The annual General Meeting was held on March 20, 1999 in Northlake. Approximately 60 people attended this event which featured three speakers:
Through the support of grants from the Hereditary Disease Foundation and the Huntington's Society of America, the Huntington Study Group (HSG) Executive Committee and other HSG members have been meeting regularly to develop research studies of persons "at-risk" for Huntington's disease.
Two at-risk projects are under development to determine the best way to carry out a study of experimental treatments aimed at slowing or postponing the onset of Huntington's disease. Pharos (Pilot Huntington's Disease At-Risk Observational Study) is a prospective double-blinded examination of persons who are at-risk but who do not know their gene status. Rush-Presbyterian St. Luke's Medical Center is looking for 25 persons between the ages of 35 and 55 who are at-risk for developing Huntington's Disease to participate in this study. You will be followed over a 3-year period (meet with doctor every 9 months) to collect data which will determine early symptom development. During this 3-year period, you WILL NOT be able to undergo any testing at Rush to determine if you are a gene-carrier. If you choose to be tested during this 3-year period, you will become invalid to continue the study.
Predict-HD (Neurobiologic Predictors of Huntington's Disease Onset) is a study of the neuropsychological, behavioral, MRI and PET correlates in healthy people who know their gene-status.
In order to find out if people at-risk for Huntington's disease might be interested in participating in these studies, the HSG has developed a Confidential Survey of Persons At-risk for HD. Over 200 people took this survey at the HDSA convention this year in Denver.
If you are interested in participating, please contact Dr. Shannon at 312/942-4500.
For the individual with HD, special considerations need to be made. The swallowing function may be affected and progressively weakened in the course of HD. There are four phases to swallowing and in each phase, there is potential for trouble.
The first phase is voluntary and involves the act of eating - biting and chewing food. This involves the tongue, cheek, lips, and various muscles of the mouth and face. If mouth and facial muscles are affected by HD, this can result in an attempt to swallow an excessively large piece of food and/or insufficient chewing of food. Choking can result.
The second phase is also voluntary. This is when the tongue propels food back towards the pharynx, the back portion of the throat. Good control of the tongue is needed for adequate command of this phase. Poor control can lead to foods being swallowed too quickly, resulting in food entering into the lungs instead of the esophagus (which leads to the stomach); also known as aspiration. That is why thin liquids like water should be avoided or taken by teaspoon only.
The third phase is the pharyngeal phase. This is involuntary; it involves the automatic closure of the vocal cords to prevent food from entering the lungs. As noted with phase two, foods swallowed too quickly may end up in the wrong place.
Phase four is the esophageal phase. This phase involves involuntary muscle contractions which push food down toward the stomach. When muscles are weakened the food moves slowly and can cause discomfort. Aspiration of food into the lungs is a serious consequence of swallowing problems. Prevention of choking is critical. The signs and symptoms of swallowing problems include:
An evaluation by a speech therapist is recommended. Be aware of the signs of choking, and also be familiar with the Heimlich maneuver. This is the first line of defense when choking is occurring.
Created and maintained by Renette Davis. Send comments to Renette by clicking here.
Created: July 12, 1999
Last updated: Dec. 1, 2010