Clinical Aspects of and Therapy for Hemophilia A. Incidence. Clinical Severity, страница 4

Hematuria    

Two-thirds of hemophiliacs will have had at least one episode of hematuria. 24 Most urinary bleeding is painless, but mild flank pain may be present, and occasionally severe renal colic occurs, the latter often associated with a clot in the ureter or renal pelvis.

Explanations of such bleeding vary from a trivial cause to significant underlying renal pathology. Hematuria is treated with increased fluid intake for several days and rest, followed by factor VIII for 2–4 days if the bleeding continues. Use of e-aminocaproic acid should be avoided because of the risk of preventing the lysis of clots that obstruct the ureter. Renal ultrasound or other studies are not done unless hematuria is chronic or recurrent or severe flank pain is present.

Intracranial Bleeding

Intracranial bleeding accounts for 25% of the hemorrhagic deaths in hemophiliacs. Antecedent trauma has occurred in one-half of such deaths. Bleeding may be subdural, epidural, subarachnoid, intracerebral, or (rarely) intraspinal. In one cooperative study of 2,500 hemophiliacs studied over 10 years, MLID78166239  25 71 episodes of central nervous system bleeding were documented; the mortality rate was 34%, and 47% of the survivors were left with mental retardation, seizure disorders, or motor impairment.

Survivors had been treated with sufficient clotting factor concentration to raise the factor VIII level to 30%–50% of normal for E10–14 days. Current regimens suggest maintaining levels approaching 100%.

Control of Pain in Hemophilia

Pain is an extremely common problem for many  persons with hemophilia. The two major causes are pain  due to pressure from hemorrhage into joints, muscles,  or other tissues and chronic arthritis pain, in which  permanent changes have occurred in the anatomy of the  joints. The control of pain is a major issue for people  with hemophilia, and extensive time and energy should  be put into training the patient in this regard. We believe  that several general rules are important.

Acute joint pain should always be assumed to be due  to bleeding. At the earliest symptom of joint discomfort  or limitation of motion, the correct therapy is correction  of the clotting factor defect. Early application of infusion  therapy prevents pain and long-term joint clotting factor  defect. Patients who are taught the general philosophy  “when in doubt, infuse” will wind up using less clotting  factor concentrate in the long run than those who adopt  a “wait and see” attitude. The latter group will have  advanced lesions requiring frequent treatment and higher  doses and will also develop chronic synovitis requiring  many days or weeks of treatment.

Chronic joint pain that fails to respond to infusion of  factor VIII or IX may sometimes be arthritic pain.  However, this determination should only be made after an  attempt has been made to correct the coagulation defect  for a period of days. If the pain that persists is  accompanied by stiffness and is accentuated early in the day, it is  more likely to be arthritic than hemorrhagic. In patients  whose pain has hemorrhagic characteristics and who do  not respond to correction of the coagulation factor  defect, the use of nonsteroidal anti-inflammatory agents  may provide considerable benefit. Aspirin must be  avoided because of its prolonged antiplatelet effect.      A major problem for most patients with hemophilia  prior to the availability of adequate means of correcting  the coagulopathy was narcotic addiction. Obviously, the  non-narcotic agents should be used whenever possible  when analgesics are required. More than 90% of patients  treated at our center never use narcotic pain medicines  and control their pain only with infusion therapy and  the use of acetaminophen. Among older adolescents and  adults with established hemarthrosis, the use of  nonsteroidal drugs for arthritic pain is helpful, and at any given  time between one-third and one-half of our patients in  this age group will be using nonsteroidal anti-inflamma tory agents.