Date: Fri 17-Oct-1997

Publication: Hea

Author: KAAREN

Quick Words:

Huntington's-Disease-Whitman

Full Text:

AGING IN HEALTH: Huntington's Disease

(with photo)

BY KAAREN VALENTA

Deidamia Whitman did not live to see it happen, but the efforts of friends

like Richard Rauner have resulted in state funding for the establishment of a

Huntington's Disease (HD) center in Connecticut.

"There is an end in sight to this nightmare disease," Mr Rauner said when Gov

John Rowland signed Bill No 6155 on August 14. The legislation will provide

$600,000 over the next two years to establish a clinic to offer genetic

testing, counseling and a core program serving HD patients and their families

so that victims of the disease can live a higher quality, healthier and

dignified life.

Huntington's Disease is a catastrophic, fatal, genetic brain disease that is

estimated to afflict more than 2,000 residents of Connecticut. Another 10,000

are at risk of inheriting HD from a parent. The early symptoms include

depression, mood swings, forgetfulness and lack of coordination. There is a

steady loss of the ability to control movements, think, and regulate emotions

and behaviors, and the disease frequently progresses to the point of

incapacitation, severe emotional disturbances and dementia. Once in the grip

of HD, there is no remission; nothing can be done to slow or stop its

relentless course.

The disease is named for Dr George Huntington, a New York physician who first

described it in medical literature in the late 1800s. When folk singer Woody

Guthrie lost his battle with HD in 1967, his widow, Marjorie Guthrie, formed

the Committee to Combat Huntington's Disease, forerunner of the Huntington

Disease Society of America (HDSA).

Rich Rauner is the patient advocate member of the board of directors of the

Connecticut chapter of the HSDA. After years of watching his beloved friend

"Whit" battle the symptoms of the disease, he knew he had to do something.

"Whit never mentioned her disease, not even to me," Mr Rauner said. "She was a

very independent person, a very intelligent person. We were total opposites

but had like causes at times. She had patience of Job with everyone. I never

heard her make a bad comment about anyone. A very quiet woman - a giving

person, always giving."

A teacher for 27 years in Monroe, Deidamia Whitman started showing symptoms of

HD about 18 years ago, but her condition was not diagnosed until ten years

later. Mr Rauner, who has been a clerk in the Newtown Post Office for the past

10 years, was her close friend and companion for more than 30 years.

"I watched her slowly lose her independence and her personality," he said.

"She started getting rebellious. All of a sudden I couldn't leave the house.

She would come to [the post office] and stand there. I'd have to leave and

bring her home. I saw her confusion in making simple decisions, her increasing

agitation and fits of super strength and semi-violence.

"She developed obsessions - drinking soda and water constantly. At first we

thought she might be diabetic, but she wasn't. This was a woman who had an

education beyond a master's degree. I knew something was desperately wrong and

that it wasn't her fault," he said.

Twice he took her to the Crisis Intervention Center at Danbury Hospital.

"A person with Huntington's Disease usually shows no emotions of any kind. She

looked great and was just calmly sitting there. I was hysterical, a mess -

totally destroyed," Mr Rauner said. "I call HD the nightmare disease because

it has so many nightmare aspects, and they differ from person to person. In

the advanced stages, you don't know what the person will do next. Whit could

stay up for 96 hours in a row. If someone you know has HD, the whole family

has the disease. It affects the whole family, it affects friends."

But there was no place in Connecticut for persons with HD or their caregivers

to go for help.

"Whit finally spent 30 useless days in a psychiatric unit where she was

heavily medicated," Mr Rauner said. "Just before she was admitted we got a

diagnosis. Dr John Reinhardt on West Street [in Newtown] said she may have

Huntington's Chorea. He worked at Fairfield Hills with doctors who knew about

it."

"Then Whit's cousin in Woodbury called and asked if she had Huntington's

Disease," Mr Rauner said. "He said it is in the family - his mother had it. We

later learned that Whit's mother had the symptoms but had not been diagnosed."

Huntington's always has been a disease hidden by the families that it strikes,

Mr Rauner said.

"There are references to it in the 17th century when its victims were thought

to be devils or witches," he said. "It was also called St Vitus Dance because,

in the advanced stages, the uncontrolled movements of the body are similar to

a dance. A lot of those with HD were executed, put in asylums or actually put

in closets by their families and kept there until their death."

Many families that were afflicted by Huntington's Disease migrated to the New

England states, Mr Rauner said. "There are many in Bristol - where Whit's

family lived - with family names that go back to 17th century England.

When persons are first diagnosed with HD, they can still work, sometimes for

years.

"But you don't dare mention it or you will lose your job and your insurance,"

Mr Rauner said. "You can lose your home and wind up destitute. HD requires a

lot of specialized, expensive treatment."

Symptoms usually begin between 30 and 45 years of age, and every child of a

parent who has Huntington's disease has a 50-50 chance of developing the

disease. It is estimated that 40,000 persons in the United States have the

disease and about 200,000 are at risk.

Three years ago the gene that causes the disease was isolated.

"A blood test will tell you if you have inherited the disease," Mr Rauner

said. "Some choose to know because they are married and want to have children.

Others don't want to know because as yet there is no cure. But with enough

money, it may be only three to five years away."

Legislative Support

After becoming active in the Connecticut chapter of HDSA, Mr Rauner was

invited in August 1996 to serve on a task force formed by the General Assembly

"to evaluate the services, accommodations and available placements for victims

of Huntington's Disease." The task force was chaired by state Rep Ann Dandrow,

R-Southington, and Eveleen McDonald of the Office of Protection and Advocacy

for Persons with Disabilities.

"We did a survey of about 500 nursing homes in Connecticut," Mr Rauner said.

"Only one or two knew anything about HD and did staff training."

The task force identified a need to provide a pre-symptomatic HD clinic for

testing and counseling and a neurobehavioral program with both an outpatient

and an inpatient program and a community impact team to provide resources to

families, caregivers, nursing homes and state agencies.

The task force's 36-page report was completed last January. In March, five of

the task force members, including Rich Rauner, were invited to speak before

the Public Health Committee, which would consider the HD bill.

"We only had four days notice," Mr Rauner said. "I took Whit to Hartford to a

hotel to be with me when we presented it to the legislature. We were expecting

a hard time because we were asking for money for staffing, salaries and

training. We were told to bring 42 copies of our presentation and only five of

us could speak, limited to three minutes each. I knew my speech - about what

it is like to be a caregiver - was four minutes long. But I begged not to be

cut off, and I wasn't."

"We had hundreds of supporters, but we were warned that asking for money would

kill us," Mr Rauner said. "The odds of getting to the next stage were nil. The

bill could be tabled or the committee could cut the money."

Three days after Diedamia Whitman died on April 1, at the age of 63, Mr Rauner

learned that the bill had been approved by the finance committee with the

money intact. It cleared the final hurdle on June 3 and became Public Act

97-264 when Gov Rowland signed it in August.

"Gov Rowland saved our bill for last that day because he wanted to spend time

with us," Mr Rauner said. "He said he was signing the bill for two years -

$600,000 - not one. He wanted Connecticut to be a front-runner in battling

HD."

On October 1, the first steps were scheduled to be taken to establish the HD

center at the University of Connecticut Medical Center in Farmington. "The

center will serve as a model for similar programs in other states and can be

used [as a model] for similar diseases," Mr Rauner said. "On September 7, a

benefit performance of The King & I at The Bushnell in Hartford was a sell-out

and brought $57,000 in corporate sponsorships. We're now hoping to have a

worldwide television concert to raise money for research into HD. If that

happens, there will be enough money to wipe out this disease and the nightmare

will end."

For more information about Huntington's Disease Society of America and area

support groups, call the Connecticut chapter's hotline at 1-800-840-9907.