       Document 0158
 DOCN  M95A0158
 TI    Hemophilia and von Willebrand's disease: 1. Diagnosis, comprehensive
       care and assessment. Association of Hemophilia Clinic Directors of
       Canada.
 DT    9510
 SO    Can Med Assoc J. 1995 Jul 1;153(1):19-25. Unique Identifier : AIDSLINE
       MED/95316809
 AB    OBJECTIVE: To present current strategies for the assessment and
       comprehensive care of patients with hemophilia and von Willebrand's
       disease. OPTIONS: Hospital care, home care, single-provider care and
       multidisciplinary care. OUTCOMES: Morbidity and quality of life
       associated with bleeding and treatment. EVIDENCE: Relevant clinical
       studies and reports published from 1974 to 1994 were examined. A search
       was conducted of own reprint files, MEDLINE, citations in the articles
       reviewed and references provided by colleagues. In the MEDLINE search
       the following terms were used singly or in combination: hemophilia, von
       Willebrand's disease, Factor VIII, Factor IX, von Willebrand factor,
       diagnosis, management, home care, comprehensive care, inhibitor, AIDS,
       hepatitis, life expectancy, complications, practice guidelines,
       consensus statement and controlled trial. The in-depth review included
       only articles written in English from North America and Europe that were
       relevant to human disease and to a predetermined outline. The
       availability of treatment products in Canada was also considered.
       VALUES: Minimizing morbidity and maximizing functional status and
       quality of life were given a high value. BENEFITS, HARMS AND COSTS: The
       optimal use of treatment procedures and home care offers patients the
       advantages of minimized disability, improved survival and financial
       benefit. It is also cost effective. Potential harm, including the risk
       of hepatitis B, hepatitis C and HIV infection, has now been minimized
       through viral inactivation of plasma-derived coagulation-factor
       concentrates and through the use of recombinant clotting factor
       concentrates and other non-plasma-derived hemostatic agents.
       RECOMMENDATIONS: Patients with hemophilia and severe von Willebrand's
       disease should be followed in comprehensive care centres that offer
       expertise in the diagnosis, assessment and management of bleeding and
       complications and that can meet the educational and counselling needs of
       patients, family members and health care providers. Eligible patients
       should be enrolled in a home self-infusion program. Patients with
       hemophilia and von Willebrand's disease should wear or carry Medic Alert
       identification. They should be vaccinated against hepatitis B and attend
       for routine follow-up examinations. Laboratory testing should be carried
       out as required, and dental and surgical care should be undertaken in
       consultation with a hematologist. VALIDATION: These recommendations were
       reviewed and approved by the Association of Hemophilia Clinic Directors
       of Canada (AHCDC) and the Medical and Scientific Advisory Committee of
       the Canadian Hemophilia Society. No similar consensus statements or
       practice guidelines are available for comparison. SPONSORS: These
       recommendations were developed at the request of the Canadian Blood
       Agency, which funds the provision of all coagulation-factor concentrates
       for people with congenital bleeding disorders, and were developed and
       endorsed by the AHCDC and the Medical and Scientific Advisory Committee
       of the Canadian Hemophilia Society.
 DE    von Willebrand's Disease/*DIAGNOSIS/*THERAPY  Activities of Daily Living
       Comprehensive Health Care/*ORGANIZATION & ADMIN
       Hemophilia/*DIAGNOSIS/*THERAPY  Human  Quality of Life  Treatment
       Outcome  GUIDELINE  JOURNAL ARTICLE  PRACTICE GUIDELINE  REVIEW  REVIEW,
       TUTORIAL

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

