SLC meeting w.Science and AG comm. - Efforts to make CJD Reportable

[Guest guest]

News article from Tuesdays Science and Agriculture committee meeting in SLC, UT

12/15/98.

The article in the SLC Tribune was posted today, here is the Deseret News

article. We also had three TV stations at the meeing and they telecast their

tapes as many as three times each. One station gave us half of their 9 pm

newscast, about 7 minutes worth, and showed it again the next morning. The

committee agreed to sending a resolution to the State Health Dept recommending

that CJD be made reportable in Utah. The State said they had already started

the actions. Banner day for CJD in Utah. Mel in SLC

Wednesday, December 16,

1998

Donor illness spurs blood quarantine

By Lois M.

Deseret News staff writer

Blood products have been quarantined after discovery that a

30-year-old blood donor has a brain disease that is distantly

related

to Mad Cow Disease.

Doug McEwen of Kaysville was diagnosed with

Creutzfeldt-Jakob

Disease (CJD), a rare member of the Transmissable Spongiform

Encephalopathy family that causes rapid progressive dementia and

is

always fatal.

The Utah Department of Health plans to require reporting of

the

disease, which after a public comment period could take effect in

two

or three months.

Doctors, epidemiologists and scientists know a lot about the

disease, which acts like the bovine strain that caused the Mad Cow

Disease outbreak in Great Britain and Chronic Wasting Disease in

deer,

among others. But they know very little about how it is

transmitted,

Craig R. Nichols, state epidemiologist, told members of the

governor's

science and technology advisory board Tuesday.

McEwen was a regular plasma donor and continued to donate

after the onset of his symptoms. A diagnosis wasn't made until

last

month, based on a brain biopsy, which aside from an autopsy is the

only way to confirm CJD. As a result, the Centers for Disease

Control

and Prevention, the Food and Drug Administration and the blood

product manufacturer are studying blood-product manufacturing and

distribution practices, Nichols said. And they have quarantined

all of

the blood product that contains McEwen's plasma donation since

last

January. Plasma donated before January is believed to have been

already used.

The problem is, his plasma was " pooled " with other

donations.

Since blood products, which have many life-saving applications,

are in

short supply and it's unknown whether (but thought unlikely) blood

can transmit CJD, they are trying to determine whether the pooled

byproducts should be released or destroyed. And because it was

pooled, it's hard to determine exactly how much blood byproduct is

potentially damaged. The state could not say how much has been

quarantined. There's no way to test the blood products for the

disease.

Since 1980, 29 Utahns have died from CJD. Six cases were

diagnosed in Utah last year. But it can take up to 30 years for

symptoms to develop and they can last for as long as 12 years

before

the patient dies. That means that, if blood products can spread

the

disease — the great unknown factor — many people could be

affected. And it's reportedly hard to kill the prion (a

malfunctioning

protein) believed responsible for infection.

Even testing for the disease is difficult. While an autopsy

will

reveal it, a brain biopsy in a living subject is only 60 percent

effective, according to the state Department of Health.

Nichols said the disease could benefit from a " good

detective. " It

has an infectious component and a hereditary component. Direct

exposure to infected brain tissue will do it. But much of the

public

policy surrounding CJD is based on speculation.

And even a good detective might get bogged down in the

various

plot twists.

Because it is hard to diagnose, many people, including Mel

Steiger, whose wife died from CJD, believe that some people with

dementia who are diagnosed as having Alzheimer's actually have

CJD.

That's significant because people with CJD are not allowed

to

donate blood or organs, since the risk isn't known. But people

with

Alzheimer's can. It makes proper diagnosis urgent, Steiger told

the

committee.

While Utah records list 16 people as having died of CJD from

1989

to early this year, 425 death certificates list dementia. The

actual

causes could be Alzheimer's or CJD, Steiger said, citing a Yale

study

that found 6 of 46 people who reportedly died from Alzheimer's

actually had CJD.

If you assume even 10 percent of Alzheimer's cases are

actually

CDC, he told the panel, then Utah would have 2,500 CJD cases.

The Health Department wants to take the possible risks

seriously,

but doesn't want to alarm the public unnecessarily, said

department

spokesman Ross .

" When people don't know what causes something, they take

extraordinary measures to avoid getting (it), " he said.

" It's a balancing of risks. We've never seen it spread by

blood

transmission, " Nichols said. " But there hasn't been enough

investigation to say for sure. "

He said that the blood-products manufacturer, the Centers

for

Disease Control and the Food and Drug Administration all believe

that

there is time to conduct more studies before a decision has to be

made about the blood-products' fate. But the country can ill

afford to

destroy it if it doesn't have to, since the products are needed so

urgently.

" I don't care if only 240 people die annually (from CJD), "

said

McEwen's wife, Tracie, in tearful comments made to the committee.

" If you add in the family and friends, potentially hundreds of

thousands of people are affected. "