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Portuguese Israeli researchers discovered a disease linked to first cousin marriages called ‘congenital neutrophil defect syndrome’ as well as how it can be treated.
Israeli researchers at the Sheba Medical Center at Tel HaShomer have uncovered an orphan disease (disease affecting a relatively small number of people) that weakens the immune system and is prevalent within Palestinian and other Arab families that practice consanguinity, otherwise known as first-cousin marriages. The Israeli researchers discovered the illness called congenital neutrophil defect syndrome by observing five Palestinian and two Moroccan children affected with the disease.
This led to the discovery of an unusual bone marrow problem that is caused due to mutations in a gene called VPS45. The Israeli researchers also realized that the replacement of bone cells could cure the illness, provided a compatible donor could be found. According to the Israeli researchers, “the identification of this disease and the discovery of its molecular mechanism creates’ a justified hope for affected patients, as young patients can now receive blood stem cell transplantation earlier.”
CONSANGUINITY WIDELY PRACTICED
Consanguinity is widely practiced within Palestinian and Arab society at large. According to a 2000 study, 27 percent of Palestinian married women have married first cousins, while an additional 19.3 to 21 percent of Palestinian married women are married to more distant cousins. In other Arab societies, the percentage of married women married to first cousins is even higher. 31 percent of Saudi married women, 35 percent of Syrian and Qatari married women, and 43 percent of Libyan married women are married to first cousins. Consanguinity has been practiced within the Arab world for thousands of years. It is viewed as a means of securing tribal relationships and preserving family wealth.
HEALTH RISKS ASSOCIATED WITH CONSANGUINITY
However, despite the fact that consanguinity is widely practiced within Arab society, there are many health problems associated with the practice. The medical risks that accompany practicing consanguinity include higher rates of infant mortality, birth defects, learning difficulties, blindness, hearing problems and metabolic disorders. As adults, the children born from first cousin marriages are at increased risk of for having miscarriages, while the Telegraph reports that one third of children affected die before their fifth birthday. Sometimes, children within Palestinian society who were born with defects resulting from first cousin marriages are ostracized and shunned, as was the case with one Gazan baby who was abandoned by his parents.
Congenital neutrophil defect syndrome is just one of the many health problems associated with marrying first cousins. Yet, in a part of the world where congenital marriages continue despite the health risks associated with it, uncovering a potential cure for congenital neutrophil defect syndrome could positively influence the lives of affected children. In addition to this research and despite regional conflict, Israeli doctors often help Arab society at large, for Israel routinely puts the conflict aside to save Arab lives.
By Rachel Avraham, staff writer for United with Israel
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