WHEN
AN ILLNESS
IS INCURABLE
Coping with an inherited disease
motivates Tammy Campbell to action
Written by SHARON ALMIRALL
Photography by KIMBERLY DAWN
Learning there’s a time bomb ticking away in their bodies could derail many. But for Tammy Campbell, the news that she has an incurable disease motivated her into action.
Campbell’s commitment to understanding how she could manage the disease and all its ramifications resulted in her being named to receive the Family of the Year Award from the Huntington’s Disease Society of America, Rocky Mountain Chapter.
“Even though I’m getting prepared, I am an optimistic person by nature. I never thought I’d be named the Family of the Year, because I go to work everyday, I live a good life,” Campbell said recently upon hearing she would receive the award.
This woman who has made family and home and living life to the fullest a priority could be a textbook case on how to cope with a serious illness. She has met with advisors of all kinds — legal, financial and medical — to organize her future so that her loved ones don’t suffer the consequences of her illness.
Huntington’s disease was passed genetically to Campbell from a family member. Though at the age of 43, she doesn’t yet see signs of the disease, she has learned symptoms typically manifest themselves in the patient’s mid-40s. Having the gene always results in having the disease.
Campbell learned her father and his mother had Huntington’s disease. Her father died when she was 21 years old and he was 71. Although she didn’t know at the time there was a name for his problems, she had noticed the symptoms, which were also verified by an aunt who was her father’s sister. Stuttering, shifting his weight, being accident-prone and experiencing mental problems, her father reached the point where he could no longer live alone. Her parents were divorced by the time his symptoms were becoming manifest, but she and her mother visited him and realized his condition. Her mother was not aware that Huntington’s disease was a family trait.
Learning from an aunt that her father suffered from Huntington’s, Campbell decided to be tested for it. “It’s just my personality,” she says of her decision to undergo predictive genetic testing. Knowing about the disease was a help to Campbell so that she could prepare. “When I found out I had Huntington’s, I felt I could control how I would react to the disease even though I couldn’t change it,” she says. Campbell says that only 8 percent of those who may have it actually have the testing done.
Huntington’s disease is an inherited brain disorder that affects the nervous system, resulting in the loss of mental capacity and physical control over a period of years. Some 35,000 people in the United States have it; another 125,000 are at risk of inheriting it.
The disease came to be known as Huntington’s chorea; chorea, the Greek word for dance, alludes to the jerky, twisting, uncontrollable muscle movement that victims exhibit. There is no cure for Huntington’s at this time.
Affirming that it is simply her personality to know what’s going on around her, Campbell has sought to eliminate as much stress as possible in her life and to focus on a healthy lifestyle, a pattern of behavior she considers important to maintaining a quality of life. She eats more organic vegetables, purposely lost weight she wanted to shed and made herself over so that she doesn’t have to worry about doing some things, such as applying makeup, every day. She is not on medication, but takes antioxidants daily to assist with healthful living.
One of the key ways in which Campbell sought to manage her life was to have an understanding with her mate, a man she has known for years. Though they live together, they are not married and do not refer to their relationship as common-law. They have their own separate financial records and their cars in their individual names. They do not file taxes together or have joint insurance. “We’re two separate people living under one roof,” she says.
She goes on to say, “I can’t get married because I don’t want to strain financial needs in Art’s daughter’s life. She knows about the Huntington’s disease and knows what to expect. She is very sweet, very smart. She lives with her mother and comes to visit on weekends. I’m teaching her to take care of herself from the inside out.”
Campbell explains that Art is “calm and laid back, in no hurry to get married,” so the arrangement they’ve made works out just fine. They do not introduce themselves as a married couple so as to not compromise their status legally when financial issues start to come into play. At a tiny church in Morrison, they participated in a spiritual ceremony to state their commitment to each other. “The ceremony satisfied my emotional side while keeping the legal side OK,” Campbell says.
A little skittish about revealing that Campbell has the Huntington’s disease gene, Art was reluctant to tell his mother about it and held off for a while. But now both their mothers know.
Planning to dedicate her brain to the Huntington’s disease brain register and donate her body to science, Campbell seeks to minimize worries for Art. He didn’t want to talk about it at first, but since she is one who wants to know what is happening with her body, she has taken an active role in determining her future. “I want to have as much power as possible and not let it control me,” she explains. She wears a “do not resuscitate” bracelet.
Campbell and Art hope to move to a mountain cabin. She’ll work fewer hours and be close to a nursing home, should that become necessary. It’s possible he could take on a care-giving role in time. “No matter how sweet a person he is, it’s still going to be a burden,” she says.
She expects to be active with the Huntington’s disease Society. She will volunteer, do interviews, participate in walks — “anything I can do, I want to do.” She hopes to hang on for the next 10 years. Always the optimist, Tammy Lynn Campbell, or TLC as she says, affirms she is not afraid of dying.
More about Huntington’s disease
An inherited brain disorder, Huntington’ disease results in a loss of mental functioning and physical control over a number of years. While it is typical for symptoms to become apparent between the ages of 30 and 50, signs of the disease can be seen in children and in adults over 70.
In 1872, Dr. George Huntington identified Huntington’s disease, which affects both men and women and many ethnic groups. It is passed on through genetics, but not everyone in a family will be affected physiologically. It is believed that chemical substances involved in normal functioning of the brain cause the death of brain cells that affect movement and emotional control. These cells are not regenerated over time, and when they are not working, the functions they control diminish.
Death comes gradually, typically occurring 15 to 20 years after the onset of the disease. The illness itself is not usually the cause of death. The patient dies with the disease after developing pneumonia, heart failure or an infection caused by the body’s weakened state. Most people with Huntington’s disease can live happy, productive lives for many years after receiving the diagnosis.
In 1993, scientists isolated the gene for Huntington’s disease and found the mutation that causes the gene to differ from the normal gene. The nature of the defect, a duplication of a piece of DNA, can predict whether the individual will have Huntingtondisease. Every child of an affected parent has a 50-percent chance of inheriting the gene.
Genetic testing for HD
Those who are at risk for Huntington’s disease may choose to undergo genetic testing to determine whether they carry the mutation and will eventually develop the disease. This is referred to as pre-symptomatic testing. People who believe they are showing symptoms of the disease may also be tested (confirmatory testing).
The actual test is a simple procedure, requiring blood be drawn and sent to a lab for analysis. However, there is an involved standard protocol that must be followed during the testing process to assure that people considering the test are as prepared as possible. This protocol requires a pre-test visit with a neurologist, a genetic counselor and a psychologist to provide counseling and an assessment as to whether testing is an appropriate option.
Blood may be drawn about one month later. A third appointment is scheduled in about a month to discuss the results of the test. A follow-up visit is then scheduled to check in with patients and discuss how they are coping with the results of their test, whether positive or negative. At any time during this process, the patient may postpone the next step or stop the process altogether. Once the test has been done, a person may choose not to receive the results at that time.
Whether to undergo the pre-symptomatic genetic test is a personal decision that must be made with careful thought. Testing is the right choice for some, but not for all. There are many issues to be considered, including the effect of the knowledge on the at-risk person, childbearing, the effect on one’s family, possible insurance or employer discrimination and the opportunity to participate in research and possible treatments. It is important to discuss these issues with a genetic counselor who is familiar with HD, as well as with one’s family.
The HDSA Center of Excellence at
Colorado Neurological Institute
The Center of Excellence is the result of a collaboration between CNI and the Huntington’s Disease Society of America to provide comprehensive services to patients with HD and their families. The clinic, generally held on the second and fourth Thursdays, is staffed by professionals whose mission is to provide top-rate assessment and treatment to patients.
When patients come to the clinic, they can meet with a neurologist, genetic counselor, physical therapist, occupational therapist, speech therapist, psychologist and social worker. This provides a setting in which all aspects of the disease may be assessed and treated as effectively as possible. Genetic testing is also available.
The Center of Excellence provides a variety of other services, including a support line staffed by a social worker; an HD support group for patients, at-risk persons and family members/caregivers; referrals to a variety of services; research opportunities; educational programs; liaison between local HD families and the Huntington’s Disease Society of America; travel assistance for patients in need; low-cost lodging options for out-of-town patients; and training and consultation for professionals and facilities.
Sources of information:
www.HDSARockyMountain.org
www.HDSA.org
