Preventing Cree Encephalitis and Leukoencephalopathy

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While there are still many Crees who might not have ever heard of Cree Leukoencephalopathy (CLE) and Cree Encephalitis (CE), for the families whose lives have been touched by it, the scars may never heal.

Both genetic disorders caused by a mutated gene, CLE and CE are serious brain (neurological) diseases that affect babies and toddlers.

According to Valerie Gosselin, a clinical nurse for the CE-CLE Education and Genetic Carrier Screening Program, both of these diseases are passed genetically through this mutated gene. The Cree Board of Health and Social Services of James Bay (CBHSSJB) sees one to three cases per year.

“In cases of CLE the baby will be born healthy and look good, but will not be able to cope with fever. So with the first fever or viral infection that the baby gets, they will get really sick and die within a few weeks to a few months of that,” said Gosselin.

“CE is a disease that affects babies but this is something that we can see at birth as it is caused by calcium on the brain that stops it from developing the way it should and so the child is severely handicapped. Some will die a few months after birth while others may survive a few years though they will be severely handicapped – they will be blind, never talk or walk or attend school.”

According to the CBHSSJB, the CLE gene occurs in about one in every 11 Crees and the rate for CE it is about one in every 17.

The program for CE and CLE began in 2006 through the Eeyou Awaash Foundation, created by the parents who lost babies to this disease. Prior to 2006 doctors and scientists focused on finding a cause for the disease.

Once it had been determined that it was caused by a genetic mutation that could be passed down through the generations, the next step was to provide testing to determine if future parents are at risk of having a child born with either disease.

If two carriers have a baby together, the chance of passing along the gene increases and so the CBHSSJB have recommended that these individuals get the unborn child screened.

Services were set up in the communities to help screen and inform prospective parents about these diseases. Gosselin said it became evident that it could be helpful to Crees if they knew whether or not they were carrying this gene before they started a family. Because of the high rate of teen pregnancy in the Cree communities, education and testing are very important.

With this in mind, the CBHSSJB decided that the best way to get the message out was to offer learning opportunities to secondary students.

“We wanted to just give out this information because a few years ago, before the program existed and the blood test was available to screen for it, there was a lot of myths about the disease. There were rumours that breastfeeding was causing it or it was a result of the parents’ bad behaviour. The families that were affected by this were being talked about because their child appeared healthy and then died,” said Gosselin.

Gosselin and Jessica Le Clerc-Blain, a researcher from the Ste-Justine Children’s Hospital in Montreal, began visiting secondary schools in the Cree communities this year to offer learning sessions about CLE and CE. Students were then surveyed to determine how useful they found the information.

Gosselin said she explains the diseases and what they can and cannot do. She emphasized that adult carriers of the gene mutation will not develop either disease.

“Our goal is to give out information so that the next generation of parents has heard about this disease and know how it is passed on. The second objective is to then offer them this blood test. It is really important to note however that these blood tests are never mandatory. You can take them if you would like to, but it is up to the students to decide whether they want to do this or not,” said Gosselin.

Results of the program show that the youth learn about CLE and CE, the more they volunteer for testing.

According to the results from visits with Secondary 3-5 students in six of the communities (267 students), eight out of 10 students who participated in the non-mandatory survey felt that they had enough or more information about CLE and CE.

“We compared answers from surveys that were done before and after Gosselin’s education session, and found that for most knowledge questions (11 out of 14 questions), knowledge improved significantly after the education session. This indicates that the education session most likely has a positive influence on students’ knowledge. By knowing and understanding the information, students can make informed decisions about carrier testing,” said Le Clerc-Blain.

Other facts that are determinable from these surveys show that:

– Seven out of 10 participants thought it is a good or very good idea to offer the CE-CLE blood test in high school;

– 2.5 out of 10 eligible students decided to do the blood test when they met the program nurse individually after the education session;

– Participants answered that the most common reasons for doing the CE-CLE blood test is to find out for sure if they are a carrier, and to avoid giving birth to a baby with CE or CLE;

– Participants answered that the most common reason for not doing the CE-CLE blood test is not wanting children right now.

“When we first go into the schools we find that there are students who have never heard of these diseases. For the Secondary 3 students, if they don’t actually know anyone who has suffered from it, then they often won’t have heard of it before. Or, they may have heard about it but are somewhat unclear about it. When we go back to the Secondary 4 or 5, we are able to see that they have understood the presentation and are getting it,” said Gosselin.

For teens who discover they are gene carriers, Gosselin said that nothing changes until they want to start a family.

“If they are carriers I will meet with them again to let them know their results and the next phase after that will be for them to reach me again when they do have a stable partner so that we can assess whether they had done this blood test before or not and whether the couple is at risk or not,” said Gosselin.

“For those who are at risk we do offer prenatal diagnosis. The goal of the program is to be able to be there, step by step with the couples that are at risk to help them to have healthy children.”

In the fall, Gosselin and Le Clerc-Blain will visit schools they have not yet visited and then revisit others to assess the impact of the information sessions on students.

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