Nursing roles in orthopaedic joint correction in haemophiliac patients.

The roles of nurses in 16 orthopaedic joint corrections of 14 haemophilia A patients (eight severe, six moderate) are described. The patients' ages ranged from 10 to 37 years with a mean age of 17 years and 4 months. The nursing tasks could be divided into three stages. The first is preoperation, the nurse acting as care provider on a team of experts involved in planning the corrective surgery for the affected joints; and as a counsellor for preparing the patients and family members to cope with the challenging operation. The second is intraoperation, the nurse having the role of care provider, giving factor concentrate either by bolus injection (seven episodes) or continuous infusion (nine episodes). The third is postoperation, as a comprehensive care provider, giving cryoprecipitate and/or factor concentrate and monitoring bleeding doses, and as a trainer, teaching the patients how to dissolve blood components and self-venepuncture with aseptic technique. Home treatment for early bleeding was given for 11 episodes, while 6-month prophylaxis was given for five. The accomplishment of these different roles required good communication and nurturing skills, a well-adjusted personality and a warm and positive attitude. The successful performance of nursing roles allows the haemophiliac patients to have a near-normal quality of life.     

Immune tolerance in a haemophilia A patient with high inhibitor using locally prepared lyophilized cryoprecipitate

The immune tolerance in a 12-year-old haemophilia A patient was carried out at the Faculty of Medicine, Ramathibodi Hospital, Bangkok in 1998. His inhibitor titres ranged from 10 to 3450 Bethesda units (BU). He suffered from serious bleeding episodes requiring prolonged hospitalization and the disarticulation of the left knee joint. After obtaining informed consent, locally prepared lyophilized cryoprecipitate (LC), heat treated at 60 degrees C for 25 h, was given in a dose of 13 units kg-1 body weight of factor VIII three times per week. His inhibitor was increased from 15 to 580 BU within the first 4 weeks of immune tolerance. Finally, it was decreased to 40 BU in the 36th week. The only adverse effect was seroconversion of anti-hepatitis C virus after receiving 108 bottles of LC for 36 weeks. In conclusion, the locally prepared LC was able to control the bleeding episodes in a haemophilia A patient with high inhibitor. To our knowledge, this is the first reported case in Thailand.