Family blasts Health Department over lack of updates on mystery brain disease

Steve Ellis has been waiting more than a monthto hear from Health Minister Dorothy Shephard after requesting updated informationabout a mystery brain disease that has so far stricken 48 New Brunswickers.

On Tuesday, he got a reply. But he didn't get answers.

Instead, he said, "I got a form letter telling me what I and everyone else in New Brunswick already knows."

Ellis's father, Roger, is one of the 48 confirmed and suspected cases identified in the province and first reported in March, when Radio-Canadaobtained a memo from Public Healthto medical professionals. He is still awaiting some test results and is a suspected case at this point, not a confirmed case.

The March 5 memonoted that 43 cases of the disease had been identified so far,most of them in the Acadian Peninsula and Moncton areas, and that five people had died.

• Families frustrated by 'wall of silence' on N.B.'s mystery brain disease

The case count has since risen to 48 and the death toll to six.Tests done on the patients have so far ruled outCreutzfeldt-Jakobdisease and other prion diseases, and scientists are looking into the possibility that this is an entirely new disease,possibly caused by an environmental toxin.

Families have frequently expressed frustration with the Health Department's handling of informationrelated to the case,repeatedly flagging what they say is a lack of transparency.

On Tuesday, Ellis said his frustration has reached a boiling point.

This is their version of transparency? The clock is ticking for these people and N.B. is dragging their feet.- Steve Ellis, whose father is suspected of having mystery brain disease

He blasted Shephard'sresponse to his questions, and the ongoing lack of answers to questions about when Public Health would be contacting families to conduct investigative questionnaires, as had been promised for weeks.

To Ellis's knowledge, none of the families of the 48 cases have been contacted yet.

Department 'dedicated to remaining transparent': Shephard

In her emailed letter, Shephardtold Ellis that Public Health is "dedicated to remaining transparent as their work progresses."

She noted that a website "has been created specifically for the New Brunswick cluster of neurological syndrome of unknown cause, and will be updated on a regular basis."

• Cases of mysterious neurological disease rise to 47

She also noted that the regional health authorities are "actively recruiting for a specialized clinic for patients suffering from this disease," and that she "has been informed that approval is underway for this unit."

Both the website and the specialized Horizon Health Network clinic were announced three weeks ago.

"This is their version of transparency?" Ellis said of Shephard's letter. "The clock is ticking for these people and N.B. is dragging their feet. I'm furious."

In an email Tuesday evening, Public Healthspokesperson Bruce Macfarlane said the department is "currently in the process of obtaining contact information" for the cases under investigation.

He said the departmenthas produced, with input from local and national subject matter experts, an epidemiological investigation questionnaire to gather exposure information from New Brunswickers suspected of having this illness.

"This questionnaire asks lots of details including ...the persons' environment, residence, exposure and travel history. ... Data collected will be incorporated into the investigation to help identify potential sources of the illness," Macfarlane said, adding the department will be in touch with patients or their caregivers "very soon."

In an interview with Information Morning Moncton on Wednesday, Ellis stressed that he does not fault the Moncton Mind clinic, led by neurologist Dr. Alier Marrero, which has beeninvestigating and diagnosing the individual cases.

"Mind clinic haskept their promise bymeeting with these patients,doing their due diligence and moving them from suspected [case status] to confirmed, and now they are being made to wait even longer by Public Health," he said.

He also said the families understand that this is a fluid and ongoing situation and that full answers will take awhile.

"We, the people who are affected by this, we understand that there's not a lot of information known yet," he said. "But just keep us informed at the very least the families but more so the public as to what's happening. There's no talk."

• Nothing held back from public about unknown neurological disorder, says minister

The information website, for example, could list what causes havebeen ruled out, something Shephard acknowledged was a good idea onCBC's Political Panel podcast on April 29.

However, Shephard also said in that podcastthat the website can only share what information it has, and the problem is that "there isn't any more information."

"I do understand theconcern in the community. The issue is that we don't have a diagnosis ... This is a methodical process, the science must do its work," Shephard said at the time.

"They are diligently working on how we get to the other side of this, because I know families want a diagnosis."

CBC News journalists are working diligently to try to learn more about this disease. If you or a familymember have information you'd like to share with us, please emailneuro-nb@cbc.ca.

N.B.'s mystery disease: What we know so far

What is it?

An unknown neurological disease withsimilarities toCreutzfeldt-Jakob disease, a rare and fatal brain disease, or prion disease.

What's a prion disease?

A prion disease is a raredisease that pertains to a misfolded protein in the brain. The abnormal folding of the prion proteins provokesa chain reaction that destroys neurons and creates holes in the brain.

When was it discovered?

The first occurrence was retroactively found to have occurred in2013,after the possible existence of a cluster of disease was first recognized by the CJD Surveillance System at the Public Health Agency of Canada in 2020.In 2019, 11 additional cases were identified, with 24 morein 2020 and 12 so far in 2021.

When was it made public?

A March 5 internal memo from Public Health to health-care professionals was obtained by Radio-Canada and reported by Radio-Canada and CBC News onMarch 17.

Where are the cases?

The disease has so far only been identified in New Brunswick.It appears to be concentrated on the Acadian Peninsula in northeast New Brunswick and the Moncton region in the southeast.

How many cases are there?

Forty-eight cases have now been identified.

How many patients have died?

Six people have died of the mystery disease, according to neurologist Dr. Alier Marrero. The six are included in the 48 cases so far.

Who has been affected?

The disease affects all age groups and affects males and females equally, according to the Public Health memo. About half of theaffected individuals are between 50 and 69 years of age.

What are the symptoms?

Symptoms include changes in behaviour, sleep disturbances, unexplained pain, visual hallucinations, co-ordination problems and severe muscle and brain atrophy.

Is it contagious?

Because the cause has not been determined, it is not yet known whether the disease is contagious.

What are the possible causes being researched?

Despite many similarities, tests for Creutzfeldt-Jakob disease have so far ruled out known prion diseases. Scientists are currently looking into the possibility that this is a new variant of a prion disease or a new disease entirely. Neurologists and scientists suspect the cause might be exposure to an as-yet-undetermined environmental toxin.

Who's researching it?

The disease is the subject of investigation by an all-Canadian team of neurologists, epidemiologists, scientists, researchers and other experts. Moncton neurologist Dr. Alier Marrero is leading the research in New Brunswick. In Ottawa, senior scientist and Creutzfeldt-Jakob Disease Surveillance System director Michael Coulthart is leading the research.

In April, Horizon Health Networklaunched a special clinic to assess people experiencing early-onset cognitive decline as cases of anunknown brain disease continue to pop up.

Province's website

The province has launched an information webpage on the mystery neurological disease, which it updates with new information. It is found on the government's gnb.ca website, and is calledNew Brunswick Cluster of Neurological Syndrome of Unknown Cause.