Thursday, October 14, 2010

The Medical Code

I spend a lot of my life thinking about language and the way that we use it. In my personal life, I'm a stickler for the precision of language; within my social group I have participated in originating several turns of phrase that cover concepts that are otherwise difficult to describe (in my social group the phrase for these phrases is "consensual language" -- i.e. language that means what it does merely because everyone in the social group consents to the definition.) On my walk to work on Saturday, I was thinking about how groups larger than my social group similarly, but less explicitly, co-opt certain words or phrases to inevitably express a specific idea.

This is particularly true in medicine -- both among doctors and between doctors and patients. Junior doctors present patients to an attending and are expected to use these phrases to paint their conclusions before stating what their opinion is. Similarly, despite the fact that taking a good history is a dying art, specific words that the patient uses can point to a differential. Perhaps the most famous example in all of medicine is the "Levine Sign" -- when a patient, while describing their chest pain, clenches their right fist closed over their mid to left heart (it is considered near-pathognomonic of angina.) In the past week, there were a couple of examples in my life that got me started thinking.

The first was also my very first role-reversal in the (misnamed) Attending Effect -- where a junior doctor, despite taking a good history, fails to be told a key piece by the family, which is then elicited by a more senior doctor (but not necessarily the Attending.) I'd sent my junior medical student to get a history from the family, while I took care of another patient. By the time I'd arrived, my medical student had spent at least 30 minutes very extensively getting the history and had done a very thorough job. Within 5 minutes of my arrival, I asked my first question: "When did your son get his Nissen fundoplication?" The father answered that it had occurred one month prior.

Afterwards, my medical student said to me, clearly frustrated: "I had just asked a past surgical history. The father swore his son had never had surgery. How did you know he had a Nissen?" And the answer was a single word: I'd walked in while the father was describing his cognitively normal 7 year old son "retching." There are only two things that I can think of that cause cognitively normal kids to retch without ever vomiting. The first is having a Nissen. The second is having a non-organic program (such as food aversion, attention seeking for some reason.) Given the rest of the history I'd heard in two minutes, the word "retch" was code for "Nissen" -- the father had essentially told me unasked that a Nissen had been placed, because I knew what to listen for.

The second story that led me to think about this was an event with an attending. I had admitted a little girl whom we suspected had epilepsy, who had been started on an anti-seizure medication, but was having adverse side effects from the med. In the course of picking a new medication, my attending became unconvinced that she even had epilepsy in the first place. Her EEG was negative. He asked me to repeat her semiology (clinical characteristics of a seizure). I started with "she has many types of events..." And he appropriately interrupted me, saying "so, she doesn't have epilepsy." I had used the code incorrectly (the code for seizures involves stereotyped movements, which should fall into one of few similar categories every time.) This time, rather than repeating word-for-word what I had been told, I instead made my case, using selective information: "yes, but one of the types involves being nonresponsive, with her eyes open, followed by rhythmic jerking, during which time she has bowel and bladder incontinence, and then is sleepy for half an hour afterwards." We started her on a new anti-seizure drug.

But perhaps the best story that I have on the code of medicine happened Saturday morning, when I arrived at work. We had admitted a new patient with "seizures" to the service. Having heard the story (by report, she was a teenager who had a history of having "jerks," which lasted seconds for years, but on the day prior to admission had had a "cluster" of "jerks," lasting minutes, which suggests a diagnosis of juvenile myoclonic epilepsy), I put in orders for an EEG and seizure precautions, and went to go see her.

The main differential diagnosis in a teenager who is having seizure-like activity is a new epilepsy disorder (and then there's a diagnostic dilemma within epilepsy, which is beyong the scope of this discussion) and pseudoseizures (also called non-epileptagenic epileptaform events, non-epileptic seizures, psychogenic-seizures and a host of other, confusing, names.) The way you tell the difference is usually with a good history and an EEG. An EEG is tough, because often, events don't get captured on EEG and then if the baseline of the EEG is abnormal, it's easy to call it epilepsy, but if the baseline is normal it's impossible to rule out epilepsy on interictal (between seizures)EEG. Therefore, a good history is the go-to tool in diagnosis.

Therefore, I started with a very careful history of what happened when she gets the jerks. She told me the jerks usually started in her head/neck, but sometimes spread down to her body. She described them as lasting seconds, but often occurring in clusters. All of that is a beautiful history for juvenile myoclonic epilepsy, and the beautiful thing about juvenile myoclonic epilepsy is that it's a type of epilepsy that will show up on EEG, even without a seizure, so I was getting pretty ready to call it a day for history taking. But then, as I was wrapping up the case in my head, she said "the movements begin with this compulsion to move." Carefully, I responded "'Compulsion' means something very specific to me, and I want to hear exactly what it means to you." She defined it: "I get this urge to move and it gets worse and worse and I can put it off, but then it overwhelms me and I have to move." And I realized that I'd also missed a huge piece of the history: she'd told me that right before her clusters started, she'd been playing in the band. Now, with a new diagnosis in hand, I asked "have you ever gotten a jerk while playing your musical instrument?" She hadn't. The diagnosis was clear. She had tics, not seizures. Tics are a different movement disorder, one which rarely gets confused with epilepsy, because the movements are typically not stereotyped and are always voluntary (not voluntary in the sense that the patient wants to do them, but voluntary in the medical code, meaning that the patient can delay doing them, and is giving in to an urge to move by choosing to move in the way that they feel the urge to do so.) The keyword in the whole case had been the "compulsion." I canceled the EEG and her seizure precautions.

We sent her home 90 minutes after admission (a new personal record), having done no diagnostic testing except a thorough history, which is why being fluent in the code is so important.

Saturday, August 7, 2010

When (and what) to disclose

It's always hard to be part of a genetics consult. It seems more encompassing than infectious disease or cards or the other organ specialties. It's a lot easier to say to a parent: "Mrs. Smith, I think that there's something wrong with the baby's liver (or even brain)" than just "I think that there's something wrong with the baby."

I wasn't thrilled to tell the mother at all that we were getting genetics to look at her new baby, which had been perfectly healthy as far as anyone knew up until 10 hours before (also known as the time of birth.) But at the same time, it's not the sort of thing you can completely withhold, either. Luckily, there was more to this particular patient than just having unusual facial features, so at least I could give the parents a reason I was calling the geneticist, instead of "your baby looks funny" (N.B. I would never actually say that, but it is hard to explain to a parent that their baby is dysmorphic in lay language. We usually use the word "differences," but I was having a particularly hard time in this case. We'd gone through me telling the family that the baby had some facial differences and the family insisting that the baby looked like their other kids. Which, fine, but when I see a picture of your other kids, I know that's not true and I don't want to disagree about it.)

Complicating the whole situation was the fact that the overnight team thought that the baby had Down Syndrome. I still am not exactly sure what the team said to the family, but when I came on it was immediately clear that the baby did not have Down syndrome. So I also wanted to let the family know that if they'd heard the words "Down Syndrome" to take those words out of their head. Unfortunately, to me and to the genetics team that came to officially consult, it was clear that the patient looked syndromic; however, there was no particular syndrome that sprang to mind.

I would say about 50% of genetics consults (fewer for experts, more for novices) fall into this category. Something is wrong and that something is...?? That's a really hard conversation to have with any family. Often, if the patient is on the floor or in the unit and going to stay for a while, we won't mention that genetics is coming until the testing is done and we have an answer (I usually will mention something vague like "we're doing some testing to see if we can come up with one answer that ties together everything that is going on with your child.") However, when the child is a well baby, they go home after 2-3 days, so we have to discuss it before results are back. It's a terribly hard conversation: "we've sent genetic testing; there may be a genetic disease that your child has, but I won't be able to give you any more information for at least 2-3 weeks. What questions do you have?" And then, of course, in this situation "well, I disagree with the overnight team; it's not Down Syndrome, but I don't know whether it's better or worse." (I didn't say that, either.)

I did my best. I told the family that until we know more, there's no point in starting to worry. I told them to enjoy the child, learn more about them as an individual and love the child for the next couple of weeks. I told them until we have answers, we'll deal with each problem as it comes up and troubleshoot each one. But it's hard. Genetics is a field people go into because they want to be able to give families answers. We hate vagaries and unconnected symptoms. I wish that there were a way to both be honest and open, but not throw parents into this diagnostic grey zone for weeks.

Thursday, July 22, 2010

The Sad Part of Pediatrics

I'm never sure where the line in the sand is between appropriate blogging and toeing HIPAA, but what I was taught in med school was follow the letter of HIPAA and this situation has been eating at me and needs to get blogged.

One of the things that I love about being a pediatrician and having my own clinic is the continuity of care. I am these people's pediatricians. I had my fourth visit with a four-week old today and it was the most awesome thing ever. Next week, I have a visit with one of my babies from the nursery, the younger brother of one of my patients and a well visit with one of my personal patients. That's also totally awesome. (The best thing about the newborn nursery is when I ask patients who they want their pediatrician to be and they say "can I come see you?")

Things that are less totally awesome: having continuity of care means I'm the one who knows when the ball gets dropped. So one of my babies is still in the "neonatal" period (first month of life) and has missed twice as many visits than it is weeks old. They've come to a whopping one visit. While at that one visit, I felt like the parent was really disengaged and they even left the room while I was examining the baby. They didn't schedule another visit, so I called the family and reminded them how important it was to be seen. They agreed, I gave them the benefit of the doubt and overbooked my schedule in order to see them at a convenient time for the family.

I think this story is going somewhere obvious, but I was still really sad when they didn't show up. So, luckily, I have good social work resources. So we did some exploring. And there are...other issues (the kid isn't really getting fed) and we've warned the family that this is likely going to become a DHS case (our version of CPS). But it makes me really sad. I feel guilty for my role in getting authorities involved, even though I know that it is absolutely the best for this kid. I want to believe that every parent is trying to make things work and is doing their best for their kid. But in a lot of ways, that's a naivete born out of primarily doing inpatient peds, where parents cared enough to at least bring their kid to medical attention when things were bad. I really hope things turn out OK for this kid, but also for this family and that having authorities involved will be a needed wake up call.

Sunday, July 18, 2010

Prenatal/Neonatal Visits

One of the problems that I think faces all doctors is that we lose perspective on the gap between what we know and what most people know. This is particularly obvious in my newborn visits. Like most doctors when doing newborn teaching for a family my goals are
1. Making sure that the family is feeding the kid correctly
2. Making sure that the kiddo is being put to sleep in a safe environment
3. Making sure that they're using a carseat and doing so correctly

Many doctors also use this time to talk about smoke detectors and smoking and carbon monoxide detectors, but I'm a rebel. I will see my newborns again in one week, and it's pretty unlikely anything will change on that front, so I can talk about those things at the two week visit. Why do I care? Because there is only so much information that parents can handle. And instead of talking about smoke detectors, etc. I try to answer the most common parents concerns (both that they have at the newborn visit and that will cause them to come back unnecessarily between the newborn and the two week visit)
1. The equivalence of the hospital brand of formula and the formula that WIC gives out (This is the number one question I get asked)
2. Things normal newborns do -- sleep a lot, get blue hands and feet, breathe funny and strain when they poop (I also try to talk about the line between the normal form of each of these and their pathologic equivalent and when it's worth bringing the kid in for a sick visit.) In infants with dark skinned parents, I like to let them know the skin will get darker (in my patient population fathers worry about mistaken paternity because the newborn is light).
3. Things many normal newborns have -- I let parents know if their kid has a murmur or an umbilical hernia or a hemangioma and that it's normal and how I expect it to change over time.
4. Things that I expect to change before they see me again -- kiddo should start eating more, jaundice should fade, if stools haven't changed from meconium they will, formula kids may get constipated (and that's OK)
5. Things that have already happened and what they meant -- most parents do not understand why their kid got a hepatitis B vaccine in the nursery, what the newborn screen was for, what "that test that they took the baby away to do" was (hearing), or why the baby had black stools at birth and I usually go over some of that.

So by the time we're done going over all of that it's a HUGE amount of information. And that's before I have the nurse come in and give them teaching on how to use a rectal thermometer and how to mix formula. It's a ton of information.

In Ohio, we had new parents come in for prenatal visits. I could talk to them about everything except the particular normal variants that their kid had. Then we would review at the newborn visit, and I would have the time to talk about smoking and home safety and all that jazz and they would have room in their brain to listen. For some unknown reason we don't offer prenatal visits here and I feel like everyone loses out from that.

The time to tell parents how to properly feed a newborn is not when the newborn has already been at home for five days. I'd love to reinforce the need for a jaundice check at day of life three or four, but when parents don't come in to see me until the kid's a week old, at that point there's nothing I can do.

I also think that there's some (a minority) of pediatricians who make up for the lack of prenatal visit and still manage to cram in home safety by sacrificing discussing parental concerns. And I think the reason that happens is that pediatricians lose sight of the knowledge gap. The result is that I've had kids come into the emergency room for glabellar hemangiomas (a normal birthmark. Also called an "angel kiss.") When this happened I asked the parents "Hasn't the kid had that since birth" and they said "well, yes, but it kept getting darker and darker and now he's crying and it's even darker." So I asked, "what did your pediatrician tell you about it?" And they said "oh, our pediatrician never mentioned it."
So it's possible that the pediatrician did give them a great talk on what the hemangioma was and what to expect and the parents didn't remember (because they were on information overload!)
But there are also pediatricians for whom hemangiomas are so routine (about 25-50% of babies have one) that they forget to teach about them. And then, as hemangiomas do, they get darker with time and darker when the kids cry and the parents panic.

Sunday, June 13, 2010

Tomorrow, I am going to walk into the largest and best children's hospital in America and pretend to be a doctor who works there.  Or at least, that's what it feels like.  It feels unreal.  In some ways, the last five years of my life have been a lot of learning how to play "the game" -- learning to put the best foot forward, make a good impression, meet the right people, get the good test scores & accumulate the symbols of ones status and knowledge.  Completing med school feels almost like a purely symbolic task, performed to sate my determination and showcase my ambition and intellect, but having nothing to do with actually becoming a doctor.
    By and large, I know that the ways in which the above are actually true are minimal compared to the other ways in which med school was important in my life -- what I've learned about really caring for other people (not just as a turn of phrase), how to perform myriad clinical tasks from procedures to telling someone that their loved one has died and learning massive amounts of biomedical science.  I know that I am the best I have ever been -- the smartest, most educated, most knowledgeable, but also the most compassionate, most understanding of ethics and least cynical I have ever been.  I know that I can be a resident and that I will be a good doctor.  I know, intellectually, at least, that I deserve to be a doctor at America's best children's hospital.  That doesn't stop me from feeling like an impostor, though.

    I think that part of the feeling of unreality is that the dream of being a resident at this particular hospital, or even a hospital like it, is less than a year old.  Just over one year ago, I decided that if I was serious about making a career of clinical genetics, I needed more training in it than what I had gained from my home hospital.  My home institution had a genetics department that I love and that treated me extremely well, but, by their own admission, was small and not equipped to prepare me to deal with a large, academic genetics department.  In search of the best place to have a genetics experience, I landed on the hospital that had trained my favorite teacher and mentor.  I never once considered that I would try training there for longer than one month -- the hospital was large, with a reputation for being snooty and, I felt, too prestigious to take me.  
    I changed my mind last August, after stepping foot in what will be my work home for the next five years.  There was something in that atmosphere -- it buzzed with scientific curiosity; little things, like the protocols for hand-washing, were clearly laid out and it was clear that from every level, there was an investment in improving the hospital even more.  But moreover, it was a giant place for kids and every single person I met from MAs to world-famous MD/PhDs cared about providing the best care for children anywhere.  I wanted to be a part of that.  I had never been exposed to such a large-scale, free-standing children's hospital before.  I had no idea that they even existed.  My home hospitals, largely out of necessity, shared the most specialized of sub-subspecialists between adult and pediatrics.  This hospital had the most arcane specialities just for kids.  Every day I came home even more impressed with the hospital.  I knew I wanted in.

    However, even for pediatricians, getting a residency spot is competitive.  I walked out my last day trying to commit details to memory, just in case it was the last time I ever set foot in the hospital.  And my application process was rocky: I was denied an interview.  It was overturned, but I knew that my dream was over.  I focused on the less "reach" programs.  When I came for my interview I repeated the ritual of committing the hospital to memory on my way out.  I knew it was goodbye.  

    For all of that, I wasn't shocked when I matched at The Hospital -- correspondence between interview day and Match Day was very encouraging -- but by that point, the journey had been so hard fought that it felt symbolic, rather than real.  Last week, when I entered The Hospital for only the second time since interviews was the first time it hit me: this is my home hospital know.  I work here.  I'm part of this.  Weird, right?

Saturday, February 13, 2010

Reflections on palliative medicine

I spend a lot of my time thinking about dying. Not in a morbid way, but in the way in which death is an inevitable portion of my job. One of the things that I had never really given any consideration to is how rare of a characteristic this is in a pediatrician . I mentioned my interest in palliative medicine to one of the younger students and he replied with "but I thought you wanted to go into pediatrics?"
It's true that kids die very rarely. However, it is also true that the sorts of conversations that happen at the end of life have a lot in common with the sorts of conversations that happen in pediatrics. In both there is a time at which we no longer make decisions as directed by the patient personally, but as directed by someone who (hopefully) loves the patient and makes decisions in the way in which they think that the patient would. Many geriatricians and palliative medicine doctors would argue that the difference is that in the care of the adult who is no longer able to make decisions for himself or herself, we have a history of decisions that he or she has made and (in the rare, but ideal case) a set of directives that has been left. However, there is a multitude of evidence now that the decisions that we say we would make in given circumstances are not the decisions we do make when we are in the circumstance. The inherent problem in the concept of "substituted judgment" (i.e. making a decision on someone else's behalf in the way in which we think that they would have made the decision were they able to themselves) is that it requires the idea of a static identity. Do you make decisions on the basis of what the person would have wanted a year ago, even if they may have had some dementia at that point or as they would have ten years ago before they had any dementia or as you predict that they would be now if the person who they were ten years ago never developed dementia?
This is in fact more complicated with kids, although I would argue it is more difficult the older the child is. With a baby one can make certain generalizations -- no one will fault a physician for assuming that an infant will value life, wellness and cognitive function. The other major wrench that makes this easier is that the younger the patient, the more likely that if you can get them to survive you can get them to good long term prognosis -- children are extremely hardy. On the other hand, teenagers have their own independent thoughts and values and those should be taken into account; however, if a teen is catastrophizing, for instance, how much of that is a normal developmental position and how much is the teen's personality.
I had a case of a teenager who was near, but not at, the age of majority. This teen had a diagnosis that was inevitably terminal, likely within the next few years at the outside. The teen wanted to transition to focusing completely on palliation, rather than curative therapy. The parents wanted to continue to try more curative therapy, reasoning that the field could change sufficiently within a couple of years that new treatment may be available. In this particular case, the prognosis was really quite grim, and ultimately, after many meetings with the family, the decision was made to transition to palliation and the patient passed away. However, what if the prognosis were not grim? Treating cancer typically requires several months of treatment, during which the patient may lose their hair, will experience extreme fatigue and be nauseated. If the prognosis is terrific, it is easy to suggest that the parents should require their minor child to undergo it. If the prognosis is truly terrible, it is viewed as cruel to force a child to undergo it. But what should be done in the intermediate cases? Patient-centered medicine dictates that we allow the patient to weigh the costs and benefits in their own value system. But for children, several months is a very long time and the future gain seems very theoretical; therefore, the gain assigned by most children will be less than most likely would be assigned by the same individual were they an adult. On the other hand, bodily autonomy is an important developmental milestone throughout childhood, and it feels uncomfortable to force a child to undergo such physical discomfort.
On the other hand, I've spent five weeks on services focused on end-of-life discussions, and at least in my experience, most of the media attention on end-of-life issues is really overblown. The vast majority of the time, the patient and the treatment team agree in their view of where the patient is at in the life course; what reasonable goals are for care and when to transition to palliation as a primary goal. When there's disagreement it seems to be roughly evenly split between the patient ready to be done with curative therapy before the treatment team and the treatment team before the patient. Both ways, the conflict is typically resolved usually by a discussion of what the patient's goals are.
My current attending likes to focus on the "levels of hope" -- that it's typical and normal even for patients who are terminal and know that they are terminal to still hope for cure. That this is not unrealistic or denial, but rather analogous to the person who buys a lottery ticket or gets average grades but applies to Harvard medical school. He asserts that this is not at all unhealthy, as long as it is not the exclusive focal point. The patient should also have hopes that are not dependent on a cure, such as resolution, relationships, spiritual closure or whatever is important to that particular patient. Patients who don't want available curative therapy usually don't have anything against curative therapy, but are hoping not to have more pain, for example and patients who do want curative therapy, when it's not really realistic, often want it for the sake of something else. By focusing on these primary goals, rather than what needs to be done to get there, it's easier to get the physician and patient on the same page. It's a good technique. I'm not sure whether it will be helpful in a pediatric setting, but it's another tool in the toolbox, which I'm sure I'll use some day.
In the last six months, especially, the majority of tools I've been gaining have been communication and philosophical skills and efficiency; not that I've stopped learning physical exam techniques or medical science, but the ratio is changing. When I interviewed, I referred to these as "ancillary skills" -- that most medical schools focus on scientific knowledge, but that to be a good doctor, it's important to know how to be a professional, to know how to have hard conversations, to know when a conversation is necessary and when to let the decision a patient or family makes stand unchallenged, etc. It's February; in five months, there will be patients for whom I am their primary care doctor. There will be babies for whom I will have been the only pediatrician that they've ever had. That, more than the idea of being the intern on-call for the NICU, more than the idea of being the first doctor in the room for a code, more than the idea of having to do lumbar punctures and arterial lines, scares me. That is what motivates me to learn as much as I possibly can (even though senioritis is kind of creeping up on me.) I want to be the sort of pediatrician that my teenage patients can come to about sex and drugs; I want to be the sort of pediatrician who can counsel worried parents about vaccines; I want to be a trusted advisor. I've learned a shockingly large amount in the last four and a half years; I think I'm going to be OK.