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Thursday, September 18, 2014

Emergency room doctors can safely use bedside ultrasound to diagnose kidney stones, saving billions of dollars and preventing some radiation induced cancers

I have been following the progress of bedside ultrasound (using ultrasound as a diagnostic tool during physical exam of patients) as it gets a foothold in standard medical practice. It has been part of my practice for almost 3 years now, during which time I have been repeatedly amazed by how helpful it is for guiding my clinical decisions. There is good research showing how useful it is for all sorts of applications, from heart problems to intestinal obstruction, but it is still slow to catch on.

An article came out just recently in the New England Journal of Medicine, which has a large circulation and should make a bit of a splash. This multi-center study looked at the option of having patients (excluding the very obese, pregnant and critically ill) with abdominal and flank pain suspected of having kidney stones evaluated first by emergency physicians with ultrasound of the kidneys and bladder before considering getting a CT scan. Normally a patient with suspected kidney stones (crampy pain in the back or abdomen, blood in the urine, suggestive history) will be referred for an abdominal and pelvic CT scan, which costs over $3000 and carries a significant amount of radiation exposure. In perfect circumstances performing the test and getting the results takes an hour, but it can end up taking several hours due to the usual delays. Sometimes patients with kidney stone type symptoms are referred by the emergency physician for an ultrasound by the radiology department, which takes about the same amount of time as the CT which takes the same amount of time, but costs a bit less and delivers no ionizing radiation. CT scans have beautiful pictures and can often find the kidney stone, if it's in there, and not finding the stone is strongly suggestive that the diagnosis of what is causing the pain must be sought elsewhere. Ultrasound can show if the kidney is blocked by showing lack of flow into the bladder or buildup of fluid in the kidney (hydronephrosis) but rarely actually visualizes the stone. This information, however, is adequate to make the diagnosis in most cases, when combined with a good clinical history, physical exam and lab tests.

It turns out that the bedside ultrasound exam done by emergency room docs (in this study they were from multiple medical centers including University of California at San Francisco, Cook County and Rush Medical Centers in Chicago, Group Health in Seattle and many more high quality locations) is adequate in cases of abdominal or flank pain as a first evaluation to rule in or out kidney stones. It is much more focused than an ultrasound performed by the radiology department and it only takes about 5 minutes or less to perform. Since it is done by the physician examining the patient it is also a time to take more history and do more general observation, which is always a good thing. About 40% of the patients initially evaluated this way got an official radiology ultrasound or CT scan which were felt to be necessary by the ER physician to clarify what was going on. About a million patients with kidney stones visit emergency rooms each year in the US and more than 10 times that many visit ER's with symptoms that sound a bit like kidney stones and have to be evaluated for them. If all of them got bedside ultrasound as the initial evaluation of their kidneys, my back-of-the-envelope calculations suggest that multiple billions of dollars could be saved on imaging costs and lives could potentially be saved due to reduced radiation exposure. The study showed no significant increase in complications in the patients first receiving bedside ultrasound. Actual cost savings were calculated, but not reported in the study (why?)

We can't just start doing this because not all ER doctors are yet comfortable performing and interpreting bedside ultrasound of the kidneys and bladder. But they could be. It is not hard. Pretty much anybody could learn to do this in maybe an hour and could certainly be competent after doing 50 exams. The implications of this are bigger than the article points out. When ER physicians start doing regular bedside (or "point of care" as it's sometimes termed) ultrasound they are going to get better at it. They will start to use ultrasound more and develop some pattern recognition skills that can't be predicted which will likely lead to more accurate diagnoses of other diseases, and possibly less dependence on ionizing and expensive radiation in the form of CT scans.

Unfortunately CT scans for abdominal pain in the emergency room  are an important source of revenue for both radiologists and hospitals which puts a little kink in the clear path toward adopting bedside ultrasound as a diagnostic procedure of choice. It's not clear what to do with this, because we could surely use the expertise of radiologists and radiology technicians in training physicians to be good bedside ultrasonographers and presently that would be a pretty big conflict of interest for them. Still, there is so much good stuff going on in the field of high tech ultrasound that is not in the scope of bedside ultrasound that radiologists and technicians could be kept gainfully occupied by doing things that other physicians can't and shouldn't do. In the journal of the American Institute of Ultrasound in Medicine there were several articles about amazing and technically challenging imaging applications that non-radiologists might be wise not to try. There were articles about ultrasound of the midbrain to evaluate Parkinson's Disease, ultrasound of the liver to look at severity of cirrhosis, ultrasound of children with intestinal intussusception (telescoping of the bowel) to follow the success of noninvasive treatments and detailed prenatal evaluations for conditions I didn't even know existed. Ultrasound to diagnose appendicitis has become nearly standard now, but is really hard to learn and ultrasonographers and radiologists do it well (some ER physicians do it well too, but it's far from an entry level skill.) Looking at the kidneys in 5 minutes in the ER is clearly fine for evaluating possible kidney stones. An abdominal ultrasound in the radiology department with their big powerful machine with the gorgeous images combined with the stunning command of anatomy of radiology professionals is a different and differently beneficial thing. This recent article may help move us as hospitalists, ER physicians and primary care providers toward doing more bedside ultrasound, which could be a very good thing. Perhaps more radiologists will find peace with that and can bring themselves to help teach other medical staff who need to learn how to do it.

Tuesday, September 16, 2014

American College of Physicians blows this one: Pri-med "free" education on safe opiate prescribing, REMS and drug companies

I am mostly a pretty big fan of the American College of Physicians (ACP), the society that (usually) represents me as an internal medicine physician. They present meetings and conferences to spread new and relevant information and they promote gifted and hard working physicians and medical teachers. They are a force for organization in our profession which often fails to pull together and sometimes resembles a group of agitated hedgehogs. Some of the educational offerings that they produce are ground breaking, encouraging us to practice medicine that is more effective and patient centered. I do pay $525 yearly to maintain my membership, but that doesn't seem unfair.

So that is why I opened the slick tri-fold large format postcard that I got in the mail today rather than recycling it immediately. It said, "Practice safe opioid prescribing with ACP's resources." Over-prescribing opiate painkillers such as morphine, oxycodone and hydrocodone is a huge problem in the US. In 2010 60% of the nearly 40,000 opiate overdose deaths were due to prescription medications, and that number is continuing, I believe, to rise every year. In addition to relieving pain, medications of this class can make people stop breathing, fall asleep at inopportune times, make poor decisions, be unable to have bowel movements and other life ending scenarios. The US uses 80% of the prescription opiates produced in the world. Many of the opiates we as physicians prescribe end up being misused or abused or sold illegally. In the 1990s there was a huge increase in understanding that pain needed treatment and in the 10 years between 1997 and 2007 prescriptions for pain medication increased 600%. I see patients frequently in the hospital whose illnesses become life threatening because they use prescription opiate pain medications. There are nearly a half a million emergency room visits each year related to prescription opiate abuse and the cost in healthcare dollars of this problem is many billions of dollars. There is a problem with our prescribing habits.

The tri-fold postcard from the ACP offered a 6 hour continuing medical education credit online course on appropriate prescribing of opiate pain medications, with a focus on avoiding overdose and addiction. The course was free to me. When I went to the ACP site I was re-directed to Pri-Med's site where the audio and slide program was available. Pri-Med is a medical education company which makes low cost (to us) online programs on all sorts of subjects. The programs are so low in cost that they can't possibly actually cover the expense of creating the content. Hmmm. Vewwy Intewwesting.

I went ahead and took the 6 modules which covered appropriate use of long acting opiate pain medications to treat chronic pain. I learned some interesting things about risk factors for prescription pain medication abuse, some obvious (active ongoing drug abuse) and some less so (age 18-45 with family history of drug abuse). I learned about different opiates' side effects and interactions with other drugs based on the cytochrome P450 system. I also heard stuff I already knew, such as the fact that long acting opiates are not supposed to be taken "as needed" but should be scheduled and that most of them must be left intact, not chewed or crushed, in order to remain long acting. Most of all I was exposed to a bunch of brand name opiates and exactly how they worked and what doses were standard and how they compared to each other. What I didn't learn was how much each of these drugs cost and how those costs and potential advantages compared to generic long acting opiates. There was nothing useful about how to help pain patients get off of opiates or alternatives to starting them in the first place.

After a rather long program I felt as if I had been cornered by a drug rep (representative from a pharmaceutical company). I am not pleased. I have been effectively inoculated with wonder drug propaganda. In return I will have a certificate that says I have been educated in safe opiate prescribing, which many state medical boards now require for licensure.

Why did this happen? Here's the story. The FDA now requires that companies that produce long acting opiates do something to make less people die of their drugs. The Food and Drug Administration Amendments Act of 2007 gave FDA the authority to require a Risk Evaluation and Mitigation Strategy (REMS) from manufacturers to ensure that the benefits of a drug or biological product outweigh its risks. For long acting opiates this consists of providing free education to prescribers and proving that at least 60% of them partake of these educational programs. Pri-Med makes its money from the healthcare industry, I'm guessing primarily from drug companies. This particular program skillfully combines risk mitigation with drug detailing. Clever pharma. 

I'm disappointed in the ACP, though. On their website they did mention that this educational activity was supported by industry, but it was in very small print. With $525 of dues money times 140,000+ members, along with other sources of income, the ACP does not need drug company support to create educational material. State boards of medicine require education in safe opiate prescribing, but they do not require that it be provided by drug companies. It sounds like drug companies may have to prove that their educational materials are being disseminated, but that is not the business of the ACP and is not my responsibility. And the shiny tri-fold postcard. Who paid for that?

Saturday, August 30, 2014

The "nocebo effect", statins and Dr. Ben Goldacre


I just recently became aware of a study that came out in March of this year which concluded that statins, drugs like lipitor (atorvastatin) and zocor (simvastatin), which people take, increasingly, to lower their cholesterol and their risk of heart disease, have NO SIDE EFFECTS. Here is a paper which explains the study. It is not possible to link to the actual study in the European Journal of Preventive Cardiology because they want me to pay for it.

The paper says that, when comparing patients who took statins to ones who took an inactive pill, the side effects of both were about the same. That is called the "nocebo effect". Many people have heard about the "placebo effect" in which a sham treatment or sugar pill has a beneficial effect due, we think, to the fact that the subjects who receive it think it will work. Placebo, in Latin, means "I will please" and nocebo means "I will harm." So the researchers who wrote the paper about statins, after reviewing the data, found that patients who believed they would have side effects on statins did have side effects, whether or not they took the real drug. This is the nocebo effect. It implies that statins have no more side effects than sugar pills.

Now this would be really interesting if it were true. But it's not, so it's just really annoying. Patients who have received these drugs and physicians who prescribe them have noticed such a marked incidence of side effects, especially muscle weakness and pain, which resolve when the medicine is stopped, that any study questioning that finding is extremely suspect. When I heard about the article, I looked a bit further to see who had written it and what data they had looked at. I suspected that the study had been funded by pharmaceutical company lackeys using faulty data. It turns out I was only half right.

One of the major authors on the paper is a British physician named Ben Goldacre who is absolutely passionate about revealing the truth in scientific research and medicine, particularly in research done by unscrupulous pharmaceutical companies. He has founded a group, AllTrials to promote honesty in reporting the results of clinical trials of medications. He actively publishes articles aimed at lay audiences about ways in which drug companies use skewed data to mislead the public about the safety and effectiveness of their drugs. He has written a book Bad Pharma about how the pharmaceutical industry distorts the truth to get patients to use their products. He is passionate about it and appears to be an excellent human being.

So what happened? Why did this guy who seems to be such a voice for truth write this paper? He explains it all quite entertainingly and in much more detail than I will here in his column "Bad Science." What happened is that he used very incomplete data about side effects from studies that were mostly performed and designed with drug company support to show that statins were safe and effective. They didn't even ask about many of the side effects that patients frequently complain about and they didn't evaluate for muscle weakness in patients unless their muscle enzymes were 10 times normal or more, which is extremely rare. Dr. Goldacre attempted to write a disclaimer to the effect that he believed his data was inadequate, but the paper had gone to press. Oops. The news that statins have no side effects was on front pages of newspapers. There must have been champagne opened in the spacious offices of the companies that produce these medications.

So we still need good unbiased data on the true side effects of statins, and that will be pretty difficult to get at this date. Statins are so commonly used that finding a cohort of patients who have never used them to participate in a double blind study to evaluate their short and long term side effects will be tough. There are several statins on the market, with different incidence of side effects based on their chemistry, and each would need to be tested. Different categories of patients have different side effects, and the side effects vary based on dosage. Most patients who are willing to take statins are already on them, since physicians love to prescribe them. Patients who don't want to take them probably also don't want to take them in a double blind fashion for a long period of time. We will probably have to settle for a re-examination of data which was collected but not released, if anyone has the time or energy to find and scrutinize that.


Hand off or second opinion--how can we make transitions be a good thing?

Healthcare is actually a 24 hour a day 7 day a week job. People get sick even when we physicians are supposed to be sleeping or eating dinner or showering or brushing our teeth. Having care available all the time often saves lives and usually reduces suffering. In the US, we have that pretty well worked out: everyone with a doctor has an after hours number to call and if that fails or the problem is too big, there is always an emergency room or at least an ambulance or fire truck to whisk one away to where help is waiting.

One of the problems with our after hours options is that a person is rarely seen by a doctor who knows them and is familiar with their medical history. It would be ideal for all of us to have rapid access to the doctor (or nurse practitioner or physician's assistant) who has been with us for years and who knows what works, what doesn't and who we can relate to and trust. Unfortunately that person has to sleep and eat dinner and sometimes even go on vacations. Most people run into the reality of seeing different doctors depending on who is available.

In my present profession, hospital medicine, I work for several days in a row taking care of a collection of patients who are in the hospital at that time. Most of them I don't know. When I have days off, I tell another hospitalist about the patients I've been seeing and write a rather complete note. We discuss how I envisioned managing the patients' medical problems, as I understand them and then I go, and Doctor Next takes the helm.

I always feel bad, at least a little bit, deserting my patients and leaving my physician partners with a job half finished, even though that is the nature of the job. But when I think about it, sometimes it is a really positive thing, and if I approach it that way it can be even more positive.

Not all hospitalist programs have "face to face signouts." It is ideal to sit with the doctor who is assuming care of my patients and explain what is going on. That becomes impractical if there are too many patients and when I am not physically working at the same time as the physician assuming my patient's care, like in places where there is a night shift physician. Telephone signouts are not bad, but are also impractical in a big hospitalist group where my 18 or 20 patients may go to several different doctors when I leave. A good signout, in person, from a good doctor is key to not being completely helpless on the first day of a set of shifts. Nevertheless, much can be gleaned from reading progress notes and reviewing labs and sometimes that's all there is, since two minutes signout times 20 patients on a service equals 40 minutes, which is way too much time and still not enough detail to really be helpful.

In short, transitions of care are difficult, no matter how you slice it, and the more intensity that is put into the communication, from departing to starting doc, the better it is. But there is a silver lining to this dark cloud. Sometimes when we treat patients we go off down a wrong path. We concentrate on one aspect of a history or a data point and head off enthusiastically, missing what is really going on. If the doctor who takes over when we go is attentive and not excessively busy, the patient gets another chance for us to get the right answer. If done right, every transition can be a second opinion.

In some of the hospitals where I did my residency training they had these wild and woolly doctor free-for-alls called morbidity and mortality conferences. They were a chance to dissect all of the decisions and actions that contributed to a patient becoming sicker or dying under our care. They were not quite blood baths, but doctors did cry regularly as they were grilled on their reasoning by more senior physicians, resting in the certainty of 20-20 hindsight. Besides being confrontational and unpleasant these were incredibly informative and it was hard to forget the lessons learned in that context. We rarely see these anymore, but I miss them. Instead, I try to keep track of situations where what my colleagues have done or have thought was going on turned out to be wrong, and to discuss it with them later. This can be tricky and needs to be done in a trusting relationship, with the understanding that they will do the same for me.

We have been discussing lately doing a small morbidity and mortality type meeting with the emergency physicians, who by necessity only see the beginning of a patient's evaluation and frequently do not have the benefit of all of the data, and the hospitalists, like me, who receive and take care of the patients from the emergency room and eventually hear the end of the story. I suspect this will be really interesting and will not only improve our medical thinking but also help us work together better. It will be a little bit tricky finding a time when even a quorum could be present together because of our very different work schedules, but I'm looking forward giving it a try.

Friday, August 29, 2014

Population Health: what might it look like?

Blue skies...
It is a beautiful day here in this little college town. The sun is shining and at 11 AM it is about 78 degrees with a barely perceptible breeze. People are out walking on Main Street and riding their bikes. The mountain nearby calls: I can go for a hike today with my dog and still be within 20 minutes or so of the hospital to respond to calls.

A mostly empty hospital
There are two patients on the hospitalist service in this fine critical access hospital. That is a tiny workload for my profession--hospitalists, who take care of patients in the hospital who have no primary care physician or whose doctor doesn't manage their care while they are inpatients, usually don't complain unless they are managing over 18 patients in a day. I am often quite busy, but not today. One of my two patients is going home later this morning. She is bright and cheerful, with progressive Alzheimer's disease and chronic lung disease from a long gone habit of smoking 3 packs of cigarettes a day. She got a little perturbation of her lung bacteria with wheezing and a low oxygen level, and is now better on medications after an 18 hour hospital stay. Her daughter, who loves her and tells me about how she used to support herself selling the cherry pies she baked, will take her home to her house in the country where she will get along well, at least for a while, with her home nebulizer.

I just visited a former primary care patient of mine who is in the emergency room after a seizure and brief respiratory arrest. She is developmentally disabled and has a cancer which is finally going to take her life, and she will be going home to the group home where she has been scrupulously and lovingly taken care of for 28 years, with hospice. We have known this was coming. Medicare would have paid us to put her in the intensive care unit and support her breathing with a ventilator and her blood pressure with chemicals with the goal of postponing her death by a few days, but that isn't what she or her family would want and it would be wrong to make her spend her last hours or days where the sounds and smells and people are unfamiliar.

A healthy community
The rest of the people in this town and outlying areas (those whose primary physicians don't admit to the hospital) appear to be fine. At least fine enough to not require hospitalization. This is a good day, medically speaking. Drinks on the house!

But wait a second...it's not that simple
Except, of course, financially for the hospital it is not so very rosy. They still have outpatients coming in for tests and treatments, but a major part of the positive side of the economic equation is payment for the treatment of inpatients. There are large fixed costs associated with a hospital being able to treat sick patients when they need it. Good nurses must be given adequate pay and adequate hours to support their families. Buildings and grounds must be maintained. Pharmacy staff and medications must be updated and ready for the huge variety of illness we see and the diverse ways we think of to treat it. Social workers and case worker nurses, who don't bill anybody for their services, must receive salaries and continue to do what they do to make sure that patients discharged from the hospital are able to remain supported at home. Hospitalists must be paid so we can be on call 24 hours a day to treat the sometimes desperately ill patients who are admitted. The hospital creates new service lines for the mostly healthy, like a women's imaging center with 3 dimensional mammography (with questionable associated benefits) but a healthy population does not a healthy hospital make.

But we all want more days like today. Not every day, of course, or we will forget how to treat sick people, but a day like today is the holy grail of effective medical care. Today, while doing very little, I am doing a great job.

Population health--everybody healthy!
Population health is a term that I have been hearing for really only a few years, though the concept is not at all new. Population health is an approach to health which aims to improve the health of a whole population, not just to treat diseases, one patient at a time. Naturally we need to also treat diseases and the individual patients who have them, with special attention to who these people are and what will restore them most effectively to a fulfilling life. But population health is about making more days like today, where most people are doing just fine, without the need to bother themselves with doctors or hospitals. Since it is inevitable that there will always be some sort of ill health related misery to deal with, population health as a concept encourages us to focus on how doctors and hospitals can be most effective to minimize the time, energy and money people spend with us.

This week in JAMA (Journal of the American Medical Association) there were a couple of articles which brought home the importance of  changing our attitudes and payment systems in ways that will actually promote population health.

Treat your own blood pressure
The first was a study out of the UK by Richard McManus and his primary care research group looking at giving patients the ability to monitor their own blood pressures and adjust their own medications, within certain parameters. In the US, in 2010, treatment of high blood pressure (hypertension) by physicians totaled $42.9 billion, and with that, it isn't actually controlled very well. Depending on which group you look at, blood pressure is uncontrolled in maybe one third or up to half of people (worse if you are hispanic or black or uninsured) which translates to more strokes, heart attacks and kidney failure. When patients are given the ability to adjust their medications according to blood pressures they measure themselves, they control their blood pressure better than doctors do, without increased side effects. Home management of blood pressure will put a dent in that $42.9 billion and even more of a dent in the money spent for treatment of strokes, heart attacks and kidney failure, but some of us health care providers will be out of a job.

Happy teenagers (well, happier)
The other article, by Linda Richardson MD of the Children's Research Institute in Seattle and colleagues, was more poignant. It looked at a collaborative model of treating adolescent depression, through Group Health Cooperative clinics in urban areas of Washington state. Depressed adolescents were assigned either to a "usual care" group who saw physicians and might have been prescribed medications or counseling based on their office visits, or a "collaborative care" group who decided, with their parents, whether they wanted medications, counseling or both, and had regular visits, in person and on the phone, with a master's level clinician. Those who chose psychotherapy received a brief 4 session cognitive behavioral therapy intervention that had been designed for adolescents. There were regular team meetings to discuss their progress and they were followed for a year, using a depression screening tool, with adjustment in treatment based on their response. In the usual care group only 34% responded to treatment, with 20% achieving remission, whereas the study group 67% responded and 50% were no longer depressed. This cost an average of a bit over $1000 per patient over usual care. Adolescent depression is huge in the magnitude of distress it causes, from increased drug abuse and suicide to school failure and parental stress. The collaborative approach was possible in Group Health's clinics because they treat whole patients, in fact whole families, so an intervention that improves health saves them money. This approach would be difficult in most healthcare settings.

Last week I treated a young person who was in the hospital with a chronic medical disease that was complicated by ongoing IV drug abuse. This was a sweet person, still had her teeth and her manners, but had been anxious and depressed since she was 16 and had found that injecting methamphetamine and heroin really helped. She will die if she doesn't find another option, but she is in deep now, with a criminal record and hepatitis C and no job or insurance. I think that is why seeing this article about adolescent depression struck me so solidly. If something had actually worked at age 16 she wouldn't be spending tens of thousands of dollars of county money in the hospital while heading unobstructed for early death or at least ignominious disability.

Basically this is what we want, but how do we get there?
I think it's important to visualize population health, like visualizing world peace (or whirled peas, as the bumper sticker says). It doesn't need to look like forcing everyone to eat broccoli or get colonoscopies or avoid butter. It can look like getting rid of health and resource disparities that make some people succeed and some people fail spectacularly. It can look like clever ways of using the health care knowledge and technology we already have to make people stronger and less dependent on us. It will also have to look like a different payment system that supports hospitals when they keep people healthy on beautiful days like today.

Wednesday, August 27, 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 two 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 US 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 one 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 US 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 $1400 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 US 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 (3 of every 100,000 people have the disease) and some other diseases are also awful and have a higher prevalence. Stroke, for instance, has a prevalence of 3 per 100, and hypertension (high blood pressure) 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 ones head and donating to the World Health Organization.

Thursday, August 7, 2014

Technology, computers, the internet and the decline of concentrated attention

If you have trouble reading this, click on the photograph of the writing and then click again to enlarge it. Happy reading!