while actively bleeding and for preventive measure before traumatic procedures. - Recombinant forms available.
It is crucial to initiate treatment as soon as possible.
A few may develop antibodies and make treatment difficult and often unsuccessful.
Aminocaproic acid (EACA; Amicar) is a fibrinolytic enzyme inhibitor to slow the dissolution of blood clots.
Desmopressin - is useful in very mild forms of the disease - It transiently increases Factor VIII
Nursing Management
Childran taught to avoid trauma.
Avoid aspirin, NSAIDs, herbs, alcohol - medicines for cough containing alcohol
Dental hygiene
If not severe applying pressure - sufficient
Nasal packing avoided
Splint and orthoppedic devices to immobilize joints.
Avoid injections.
Invasive procedures to be minimized
Should carry medical identification (information)
Cold compresses to alleviate pain
Beware of HIV and hepatitis while treating.
Those with antibodies may require plasmapheresis or concurrent immunosuppressive therapy.
