Dr. Ria Maslog
Medical insider
Classic Hemophilia
Hemophilia A or Factor VIII deficiency is the most common
among the hemophilias. It constitutes 80% of cases and it
is caused by a defective gene carried on the X
chromosome.
To review, hemophilias are the most common and serious
of the congenital coagulation disorders.
In this case, it is the mother who is the carrier of the
disorder and will manifest this to her affected son.
Because Factor VIII does not cross the placenta, a
bleeding tendency may be evident in the neonatal period.
Clinically, the affected son will have hematomas after
injections and bleeding after circumcision. As he grows
older, because of mobility, there will appear some
bruising.
Clinically, affected individuals will also show hemarthrosis.
These are hemorrhages in the elbows, knees and ankles
and that may cause pain and swelling thus resulting to
limitation of movement of the affected joint.
Repeated hemorrhages may result to degenerative
changes, osteoporosis, muscle atrophy and a fixed
unusable joint.
Other patients will also have spontaneous blood in the
urine and intracranial hemorrhages.
These children with hemophilia A are referred to and
managed by a pediatric hematologists for the factor 8
transfusions and other blood transfusions.
However, at home, parents and caregivers are being
advised to protect babies against trauma. Their crib and
playpen should be padded and they should be carefully
supervised while learning to walk.
As they grow older, they should be warned against
physical activities that have risks for trauma. Since they
will be receiving blood transfusions, it should be given
attention that these children should be immunized against
hepatitis B virus.