The Huntington's Disease Society of America (HDSA) has designated the Barnes-Jewish Hospital and Washington University Department of Neurology as a Center of Excellence for Huntington’s Disease care. Our center offers the best clinical care and services for those affected by Huntington’s disease in the Midwest area, including Missouri, Kansas, Iowa, Arkansas and Southern Illinois.
Understanding Huntington's Disease
Huntington's disease (HD) is a hereditary degenerative brain disorder which leads to both physical and mental disabilities. Symptoms usually appear between the ages of 30 and 50, but can begin as early as two and as old as 80. Symptoms range from forgetfulness, personality change, and involuntary movement to difficulty swallowing, although each person is affected individually and may display different symptoms.
Every child born to an affected person has a 50 percent chance of inheriting the gene which causes the disease. It affects women and men, as well as all races. Currently there is no cure or treatment for the disease; however, symptomatic treatments are available and a great deal of research is ongoing throughout the world.
As a Center of Excellence, we are committed to providing comprehensive services for individuals with Huntington's disease and their families.
- Pre-symptomatic genetic testing
- Occupational therapy
- Support groups
- Genetic counseling
- Research opportunities
- Information and referral
- Social work support
- General HD information
- In-home visits/nursing home visits (limited area)
- Professional education/speakers’ bureau
Pre-symptomatic Genetic Testing
The HDSA Center of Excellence at Washington University School of Medicine offers pre-symptomatic genetic testing combined with patient education to at-risk individuals interested in learning their HD gene status.
People who have a family history of HD—and therefore an increased likelihood of being diagnosed with it—can undergo predictive genetic testing. This testing reveals whether or not they have inherited the HD gene, before any symptoms start.
The decision of whether or not to have predictive testing is a personal one, and not to be made lightly. The implications of learning a person’s risk status for developing this degenerative illness are far-reaching, so it is important that those seeking this testing receive adequate support, education and assessment of readiness for this information.
What to Expect with Pre-symptomatic Genetic Testing
Some of the issues to consider before testing include cost/insurance matters, how you will handle the results emotionally, how your family will be affected, practical considerations for long-term planning, and other concerns.
The costs associated with pre-symptomatic testing are used to cover the genetic counseling, neurology assessment, neuropsychiatry assessment, the genetic test itself, and in some cases counseling afterwards. When you contact us about predictive testing, we will discuss your payment options with you. Please contact us for detailed information about the cost of testing.
Preparation and Testing
An individual interested in going through pre-symptomatic genetic testing will first talk with our center social worker. During this conversation, you will discuss your family's history of HD, your motivation for testing at this time, and the schedule of our testing protocol, if it is decided that you wish to proceed. Costs of testing will be discussed prior to beginning the testing protocol. Testing involves at least three visits to our center:
The first visit is a lengthy one. You will meet with the social worker for genetic counseling and a discussion of the practical steps to take before receiving your results. You will next meet with the neurologist for a neurological exam to determine if there is any evidence of manifest HD. Next you will meet with the neuropsychiatrist for an assessment of your emotional and behavioral risks. While it is rare, it is possible that after these initial appointments the pre-symptomatic testing team may determine that it is not appropriate to move forward with testing or may recommend delaying testing until a later date. In these cases, the cost for the initial appointments is not refundable.
During the second visit you will meet with the social worker who will accompany you as your blood is drawn for the genetic test. This visit is a minimum of one month after your initial counseling appointments, as described above. Any additional questions will be answered at this time.
The final visit occurs no less than one month from the date of the blood draw. This is where your test results will be revealed to you. Both the center social worker and the neuropsychiatrist will be present. At this time, regardless of results, we may recommend, or you may choose to have, ongoing counseling and support. Please discuss this with our center social worker if you have questions.
We strongly recommend that you have a support person with you for all of your visits to our center. Generally, it is best if your support person is not also an at-risk person. We will consider requests outside of our recommendations on a case-by-case basis.
It is acceptable for you to decide to forego testing at any point during the protocol. For example, if after your initial counseling appointments you decide not to have your blood drawn for testing you may tell us and we will cancel any future appointments. You may also, even after having the blood drawn, decide not to receive the results of your test. In this case, we will hold the copy of your results and if in the future you wish to receive them, they will be available to you. We may require additional counseling visits if it has been a long time since the testing. Any costs already incurred will not be refunded, but you will not be charged for any services not yet provided.
Confidentiality is an important feature of the predictive testing protocol. There are numerous safeguards in place to protect your confidentiality. At this time, anonymous testing is not available.
Important Considerations of Predictive Testing
- We will not test at-risk, non-symptomatic minors. There are no exceptions to this policy.
- We can provide counseling and referral for those who wish to consider prenatal genetic testing or receive information on pre-implantation genetic testing. In some cases, this can be done without disclosure of the genetic status of the mother.
- In some cases, the results of one individual may reveal another person's gene status, such as in the cases of identical twins or in cases where the parent of the person seeking testing is at-risk but not symptomatic.
In all of these situations, significant counseling is required before going forth with the testing protocol.
For a referral to a Washington University neurologist or neurosurgeon at Barnes-Jewish Hospital, call 855.925.0631.