Everyone living with HD should "refresh" themselves on the principles of treatment for HD periodically.
Every physician with an HD patient should have a copy of this book too which is free to physicians requesting one from the HDSA 1-800--345-4372 or the online version can be found at HDL http://hdlighthouse.org/see/pg2/0.htm
- The medication list and the rationale for each medication needs to be reevaluated at regular intervals.
- Sometimes the most helpful intervention a physician can perform is to discontinue an unnecessary drug.
- HD patients are highly vulnerable to side effects, particularly cognitive side effects, of medications.
- The physician should begin with low doses and advance medicines slowly.
- Pharmacologic interventions should not be launched in isolation, but in a setting of education, social support, and environmental management.
- Patients should understand that there is no substance, no matter how "natural," which has pharmacologic activity without side effects, and that all treatments carry an element of risk.
A Physicians Guide to the Management of Huntington's Disease
Principles of Treatment
Caring for patients with HD is both challenging and rewarding. At times, the lack of definitive treatments can be frustrating, but careful attention to the changing symptoms and good communication between professionals,
family members, and affected individuals all contribute to the successful management of the disease.
HD is a progressive disease. The symptoms evolve over time such that treatments which were effective in the early stages may be unnecessary, or problematic later on, and vice versa.
For example, medications such as neuroleptics may be started in the early to middle stages to control chorea. However, this category of medications may exacerbate the rigidity and bradykinesia of the later stages, and result in delirium or oversedation as the cognitive disorder progresses.
The medication list and the rationale for each medication needs to be reevaluated at regular intervals.
Sometimes the most helpful intervention a physician can perform is to discontinue an unnecessary drug.
Symptoms vary over time as a patient passes through different stages of the disease. Symptoms also vary fromindividual to individual, even within a family. For example, one patient may develop a severe mood disorder,
requiring multiple hospitalizations, but have little motor disability. The patient's brother may have debilitating motor symptoms, but no mood disturbance at all. Thus interventions need to be tailored to individual symptoms,and fearful patients should be reassured that their symptoms may not necessarily resemble those of their relatives.
HD patients, like others with injuries to the brain, are highly vulnerable to side effects, particularly cognitive side effects, of medications. The physician should begin with low doses and advance medicines slowly.
Polypharmacy should be avoided where possible. Many of the drugs used in treating symptoms of HD, such as neuroleptics and antidepressants, will not have immediate efficacy and patients need to be told that they may feel worse before they feel better, because they will experience the side effects, before the beneficial effects have appeared.
"HD patients... are highly vulnerable to side effects, particularly cognitive side effects, of medications."
Pharmacologic interventions should not be launched in isolation, but in a setting of education, social support, and environmental management. Symptomatic treatment of HD needs to be approached like any other medical problem.
The clinician should elicit the details of the symptom, its character, onset and duration, and its context including precipitating, exacerbating and ameliorating factors.
A differential diagnosis should be generated, non-pharmacologic interventions should be considered, and the clinician should have a way ot determining whether the goals or treatment are being met and should formulate a contingency plan if treatment is not working. Sharing some of this reasoning process with patients and famlies can be reassuring.
A few words should be said on the issue of "alternative treatments" for Huntington's disease -unproven remedies such as herbs, megadose vitamins, homeopathic preparations, or magnetic devices, which are to be distinguished from experimental treatments taking place as part of a scientific study.
Patients should be encouraged to discuss their ideas about these therapies and not to be afraid to tell their physicians that they are trying them. This will allow the doctor to help the patient think through the pros and cons of such a decision, to avoid notoriously dangerous or ineffective nostrums, and to monitor for side effects.
Patients should understand that there is no substance, no matter how "natural," which has pharmacologic activity without side effects, and that all treatments carry an element of risk.
We have found it useful to share certain caveats with patients to minimize the risk for those who have chosen to pursue these alternative therapies:
1) Don't spend too much money
2) Don't do something that common sense suggests is dangerous and
3) Don't neglect or discontinue effective medical treatments in favor of an unproven therapy.
By following these principles patients are likely to avoid harm.
I read this on a PD news update and know they probably apply to HD too, so it might help us to know what the desired outcome is in research/clinical trials etc, ie WHICH type of treatment for HD is being considered!
Treatments that improve symptoms
Those that slow the progression of disease are neuroprotective because
they protect or slow the loss of cells.
Those that increase cells or cell function