Although there surely are others, four non-drug "things" that this author believes can make a huge difference for those with HD or at risk of it is the focus of this article.  The "things" include:

  • Exercise for body and brain
  • Knowledge of and early recognition and management of symptoms
  • Healthy Sleep
  • Attention to carer needs

Although a study testing bupropion (Wellbutrin) for treating apathy has recently been completed, there has been no high level study of any drug for treatment of any behavioral or neuropsychiatric symptom in Huntington's disease (HD).  Nor is there study on adverse side effects of the drugs commonly used in HD.  However experts believe, and families know that available drugs can be helpful. Unfortunately there is little available guidance for doctors or families on when and how to use these drugs, or what side effects to look for.

Lack of guidance leads to opposing problems: Some with HD have too little drug therapy, while others likely have too much . . .

Psychosis is a severe but relatively rare behavioral/neuropsychiatric symptom in Huntington's disease (HD).  Psychosis in HD is defined by the presence of delusions or hallucinations.  Delusions are fixed beliefs not grounded in reality.  Hallucinations are the sensory experience of seeing, hearing, or feeling something not based in realtiy.

Psychological distress and psychiatric symptoms are terms that we can use to describe a range of situations that occur in Huntington's disease (HD) that are emotionally troubling and have negative impact on how we function as individuals and families. And we certainly have more than our share of psychological distress living and and coping with this disease.

All in our community, families and professionals alike tend to describe various symptoms, like depression, anxiety, irritability and others as if they are rigidly separate disease symptoms. Though there can be value in separating symptoms, this author believes this separation may also create barriers to understanding and optimally treating the individual with HD experiencing them.

There have been a number of studies about the impact and burden of Huntington's disease (HD) on both those affected and their family carers. Though there are many other factors, the major recurring theme boils down to "lack of care". This includes lack of access to HD subspecialty medical care, lack of community medical or service provider knowledge about HD, and lack of support for family or other carers. It is unfortunate that the magnitude of burden imposed by "lack of care" for HD has not substantially changed over the two decades or so covered in these studies.

Care is described as informal when provided by unpaid family members or friends, and formal when caretakers are paid. Though a small subset of individuals with HD are cared for in the home through end of life, formal paid home healthcare services are frequently utilized. However the vast majority of individuals with HD, as the intensity of care needs increase will transition to long-term care facilities. In this situation, many family caretakers continue to advocate for and provide care.

What is known about informal care in HD?

A recent article reports that aggression is common in individuals with Huntington's disease. Authors report rates of aggressive behaviors between 22% (for clinic patients) and 66% (for hospitalized patients) among individuals with HD. What do they mean by aggression, and how should we interpret these numbers? It is important to remember that aggression (as defined in this article) and violence are NOT the same thing.

However, the most important thing to learn is not how often it occurs, but how we can understand, cope with and treat aggressive behaviors in HD.

Auspex Pharmaceuticals announced quite positive results for their drug SD-109. This drug treats chorea with many fewer side effects associated with tetrabenazine. And most important it improved quality of life.

Particularly during this past year, living with Huntington's disease (HD) has been full of its ups and downs. First there was the hope in ongoing clinical trials of both coenzyme Q-10 and creatine. Then there was sadness and fear that came with their failures. And now hope is springing again for new drugs coming to clinical trials now that will take a few years to complete. No doubt this Yin-Yang cycle will continue for HD, just as it does for other diseases.

However, there may be reason to believe in the chance of greater success this next time around.

"Bummed" was what this author felt after the recent announcement of the failed trial of creatine for Huntington's disease (HD). We can put some positive spins on recent trial failures; we have learned how to run large clinical trials, we can learn from negative outcomes, and best of all hundreds of HD individuals are freed up to participate in other (potentially better) trials. But the reality is that this is one more disappointing failure in an increasingly long string of negative trials. The community is bummed. Saying it straight is better, helps us to get over it, get up and go on to what comes next.

A story comes to mind . .