Therapeutic Management of Hemophilia Especially in Children

The primary therapy for hemophilia is preventingas safe as possible to minimize the incidental
spontaneous bleeding by replacement of theinjuries and maintaining close supervision during
missing factor.playtime.
Vigorous therapy is instituted to prevent chronicFor older children, the family usually needs
crippling effects, from joint bleeding. Ifassistance in preparing for school. A nurse who
replacement therapy is begun immediately, localknows the family can be instrumental in discussing
measures such as Ice applications and splinting arethe situation with the school nurse and in jointly
seldom needed. Local application of epsilonplanning an appropriate schedule of activity. Since
aminocapronic acid prevents clot destruction;almost all hemophiliac persons are boys. The
however, its use is limited to mouth or traumaphysical limitations in regard to active sports are a
surgery.difficult adjustment. Encouraging the pursuit of
A regular program of exercise and physicalintellectual and/or creative endeavors from early
therapy is an important aspect of management.childhood helps foster a life-style that is fulfilling
Treatment without delay results in more aspectand less conflicting than one focused on unrealistic
of management. Treatment without delay resultsgoals. Using the group approach with hemophiliac
in more rapid recovery and a decreased likelihoodchildren is often very successful because within
of complications; therefore, most hemophiliacthe group, they can find acceptance, healthy
children over 3 years of age. The child learns thecompetition, and a sense of camaraderie. Such a
procedure for self-administration between ages 9setting may be the first time that these children
and 12. Home treatment is highly successful, andhave felt a sense of belonging; even with their
the rewards, in addition to the immediacy ofsibling relationships, they commonly see
treatment, are less disruption of family life, fewerthemselves as outsiders.
school of work days missed, and enhancement ofSince the mucous membranes bleed easily, the
the child's independence.young child is given food that is easily masticated.
Nursing considerationsFor the older child, food choices need not be
The objectives for nursing care can be dividedlimited as long as he understands the necessity of
into immediate meeds and long-term goals.chewing thoroughly and eating slowly. Dental care
Obviously the most immediate consideration isis very important and may require some
control of bleeding episodes. However, thereadjustment in terms of daily hygiene. For
ultimate adjustment and prognosis for theexample, the nurse can recommend the use of a
hemophiliac person rely heavily on the family'sWater Pik, softening the toothbrush in warm
ability to cope with the disorder, to providewater before brushing, or using a sponge-tipped
effective methods of control and prevention, anddisposable toothbrush available in many
t temper child-rearing practices with judiciousdrugstores. A regular toothbrush should be soft
protection from injury while fosteringbristled and small in size. Adolescents also need to
independence and development.be advised of the dangers of shaving with razor
Prevent bleeding by decreasing risk of injuryblade and encouraged to use an electric shaver.
Prevention of bleeding through control of behaviorDiet is also an important consideration, since
is no easy task. During infancy and 'toddler-hood'excessive body weight can increase the strain on
the normal acquisition of motor skills createsaffected joints, especially the knees and
innumerable opportunities for falls, bruises andpredispose to hemarthrosis. Since limited activity is
minor wounds. Restraining the child fromfrequently the result of a more sedentary
mastering motor development can herald morelife-style and occasional periods of bed rest aster
serious long-term problems than allowing thetissue bleeding, calories need to be supplied in
behavior. However, the environment can be madeaccordance with energy requirements.