During my years as an internal medicine physician, I have used standard of care guidelines for my patients with conditions like diabetes and heart disease. Guidelines are developed by experts in each disease who translate clinical trial evidence and/or expert experience into recommended care patterns for use in medical offices or at the bedside. When followed, guidelines have been central to improving the quality of care provided by all physicians whether they are specialists or generalists.
Huntington's Standard of Care Guides
Though the Huntington's Disease Society of America (HDSA) has provided the "A Physicians Guide to the Management of Huntington's Disease" for more than a decade (3rd edition recently available), there have been no published guides that have been peer-reviewed by physician leaders from the European Huntington's Network (EHDN) or Huntington Study Group (HSG). Fortunately over the last 3 years both groups have spearheaded efforts within working-group committees to establish HD standard of care guides; however, this effort has proved difficult because individual experts may disagree, and there is lack of evidence from clinical trials to support any opinion.
Though it isn't equal to an evidence base, expert experience can be valuable. As part of a project to help develop HD treatment guides, I worked with EHDN and HSG doctors to identify (through surveys) the drug treatment patterns of a large number of international experts for three symptoms of HD. And also important to getting this information out, we worked to put the combined results into a format that would be useful, accessible, and could effectively fit into times frames of general physician office visits. Why is this important? General physicians and general neurologists provide medical care for many, indeed probably most, HD patients. Having easy access to the experts' care patterns can improve the quality of care delivered by busy doctors who have limited experience with HD and need time-efficient guides.
Expert Experience-based Treatment Algorithms
In three recent articles on PLoS Currents: Huntington's Disease, which can be down-loaded from PubMed (references below), I joined with HD experts from Europe, North America and Australia to present algorithms for the pharmacologic treatment of three HD symptoms: irritability [Groves M 2011], obsessive-compulsive behaviors [Anderson K 2011], and chorea [Burgunder JM 2011]. The algorithms are based on the combined knowledge and experience of more than 50 expert clinicians from Europe, North America, and Australia. Treatment algorithms, whether evidence-based or experience-based, are common in medical practice, providing a general and sequenced guide to care that is particularly useful in busy office schedules.
The entire articles and one-page summaries of the algorithms are freely accessible on the web, and can be printed for you or your doctor's use. You may want to print these out before a visit, and bring them to your doctor's office.
Algorithms Aren't Perfect
Algorithms, or any other standard of care tools are useful general guides for many but won't fit all patient care situations. Because each Huntington's patient is unique, drug treatment must be individualized. Unlike a recipe that gives a rigid step-by-step guide, algorithms give choice of alternative steps. Also remember that not all drugs are covered. Your doctor may prefer different drugs. It is imperative to tailor treatment to match the complexity of each patient. Further, these algorithms acknowledge that a first drug or combination of drugs may be unsuccessful, and there is need to try other drug or drug combinations. Specifically the algorithms:
(1) Give starting points, or the experts' first choice drug (or more than one) for irritability, chorea, and obsessive compulsive symptoms.
(2) Emphasize that first choice drug is greatly influenced by severity of symptom. For instance an SSRI drug (like citalopram or sertraline) is first choice if the irritability symptom is not severe or aggressive; but an antipsychotic drug (like olanzapine or risperidone) is first choice if it is severe or aggressive.
(3) Emphasize that first choice is greatly influenced by the presence of other symptoms. For instance, Xenazine® (tetrabenazine) should not be considered best treatment for chorea in a patient who is depressed, but that the majority of experts use this drug after depression is adequately treated.
(4) Point out the complexity of HD care by acknowledging that single drug treatments are often unsuccessful and require addition of other drugs or drug combinations. And often two or three trials may be necessary in more problematic patients. Or, don't stop if a first drug trial doesn't succeed -- try another.
Not All Inclusive: The algorithms do not cover drug treatments for all symptoms of HD. However the authors thought these three symptoms most important to study, because other HD symptoms (anxiety, depression, psychotic behaviors, insomnia, etc.) follow guidelines already established for these conditions in the general population.
Author's comment: Guides to care in Huntington's or any other disease -- no matter how good -- will not improve quality of care unless they get into the hands of the treating doctor. This can take a very long time without help from the HD community. For instance HD consulting doctors can supply the algorithm, or links to the articles (which are open access) to general doctors as part of longer term care for consult-referred patients. HD patients and families, particularly those who don't have access to HD specialty centers, can provide the algorithms and article link to your local doctor. Part of the strength of peer-reviewed guides -- as has been shown for other diseases -- is the ability to empower patients and families as partners in quality improvement in their own health care [Peterson PN 2011].
Groves M, van Duijn E, Anderson K, Craufurd D, Edmondson MC, Goodman N, van Kammen DP, Goodman L. An International Survey-based Algorithm for the Pharmacologic Treatment of Irritability in Huntington's Disease. PLoS Curr. 2011 Aug 30;3:RRN1259. PubMed abstract
Anderson K, Craufurd D, Edmondson MC, Goodman N, Groves M, van Duijn E, van Kammen DP, Goodman L. An International Survey-based Algorithm for the Pharmacologic Treatment of Obsessive-Compulsive Behaviors in Huntington's Disease. PLoS Curr. 2011 Sep 20;3:RRN1261. PubMed abstract
Burgunder JM, Guttman M, Perlman S, Goodman N, van Kammen DP, Goodman L. An International Survey-based Algorithm for the Pharmacologic Treatment of Chorea in Huntington's Disease. Version 2. PLoS Curr. 2011 Aug 30 [revised 2011 Oct 11];3:RRN1260. PubMed abstract
Peterson PN, Rumsfeld JS. The evolving story of guidelines and health care: does being NICE help?. Ann Intern Med. 2011 Aug 16;155(4):269-71. doi: 10.7326/0003-4819-155-4-201108160-00013. PubMed abstract