Also in this week's column:

• What's this 'scotomisation' in The Da Vinci Code?
• What lies within
• Is the brain-sex theory founded in fact?

What happened to haemophiliacs before blood supplies were safe?

Asked by Alicia Rauzok of Greensboro, North Carolina

According to the National Haemophilia Foundation in New York City, nearly 90 per cent of Americans with severe hemophilia became infected with AIDS in the 1980s when the blood supply needed for transfusions was contaminated. Sadly, more than 50 per cent of those infected with HIV have died.

Yet there are several myths about haemophilia. In the developed world, haemophilia is not the death sentence many people believe it is.

"Haemophilia" is from the Greek words haima (blood) and philia (to love). The disease involves only an impairment of clotting, but not actually a complete inability of the blood to clot.

Haemophilia is a genetic-based inherited disorder involving a deficiency of a specific clotting factor in the blood. There are two forms of haemophilia. Haemophilia A (factor VIII deficiency) occurs in about one in 5,000 live male births. Haemophilia B (factor IX deficiency) occurs in about one in 10,000 live male births. The symptoms range from mild to severe.

Haemophilia occurs mostly in males while the defective gene is carried by females. However, women carriers can experience mild symptoms of the disease. Most haemophiliacs can now lead fairly normal lives, while some are seriously debilitated, and a few may die prematurely.

However, the true nature of the disorder contrasts markedly with the popular image of the disease largely derived from its historical association with Europe’s royal families, especially the Russian royal family, the Romanovs.

In haemophilia, coagulation time is relatively normal and bleeding is characteristically a delayed or prolonged oozing or trickling occurring after minor trauma or surgery such as tooth extraction. Even in severe haemophilia, coagulation time ranges from 30 minutes to several hours. Rarely does a haemophiliac have massive bleeding, and never does one bleed to death from a small cut as it is commonly believed.

Joint hemorrhages, gastrointestinal bleeding, and bruising are actually more serious concerns for the haemophiliac than is bleeding to death.

Modern treatment methods have significantly lowered risks through the use of new clotting serums given during periodic blood transfusions. However, it is estimated that 70 per cent of the haemophiliacs around the world do not have access to treatment that would make this disease far less fatal.

Stephen Juan, Ph.D. is an anthropologist at the University of Sydney. Email your Odd Body questions to s.juan@edfac.usyd.edu.au