Patients should be taught that literally hundreds of medications available over the counter contain aspirin. Lists of aspirin-containing compounds are available from a variety of sources including the National Hemophilia Foundation in New York. Our own general rule is that patients with hemophilia take no new medication of any sort until they have checked with the center.
Occasionally the relief of chronic pain in hemophilia will require the use of alternative therapies, such as an exercise program administered by a physical therapist, biofeedback, acupuncture or acupressure, transcutaneous nerve stimulation, or hypnosis. A risk with hypnosis is that the patient may neglect to pay attention to episodes of pain, which indicate new hemorrhage for which the patient should be seeking medical intervention.
In the last analysis the best way to control pain in hemophilia is to prevent it through the early and adequate application of replacement therapy and through the use of home infusion incorporated into a program of patient education and comprehensive hemophilia care.
Other Sites of Hemorrhage
Gastrointestinal hemorrhages, manifested by hematemesis, melena, or hematochezia, are unusual in hemophilia. They are usually caused by organic gastrointestinal lesions.
Prolonged gingival oozing is common after shedding of deciduous teeth, eruption of new teeth, or instrumentation. If such oozing is sufficiently prolonged or severe enough to require therapy, several days of e-aminocaproic acid (Amicar) therapy will usually suffice. Infusion of coagulation factor is only occasionally needed.
Epistaxis is not unusual in the severe hemophiliac, but it is unusual for mild hemophiliacs in the absence of a local nasal lesion. It is largely treated by local measures but may require factor infusion and cauterization.
Post-traumatic hemorrhage requires a special comment. Normal platelet plug formation may initially provide good hemostasis but delayed bleeding usually follows trauma. For this reason, many centers advocate treatment after significant trauma whether or not evidence of hemorrhage is yet apparent.
Therapy
General Considerations
The many ramifications of this lifelong expensive and crippling illness must be considered or the efficacy of treatment will be impaired and the outcome will be poor. Hemophilia societies are useful sources of paramedical support and information. Many problem areas exist, including the following: (1) interactional difficulties between hemophiliacs and the health care system 26; (2) psychological sequelae of overprotectiveness, such as the daredevil syndrome 27; (3) poor adjustment of the hemophilic child to school 28; (4) the enormous financial cost—in the range of $20,000–100,000/year for each patient depending on weight and severity; (5) important vocational problems in adults with established arthropathy 29; and finally, (6) a low level of education about hemophilia on the part of health care professionals, owing to the relative rarity of the disease and the pace of recent therapeutic advances.
Principles of Replacement Therapy for Factor VIII
The hemostatically effective plasma level for each coagulation factor is different and depends in part on the nature, extent, and duration of the bleeding lesion. The dose of replacement factor is calculated in units: 1 U is the activity of a given coagulation factor found in 1 ml of pooled, citrated fresh frozen human plasma. The factor must be given in sufficient quantity to allow for its clearance, metabolic half-life, and volume of distribution with the body.
The half-life of factor VIII in plasma is between 8 and 12 hours, which includes an initial rapid decline in level owing to diffusion into extravascular pools. 30 The minimum hemostatic level of factor VIII for relatively mild hemorrhages is 30% (0.30 U/ml of plasma), while that for advanced joint or muscle bleeding or for other major hemorrhagic lesions is 50% (0.50 U/ml). One to several days of maintenance therapy is needed for such advanced lesions to resolve. Resolution is achieved by repeating the infusion at 24-hour intervals at approximately 75% of the original dose. For life-threatening lesions or surgery, levels of 80%–100% (0.80–1.00 U/ml) should be achieved and the factor VIII level should be kept above the 30%–50% range by means of appropriate doses of factor VIII infused at intervals of 8–12 hours. 31 This more frequent infusion regimen decreases the incidence of excessively low levels just prior to an infusion and also decreases the total amount of factor needed to maintain given in vivo minimum plasma levels. Constant infusion regimens are another option when levels need to be maintained above a set minimum. MLID89333689 32
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