Hemophilia.
Synonyms:—Hemorrhagic diathesis; bleeder's disease.
Definition:—An hereditary tendency to hemorrhage transmitted by the mother to the child, in which the mother herself may not exhibit the disease.
Etiology:—This is emphatically an hereditary disorder and has been traced back for centuries in the same family. In only about one case in eight or ten does the mother have the disease, but she transmits it to her sons, or through her daughters who may not show it, to her grandsons.
It depends upon imperfect, impaired or deficient coagulability of the blood, and probably upon other conditions of the blood, or of the blood vessels and their nervous control, which are not as yet determined. It manifests itself in infancy and in early life and often in those who are apparently in vigorous health and of unimpaired vitality.
Symptomatology:—Sudden, persistent bleeding, either spontaneous, from some very slight cut, abrasion or laceration, or from a tooth, where it is difficult of control, is the first evidence of the disease. Oozing from injured capillaries is a common form, when no blood vessel of size has been opened. A bruise from a direct blow, with no laceration, or a severe muscular strain may result in an extravasation of blood into the intercellular tissues, which may be extreme, and which may thus constitute a hematoma. Hemorrhages occur most frequently from the nose, as well as from other parts of the body, and joint affections are not uncommon. These consist of enlargement from hemorrhage into the joints, with tenderness, and ultimately there may be signs of local synovitis or other inflammation. There may be stiffness and soreness of the joints, resembling rheumatism, and finally more or less immobility.
The hemorrhages are most frequent in early life, and often severest in early childhood, but if restrained, will recur with less severity and less frequency as the child grows older, until the tendency with those who survive until adult life has greatly diminished, or in rare cases has disappeared entirely. More than one-half, however, of those who inherit the hemorrhagic tendency die from hemorrhage before they reach the age of six or seven years. Women may show no other tendency than profuse menstruation, but this is not often productive of serious results.
Diagnosis:—The diagnosis depends upon the knowledge of the inheritance of the diathesis and the occurrence of sudden and intractable hemorrhage.
Prognosis:—In distinctly marked cases the children will die early. In cases which do not develop early or with any degree of severity the condition may be controlled until the child passes the age of puberty, when there is a gradual decrease in the inclination, the improvement increasing with each added year of life.
Treatment:—These patients should be kept under close surveillance. They should be placed in early life, and keep themselves in later life, always under the observation and care of a physician who appreciates the condition and realizes the responsibility. They should be schooled in the fact that the existence of the inherited tendency is a constant menace to their life, but that with constant care, each year will bring increasing respite with decreasing liability. The infants should be taken through the period of dentition with great care. At no time should teeth be extracted, and they should be prevented from bruising, scratching or in any way breaking the skin. In adult life they should engage in quiet indoor employment or that in which there is no danger of physical injury; they should submit to no cutting operations of any character, unless absolutely necessary.
The treatment of an attack of hemorrhage should be the same as advised for other bad cases. The patient should be put to bed and kept quiet and free from agitation. Gallic acid, geranium, the compound tincture of erigeron and cinnamon, or ergot, may be used in sufficient doses. Iron should be administered and if there is a tendency to petechia or ecchymosis, arsenic should be given for quite a time in conjunction with the iron. In severe cases, the sub-sulphate of iron, or the acetate of lead, or other inorganic astringents may be necessary. The remarkable influence of hydrastis canadensis in large and frequently repeated doses at the onset, and during an attack, and in smaller doses in conjunction with other appropriate tonics for protracted periods between attacks, must be impressed upon each practitioner. From ten to twenty drops of colorless hydrastis, or fluid hydrastis, or a good fluid extract may be given every hour for three or four doses during the tendency to an attack, and the remedy may be continued to children, in from two to five drop doses, five or six times daily, for several weeks. Gelatin internally or by subcutaneous injection has exercised a frequent beneficial influence upon this disease.
