An historic meeting this week in Montreal promises that future generations of Cree will have more control over their health.
“A lot of parents wanted to know why it was happening,” Annie Bearskin says of the Cree disease, which took her granddaughter’s life in 1998. “Right now, they’re very happy with the information and the fact people will have a choice in the future.” Because of her role in the Eeyou Awash Foundation, a support group for parents of children with the disease, Bearskin will be in Montreal this week to discuss the course for the next stage in the struggle against the disease.
She’ll be joined by a remarkable coalition of scientists who are researching the genetic secrets of the disease and Cree health authorities who will decide how to best apply that knowledge in the nine Cree communities.
“So far, all the work has been done in the lab,” said Dr. Deborah Black, a neurologist who works in Montreal and Vermont and who did early, groundbreaking research on the disease while working in Chisasibi in the 1980s. “The next step is for the Cree to decide what they want to do with that information.” After Dr. Black and her team first discovered the Cree disease is actually two similar neurological disorders in 1988 — Cree encephalitis and Cree leuco-encepha-lopathy — work turned towards finding the specific genes that caused the diseases. Scientists and Cree had long suspected the cause of the disease lay in the parents’ genes, as the disease seemed to run in some families but not in others.
In the last two years, researchers in Leeds, England, Paris and Bethesda, Maryland, working together with affected Cree, found the genes. Now, scientists know that about one of every 20 Cree carry the gene. However, the disease only develops in children who have parents who both carry the gene. Even then, the chance is only one-in-four they will be affected, according to Dr. Black.
“The mother and the father could both have the gene and none of their kids would get the disease. That’s a chance they could take.” Armed with this scientific information, Cree health officials now have to decide how to help Cree carrying the gene.
“There are really three ways to go,” explained Dr. Black, laying out the chief issues for discussion at this week’s meetings. “Before they have kids, the mother and father can be screened for the genes and told what chance their kids will have of developing the disease. Or, when mothers are pregnant they can test the fetus and could choose to abort. Or Cree officials could screen everyone for the genes.” Each strategy comes with tricky ethical and moral issues. Fortunately, the genetic research into the Cree disease revealed the Cree do not suffer alone.
“Both of these diseases have homologues; they’re not exactly the same disease but it means there is more scientific interest,” explained Dr. Black.
Cree encephalitis shares the same genetic cause as Aicardi-Goutieres syndrome, an extremely rare disorder that affects approximately 40 children each year, primarily in Europe. The disease is especially prevalent among an immigrant Pakistani community in London. Cree leuco-en-cephalopathy shares the same gene with a disease that affects a small population in the Scottish highlands. Cree genealogists have traced the disease back to three Scottish fur traders who intermarried with Cree women.
Whatever course Cree health officials chose to recommend to those who carry the gene, the fight against the Cree disease — linking Cree communities with laboratories in Montreal and Europe — is hailed by Dr. Black.
“This couldn’t have happened without close ties between the scientific communities and the Cree,” she said. “If Bearskin hadn’t started the foundation there wouldn’t have been any way to mobilize the population.
“It’s been a remarkable collaboration. You hear all sorts of horror stories from the past about doctors marching into communities, taking blood, doing whatever they wanted. This wasn’t like that. And from here on, the Cree will run the show.”