Monthly Archives: November 2013

Tonight my 5 year old son chased me around the house for a very long time.  We circled tables, climbed over couches, and tumbled across beds.  As he ran after me with all of his energy, he laughed with rapturous delight.  And I got to thinking about the emotions of children.  Their feelings are like bright colors, while those of us adults are so subtle we often hardly realize their presence.  

Why do adults stop having fun?  Is it because we have responsibilities to which care-free kids are oblivious?  Perhaps.  But I live near some tennis courts and when I watch people play, they look deadly serious, as if their lives are hanging in the balance.  In those moments, these adults are not working for their livelihood or engaged in some other activity essential to their survival.  They are playing a game and yet it takes hitting a spectacular shot to bring a smile to one of their faces.  And I am probably the same way when I play.

Is children’s ability to more easily delight in life due to its freshness and novelty?  Do adults get bored with what we’ve become used to?  I think that is part of the answer.   

There are also the expectations we adults place upon ourselves and each other.  In childhood you are supposed to discover and be surprised by what you find.  In contrast, we adults take pride in not being caught off guard.  We are wise to the world.  Our humor is that of wry irony.   

Does being an adult require leaving the frivolous fun of childhood behind?  Or should we cultivate a space in our days to do nothing but delight in the miracle of our existence?

As many of you know, about 1 year ago my younger son was diagnosed with autism.  Before that time, I had brief encounters with children on the autism spectrum during my pediatric rotations of medical school.  But my first sustained exposure to it was with Jack Robbins, the son of my wife’s cousins Brent and Carla.  We lived in the same neighborhood with them when they received Jack’s diagnosis.  Since then we have witnessed how they’ve given all of themselves to provide him with the best life possible.  Their courage and love serve as an inspiring example for us in our experience with our son.  

In recent days, Jack has become a bit of a celebrity. His family realized that what they thought were meaningless sounds were actually Jack singing Katy Perry’s song “Roar.”  This was the first time he had meaningfully put words together on his own.  Carla and Brent put the video on YouTube and it’s become a viral hit with features on Yahoo and Huff Post.  Here is a link to Carla and Jack being interviewed on their local TV station.  

You will consider it time well spent if you listen to Terry Gross’ interview with astronaut Chris Hadfield.  Here is a link to the NPR website with the full interview, key excerpts, and a beautiful video of Hadfield singing his own version of David Bowie’s “Space Oddity” from outer space.  

He describes what it is like to be in space outside of the shuttle or space station. 

The contrast of your body and your mind inside … essentially a one-person spaceship, which is your spacesuit, where you’re holding on for dear life to the shuttle or the station with one hand, and you are inexplicably in between what is just a pouring glory of the world roaring by, silently next to you — just the kaleidoscope of it, it takes up your whole mind. It’s like the most beautiful thing you’ve ever seen just screaming at you on the right side, and when you look left, it’s the whole bottomless black of the universe and it goes in all directions. It’s like a huge yawning endlessness on your left side and you’re in between those two things and trying to rationalize it to yourself and trying to get some work done.

When Terry Gross asks for him to relate an especially anxiety-provoking moment from his time in space, Hadfield recalls once suddenly becoming blind in one while working.  He realized some liquid had somehow entered his eye.  He tried to work through the pain, but rather than falling to the ground, the tears streaming from his eyes migrated to the other eye, leaving him temporarily blind.  This prompts him to describe what it feels like to close your eyes in space.

What does it feel like when you close your eyes when you’re weightless? Normally on Earth when you close your eyes you can feel your feet on the floor or your rear end on your chair or something and that gives you a sense of up. You can balance with your eyes closed, you can walk with your eyes closed because of all of the external references. When you’re weightless and you close your eyes it’s as if you just stepped off a cliff into complete blackness and you’re falling forever, so the perception of that is really odd. You can do it as like a thought experiment and instead of closing your eyes and thinking that you’re just floating, close your eyes and picture that you’ve just stepped off the Half Dome in Yosemite and are now falling into the blackness, and it’s interesting to see how your body reacts to it.

One of things that struck me in listening to the interview is that Hadfield never seemed to lose his awe for what he experienced in space.  Here he is talking about doing a spacewalk amid the Southern Lights.

I was coming across the Indian Ocean in the dark. I was riding on the end of the robot arm … [and] I thought, “I want to look at Australia in the dark,” because everyone lives along the coast, starting with Perth and across and it’s like a necklace of cities. So I shut off my lights, and I let my eyes completely adjust to the darkness, but as we came south under Australia instead of seeing just the lights of the cities of Australia we flew into the Southern Lights. Just like the Northern Lights they erupt out of the world and it’s almost as if someone has put on this huge fantastic laser light show for thousands of miles. The colors, of course, with your naked eye are so much more vivid than just a camera. There are greens and reds and yellows and oranges and they poured up under my feet, just the ribbons and curtains of it — it was surreal to look at, driving through the Southern Lights. …

To me it was taking time to notice something that is almost always there but that if you didn’t purposefully seek it out you would miss — and that is our planet and how it reacts with the energy from the sun and how our magnetic field works and how the upper atmosphere works — what it really is, is just beauty.

Hadfield also relates the effects of space and space travel on the astronauts’ bodies.  He developed osteoporosis, or thinning of the bones, a condition I often see and treat in my patients.  Thankfully, this condition at least partially reverses as an astronaut spends time back on Earth.  Scientists are seeking to understand how this happens and perhaps someday apply this knowledge to preventing hip and vertebral compression fractures in us regular, non-astronaut folks.  His description of the punishing collision astronauts endure when they land on Earth left me in awe of their bravery and toughness.

When Hadfield details how astronauts learn to cope with the many fears that their work evokes, it reminded me of cognitive behavioral therapy (CBT).  You confront your fears by thoughtfully planning for the realities you will face. 

Half of the risk of a six-month flight is in the first nine minutes, so as a crew, how do you stay focused? How do you not get paralyzed by the fear of it? The way we do it is to break down: What are the risks? And a nice way to keep reminding yourself is: What’s the next thing that’s going to kill me? And it might be five seconds away, it might be an inadvertent engine shutdown, or it might be staging of the solid rockets coming off. … We don’t just live with that, though. The thing that is really useful, I think out of all of this, is we dig into it so deeply and we look at, “OK, so this might kill us, this is something that would normally panic us, let’s get ready, let’s think about it.” And we go into every excruciating detail of why that might affect what we’re doing and what we can do to resolve it and have a plan, and be comfortable with it. …

It’s not like astronauts are braver than other people; we’re just meticulously prepared. We dissect what it is that’s going to scare us, and what it is that is a threat to us and then we practice over and over again so that the natural irrational fear is neutralized.

At the end of the interview, Terry Gross asks Hadfield about his own spirituality and how his experience in space has influenced it.  

The big pervasive feeling onboard looking at the Earth [from space] is one of tremendous exquisite privilege that it exists. … But I think what everyone would find if they could be in that position — if they could see the whole world every 90 minutes and look down on the places where we do things right, and look down where we’re doing stupid, brutal things to each other and the inevitable patience of the world that houses us — I think everybody would be reinforced in their faith, and maybe readdress the real true tenets of what’s good and what gives them strength.

The Washington Post reports on a new study that reveals the prevalence of depression around the world.  Researchers at Australia’s University of Queensland found that the Middle East and North Africa suffer the world’s highest depression rates.  Globally, depression is the second-leading cause of disability, with slightly more than 4 percent of the world’s population diagnosed with it.  The article reports that 

the most depressed country is Afghanistan, where more than one in five people suffer from the disorder. The least depressed is Japan, with a diagnosed rate of less than 2.5 percent.

What accounts for the higher rates of depression in certain countries?  Researchers theorize that military conflict is one important cause.  The article also points to a 2010 paper by the Inter-American Development Bank which found that unemployment, low incomes and high income inequality correlate with high depression rates.  Finally, there is the age factor.  Studies have shown that people between 16 and 65 tend to suffer depression at much higher rates.

That age factor, along with massive population growth, actually explains the fact that the burden of depression has grown by nearly a third since 1990.  And since both aging and population growth are likely to continue, that makes low-cost depression interventions even more of a priority for both global organizations and national governments. 

The November 6, 2013 issue of the New England Journal of Medicine has an important editorial entitled Grading a Physician’s Value: The Misapplication of Performance Measurement. It begins by acknowledging that

perhaps the only health policy issue on which Republicans and Democrats agree is the need to move from volume-based to value-based payment for health care providers. Rather than paying for activity, the aspirational goal is to pay for outcomes that take into account quality and costs.

 

But even as the authors  

agree that value-based payment is appropriate as a concept, the practical reality is that the Centers for Medicare and Medicaid Services (CMS), despite heroic efforts, cannot accurately measure any physician’s overall value, now or in the foreseeable future.

They argue that “physicians simply do not respect the (current quality) measures, and for good reason.”  Such quality measures

reflect a vanishingly small part of professional activities in most clinical specialties. A handful of such measures can provide a highly misleading snapshot of any physician’s quality. Research shows that performance on specific aspects of care does not predict performance on other components of care. Primary care physicians manage 400 different conditions in a year, and 70 conditions account for 80% of their patient load. Yet a primary care physician currently reports on as few as three (quality) measures.

They continue:

One definition of physician professional competence is “the habitual and judicious use of communication, knowledge, technical skills, clinical reasoning, emotions, values, and reflection in daily practice for the benefit of the individual and the community being served.” Patients place emphasis on physicians’ confidence, empathy, humanity, personability, forthrightness, respect, and thoroughness. A global measure of value should capture most, if not all, of these diverse elements of desired performance. Yet available measures in the Physician Quality Reporting System (PQRS) and elsewhere are relevant to few of these professional qualities.

 

More concretely, examples of important but mostly overlooked aspects of physician performance that we would want to measure include making accurate and timely diagnoses, avoiding overuse of diagnostic and therapeutic interventions, and caring for the growing number of patients with multiple chronic conditions and functional limitations.  A radiologist’s primary role is to provide accurate and complete interpretations of imaging studies. Yet because we lack measures of accuracy for radiographic diagnoses, PQRS measures include “exposure time reported for procedures using fluoroscopy” and “inappropriate use of `probably benign’ assessment category in mammography screening.” The PQRS is predicated on the dubious proposition that measuring and rewarding performance on such obscure clinical aspects of care is worthwhile. Even if such activities are beneficial, performance on these measures is not indicative of a radiologist’s quality as part of the CMS value calculation.

 

Consider quality for surgeons. We want to be able to measure performance on core competencies that affect outcomes, such as judgment about whether and when to operate and which procedure to use, as well as the surgeon’s technical skill in the operating room. Yet because these characteristics are difficult to quantify accurately and routinely, PQRS measures for surgeons instead include adherence to guidelines for antibiotic and anticoagulation prophylaxis. Again, these measures assess worthy prevention activities but do not reflect a surgeon’s contribution to producing value.

 

The bottom line is that the things in health care that are most easy to measure quite often are not what translates into healing, value, and a higher quality of life for patients.  

Another issue the authors address is judging a physician by how much money is spent in the care of his or her patients.  The government and insurance companies will be increasingly penalizing and rewarding primary care physicians on this basis.  This is problematic since

current methods for case-mix adjustment do not adequately capture variations in patients’ illness severity, complicating coexisting conditions, or relevant socioeconomic differences — differences beyond the physician’s control that affect the cost of care. 

I am very much for a health care system that rewards high quality, cost-efficient care.  But for that to become a reality, researchers must develop much better methods of accurately measuring quality and cost-efficiency.

Here is a review I wrote on a book about blood called Five Quarts.  

It is only natural to take for granted that with which we are most familiar. Just as a cell down- regulates its receptors in response to activating hormones, so humans quickly become habituated to novel experiences. The surgeon who at the beginning of her training found the operating room to be a terrifying and exciting place, years later experiences it as a zone of comfort and security. In relationships, the thrill of reciprocated affection in time gives way to the quieter love of friendship, marriage, and family. But even as we cherish our dependable routines, we sometimes feel nostalgia for a time when the familiar was new. This is why the long-married couple still takes time out for formal dates. It may also explain why many physicians strive to impart their knowledge to younger generations. Through teaching, these seasoned professionals vicariously relearn their craft. Perhaps procreation is the most obvious example of the human desire to make the familiar new. Parents know that a child brings fresh eyes to the world, making the ordinary extraordinary again.

A layman’s naiveté can likewise bring a unique sense of wonder to a complex subject like blood. This is certainly true with respect to Bill Hayes’ new book, Five Quarts: A Personal and Natural History of Blood. Incorporating mythology, history, literature, medical science, and personal experience, the freelance writer Hayes has composed a fascinating exploration of the crucial red liquid that we physicians so matter-of-factly extract and study every day.

Hayes’ discussion of the mythological figure Medusa near the beginning of Five Quarts serves as a launching point for the rest of the book. Besides having snake-hair and a face that turned all of its beholders to stone, Medusa’s blood also possessed special qualities. If drawn from the left of her body, her blood brought instant death. But if extracted from Medusa’s right side, it restored life. Hayes remarks that “this duality was an especially prescient invention of the ancient mythmakers, for we now understand in cellular detail how blood can bear both disease with deadly efficiency and save a person’s life, as with vaccinations and transfusions.” This idea of blood as both a killer and a savior, as a source of both dread and fascination, recurs throughout Five Quarts. Indeed, it flows from the deeper purpose of his book. Hayes’ long-time partner is HIV positive, and one senses that the author’s exploration of blood is a quest to understand the mysterious substance that has afflicted his beloved.

As part of this quest, Hayes recounts the history of humanity’s efforts to grasp blood’s content and meaning. Particularly interesting in this respect is the story of Paul Ehrlich’s life and work. Born in 1854 in a small German village, Ehrlich was the only son of Jewish parents who operated an inn. As a teenager, he was fascinated by his older cousin Carl Weigert’s research staining cells with synthetic dyes. Generated by the flourishing German dye industry, these dyes revealed contrast and texture in biological specimens, making them easier to view under the microscope. In his own studies, Ehrlich observed that different dyes responded uniquely to certain cells or parts of cells. To him, this suggested the existence of distinct cellular receptors (“side chains”) for the various dyes. The concept of cellular receptors ultimately led Ehrlich to his side-chain theory. It proposed that cells have receptors or side chains that bind invading toxins “like a key in a lock,” thereby neutralizing them. The theory further held that a cell under threat grows additional side chains to bind the toxin and that these additional side chains break off to become antibodies that are circulated through the body. As Hayes reports, Ehrlich’s revolutionary side-chain theory was just one of his many crucial contributions to the field of hematology.

Exploring blood’s role in literature, the author considers Bram Stoker’s Dracula. It turns out that the historical inspiration for the great novel’s vampirism may have been the hematological disorder of congenital erythropoietic porphyria. Inbreeding within the isolated and remote Eastern European communities, such as the valleys of Transylvania, may have resulted in an unusually high prevalence of this rare condition during the Middle Ages. As Hayes writes, “The corpse-like appearance and odd behavior of sufferers may have given rise to whispers of vampirism.” Folk knowledge about the disease may have gradually evolved into legend. Certain chemicals in garlic, for example, can exacerbate porphyria symptoms, thus giving rise to the idea that garlic repels vampires.

Toward the end of Five Quarts, Hayes writes about Dr. Jay Levy’s codiscovery of HIV and his remarkable contributions to understanding and fighting the disease. The medical progress made possible by Levy and other scientists profiled in the book has prolonged the life of Hayes’ HIV-positive partner. Much of the book can be read as a grateful tribute to their efforts.

For its beautiful writing, captivating stories, and eclectic approach to the subject of blood, I heartily recommend Five Quarts. It just might affect the way you look at your next blood smear.

Francis Collins is the current director of the National Institutes of Health (NIH) and oversaw completion of the Human Genome Project.  He wrote a book called the Language of God in which he shared his Christian faith and its relationship to his work as a scientist.  Here is my review of it.

Stepping into the crossfire between these two warring camps is Francis Collins, a scientist with impeccable credentials and a devout Christian. Among Collins’ accomplishments is a gene-hunting technique called positional cloning, which his laboratory used to isolate the genes responsible for cystic fibrosis, Huntington disease, and neurofibromatosis (2). In 1993, Collins was selected to succeed James Watson, the codiscoverer of DNA, as the director of the Human Genome Project. Under his leadership, a working draft of the human genome was announced ahead of schedule in June 2000. In The Language of God, Collins seeks to bring his credibility in both scientific and spiritual circles to bear in an attempt to achieve a harmonious synthesis between science and faith.

Collins begins the book by telling the story of his journey to belief. His parents were Yale graduates who did the “60s thing” in the 1940s, seeking a simple agricultural lifestyle on a farm without the use of machinery. Homeschooled in a place with no running water and few other physical amenities, Collins treasured his unique upbringing for the remarkable culture of ideas created by his parents. He writes, “Those early years conferred on me the priceless gift of the joy of learning” (p. 13). One area in which Collins did not receive instruction was religion, as his parents were relatively indifferent to faith. Collins likewise gave spiritual questions little thought growing up and described himself as a skeptical agnostic through his early adulthood. During his PhD program in physical chemistry at Yale, he read the biography of Albert Einstein. Learning that despite Einstein’s Zionist position after World War II he did not believe in Yahweh, God of the Jewish people, only reinforced Collins’ conclusion that no thinking scientist could seriously entertain the possibility of such a deity.

A turning point in Collins’ spiritual journey occurred during medical school when he encountered patients whose faith provided them with reassurance and peace during terrible suffering. This led him to question if belief could have a rational basis and to survey the world’s great religions. During his investigations, he was most influenced by C. S. Lewis’ Mere Christianity, a collection of radio talks delivered by the legendary Oxford scholar on the BBC during World War II. Lewis argued that the human conscience provides genuine insight into reality, just as our senses or mathematics do. In his view, for example, the statement that the Nazis’ treatment of Jews and slavery in America were morally wrong more closely resembled a fact than a cultural sensibility. Lewis contended that holding up morality as an objective reality rather than merely a human construct requires positing a Being that defines right and wrong. Collins was persuaded by this moral argument for God’s existence and impressed by the rest of Lewis’ case for belief. And though he continued to have doubts, Collins decided to take a leap of faith.

Collins goes on to share some of the rest of his rational basis for belief. He points out that 15 physical constants, such as the strength of weak and strong nuclear forces and the speed of light, all have values within the narrow range necessary for the existence of a stable universe capable of sustaining complex life. The odds of this happening by chance are almost infinitesimal. One theory proposed to explain this coincidence is assuming the existence of an infinite number of universes, each with their own physical constant values. Among these universes, it is postulated, ours happens to contain the physical properties permitting life and consciousness. The alternative explanation for the improbable conditions that make intelligent life possible is that rather than occurring by chance, they reflect the action of the One who created the universe. Collins finds this second account to be more elegant and compelling.

Collins’ next task is proposing a framework for reconciliation between science and religion. He first reviews what astrophysics, the geological and fossil record, and the study of genetic material across species have to say about the question of origins. He concludes that the evidence overwhelmingly reveals a universe billions of years old and that evolution through natural selection is the crucial generator of the diversity and complexity of life.

Collins then presents a case that this modern scientific account of origins is compatible with belief in God and the biblical narrative. Examining the Genesis creation story, he argues that the text intends to impart theological truths rather than provide a natural history. Here he follows a long tradition of biblical exegesis. For instance, in the fifth century, St. Augustine wrote contemptuously of a literal interpretation of the Genesis creation account: “The shame is not so much that an ignorant individual is derided but that people outside the household of faith think our sacred writers held such opinions … and are criticized and rejected as unlearned men”.

Collins also rejects intelligent design (ID) theory, which points out the explanatory shortcomings of evolution theory and on this basis postulates the involvement of an intelligent designer. He argues that ID fails to function as a viable scientific theory since it does not predict other findings or suggest approaches for further experimental verification. ID also fails to provide a mechanism by which its hypothesized supernatural interventions took place. Finally, many of the cases in nature that ID points to as examples of the inadequacies of evolution theory are now being shown to be consistent with it after all.

Collins points to theistic evolution as an explanation that reconciles faith and science. It is a view espoused by most serious scientists of all faith traditions and includes among its adherents Asa Gray, Darwin’s chief advocate in the USA, and Pope John Paul II. Theistic evolution holds that God used the elegant mechanism of evolution to create all of life, including human beings.

Francis Collins is under no illusion that his book will settle the often rancorous disputes between the religious and scientific communities. But he hopes at least to offer a model for more civil and reasonable dialogue. In this respect, I believe he has succeeded.

Vitamins are carbon-containing substances that the body relies upon to perform essential functions.  With the exception of  vitamin D, vitamins cannot be synthesized by humans.  They, therefore, need to be ingested in the diet or through supplements to prevent severe disorders.

A multivitamin usually contains the recommended daily intake of all the vitamins.  They are recommended for people at risk for vitamin deficiencies, such as those with alcoholism, malabsorption, a vegan diet, a history of gastric bypass surgery, or some inborn errors of metabolism.  For the rest of the population, the available evidence suggests taking a multivitamin does not offer any significant health benefits.  This is because we are already receiving a sufficient amount of vitamins in our diets.

It would be a grand task to comment upon all of the vitamins, so I’ll briefly discuss a vitamin that commonly comes into play in my medical practice: Vitamin B12.  

Vitamin B12 deficiency is most commonly caused by poor absorption and inadequate intake of B12-containing food sources such as liver, milk, fish, and meat.  Why might somebody not absorb Vitamin B12?  In some people, the body forms an antibody against a protein called Intrinsic Factor, that is needed to absorb Vitamin B12 in our gut.  This is called Pernicious Anemia.  Malabsorption can also occur in people taking certain medications.  For instance, Metformin is a commonly used medication for diabetes that prevents the liberation of Vitamin B12 from foods. Strong antacids for acid reflux can also decrease the gut’s ability to absorb B12.  Finally, surgeries that remove part of the stomach, such as gastric bypass weight-loss surgery, can make it difficult for the gut to absorb adequate Vitamin B12. 

Vitamin B12 deficiency can damage the central and peripheral nervous system.   Low levels can cause cognitive impairment and even dementia, as well peripheral neuropathies and difficulty with balance.  It can also cause anemia, which is a low level of the red blood cells that carry oxygen to our body’s tissues.

Before Vitamin B12 was identified, people with B12 deficiency syndromes were treated by being fed a copious amount of liver.  Upon B12’s identification, Vitamin B12 injections became the standard means of replacement.  More recently, it has been found that most people with Vitamin B12 deficiency can attain an adequate level of B12 through high-dose oral supplementation.  When I detect low B12 levels, I offer people the oral or injection route of repletion, then later check a blood test to ensure they have achieved an adequate level.

In my next post, I’ll discuss Vitamin D.