Home treatment for patients with congenital bleeding disorders in a developing country

• Main Authors: Ampaiwan Chuansumrit, Parttraporn Isarangkura, Aroonrat Chantanakajornfung, Wanpen Panthangkool, Phongjan Hathirat
• Other Authors: Mahidol University
• Format: Article
• Published: 2018
• Subjects: Medicine
• Online Access: https://repository.li.mahidol.ac.th/handle/123456789/25561

Ninety-six patients with congenital bleeding disorders were enrolled in a home treatment program from 1979 to 1997. The diagnosis included severe and moderate hemophilia A and B (n = 63), mild hemophilia A (n =18), von Willebrand disease (vWD) (n = 12) and congenital factor VII deficiency (n = 3). The median age was 9 years and the median duration of follow-up was 4 3/12 years. The home treatment was modified 4 ways: (1) Using locally-prepared single units of fresh dry plasma in the majority of the patients while mild hemophilia A and vWD patients received 1-deamino 8D-arginine vasopressin. (2) Recruiting local health personnel as the primary care providers. (3) Teaching and training patients and parents intensively. (4) Maintaining an effective control system. The heartful effort of the health personnel was not in vain; the patients learned to take good care of themselves. Twenty patients and 20 parents or relatives were able to perform venipuncture properly and no adverse effect was observed. Since the hemorrhage was treated very early, the severity and sequelae of bleeding were decreased. The utilized blood components and days of hospitalization were reduced. Impressively, the absenteeism from work or school was minimized so that the patients could enjoy a near normal life in their family, school and society.