Tuesday, September 2, 2014
The Ice Bucket Challenge and ALS: a matter of priorities
For a few months I have been noticing that several of my friends and acquaintances, as well as all sorts of famous people, have been filming themselves dumping ice water over their heads and then talking a little bit about Amyotrophic Lateral Sclerosis (ALS). Then they suggest that I donate money to the ALS Association. It has been a particularly hot summer, which partly explains the popularity of the phenomenon, and mostly all of these people look adorable soaking wet and spluttering, which is an added bonus.
The ALS Association, and the British version, the Motor Neurone Disease Association, have made boatloads of money off of the challenge. Originally the idea was that a person would challenge another person to donate money for ALS research and if they hadn’t done so in 24 hours, they would dump a bucket of ice water over their head. It turns out that people like dumping ice buckets over their heads (at least this summer) and posting video clips of it and also like donating money to ALS research. So that’s how it goes now.
People (like George W. Bush) dump a nice bucket of ice water over their head and then challenge another person (like Bill Clinton) to donate money to ALS research and also dump a bucket of ice water over their own head. It looks like the ALS Association is getting lots of money, tens of millions of dollars so far. It feeds not only research but peoples’ desires to be courageous and generous and a little bit vulnerable and to connect to other people in a teasing way. Cool, I think, that George W. Bush tagged Bill Clinton. If the 2 parties in Congress could work together like that, perhaps good things would happen in Washington.
But being filmed soaking wet isn’t really the whole point of this exercise. Amyotrophic Lateral Sclerosis is. The British call this disease “Motor Neuron Disease” which is much easier to pronounce and a bit more descriptive, since it is a disease of the motor (muscle activating) neurons. The disease is called Amyotrophic Lateral Sclerosis in the U.S. because it affects the outside columns of the spinal cord which carry the neurons responsible for muscle activity. In pathology specimens, these lateral columns become hard and scarred, sclerotic in medical terms.
I have not seen much ALS in my quarter century of practice because it is really quite rare. It is tragic, though. The most recent patient who I was involved in treating came to me for a treadmill test because he had been losing stamina and his doctor couldn’t figure out what might be causing this after examining him and doing various blood tests. His treadmill test was fine, but he had clearly declined. His description of the decline put me in mind of some kind of neurological disease involving the muscles. ALS usually presents with weakness of the legs or the arms or sometimes the face or muscles of breathing or chewing. There can be some tingling, but the disease primarily affects strength. There may be uncontrolled twitching of muscles, primarily in the tongue (not those pesky little twitches around the eyes that we all get.) As the disease progresses all muscles become weak and eventually paralyzed. Thinking and memory are normal. I referred my patient to an excellent neurologist who performed electrical testing of muscles and nerves and gave the patient his diagnosis. I saw him 1 more time and then several months later there was a beautiful obituary in the paper that he had written himself.
ALS affects about 3 out of every 100,000 people in the U.S. and is more common in the elderly. It does occur in young people, though, and in those it is more common in men than in women. Stephen Hawking, the Nobel Prize winning physicist, was diagnosed with it at age 21. His progression was surprisingly slow, and he has survived, with the help of much technology, for decades. Most patients survive about 2 years, and there is really very little to offer as far as treatment. There is one approved drug, Riluzole, which costs about $1,400 a month and prolongs life a bit. Its mechanism of action is not well understood. As patients lose strength they can be fed with a feeding tube and their breathing can be supported with a tight fitting positive pressure face mask or even a ventilator attached to a tracheostomy. Lou Gehrig ended his career at age 36 due to ALS, which has held his name in the U.S. since then (Lou Gehrig’s Disease) and died 2 years later.
It looks like there is lots of active research on treatments for ALS. There are not only new drugs in the pipeline but older ones that have been used for other things are being tried. Scientists are working on stem cells which might replace the function of the sick and dying motor neurons. It is unclear how much all of this money will do to hasten the development of effective treatments or lessen the misery of the disease, but now there is lots of money. It will be interesting to see how the money is spent, since drug companies often finance their own research. A rare disease, though, doesn’t attract nearly as much research and development as a more common disease because it cannot be expected to have many potential consumers. A bolus of financial support might just spur an important discovery.
I do have some qualms about the ice bucket challenge. ALS, though awful, has a prevalence of about 3 in 100,000 people, and some other diseases are also awful and have a higher prevalence. Stroke, for instance, has a prevalence of 3 per 100, and hypertension, which causes stroke, has a prevalence of 3 in 10. We do have medicines for hypertension, and we have some treatments for stroke as well, but that’s really just true in the developed world. High blood pressure and its effects, stroke, heart attack and kidney failure, are rapidly surpassing infectious diseases as the major causes of death in developing countries such as China, India, and countries in Africa and the Caribbean. This is at least partly due to the globalization of western dietary habits. Strangely enough, they also don’t have enough clean water. This makes it hard for me to donate generously to the ALS Association while watching people dumping delicious ice water on their healthy and well-hydrated heads. Perhaps the next challenge should be dumping dry dirt on one’s head and donating to the World Health Organization.
Janice Boughton, MD, ACP Member, practiced in the Seattle area for four years and in rural Idaho for 17 years before deciding to take a few years off to see more places, learn more about medicine and increase her knowledge base and perspective by practicing hospital and primary care medicine as a locum tenens physician. She lives in Idaho when not traveling. Disturbed by various aspects of the practice of medicine that make no sense and concerned about the cost of providing health care to every American, she blogs at Why is American Health Care So Expensive?, where this post originally appeared.
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Members of the American College of Physicians contribute posts from their own sites to ACP Internistand ACP Hospitalist. Contributors include:
Albert Fuchs, MD, FACP, graduated from the University of California, Los Angeles School of Medicine, where he also did his internal medicine training. Certified by the American Board of Internal Medicine, Dr. Fuchs spent three years as a full-time faculty member at UCLA School of Medicine before opening his private practice in Beverly Hills in 2000.
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Amanda Xi, ACP Medical Student Member, is a first-year medical student at the OUWB School of Medicine, charter class of 2015, in Rochester, Mich., from which she which chronicles her journey through medical training from day 1 of medical school.
Ira S. Nash, MD, FACP, is the senior vice president and executive director of the North Shore-LIJ Medical Group, and a professor of Cardiology and Population Health at Hofstra North Shore-LIJ School of Medicine. He is Board Certified in Internal Medicine and Cardiovascular Diseases and was in the private practice of cardiology before joining the full-time faculty of Massachusetts General Hospital.
Zackary Berger, MD, ACP Member, is a primary care doctor and general internist in the Division of General Internal Medicine at Johns Hopkins. His research interests include doctor-patient communication, bioethics, and systematic reviews.
Controversies in Hospital
Run by three ACP Fellows, this blog ponders vexing issues in infection prevention and control, inside and outside the hospital. Daniel J Diekema, MD, FACP, practices infectious diseases, clinical microbiology, and hospital epidemiology in Iowa City, Iowa, splitting time between seeing patients with infectious diseases, diagnosing infections in the microbiology laboratory, and trying to prevent infections in the hospital. Michael B. Edmond, MD, FACP, is a hospital epidemiologist in Richmond, Va., with a focus on understanding why infections occur in the hospital and ways to prevent these infections, and sees patients in the inpatient and outpatient settings. Eli N. Perencevich, MD, ACP Member, is an infectious disease physician and epidemiologist in Iowa City, Iowa, who studies methods to halt the spread of resistant bacteria in our hospitals (including novel ways to get everyone to wash their hands).
db's Medical Rants
Robert M. Centor, MD, FACP, contributes short essays contemplating medicine and the health care system.
Suneel Dhand, MD, ACP Member
Suneel Dhand, MD, ACP Member, is a practicing physician in Massachusetts. He has published numerous articles in clinical medicine, covering a wide range of specialty areas including; pulmonology, cardiology, endocrinology, hematology, and infectious disease. He has also authored chapters in the prestigious "5-Minute Clinical Consult" medical textbook. His other clinical interests include quality improvement, hospital safety, hospital utilization, and the use of technology in health care.
Juliet K. Mavromatis, MD, FACP, provides a conversation about health topics for patients and health professionals.
Dr. Mintz' Blog
Matthew Mintz, MD, FACP, has practiced internal medicine for more than a decade and is an Associate Professor of Medicine at an academic medical center on the East Coast. His time is split between teaching medical students and residents, and caring for patients.
Toni Brayer, MD, FACP, blogs about the rapid changes in science, medicine, health and healing in the 21st century.
Vineet Arora, MD, FACP, is Associate Program Director for the Internal Medicine Residency and Assistant Dean of Scholarship & Discovery at the Pritzker School of Medicine for the University of Chicago. Her education and research focus is on resident duty hours, patient handoffs, medical professionalism, and quality of hospital care. She is also an academic hospitalist.
John H. Schumann, MD, FACP, provides transparency on the workings of medical practice and the complexities of hospital care, illuminates the emotional and cognitive aspects of caregiving and decision-making from the perspective of an active primary care physician, and offers behind-the-scenes portraits of hospital sanctums and the people who inhabit them.
Ryan Madanick, MD, ACP Member, is a gastroenterologist at the University of North Carolina School of Medicine, and the Program Director for the GI & Hepatology Fellowship Program. He specializes in diseases of the esophagus, with a strong interest in the diagnosis and treatment of patients who have difficult-to-manage esophageal problems such as refractory GERD, heartburn, and chest pain.
Mike Aref, MD, PhD, FACP, is an academic hospitalist with an interest in basic and clinical science and education, with interests in noninvasive monitoring and diagnostic testing using novel bedside imaging modalities, diagnostic reasoning, medical informatics, new medical education modalities, pre-code/code management, palliative care, patient-physician communication, quality improvement, and quantitative biomedical imaging.
William Hersh, MD, FACP, Professor and Chair, Department of Medical Informatics & Clinical Epidemiology, Oregon Health & Science University, posts his thoughts on various topics related to biomedical and health informatics.
David Katz, MD
David L. Katz, MD, MPH, FACP, is an internationally renowned authority on nutrition, weight management, and the prevention of chronic disease, and an internationally recognized leader in integrative medicine and patient-centered care.
Richard Just, MD, ACP Member, has 36 years in clinical practice of hematology and medical oncology. His blog is a joint publication with Gregg Masters, MPH.
Kevin Pho, MD, ACP Member, offers one of the Web's definitive sites for influential health commentary.
Michael Kirsch, MD, FACP, addresses the joys and challenges of medical practice, including controversies in the doctor-patient relationship, medical ethics and measuring medical quality. When he's not writing, he's performing colonoscopies.
Elaine Schattner, MD, FACP, shares her ideas on education, ethics in medicine, health care news and culture. Her views on medicine are informed by her past experiences in caring for patients, as a researcher in cancer immunology, and as a patient who's had breast cancer.
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Alexander M. Djuricich, MD, FACP, is the Associate Dean for Continuing Medical Education (CME), and a Program Director in Medicine-Pediatrics at the Indiana University School of Medicine in Indianapolis, where he blogs about medical education.
Rob Lamberts, MD, ACP Member, a med-peds and general practice internist, returns with "volume 2" of his personal musings about medicine, life, armadillos and Sasquatch at More Musings (of a Distractible Kind).
David M. Sack, MD, FACP, practices general gastroenterology at a small community hospital in Connecticut. His blog is a series of musings on medicine, medical care, the health care system and medical ethics, in no particular order.
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Kimberly Manning, MD, FACP, reflects on the personal side of being a doctor in a community hospital in Atlanta.
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Paul Sufka, MD, ACP Member, is a board certified rheumatologist in St. Paul, Minn. He was a chief resident in internal medicine with the University of Minnesota and then completed his fellowship training in rheumatology in June 2011 at the University of Minnesota Department of Rheumatology. His interests include the use of technology in medicine.
Technology in (Medical)
Neil Mehta, MBBS, MS, FACP, is interested in use of technology in education, social media and networking, practice management and evidence-based medicine tools, personal information and knowledge management.
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Peter A. Lipson, MD, ACP Member, is a practicing internist and teaching physician in Southeast Michigan. The blog, which has been around in various forms since 2007, offers musings on the intersection of science, medicine, and culture.
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Janice Boughton, MD, FACP, practiced internal medicine for 20 years before adopting a career in hospital and primary care medicine as a locum tenens physician. She lives in Idaho when not traveling.
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Other blogs of note:
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Also known as the Green Journal, the American Journal of Medicine publishes original clinical articles of interest to physicians in internal medicine and its subspecialities, both in academia and community-based practice.
A collaborative medical blog started by Neil Shapiro, MD, ACP Member, associate program director at New York University Medical Center's internal medicine residency program. Faculty, residents and students contribute case studies, mystery quizzes, news, commentary and more.
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The Public Library of Science's open access materials include a blog.
One of the most popular anonymous blogs written by an emergency room physician.