Faces of HD – Katelyn and 2 of the button brothers at June farm meeting

Huntington’s disease (HD) is an autosomal dominant genetic mutation in either copy of an individual’s Huntingtin gene which results in a neurodegenerative disorder that causes nerve cells in certain parts of the brain to waste away.  It affects muscle coordination, resulting in abnormal writhing movements called chorea, and leads to cognitive decline and psychiatric problems. It typically becomes noticeable in mid-adult life, although there is a juvenile form.

To help bring a treatment to clinical trials donate at The Shepherds Gift: GM1 for HD

Produced by Aaron Cooley, Brookings High School Student

For more information visit the following links:







See www.hdsa.org for more information and resources.

Contributed 2/24/13 by Tricia Wempe
BS 97 Psychology, MA 01 Political Science, University of South Dakota

HDSA Clinical Trial Diplomat http://www.hdsa.org/research/clinical-trials-1/cldip/clinical-trial-diplomats-map/ar.html,

Lifetime Costs of HD – Estimated burden to society in economic terms Lifetime Costs: The average yearly health care and living expenses and the estimated lifetime costs that are directly attributable to HD vary greatly according to the stages of disease progression. It is known that the latest research estimates the duration of HD to be 25 up to 30 years http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2569211/). This disease is a long-term illness. Two types of costs are associated with the treatment of HD, they are indirect costs and direct costs. Please keep in mind that the discussions below are preliminary findings, have not yet been peer-reviewed, and the data below came from personal communications from Dr. John Warner, Director of Biostatistics at CHDI Management/CHDI Foundation and Dr. Nancy Downing, Assistant Professor at University of Iowa (UI). Indirect Costs: Indirect costs include loss of wages, fringe benefits, and productivity. People with HD typically have to stop working early and the loss of income is significant. Dr. Downing’s (UI) calculations, which again are preliminary, provide a good faith estimate based on available data. Please note that the figures below represent widely accepted statistics among professionals in a variety of fields which would consider the sources legitimate and trustworthy.  For example, the median annual US income for 2012 was $62,273. This information was taken from the US census website: http://www.census.gov/newsroom/releases/archives/income_wealth/cb12-172.html Consider the course of HD is 15-20 years post onset of motor symptoms, therefore, this represents a potential loss of 15 years of income (being conservative). Therefore 15 X 62,273 = $934,095 in personal loss of income over the disease course. It is known that the loss of income would vary substantially based on education, the age of onset due to CAG repeat, and employment history prior to disablement. If you multiply that by the number of people with diagnosed HD, now estimated to be 10:100,000 (2 websites below provide prevalence statistics): http://www.thelancet.com/journals/laneur/article/PIIS1474-4422(10)70287-8/fulltext and http://www.hdsa.org/ohvch/ohvhd/index.html Current population (as of today = 315,363,366): http://www.census.gov/population/www/popclockus.html Divide that by 100,000 = 3,153 X 10 = 31,530. 31,530 X $62,273 = $1.9 Billion United States Dollars (USD) each year of  lost income. This represents a very significant amount of earned income that is lost due to HD. This represents money that would have been used to provide food, shelter, transportation, and educational costs for themselves and their family. Direct Costs: Dr. Warner, CHDI, provided a poster that was presented at the International Society for Pharmacoeconomics and Outcomes Research (ISPOR) conference last June 2012. This poster gives a good summary of the direct costs portion of our analysis. The statistics below and discussions to follow were contributed by Dr. Warner from CHDI. It needs to be noted that this is unpublished data and is rough approximations.   The following are cost estimates by insurer type and disease stage.

Commercial Medicaid Average
Early 4,947 3,257 4,102
Middle 15,066 12,330 13,698
Late 22,582 37,495 30,039

Dr. Warner assumed that patients will spend 10 years in each stage of the disease so that total lifetime direct costs per patient would be about $478,385. Assuming that the incidence of HD in the US population is 1 in 10,000 it follows that there are approximates 30,000 HD patients. Furthermore, if it is assumed that HD patients are equally likely to be in each of the three stages of the disease, it follows that the total annual direct costs of HD in the US are about $478 million. Please bear in mind that the calculations in the above paragraphs are very approximate. Dr. Warner states, “Also, note that they take only direct costs into account. We are currently working on developing estimates of indirect costs (including lost earning for HD patients and caregivers) which may be much larger than the direct costs. Indirect cost estimates will be based on a survey instrument that has been developed but not yet fielded.”