Guideline for the treatment of haemophilia in South Africa.

This guideline has been prepared by the authors for and on behalf of the Medical and Scientific Advisory Council (MASAC) of the South African Haemophilia Foundation to facilitate the appropriate management of individuals with haemophilia in South Africa. Individuals with haemophilia and their physicians should be advised by a Comprehensive Haemophilia Treatment Centre. Strategies that help to prevent bleeds include regular exercise to strengthen muscles, protect joints and improve fitness; maintaining a healthy body weight to avoid extra stress on joints; and avoiding contact sports. Acute bleeds should be treated early, ideally within 2 hours of onset. Patients with mild or moderate haemophilia A may be treated with desmopressin. Bleeding in patients with severe haemophilia A without inhibitors should be treated with factor VIII concentrate. Bleeding in patients with haemophilia B without inhibitors should be treated with factor IX replacement. Tranexamic acid can be used for mucous membrane bleeding in surgical or dental procedures. Bleeds in patients with inhibitors must be managed in consultation with a haemophilia treatment centre. Major bleeding episodes are large muscle or joint bleeds, bleeds resulting from severe injury, or bleeds that affect the central nervous system; gastrointestinal system; neck or throat; hip or iliopsoas; or the forearm compartment. These bleeds may cause death or musculoskeletal deformities, and advice on their treatment should be sought from a haemophilia treatment centre physician. Appropriate factor replacement therapy must be started urgently for major bleeds, and hospitalisation is usually required to maintain adequate factor levels.