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The ALS Association is proud to host this American Academy of Neurology presentation on ALS practice guidelines. Report of the Quality Standards Subcommittee of the American Academy of Neurology. - PowerPoint PPT Presentation
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© 2010 AMERICAN ACADEMY OF NEUROLOGY
The ALS Association is proud to host this American Academy of Neurology presentation
on ALS practice guidelines.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Update: The Care of the Patient with Amyotrophic
Lateral Sclerosis Report of the Quality Standards
Subcommittee of the American Academy of Neurology
R. G. Miller, MD, FAAN; C. E. Jackson, MD, FAAN; E. J. Kasarskis, MD, PhD, FAAN; J. D. England, MD, FAAN; D. Forshew, RN; W. Johnston, MD; S. Kalra, MD; J. S. Katz, MD; H. Mitsumoto, MD,
FAAN; J. Rosenfeld, MD, PhD, FAAN; C. Shoesmith, MD, BSc; M. J. Strong, MD; S. C. Woolley, PhD
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Presentation Objectives
• To describe the process of making an evidence-based guideline
• To present the findings on and recommendations for care of people with amyotrophic lateral sclerosis (ALS)- Drug, symptom, nutritional, and breathing therapies - Multidisciplinary care in specialized ALS clinics- Behavioral and thinking problems
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Practice Guidelines
Evidence-based statements to helpdoctors and patients make smart decisions about health care
© 2010 AMERICAN ACADEMY OF NEUROLOGY
AAN Guidelines Are Evidence-based
?
Evidence-basedConsensus-based
© 2010 AMERICAN ACADEMY OF NEUROLOGY
AAN Guideline Process
Clinical Question
Evidence
Conclusions
Recommendations
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Clinical Questions
• The first step in developing guidelines is to ask important clinical questions.
• Questions look at areas of medicine where practice varies because of doctors’ understanding, beliefs, or attitudes.
• Questions must be answerable with published data.
• Answering the question must have the potential to improve care/outcomes.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Strong Weak
Randomized Masked Study
SingleCase Report
Class I Class II Class III Class IV
Rating the Evidence
Level A B C U
© 2010 AMERICAN ACADEMY OF NEUROLOGY
AAN Level of Recommendations
• Strong evidence = more than one high-quality scientific study
• Good evidence = at least one high-quality scientific study or two or more studies of a lesser quality
• Weak evidence = the studies, while supportive, are weak in design or strength of the findings
• Not enough evidence = either different studies have come to conflicting results or there are no studies of reasonable quality
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Introduction
What is ALS? • ALS is a disease of weakening muscles. It is also called
Lou Gehrig’s disease. The cause is unknown.• In ALS, certain nerve cells in the brain and spinal cord
become damaged. The affected cells are called motor neurons. These nerve cells send signals to the muscles in your arms and legs, and to the muscles for speaking, swallowing, and breathing.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Introduction
Is there a cure for ALS? Will I die from it?
• ALS is serious. However, several therapies are now available to make life longer and easier.
• ALS progresses quickly and eventually leads to death. Most people with ALS die within three to five years from the onset of their first symptoms. However, 20% of people live longer.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Drug Therapies for Lengthening Life
Are there any medications I can take to help me live longer?
• As of January 2010, riluzole is the only medication approved by the US Food and Drug Administration (FDA) to treat ALS. To a modest degree, riluzole slows the rate at which the disease worsens.
• Strong evidence shows that riluzole can prolong survival by two to three months. Experts suggest a greater benefit when riluzole is started early in the course of the disease.
• Riluzole is very safe but quite expensive.• People who are newly diagnosed with ALS should discuss riluzole with their
neurologists.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Multidisciplinary Care
My doctor says my ALS will greatly affect my health and how I live my life. How should I go about getting the care I need?
• There is good evidence that visiting a multidisciplinary clinic can help people with ALS live longer and get the best possible care for their condition.
• Weak evidence shows that people with ALS who visit a multidisciplinary clinic have better quality of life.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Multidisciplinary Care My doctor says my ALS will greatly affect my health and how I live my life. How should I go about getting the care I need?, cont.
• These clinics are called multidisciplinary clinics because they provide a central location with many services that can help meet the needs of people with ALS.
• These services include sessions with a neurologist or rehabilitation specialist experienced in treating ALS, as well as a nurse case manager. These people will work to coordinate your care.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Multidisciplinary Care My doctor says my ALS will greatly affect my health and how I live my life. How should I go about getting the care I need?, cont.
• You also will meet with several other types of specialists, each of whom will focus on a particular area of care if you eventually need it.
• A physical therapist will work with you on toning muscles and maintaining range of motion and mobility. He or she also will assist you with choosing devices that help with movement.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Multidisciplinary Care My doctor says my ALS will greatly affect my health and how I live my life. How should I go about getting the care I need?, cont.
• An occupational therapist will help you and your caregiver with performing daily activities and adapting your home environment to meet your changing needs.
• A speech pathologist will help you develop different ways of communicating and give advice to make swallowing easier.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Multidisciplinary Care My doctor says my ALS will greatly affect my health and how I live my life. How should I go about getting the care I need?, cont.
• A dietitian will guide you with nutrition and maintaining your weight.
• For help with managing your breathing, you will work with a respiratory therapist.
• A social worker will work with you on how to cope with the disease and will identify additional resources to help you make the needed changes to your daily routine.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Nutritional Therapies
Will having trouble swallowing affect my health? • When you have problems swallowing, eating becomes very difficult. It
is common to begin to choke and accidentally breathe in food you are trying to eat.
• Eventually weight loss and dehydration (not enough fluid in your system) can occur.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Nutritional Therapies
How can this problem be treated? • It is important to manage your swallowing problem so that you maintain good
nutrition. Changing the texture of your food, taking smaller bites, and tucking your chin slightly while you swallow can help.
• You can also use a feeding tube called a percutaneous endoscopic gastrostomy (PEG) tube. These types of tubes are inserted through the skin into the stomach. or radiologically inserted gastrostomy (RIG) tube.
• There is good evidence that a PEG or RIG tube will likely help stabilize body weight and prolong survival.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Nutritional Therapies
Do vitamin and nutritional supplements help people with ALS live longer?
• There is not enough evidence to show if vitamin or nutritional supplements help lengthen the lives of people with ALS.
• Specifically, studies of creatine, CoQ10, and Vitamin E (taken in high doses) do not show a benefit.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Drug Therapies for Symptoms
Are there any medications I can take to treat my symptoms?
• There are medications available that can be very helpful in treating some symptoms of ALS.
• One symptom, uncontrolled laughing or crying, can be embarrassing and hard to manage.
• A person with ALS at times may cry excessively or laugh longer than expected at something that is only mildly funny.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Drug Therapies for Symptoms
Are there any medications I can take to treat my symptoms?, cont.
• There is good evidence that a combination of the medications dextromethorphan (DM) and quinidine (Q) will lessen episodes of uncontrolled laughing or crying—both how severe they are and how often they happen.
• The side effects associated with DM and Q are dizziness, nausea, and sleepiness, so some people stop taking them. This combination of medications is currently awaiting FDA approval.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Drug Therapies for Symptoms
Are there any medications I can take to treat my symptoms?, cont.
• Another symptom, drooling, happens because of swallowing problems.
• Drooling can be embarrassing, and trouble with swallowing the drool can be a health risk. The excess saliva can be accidentally breathed in, which in some cases contributes to pneumonia.
• There are several medications used to dry up the drool, but none of them has been studied for use in ALS.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Drug Therapies for Symptoms
Are there any medications I can take to treat my symptoms?, cont.
• There is good evidence that injections of the medication botulinum toxin type B (BTxB) can help with drooling. The medication is injected into the glands near the jaw that make saliva.
• No serious side effects have been reported from use of BTxB.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Non-Drug Therapies for Symptoms
Are there other ways of treating these symptoms?
• You can get radiation therapy for drooling if BTxB does not give relief.
• There is weak evidence that small amounts of radiation therapy might help. The side effects of this therapy are sore throat, nausea, and reddening of the skin.
• Ask your doctor whether radiation therapy would be a good choice for you.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Non-Drug Therapies for Symptoms
Are there other ways of treating these symptoms?, cont.
• Also, services such as physical therapy, occupational therapy, and speech and language therapy can help you live better and more fully with your symptoms.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Non-Drug Therapies for Symptoms
There doesn’t seem to be much evidence for therapies for symptoms. Why?
• In this guideline update, the authors were able to review only those studies that were specific to ALS. At this time, there are no studies available on use of these therapies in people with ALS.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Behavioral and Thinking Problems
Is ALS a purely physical disease? • ALS is mainly thought of as a physical disease. Many
people with ALS have problems only with weakness of voluntary muscles.
• However, some people with ALS also develop problems with thinking or behavior changes, or both.
• For a small number of people with ALS, the effects on the brain can lead to a form of dementia.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Behavioral and Thinking Problems
Is ALS a purely physical disease?, cont. • The dementia that can occur with ALS progresses slowly
and leads to changes in social behavior, loss of the ability to express feelings clearly and appropriately, and loss of the ability to reflect on things deeply.
• Other problems in this type of dementia are language problems and poor self-care.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Behavioral and Thinking Problems
Is ALS a purely physical disease?, cont. • Because these problems with thinking ability and behavior
changes usually appear slowly, you may not notice them until they have become serious.
• It is important to try to detect and manage these problems as early as possible.
• Some people with ALS who develop these types of problems might not be willing to use important therapies that may prolong their survival or improve their quality of life.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Behavioral and Thinking Problems
Is ALS a purely physical disease?, cont. • There are tests available that screen for behavioral or
thinking problems in people with ALS.• Good evidence shows that people with ALS should be
screened for possible problems with thinking ability or behavioral change.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Behavioral and Thinking Problems
Is ALS a purely physical disease?, cont. • If you are screened for a problem with thinking ability and
the results show some problems, your doctor may suggest further testing.
• Talk to your doctor about whether such tests would be useful for you. Also ask your doctor about therapies that may help you and your caregivers to manage these changes.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Breathing Therapies
How can problems with breathing be treated?
• Breathing problems develop in people with ALS because of weakening of the muscles that help with breathing.
• Treating breathing problems is very important. • The most common cause of death from ALS occurs from
weakening of breathing muscles.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Breathing Therapies
How can problems with breathing be treated?, cont.
• It can be hard to know if you have a breathing problem because you may not notice it until it becomes serious.
• Talk to your doctor about available tests to detect a breathing problem.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Breathing Therapies How can problems with breathing be treated?, cont.
• If your doctor discovers that you have a breathing problem, he or she may recommend that you use an assisted-breathing device, also known as a noninvasive ventilation (NIV) device.
• Good evidence shows that using an NIV device will likely lengthen survival and slow the development of breathing problems.
• There is weak evidence that shows use of an NIV device will also improve quality of life.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Breathing Therapies How can problems with breathing be treated?, cont.
• Muscle weakness that affects breathing can also affect the ability to cough. If your cough is weak, you might have trouble clearing mucus and other fluids from your throat, which may lead to a chest infection.
• Assisted-coughing devices, also known as mechanical insufflation/exsufflation (MIE) devices, are available to help with coughing.
• Weak evidence shows that using an MIE device may help clear fluids from the throat, especially during a chest infection.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Conclusions•There has been some progress in evaluating new therapies for people with ALS.
•More high-quality studies are available on multidisciplinary clinics, riluzole, breathing and nutrition, and therapies for some symptoms (laughing and crying behaviors, drooling).
•Unfortunately, many of these important treatments are not being used to their full potential.
•What’s more, there is still a great need for more and better research in many areas.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Future Research
•Lithium carbonate Larger studies are in progress on whether lithium slows
the rate at which the disease worsens and whether lithium lengthens survival.
•Nutrition Studies are needed on the best timing for inserting a
feeding tube and for beginning antioxidant and supplement use.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Future Research
•Respiratory Management Evaluation is needed on when to start use of an NIV
(breathing) device: impact on quality of life and survival. Comparison of the best techniques is needed for
clearing phlegm and saliva.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Future Research
•Breaking the News Measures are needed on how and when to disclose an
ALS diagnosis. Evaluation is needed on neurologists’ and patients’
attitudes toward strategies for breaking the news. Studies are needed on the effects of different strategies
on patient satisfaction, preserving hope, and outcomes.•Multidisciplinary Clinic
−Evaluation is needed on factors essential to benefits, best visit frequency, and economic concerns.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Future Research
•Symptom Management Studies are needed on medication treatment for
spasticity, cramps, constipation, drooling, laughing and crying behaviors, pain, depression, anxiety, fatigue, and exercise.
•Behavioral and Thinking Problems−Evaluation is needed on diagnosis and research criteria,
screening tests, and the history of and treatments for these problems.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Future Research
•Communication Evaluation is needed on communication strategies for
people with weakness of their muscles for speech. Studies are needed that compare different
communication strategies.•Palliative Care
−Studies are needed on end-of-life symptom management, advanced directives, hospice, and spiritual care.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Acknowledgments
The authors thank Gary Gronseth, MD; Thomas Getchius; Valerie Cwik, MD; Larry Brower; and Sid Valo for contributions to the practice parameters; Christina Metzler and Barbara Phillips, MS, OTR/L, for their contributions to the patient summary versions of the guidelines; and Sharon J. Matland, RN, MBA, for her contributions to both the practice parameters and the patient summary versions.
© 2010 AMERICAN ACADEMY OF NEUROLOGY
References
For a complete list of references, please access the full guidelines at
www.aan.com/guidelines
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Questions/Comments
© 2010 AMERICAN ACADEMY OF NEUROLOGY
Thank you for your participation!
© 2010 AMERICAN ACADEMY OF NEUROLOGY
The ALS Association thanks the American Academy of Neurology and
all of today’s presenters and participants.