Haemophilia the cause of circumcision deaths?

Reports of deaths during traditional initiation rites often hit the headlines in South Africa, and there is always a devastating sense of young lives lost too soon. Yet, partly because these centuries-old practices are swathed in mystery, insight into the reasons for the loss of life following ritual circumcision is often limited and not fully understood.

Poor surgical practices in non-sterile conditions are often blamed, but there may be another factor, which needs to be considered. The hereditary bleeding disorder, haemophilia, may indeed be a reason for the deaths in some of these rituals.

The Igazi Foundation, based in Port Elizabeth and working throughout the Eastern Cape, has identified haemophilia as a definite risk factor in traditional circumcisions, especially since they take place in remote areas far from hospitals and clinics. The foundation had a dedicated haemophilia project, which was also aimed at reducing the risk of excessive bleeding amongst initiates. 

What is haemophilia?

Haemophilia is an inherited condition in which the patient’s blood fails to clot. It is caused by genetic mutations that lead to reduced clotting factors in the blood of  people living with the condition. The disorder normally only affects men and boys, although it is carried by women, who may exhibit bleeding-related health issues such as very heavy menstrual periods. Haemophilia does not necessarily need to have a family history. Around 30% of patients with this condition have had a spontaneous gene mutation.
Symptoms of bleeding are very variable and can range from mild to severe. It is in the mild to moderate range where the greatest risk of uncontrolled bleeding during a traditional circumcision may occur.

The reason for this, says Henry Steenkamp, a registered haemophilia nurse working on the Igazi education and outreach programme, is that severe cases are usually diagnosed in infancy, and are already being managed by the time a youngster reaches puberty.

“Mild or moderate cases of haemophilia, however, often only come to light after an injury, dental work or surgery,” he says. “As a result, initiates who may have a less severe form of the condition - and who may not previously have experienced an uncontrolled bleed - may not be aware of the fact that they are at risk following a traditional circumcision.” 

While reliable statistics relating to the causes of circumcision deaths are not available, hospital admissions suggest that excessive bleeding is often a factor. In South Africa are currently around 2,000 people that have been diagnosed with haemophilia.. “We know that around 1 in every 10,000 persons live with this condition. As a matter of fact this means that in South Africa we should have at least 4000 people living with this condition in total, we have identified only half the number expected”, says Prof Mahlangu, Haemophilia Specialist from the Charlotte Maxeke Hospital in Johannesburg.

He continues saying that “this is a serious problem: surgical interventions - circumcision is one - in these persons can lead to life-threatening bleeding in the worst case or cause injuries that might take a long time to heal”.

Igazi’s mission, therefore, is to assist in the set-up of improved diagnostic capacities and the case management procedures for initiates who are found to have haemophilia.

Managing the risk

In accordance with the Traditional Circumcision Act (2001), initiates must be over the age of 16, must consent to the procedure willingly, and must undergo routine testing before retreating to the rural areas where initiation rituals are performed. However, while the mandatory routine tests include a full blood count, HIV screening, viral studies and chest x-rays to determine whether the individual has tuberculosis, they do not include a PTT test. This relatively simple screening test is the first test used to diagnose haemophilia.

“If we could get the PTT test included as part of the routine screening procedure,” says Steenkamp, “we could identify high-risk individuals, and be in a stronger position to assist both initiates and traditional authorities to manage the risk of excessive bleeding following circumcision.”

While medical circumcision in a provincial hospital is always available to all high-risk individuals, many still opt for traditional circumcisions for spiritual and social reasons. In this case, individuals who have been diagnosed receive pre-initiation counselling, and are supplied with clotting factor. Having been taught to self-infuse, the clotting factor needs to be injected by the patients every 12 hours for a period of five days in order to limit the risk of uncontrolled bleeding. They are also supplied with the contact numbers of haemophilia councillors who can assist with any queries or in case of an emergency.

Funded through a grant of an international non-profit organization, Igazi organised educational sessions about haemophilia for circumcision coordinators. Circumcision coordinators are the link between the haemophilia centres and the traditional authorities. These coordinators support the medical evaluation of the boys before going for the initiation. Raising their knowledge is an important entry point into this otherwise quite closed ritual. "The coordinators attended these sessions with great interest. We from Igazi are especially proud that the Department of Health also saw the benefit of this training and acted straight away by themselves, funding an additional two more sessions for which we provided lecturers" explains Richard Johnson, member of Igazi and responsible for this project.

Since people with moderate and mild haemophilia will not show clear clinical symptoms, running laboratory testing during these evaluations remains to be set-up. “In cases where we have been able to test initiates and manage high-risk individuals, there hasn’t been a single death,” says Steenkamp. “This is an excellent result, and shows that there’s a good argument to be made for including PTT tests for haemophilia in the standard screening of all initiation candidates.

“If we can identify high-risk individuals in this way, we can work with traditional authorities through the circumcision coordinators to manage the risk of excessive bleeding within the context of the initiation ritual. It’s a win-win solution. And hopefully, in the long run, we can eliminate circumcision deaths caused by an undiagnosed bleeding disorder altogether.”

For further information about the Igazi Foundation and its work, visit www.igazi.org.za.

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