Obsessive compulsive behaviors (OCBs) occur frequently in Huntington's disease (HD) and can cause great distress. These behavior symptoms are often hard for patients and families to describe, and doctors often do not routinely ask about or treat them. It is important for you to recognize OCB symptoms and learn about treatment, because, though it is estimated that as many as 50% of HD patients experience these symptoms, a study of European HD specialty centers found that fewer than 2% were prescribed OCB-specific medications. This is unfortunate because OCB treatment can make a big difference in the quality of life for both HD individuals and care-partners.

Though there has been little research done on this symptom in HD, there is much you can learn from the experts . .

According to two recent studies from the United Kingdom, bodily discomfort (which refers to total body physical suffering) and pain (which refers to localized physical suffering) are problems in Huntington's disease (HD) that significantly affect quality of life. Both studies, which included a total of 140 home-dwelling individuals in middle stages of HD, reported that more than 50% suffered from at least moderate discomfort/pain, and about 10% more severely. This is a large number, much higher than occurs in an age-matched general population. Unfortunately, very little is known about this symptom in HD . . .

CHDI Foundation, privately funded by a small group of anonymous donors started work on Huntington's disease (HD) about a decade ago. Many have wondered what criteria this organization would require to launch a new drug into clinical trial. The PDE10A inhibitor story answers that question and shows the careful and powerful process that CHDI utilizes before promoting a drug candidate to clinical trial. "Aligning the stars" has nothing to do with serendipity or luck. It is about a lot of hard work, with the CHDI role that of "collaborative enabler" with financial clout: to guide, support, and coordinate a multitude of scientists and drug developers.

Get your telescope ready: The stars are being moved into place...

What is empathy, and what does it have to do with Huntington's disease (HD) care? Social scientists define empathy in many different ways, but it boils down to the complex neurobiologic, emotional process that allows us to "walk in someone else's shoes". This is different than sympathy or compassion, which corresponds to our feeling "for" another. Empathy goes a big step further to feeling "as" another. Empathy is an active process, because "walking in someone else's shoes" requires learning and understanding what the other is going through.

What's empathy got to do with it? Empathy can take HD "care" to a higher level.

Anosognosia is the medical term for lack of self awareness of one's own disability or disease symptom. This condition, which is different from denial, occurs in many brain diseases including Huntington's disease (HD). Anosognosia is caused by nerve circuit damage to brain structures that interpret and organize information. Lack of awareness in HD creates problems that can range from refusal of therapy: "I don't need treatment if I'm not ill" to more extreme situations that can be dangerous for HD individuals or others. Families know that dealing with this symptom when it is severe -- is like walking through an emotional minefield, knowing the explosion is likely to come.

In a recent article by Gene Veritas at curehdblogspot.org, Jonathan Monkemeyer suggested that "advocates must actively participate in the search for treatments, perhaps even trying drugs and substances approved for other purposes in their own off-label studies, seeking advice from researchers on dosing, and having people reporting their observations via a website". While I agree with the first part of the statement, I have some concerns about the remainder.

No question that we need strong HD family advocates like Jonathan to increase HD awareness and bring new ideas. However . .

Predictive testing for Huntington's disease (HD) isn't just hard, but likely comes closer to the description given by Charles Sabine, a former NBC war correspondent. He said it was more terrifying than having a rebel hold a gun to his head. No wonder the HD "at risk" community and their families and friends struggle with testing decisions.

What to consider while making this decision for predictive testing? And if the decision is to test, what is the recommended procedure?

Many families dealing with Huntington's disease (HD) know too well that most of their community doctors know little about HD. But why do they know so little? The answer is quite straightforward: U.S. doctors get only a few hours of HD education over their entire 5 to 8 years of medical education and training. No wonder they have difficulty providing care for patients with this complex disease. This problem is huge because in any given year, the majority of HD patients get all of their medical care from doctors who have not been well educated. How can this problem be solved?

More than 250 individuals from Huntington's disease (HD) families and their friends participated in the Huntington Study Group (HSG) Symposium and Clinical Research Workshop held in Seattle on November 10, 2012. Attendance was higher in Seattle than any of the prior cities hosting the symposium over the previous 5 years. At this "one of a kind" meeting HD families sat at circular tables with clinical researchers from all over the world, sharing information, hearing about new research -- and most important -- learning from each other.

The day was divided into 3 parts: symposium, poster, and workshop sessions . . .

HSG-2012-Huntington’s Disease in Clinical Practice

Dates: 11/8/2012 – 11/10/2012

Tuition: Free for preregistration, $25.00 at the door. Location: Hyatt at Olive 8, 1635 8th Avenue, Seattle, WA

Course Director: LaVonne Veatch Goodman MD, in coordination with members of the Huntington Study Group Education Committee

Course Description: This course will coincide with the Huntington Study Group Annual Meeting and Research Symposium. Despite the availability of symptomatic therapies, Huntington’s disease patients and families are often under-treated. To address this problem, national and local Huntington’s experts will present practical information for community providers (physician generalists and subspecialists, nurses, and social workers) on the symptomatic treatment and care for Huntington’s disease patients.

Topics on the first two days will include current expert recommendations for genetic counseling, treatment of both motor and behavioral/psychiatric symptoms, use of ancillary speech and physical therapies, advocating for disability benefits, and palliative care for late and end-stage patients. On the final day, there will be a poster session and presentations by the experts on recent clinical research.

Thursday, November 8, 2012 (3 CME Hours)

2:00-3:00 pm

Registration

3:00-3:30 pm

Welcome and Introduction

Ira Shoulson, MD

LaVonne Veatch Goodman, MD

3:30-5:00 pm

Breakout Sessions

UHDRS Training

Physical, Occupational and Speech Therapy for HD

Ramon Rodriguez, MD

Karen Anderson, MD

Leigh Beglinger, PhD

Ashwini Rao, EdD, OTR

Deanna Britton, PhC, CCC-SLP

5:00-5:30 pm

Break

5:30-6:30 pm

Evolution of Genetic Counseling for Huntington’s Disease

Clare Gibbons, MS, CCGC, CGC

Thomas Bird, MD

Susan Creighton, MS, CGC

Corrie Smith, MS. CGC

6:30-7:30 pm

Welcome Reception

Friday, November 9, 2012 (7 CME Hours)

7:00-7:45 am

Registration and Breakfast

7:45-8:00 am

Welcome and Introduction

LaVonne Veatch Goodman, MD

8:00-8:30 am

Clinical Genetics of HD

Thomas Bird, MD

8:30-9:00 am

Overview of Pre and Early HD

Blair Leavitt, MD

9:00-9:30 am

Overview of Middle Stages of HD

Ali Samii, M.D.

9:30-10:00 am

Break

10:00-11:00 am

Successfully Advocating for HD Disability

Sam Frank, M’D.

Jane Kogan, LMSW

Bonnie L. Hennig, MSW, LCSW, QCSW, DCSW

11:00-12:00 am

Overview and Management of Late and End-stage HD

Martha Nance, MD

12:00-1:00 pm

Lunch

1:00-3:00 pm

Behavioral Treatments for HD with Case Presentations

Mary Edmondson, MD.

Karen Anderson, MD

Mark Groves, MD

3:00-3:30 pm

Break

3:30-4:30 pm

Identification and Management of Motor Symptoms of HD

Ralf Reilmann, MD

4:30-5:O0

Support Services and Clinical Research Opportunities in the Northwest

Chris Wick, MSW, LICSW

LaVonne Veatch Goodman, MD

Saturday, November 10, 2012 (3 CME Hours)

Sixth Annual Huntington Disease Clinical Research Symposium: 8:00am -12:30pm

Symposium Poster Session from 8:00-9AM

The Symposium will feature keynote addresses beginning at 9:00am by:

  • Charles Sabine, HD Advocate and Mary Edmondson, MD, Duke University Medical Center
  • Ashwini Rao, EdD, OTR, Columbia University
  • Pierre Tariot, MD, Banner Alzheimer's Institute
  • Blair Leavitt, MD, University of British Columbia

In addition to these keynote addresses, clinical scientists will be presenting their original research on relevant topics of interest to the HD community today. The Symposium will conclude with an open panel discussion, which will present audience members with the opportunity to interact with the day's presenters.