A Balancing Act: Ataxia and Living

Denise Drake
Donna Guillory

The dictionary offers many definitions of the word “balance.” Perhaps the most commonly used description refers to the harmony and equilibrium that occurs through the synchrony of parts working together with equal force. When parts work together there is evenness and a sense of stability; when they don’t, there is disequilibrium or loss of balance.

The hereditary ataxias change the way that the area of the brain controlling coordination and balance performs. As a result, these diseases physically disrupt balance. From information in the National Ataxia Foundation’s “Frequently Asked Questions About Hereditary Ataxia,” the word “ataxia” comes from the Greek word ataxis, meaning “without order” or “incoordination” (2000). But balance can occur even when order is disrupted, as long as something also acts as a counterbalance.

Classification of Ataxia
Ataxia is a progressive, degenerative, neurological disorder. In the years since it was identified, it has gone through several levels of classification, from very simple to very complex. With the discovery of genes and the progress of genetic research, classification has become more sophisticated and more accurate. Ataxia is classified as either sporadic or inherited. Sporadic ataxia is not an inherited condition, and is a by-product associated with other medical processes. Inherited ataxia is passed on through genes carrying the abnormal disease tendency. Inherited ataxia may be dominant or recessive. Dominant ataxia is passed directly from an affected parent to his child. If the child does not inherit the affected gene, he cannot pass it on to his own offspring. There is a 50-50 chance that a child born to a parent with dominant ataxia will develop ataxia, as it takes only one abnormal gene to produce the disease. Recessive ataxia is passed on from parents who each carry the recessive gene. There is a 25% chance that offspring will receive an abnormal gene from each parent and thereby have the disease and pass it on. There is a 25% chance that an offspring will receive two normal genes and be free of the disease. There is a 50% chance that a normal and abnormal gene will be passed, and the offspring will not exhibit symptoms but can pass the disease to their offspring.

Symptoms of Ataxia
Balance problems and unsteadiness are the most common early symptoms of ataxia, and these usually progress over time until walking becomes so difficult that a wheelchair may become necessary for ambulation. Coordination is affected with shakiness and increased difficulty maneuvering movements of the arms and legs. Seemingly simple tasks such as holding a glass of water or a cup of coffee may become more and more difficult. Speech becomes thick or slurred, and there may be swallowing difficulties. There are often other disorders that affect those with ataxia, such as heart disease, diabetes, loss of hearing, spinal curvature abnormalities, and high-arched feet. Although these disorders are possible in those with ataxia, they are not seen in everyone affected.

A Balancing Act
Those affected by ataxia cannot control losing their balance physically, but it is very important that they not lose it mentally. Personal balance is far more important than physical balance. But it’s not always easy. No one wants to have to deal with a disabling disease. And there often seems to be a particular burden in dealing with a hereditary disease. No one wants to have to use a wheelchair. No one wants to need help to do normal daily activities. No one wants new odds. Denial, resentment, and fear are powerful opponents. It may seem easier to give in to the negatives. After all, that is what is expected of you, isn’t it? The effect of the shortsighted words and actions of others on your self-esteem is often the most difficult thing to balance.

It is not uncommon that disabled individuals get more stares than the average person, and the stares are often not rooted in kindness. Comments made out of ignorance or callousness still hurt. And even when the stares are not unkind, the scrutiny tends to place more marks in your personal "minus" column than in your "plus". It takes personal time to weed the ugly people from your life, and many are too preoccupied with what has happened to them to be able to do that. It is easier to retreat into the safety of a closed world where no one has to know that something is wrong with you. It may be easier to let others think that your slurred speech and staggering are the result of drugs or alcohol, or it may be easier to justify these indulgences because it hides your handicap. Fear can breed isolation, but detachment creates a loss of self. And suddenly, you truly lose your balance.

You must know and respect your limitations but not allow them to keep you from putting forth your best effort. The choice to accept the freedom to live to the best of your ability is much more powerful than constantly embracing your limitations or other’s expectations. Yes, there are certain inevitable changes that must be faced. Certain activities may become impossible to do safely, but many other activities can be adapted to an individual’s capability. There are always choices to be made, but they need not revolve around expectations. A disability does not define a person. Only the person can do that. Constantly questioning the “whys” can interfere with the time that can be better used to appreciate those things around you, those things that give you enjoyment, those things that help you to live to the best of your personal ability. One must find what they are capable of – what works for them. What works for one person may not work for another without adaptation. Adaptability is the key to balance.

Hereditary Healing Through Helping Hands
Although the Acadian population of Louisiana has shown a higher than normal incidence of hereditary ataxias, these diseases are not exclusive to this population. The record keeping that took place within this isolated populace allowed medical researchers to effectively chart family histories and note hereditary tendencies within extended families. The data compiled in the last decade, as a result of this population’s thorough genealogical research, illustrates the important findings that can emerge from knowing as much as you can about a disorder and about those that have been affected by it.

One of the greatest support mechanisms to those affected by hereditary ataxia emerges as a result of the awareness generated through patient health and education services like “Genetics of the Acadian People” forums. Open to the public and free to all who care to participate, this event presents the latest information in medical research and treatment. It provides access to physicians and other professionals dedicated to increasing educational information and offers “break-out” sessions designed to answer questions in specific topics areas, ranging from medical ethics issues to diet management. It puts a sharing process into motion. Professionals share their expertise and the latest information with their audience, and audience members share their personal experiences. There is a real sense of family fostered by the amount of caring and sharing that goes on at these events. And the sharing and caring allows healing.

How to Learn More
For more information contact the National Ataxia Foundation (NAF) at (763) 553-0020, or visit their website: http://www.ataxia.org or visit the LA Chapter NAF website at: http://www.angelfire.com/la/ataxiachapter/index.html

Contact the Author
Denise Drake
Email: dd3@prodigy.net

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