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INTRODUCTION
Huntington’s
disease (HD) is one of the most devastating diseases of mankind.
It incapacitates patients by affecting their ability to move,
think, and behave normally. It causes uncontrollable and disabling
movements of the face, neck, trunk and limbs, loss of balance,
and uncoordinated movements. In addition to these motor dysfunctions,
memory, thinking, and judgment become progressively impaired.
Furthermore, patients with HD exhibit a wide variety of psychiatric
problems. At the beginning of the disease, the patient’s
job-related capacity is frequently overestimated because they
can perform isolated tasks reasonably well for a short period
of time. In a real job environment, this is not the case. This
deceptively mild disability on a casual neurological examination
frequently delays qualification of the patient for the disability
status and income.
Physical disability and intellectual loss are already devastating
to HD patients and their families. However, severe psychiatric
problems are the ones that often render the entire family dysfunctional.
The disease can change the sweetest persons into angry, aggressive,
irrational individuals. To make matters worse, the caregivers
are frequently the main targets of HD patients’ aggression.
HD is an eventually fatal disease although the lifespan considerably
varies from patient to patient. There have been no medications
proven to slow down, halt, or reverse the progression of the
disease. However, many medications can alleviate many symptoms
of HD, particularly involuntary movements and psychiatric problems.
As the disease progresses, it overcomes the therapeutic effects
of these medications. It is important to note, however, that
cautious optimism exists for the future. Many scientists and
clinical investigators believe that recent advances in research
are about to change this dismal prognosis of HD.
HD patients and their caregivers need psychological, financial,
social, and legal supports. It is also very important to proactively
address a number of problems expected to occur in HD. Advance
directives and durable power of attorney play important roles
in deciding the treatment options in the later stages of this
disease. Protection of patients’ dignity often becomes
a challenging task as the disease advances. Educating patients
themselves, caregivers, family members, physicians, nurses,
and other healthcare professionals about the complex medical
and non-medical problems of this disease is essential for optimum
care. A multidisciplinary approach involving a wide variety
of healthcare professionals is an effective way to optimize
the care of HD patients. The HD Clinic at UTMB is established
to answer the complex needs by HD patients, caregivers, and
family members.
It is extremely important to let HD patients know that there
are teams of physicians and scientists all over the world challenging
the dismal outcome of this disease. Research is the best hope
for developing effective treatments to change the course of
HD. The hope has become increasingly promising. In 1993, the
genetic mutation causing HD was discovered. Before then, we
had no clue how the disease process was started. Since the
discovery of the HD gene, scientists have been accumulating
an increasing amount of data that are important for understanding
the disease-causing mechanism. Furthermore, the genetic mutation
of HD was experimentally introduced into animals. These “transgenic” animals
show a disease similar to human HD, and have become an important
experimental system to study the disease-causing mechanisms.
Transgenic HD animals are also used to screen drugs to find
new treatments for HD. These efforts are paying off. New drugs
that show promising results in animals are expected to come
to human therapeutic trials in the near future under the auspice
of the Huntington Study Group (HSG). As a member of the HSG,
the Department of Neurology at UTMB is preparing for participation
in such studies.
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March 06, 2008
