(BLEEDER’S DISEASE).
Hemophilia is a hereditary condition manifested by a tendency to
uncontrollable hemorrhage with or without injury. The usual mode of
transmission is through the female line, rather than by the male. The
mother does not necessarily have to be a bleeder, but the daughter
of one, in order to transmit the disease to her offspring. Atavism
through the female alone is almost the rule. Not all the children of a
bleeding family are afflicted; the male children are more subject to
the condition than the female children. The tendency usually appears
within the first two years of life. The families of bleeders are often
large and are commonly healthy looking and have fine soft skins. It is
claimed blondes are most likely to be afflicted.
=Pathologically=, an unusual thinness of the blood-vessels with a
fatty degeneration of the intima has been noted. In many cases there
is deficient coagulability of the blood and a lessened number of
leucocytes. Hemorrhages have been found in and about the capsules
of the joints, and in a few instances inflammation of the synovial
surfaces. The arteries are situated superficially, but that does
not explain anything. The real nature of the disease has not been
determined. Emotional excitement is a factor, consequently vasomotor
disturbances may be important. The frailty of the blood-vessels and the
peculiar constitution of the blood preventing thrombotic formation are
the two facts of importance that have been recognized.
=Symptoms.=—Hemorrhages occur from the most trifling injuries. Blowing
the nose may cause severe epistaxis; the extraction of a tooth is a
frequent cause of hemorrhage; the prick of a pin, a slight cut, a
scratch, or a blow may result in profuse bleeding. The bleeding may
occur spontaneously from the mucous membrane of the mouth, nose,
lungs, intestines, etc.; or it may occur directly from the fingers,
toes, back of the hands, and lobes of the ears. The hemorrhages may
last several hours. As soon as checked the patients rapidly resume
natural appearance providing the bleeding is not often repeated,
thereby causing a permanent anemia. There may be attacks of arthritis
with fever, as with acquired hemorrhagic tendency, closely resembling
rheumatism.
=Diagnosis.=—Hereditary tendency and persistent hemorrhage from slight
injury.
=Prognosis.=—In a few cases the tendency to bleed gradually diminishes
until at last it entirely ceases. The younger the subject the more is
it liable to prove fatal. In the majority of cases death occurs between
the first and eighth years. After maturity the chances of an attack are
much lessened.
=Treatment.=—Members of the bleeder’s family, particularly the boys,
should be guarded against traumatic influences, and operations of
all kinds should be avoided. Outdoor exercise, fresh air, bathing and
plain nourishing food, in fact, the hygienic surroundings, and all food
should be carefully watched so that the threatened subject may become
strengthened and hardened. Marriage should be discouraged, especially
with the daughters, as it is through them the tendency is propagated.
Possibly, coupled with the foregoing prophylactic treatment, a
stimulation of the glands of elaboration of the blood will be of
service to build up the physical constitution of the patient. During
attacks absolute rest and the required symptomatic treatment should be
given. For resultant anemia the usual treatment is to be employed.
In severe cases direct transfusion should be considered.