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Issue 99, December 2007

Huntington's Disease World Congress

Kim Smith, Auckland Committee Chairperson and Jo Dysart, Auckland Family Liaison Coordinator attended the Huntington's Disease World Congress in Dresden Germany. The three-day World Congress was preceded by two days of the European HD Network. The following summary, written by Kim, encompasses these five days in a few sentences. If you are interested in the full report from Kim please contact her at kim.smith@pobox.com or 021 336 757.

  • There is an increasing recognition that the disease is much more than a movement disorder. The cognitive and psych symptoms are just as, if not more important to deal with and there is increasing research in those two additional areas.
  • The disease is a family one and more attention should be paid to how families are affected by the disease, not just the person afflicted with it.
  • There is a real push for an integrated, multi-disciplinary approach to care combining several specialists as well as social services (sound familiar? It should, it's exactly what we are creating in Auckland).
  • Most current research is done on late stages of the disease. Drug therapies are primarily geared towards halting cell death, switching the gene off or interfering with the RNA, which eventually creates the toxic protein that results in cell death. There was a real call (and the beginnings of some research) for study into what is happening to the brain pre-diagnosis. What can be done when the neurons are just compromised, before they die?
  • There was another call that said that we've learnt what we need to know about the mechanisms of the disease. We certainly don't know all about how it works, but there was a general feeling that we know enough so that some of the research can be shifted more into what to do about it. How to halt or slow its progression or how to ensure that gene positives don't become symptomatic or that that day is delayed far into the future.
  • There was some discussion of the Track HD group, which is being run by the European Network. This is a multi- centre, multi-national biomarker study, which is studying early and pre-symptomatic HD patients with the aim of developing a methodology to undertake clinical trials of potential therapies in this population.
  • The European Network will be coming up with behavioural and cognitive rating training videos to make diagnoses of these symptoms more standard.
  • Weight loss is now starting to be taken very seriously in HD Patients. It has been shown that weight loss can affect the patients' movements as well as cognitive and behavioural symptoms.
  • The hot topic of research these days seems to be RNA inhibitors. Now, I'm not a scientist by any means, but as I understand it…the problem gene is of course, in our DNA. This DNA is translated to RNA (don't ask me what that actually is) and the RNA then creates the protein, which aggregates, turns toxic and kills the cells. RNA therapies are about destroying the message that is carried into the cells that tells them to aggregate the protein. If we can do that, the protein will not be expressed in a toxic way, such as it is in people with normal CAG repeat counts.
  • Jimmy Pollard, a nursing home administrator who has worked with HD patients and their families since the mid 80's gave two sessions on how people with HD think and how we, as care givers can make our lives and theirs easier. See synopsis below:
Jimmy started out by saying that the cognitive characteristics of HD include the following:
  • Slower thinking
  • Recognition easier than recall
  • More difficult to change topics
  • Difficulty organising and planning
These cognitive characteristics create:
  • Apathy
  • Short fuses
  • Irritability
To accommodate for the above caregivers need to:
  • Make sure there are no surprises (always let the PHD know what's coming)
  • Do things in the same sequence or order (the example he gave was bathing…always do body parts in the same sequence and let them know what's coming next).
  • Go slower but not slow
  • Preview what's to come…tell them "At 2pm you and I are going to the store".
  • Do a play by play of what's happening, "okay we're almost at the store, only about another 5 minutes. We're just passing by…."
  • Recognise milestones, "we're about half way through your bath".
  • Have a clear beginning and ending.

Remember, with these cognitive challenges things that people with HD used to do automatically now take a lot of effort. Combine that with having to concentrate on you and they may feel frustrated or exhausted. Let them catch up. Don't ask them a question and not wait for an answer. Them not answering doesn't mean they don't want to or the answer is negative.

When working with people, especially in later stages keep them involved. A lot of people ask what types of activities you can engage people in, especially in later stages. His answer was anything! They just enjoy being with you…even though they may not show it.

He ended his session by explaining the HD disguise or mask as he called it. Because of what HD does with muscle tone and posture, people suffering from the disease often have body language that conveys, "He doesn't understand, He's not paying attention, or He just doesn't care". Add to that the cognitive disguise (delays in responding and finding it hard to form answers) and caregivers (especially loved ones) feel distant from the person they are caring for. It's difficult, certainly but if you follow the above recommendations and learn to look past the disguise an intimate relationship can be maintained well into the disease.

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