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 that work 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 common and increasing 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 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 that are 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 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