P. Isarangkura

Haemophilia care in the developing world: benchmarking for excellence

Seventy‐five percent of patients with haemophilia receive no or inadequate treatment, and often do not survive to adulthood. With efficient organization, the disorder is treatable and becomes part of normal life. In developing countries there is a large discrepancy in haemophilia care. Some have zero treatment levels, while others already have comprehensive care centres. This paper attempts to assess and standardize the levels of haemophilia care for developing countries, setting up benchmarks or guidelines for future development. Four major areas are emphasized: clinical care, laboratory, blood products and patient organization. For each country or community, development work begins after the assessment of competency level in each area. The next step is then to plan, organize, improve and move up to the next level. To become successful, a sound and realistic strategy should be employed, starting from the identification of key leaders and the recruitment of an expert team. To obtain recognition and support from health authorities, the haemophilia care programme should not limit itself to haemophilia care but should also include medical care for all bleeding disorders, including the improvement of blood banks, blood products, coagulation laboratories and other medical facilities. This would directly improve the overall medical care standard of the whole hospital. It is also important to emphasize the need for selfreliance, employing simple yet effective methodology, equipment and mechanical facilities. The effective coordination of World Federation of Hemophilia assistance and the host countrys committed action will ensure success in the emerging trend of better haemophilia care in developing countries.

Survival analysis of patients with haemophilia at the International Haemophilia Training Centre, Bangkok, Thailand

Summary.  Haemophiliac treatment in less developed countries is limited to locally prepared fresh frozen plasma, cryoprecipitate, cryo‐removed plasma and lyophilized products as replacement therapy. Factor concentrate is seldom used because of the high price. The present study reports the survival analysis of 164 patients comprising 138 haemophilia A and 26 haemophilia B cases from 134 families registered at the International Haemophilia Training Centre‐Bangkok, Faculty of Medicine, Ramathibodi Hospital, Mahidol University from 1971 to 2000. The duration of follow‐up ranged from 1 to 27 years and 8 months with a median of 9 years and 1 month. They received treatment on demand of bleedings, and 85 patients received additional home treatment for early bleedings. The proportion of death was 15.2%. The Kaplan–Meier survival curves revealed the median (95% CI) survival time of patients with severe and moderate degrees of 35 years and 6 months (21.4–49.7), and 38 years and 1 month (28.8–47.3), respectively. To compare the progressive achievement of haemophilia care services, the treatment period was divided into three decades: 1971–1980, 1981–1990 and 1991–2000. The patients with unaided proper walking increased from 62.8% in the first decade to 84.5% in the third decade. However, one‐third of the patients developed one to four chronic haemarthrosis determined by clinical evaluation, especially patients with severe degree. Moreover, the estimated probability of a survival time beyond 13 years of age among patients with severe degree increased from 0.85 in the first decade to 0.94 and 1 in the second and third decades, respectively. Thus, established haemophilia care, even with limited resources, has significantly decreased the risk of death and increased the survival time among patients with haemophilia.

Acquired bleeding disorders: the impact of health problems in the developing world

Summary.  Several acquired bleeding disorders in the developing world have impacts on health, including late vitamin K deficiency bleeding (VKDB) in infants, dengue haemorrhagic fever (DHF), and malaria. This paper describes their clinical manifestations, mechanisms involved, and treatment.

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.

Disarticulation of a knee joint in a haemophiliac with high inhibitor titre

Summary. Disarticulation of a knee joint in an 8‐year‐old haemophilia A patient with high inhibitor of 3450 Bethesda units (BU) is described. He had an infected compound fracture of the tibia and fibula. Surgery was successfully performed after extensive plasma exchange; administration of immunosuppressive agents such as cyclophosphamide, methylprednisolone, intravenous immunoglobulin and cyclosporine were combined with a loading dose of 100 units kg‐1 of factor VIII concentrate, followed by continuous infusion of 16 units kg‐1 h‐1 of factor VIII in the form of factor VIII concentrate and cryoprecipitate for 7 days and decreased to 8 units kg‐1 h‐1 in the form of cryoprecipitate for 19 more days. During the 1st to 7th post‐operative days, the lowest factor VIII inhibitor was 18 BU and the factor VIII level ranged from < 1–2.1 IU dL‐1. On the 9th and 13th post‐operative day, although the inhibitor rose to 330 and 2700 BU, respectively, there was no serious bleeding. The suture was removed on the 21st post‐operative day. The inhibitor spontaneously decreased to 550, 232 and 14 BU at 1, 7 and 10 months, respectively.

Fresh dried plasma: A solution for the shortage of blood products in developing countries

SummaryThe availability of blood products for replacement therapy of hemophilia and allied disorders is still a formidable problem in most developing countries which consist of 80% of the world population. Fresh dried plasma (FDP) is a lyophilized form of fresh frozen plasma which can be stored at 4 °C, whereas fresh frozen plasma must be stored at −20 °C. A large-scale production of FDP from single units of fresh frozen plasma was achieved by the National Red Cross Blood Center in Thailand. This paper describes the method of preparation, biochemical and blood coagulation properties of FDP during one-year storage as well as its clinical usefulness. A batch of 179 bottles of FDP stored at 4 °C for one week, 3, 6, 9 and 12 months was studied in order to determine the biochemical and blood coagulation parameters. FDP has pH higher than that of fresh frozen plasma, but its value is within the safety range for infusion. Electrolyte and protein levels were comparable with those of fresh frozen plasma. Coagulant activities of factors I, II, V, VII, VIII, IX and X were about 10% lower than those of fresh frozen plasma; however, they are in the acceptable therapeutic range and all were stable during storage at 4 °C for up to one year. Over 4,000 bottles of FDP were used with good clinical and laboratory response and no serious side effects were observed in the treatment of bleeding in blood coagulation disorders and home care therapy of hemophilia.