A short while ago a patient of mine died after a long struggle with Huntington's disease. Here I tell the uplifting story about him and the last four years of his life. This story would not have been the same -- for him or for me -- without Elizabeth, our mutual and ordinary/extraordinary friend.

Over the years there have been a few studies regarding the risks of substance abuse with tobacco, alcohol or other drugs in Huntington's disease (HD). Does using -- or abusing these substances cause an earlier onset of disease or faster rate of progression in Huntington's disease?

Although "common sense" and anecdotal experience suggest that these substances are likely harmful in both the short and long term in HD, what can we learn from the scientific literature?

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?