Haemophilia in South Africa
These guidelines have been compiled by the South African Haemophilia Foundation Medical and Scientific Advisory Council (SAHF MASAC) to facilitate the appropriate management of people with haemophilia (PWH). The current guidelines are based on the publication noted below.
Mahlangu J, Gillham A; South African Medical Journal, February 2008, 98(2):127 – 138
This guide is intended for healthcare personnel who might not be familiar with Haemophilia. People with Haemophilia (PWH) and their physicians should be advised by a comprehensive Haemophilia Treatment Centre staffed by a multidisciplinary team skilled in the care of this uncommon chronic bleeding disorder.
Parents of patients with severe Haemophilia are usually trained in home infusion of the clotting Factor when their child is about four years old and self infusion is normally accomplished by 12 – 14 years of age. However, infants and boys with mild Haemophilia must rely on a Haemophilia Centre or other medical facility for clotting Factor infusions.
Acknowledgement is made of all the past and current members of MASAC, who have produced the previous edition, and reviewed the current edition.
Haemophilia is classified as severe, moderate, or mild according to the levels of circulating Factor VIII or IX and indicates the expected frequency of bleeding:
Severe
Moderate
Mild
Factor VIII Inhibitors in Haemophilia
Inhibitors may develop in 10 – 15% of persons with haemophilia A but are much less common in haemophilia B (1 – 3%). Inhibitors are neutralising antibodies that limit the effectiveness of Factor infusions.
