September 12, 2009

H1N1 Influenza—Swine Flu Update

When the H1N1 flu came to my office in April and May of 2009, I had been hearing a lot about it, but with few credible statistics. In a few weeks, I saw maybe a dozen kids and parents with the right symptoms to make the diagnosis. One or two actually had confirmed diagnoses with laboratory testing. I have a tiny practice, so that seemed like a lot at the time.

Perhaps it's obvious that everybody in Washington is expecting worse to come. The mini-epidemic last spring was relatively removed from typical winter conditions in most of the country, so if it returns in mid-winter, as the flu usually does, it might be worse.

In the winter, kids generally spend more time indoors with other kids, often playing actively or in close proximity to each other in school. That's a good way to spread germs. Even at home, that closeness favors the spread of contagious diseases. In the winter, the air is drier and our respiratory secretions are thicker and stickier. That, too, is kind of like flypaper for diseases.

There is another hand to this. So far, I haven't really read much about this particular influenza being more virulent than the regular influenza that comes around every season. That's not terribly reassuring in my line of work, since the regular old flu can be a serious problem for newborns, as well as for kids with a breathing condition like asthma. That's the good news.

The reason, I think, that there's so much worry is that it seems that nearly everybody who's exposed to this H1N1 flu gets sick. Think of what that could mean—you and your whole family all sick at once, your neighbors and friends sick, the pharmacist, and so on. It's not a pretty picture.

The nice folks at the CDC have said there will be a H1N1 vaccine available, probably in October or so. I don't know what the final recommendations will be for who will be the first priority of people to get it. At the moment, it’s recommended for all children and young adults, all pregnant women, and people with other risk factors. I'll get it if I can. There's a regular seasonal flu shot too—it's available now. It's worth a shot.

Note to my patients and their parents: if you want your child to have flu shots this season, please let me know ASAP. I might still be able to order them.

The Centers for Disease Control (CDC) is one branch of the government that works superbly. They are the most authoritative source on this planet for all news of epidemics and new diseases. There is no physician or expert who knows more than they do. All the major news organizations get their information from them, and you should too. The H1N1 Flu page is where you can find the best and most up-to-date information. It's updated at least once every couple of days. Don't be sold on something from the alarmist salesperson at the alternative-medicine pharmacy. Trust yourself and get the straight story from the CDC. They have no hidden agenda and aren't trying to sell you anything.

September 9, 2009

Mystery Diagnosis: Jocelyn

In April, 2008, Jocelyn came in to my office complaining of chest pain. Most 8-year olds don’t complain of chest pain. There are lots of stomach aches, occasional headaches. Many, many minor injuries from active play. Chest pain doesn’t occur to them. Her parents feared there was a problem with her heart.

In children, chest pain is almost never heart-related. The heart is made from powerful muscle, and it’s working constantly. Adult heart rates are about 80 times a minute. Kids can be well over 100; when they are running around, much higher than that. With active children, it often works even harder. When adults get heart-heartrelated pain, it’s almost always because the heart muscle isn’t getting all the blood it needs to meet its demands. Activity increases the demands on the heart, and the chest pain usually gets worse. In Jocelyn, if there were something wrong with the heart’s blood supply, she would have had to have been born with the problem. So it would have shown up before now. Still, I asked her some basic questions. The pain didn’t come with activity. It never caused her to stop what she was doing and rest. She had no problems breathing, even when playing soccer. Her chest didn’t hurt during activity, but her pain was worst on the nights of her most active days.

She wasn’t sick in any way. She had no rashes. She denied any falls or collisions with other people or objects. Nobody hit her. None of her joints hurt or were red or swollen. She had no headache. Her mother confirmed that she was acting just as usual. Which was always enjoyable for me. Even at 8, she seemed like she was going on 18. Unusually articulate and good-humored, she had an endearing confidence and an uncanny ability to participate in adult conversation as a peer.

I had been Jocelyn’s doctor for years. I knew her well and knew that she was very intelligent. She didn’t have anxiety, but she was worried that this could be her heart. She told me so.

Everything in her physical exam was normal. I listened for a long time on her chest and back with my stethoscope. I didn’t think it was her heart, and told her so as gravely as I could. She seemed relieved.

I asked her to stand and do the following maneuver. I had her hold her hands together behind her back with her arms completely straight. thorax3Then lift the hands as far as she could. This hurt her chest! I asked her to do it again, but this time I pressed gently on the spots where her sternum (breastbone, shown in green in the picture (but not green in real life)) meets her ribs. This hurt a lot.

Her diagnosis was costochondritis, an inflammatory condition of the joints where the ribs meet the sternum. (Yes, it’s considered a joint because it moves, every time you take a breath.) It’s usually treated with anti-inflammatory medicines, like ibuprofen, and rest.

It went away just as I had predicted. A couple of months later it happened again. At one point, I ordered a chest x-ray. it was cxr normal. Since April of 2008, I have seen her 4 or 5 times for this problem, and dealt with the problem over the phone another half-dozen times. Why does she keep getting this?

In March of this year, I did an extensive panel of blood tests to look for possible diagnoses. All of them are unusual in someone her age, but all remotely possible. Immune deficiencies, Rheumatoid Arthritis, Lupus.

Then, this Spring, she had the pain—and her knee hurt at the same time. It was so intense this time that she was crying and didn’t want to get out of bed. I rushed through an insurance bone scanauthorization request for a bone scan. In a bone scan, radioactive technetium-99 would get injected directly into her bloodstream. It is taken up by her bones, and concentrates in areas of disturbance such as infection, inflammation, and cancer. Special photography (not too different from the cathode-ray tube of an old-fashioned television set) is used to capture the radioactive hot spots. I hoped that the bone scan would show areas of increased bone activity and provide a clue to her diagnosis. From that, I could fashion a treatment, or at least know what specialist to send her to.

It was completely normal.

I’m not completely back to square one. I now have a pretty long list of diseases and conditions nobody would want to have that she probably does not have either.jocelyn growth curve2

I’ve been thinking about her growth. As I pointed out, she’s really smart and developmentally precocious intellectually. Physically, she’s developmentally normal except for her short stature. Is there something wrong with her growth? Are her bones growing unevenly or in a way in which there are subtle pressures concentrated in the center of her chest? How can I tell? In past years, I’ve sent her to both an endocrinologist (to look at growth hormone issues) and an orthopedist (to look at bone and joint issues), but they said she was normal. Who do I ask now?

September 3, 2009

Mystery Diagnosis: Martin

Who gets to make a diagnosis? Do I have the authority? Who would believe me?snowboarding

Martin’s story starts on December 28th, on a snowboarding vacation. He was a slender 18 at the time, healthy, athletic, and an avid snowboarder. He spent the first week in Idaho, and called me near the end of his second week of vacation, in Colorado. He had a fever of 102, had a really sore throat, and had been sick for a couple of days. Today he was much worse, and had vomited twice so far.

It was winter, he was staying with a big group of people, and he had different kinds of symptoms. Strep throat can cause sore throat and fever, but doesn’t usually cause vomiting. I suspected the flu. I told him to make sure he had lots of sips of clear fluid, and call again if he got worse in any way. He was scheduled to return home tomorrow, on the weekend, and I told him that if he wasn’t better, I would see him right away.

In the office, he told his story. He started getting sick about 9 days before. His first symptoms were fatigue and nausea. He vomited once that day and had no appetite. From that evening a week and a half ago, he has had a fever up to 103. He’s had muscle aches and backache. Then the rash appeared. It’s not itchy, but it kind of hurt a little when touched and gave him a strange sensation he couldn’t describe. As I questioned him about every possible part of his body, he recalled that it hurt just a little to pee from the beginning of the illness. Over the previous 24 hours or so, it’s been hurting to pee more consistently, though he was sure there was nothing unusual leaking out of there. He’s vomited once or twice a day, but hasn’t had diarrhea. He wonders about a curious thing that he doesn’t think is related. He last weighed himself about a month ago; in my office that day, the scale showed that he had lost 10 pounds. I asked him about everything he did and everyone he was with. I asked him again about every part of him, from his scalp to his toes.

He looked thin and tired. It would be a thorough exam and I asked him to put on a gown but leave on his shorts. His eyes were a little pink and had a hint of crust on the eyelashes. He had denied any eye symptoms. His nose was stuffy and there were flakes of dried blood I could see when I looked in there. He hadn’t mentioned that, either. His abdominal exam was normal, but I noticed something that he said he hadn’t. On his undershorts, there was a little dark brown stain that looked like a dried drop of blood. His skin rash was very faint but I could just barely feel it. It covered his arms like a delicate pink lace all the way from shoulders to hands.

I sent him to the lab for blood and urine testing, and started him on antibiotics.

The next day I saw him again. He had called and said that over the last 24 hours he has gradually developed joint pain. By the way, he added, he cannot straighten his arms.

I ordered more tests, and x-rays of his elbows.

By January 4, both ankles were swollen. Not his feet, not his legs, just his ankles. Even short walks caused intense pain in both ankles. It didn’t hurt to pee any more. He had some diarrhea. The following day his right eye started to hurt whenever he looked upward. I pushed appointments aside and made phone calls. I was able to convince a busy local ophthalmologist to see him right away, and an orthopedic surgeon to examine his joints, and perhaps put a needle in one of them to draw off some fluid for analysis.

Every night, I was driving to the medical library at UCSF. ucsf library One by one, I eliminated the likely diagnoses and most of the unlikely ones. There was one that kept fitting in: Reiter’s Syndrome. One of the older reference books I found had this mnemonic: ‘The patient can’t see, can’t pee, can’t bend a knee.’ I learned that it is most common in young white men, like Martin.

I saw him again on January 8th. He said that sometimes his right eye ‘doesn’t work’ and the lid is mostly closed. He has to open the lid using his hand. His fever was gone now, and he felt generally better. His elbows were better and his ankles no longer swollen but still painful. He noticed some bumps today under the skin of both his hands. The bumps I felt were like firm BBs under the skin on the tendons of his hands and the back of the wrist.

As a pediatrician, I hadn’t seen or heard of Reiter’s Syndrome since medical school, and it had not naturally come to mind. But then, suddenly, the bumps under the skin. Martin mentioned a sore throat at the very beginning of the story. When I did my residency in Utah, I learned that though it’s quite rare in the United States, the Rocky Mountain states have the highest incidence of rheumatic fever in the country. No one understands why. This brought on a new surge of investigation and laboratory testing. Among others, I tested him for evidence of a past Strep infection. That was positive.

Rheumatic fever is a complication of Strep Throat infections. To diagnose it, the patient needs to have 2 of these 5 criteria:

  • arthritis of joints that seems to move from one joint to another
  • nodules under the skin
  • a long-lasting red rash that usually begins on the arms
  • uncontrollable muscle movements
  • inflammation of the heart

Martin had 3 of the 5, plus a couple of other things, considered minor criteria for this diagnosis:

  • fever
  • raised inflammation factors in his blood
  • raised infection-fighting cells in his blood
  • evidence of strep infection

He had started to get sick on a visit to the Rockies. Rheumatic fever can cause permanent damage to heart valves, so I arranged an urgent echocardiogram, which was normal.

So this is what he had, right? Well, that eye trouble didn’t fit in just right. And what about that pain when he peed? That’s not on the menu for rheumatic fever.

Reiter’s syndrome is diagnosed by meeting 3 criteria, called the ‘clinical triad’:

  1. arthritis of multiple joints
  2. urethritis
  3. conjunctivitis

Martin had all of those.

Rheumatic Fever can be serious when it causes permanent damage to the heart. Knowing Martin’s heart was OK reassures me that if that’s what he had, it shouldn’t bother him any more.

Reiter’s Syndrome is a little more mysterious, and there are reports that some people who have had this have recurring arthritis.

If I really get to make the diagnosis, maybe he had both at the same time. He seems to be better now.