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.

The efficacy and safety of lyophilized cryoprecipitate in hemophilia A.

This prospective study of assessing the efficacy and safety of lyophilized cryoprecipitate (LC), which was heat-treated at 60 degrees C for 25 hours, was conducted in 23 patients with hemophilia A (severe 13, moderate 9, mild 1) at the International Hemophilia Training Center, Bangkok from 1997 to 1998. A total of 223 infusions of LC were given. The status of the patients could be classified into 4 groups: group I, non-bleeding (n = 13); group II, severe bleeding requiring hospitalization (n = 9); group III, appendectomy (n = 1) and group IV, early bleeding controlled by modified home treatment (n = 200). Pharmacokinetic studies were conducted in groups I and II. The mean in vivo half-life of factor VIII clotting activity (F VIII:C) was 12.6 hours and the mean in vivo incremental recovery at baseline was 2.1 per cent/unit/kg. The mean clearance was 3.22 ml/kg/h. There was no statistically significant difference in these parameters between groups I and II (p > 0.05). The hemostasis was successfully achieved and 1 to 2 small urticarial wheals were observed in only 2 infusions. In addition, 9 out of 23 patients received LC exclusively for 1 year. None of them developed inhibitor to F VIII:C nor did any contract additional transfusion-transmitted infection except one who developed anti-hepatitis C virus seroconversion after receiving 16 bottles of LC in 4 months. Therefore, the more efficient virus-inactivation in the preparation of LC should be established.

Limb salvage for extremity sarcoma in Ramathibodi Hospital.

From May 1986 to July 1991, a retrospective review of 27 patients treated with limb salvage surgery for extremity sarcomas, was evaluated by our team. There were 12 males and 15 females with a mean age of 22.7 years (ranging from, 9 to 53 years). The mean of the follow-up period was 27.6 months. (ranging from 6 to 62 months). Twenty-four patients had bone sarcomas which included 20 osteosarcomas, 3 chondrosarcomas and one adamantinoma. The locations of bone sarcomas were the proximal tibia (6 cases), distal femur (5 cases), proximal humerus (4 cases), proximal fibula (3 cases), scapula (3 cases), proximal femur (1 case), tibial shaft (1 case) and pelvis (1 case). The remaining three patients had malignant fibrous histiocytomas located in scapular, tibialis anterior muscle, and knee regions. In total there were 1 stage IA, 2 stage Ib, 1 stage IIA and 23 stage IIB. Twenty-four cases had wide excisions; two had marginal excisions and one radical excision. There were 17 bone reconstructions consisting of 11 allografts and 6 autografts. At the most recent follow-up examination, 74 per cent of the patients are alive and the overall disease free survival was 63 per cent. Local recurrences occurred in 11 per cent. The major complication rate was 3 per cent and the minor complication rate was 33 per cent. With respect to a functional outcome, 84 per cent of the patients achieved excellent or good results.(ABSTRACT TRUNCATED AT 250 WORDS)