July 19, 2009

Fever -- 8 hot days

In my last post, Fever, I urged parents not to ask a doctor 'What could it be?' The list of possibilities is invariably a long one, and includes some extremely scary diseases that your doctor—like most doctors—has never seen in person. Good physicians always have in mind that if it walks like a duck, quacks like a duck...it's probably a duck. Any sound of hoofbeats is much more likely to be from horses than from a herd of zebras.

So when Matthew's mother called me to say that her 21-month-old has had a fever for about 4 days, I wasn't particularly worried, and told her that. I cared much more about how the child was doing—how sick was he?

Not very, she said. He had a fever pretty much continuously, up to the mid 104's (40°C). He felt lousy at those times, but his mother said that he perked up right away when the Tylenol kicked in. He was eating, keeping up with his fluids, acting like his usual self when the fever was kept down. I didn't need to see him—what was I going to do for him? If he was OK, then I was OK.

On day 5 of the fever, I called the family to check on him. Nothing much was new. Still had high fevers, but nothing in particular was hurting. I told the parents that as a rule-of-thumb, I usually order some basic tests if a child has about 5 days of fever without giving us any clue of what its from.

Most of the time, we don't find anything when we do these tests. And very often, the tests come back normal just as the rash is starting. One of the most interesting and sometimes frustrating common diseases of toddlerhood is Exanthema Subitum, also known as Sixth Disease, also known as Roseola Infantum. It's probably a deep truth about medicine that if you or somebody you know has a disease which has 3 different medical names, it's a good bet that medicine doesn't know much about it. In the 19th century and earlier, the cause of most diseases was completely unknown. So the classification system was based not on the cause of an illness, but on how the physician would witness it progressing. These days, we think of illness as either from a cause of some sort. Back then, there were 6 numbered rashes of childhood. First Disease, for example, is measles. Sixth Disease was a longstanding mystery until 1988 when the viral cause of it was discovered. Luckily, it seems to be the least dangerous of the numbered diseases. What makes it frustrating, and often results in lab tests that turn out to be unnecessary, is the high fever for day after day. Just like Matthew, the kids aren't very sick when we control their fever, which can last for days—usually 3 to 5 days, but I've seen it go a week. Then, mysteriously, the fever goes away and the child gets an awful-looking red rash over their whole body in a matter of hours. The rash doesn't hurt, doesn't itch, and goes away in 1-2 days without a trace and without treatment.

So I knew it was just a matter of patience. The parents, both scientists, were fine with that when I explained the plan. We'd take it day by day, and wait for the rash to appear.

On the morning of day 6, I called for a report. Nothing much was new with Matthew. But in the back of my mind was the fact that this was a boy, with a father is of Asian descent. The longer he went without getting the rash, the more I needed to question if I was right. While I was on the phone, I asked his parents to look at him and tell me if he had any rash of any kind, or if his tongue looked odd or if his eyes were red. I made them describe to me the skin on his hands and fingertips.

About 6pm on day 6, I called and was told that his eyes were a little pink. I drove to their house to look at him. Matthew was watching TV with his older brother, eating a banana. The boys didn't look up when I came in. His eyes were, in fact, a little pink. He wasn't rubbing them, and there wasn't any goopy stuff in the eyes. Everything else looked just as usual. I told the parents that I could wait no longer, and insisted that they take him for a blood test first thing in the morning.

On the evening of day 7, his fever still going, I got the lab results. There wasn't any particular evidence of infection in his blood, but his platelets were about 550, about twice the normal level. That was enough for me. I told them to take him right then, as soon as we got off the phone, to the emergency room at Children's Hospital Oakland. I called the ER and spoke to the doctor in charge so they'd be prepared for him. I told them he had Kawasaki Disease.

I came to the hospital later that night. The treatment was just getting started. We give an intravenous product of filtered, donated human blood called IVIG [Intravenous Immunoglobulin]. It's a liquid packed with all the antibodies and immune cells and chemicals from lots of people, with all the other blood components removed. It's like an immune-system sponge, which vacuums the system of illness-causing agents. We also give them aspirin, the ancient concoction originally from willow bark. It keeps platelets from sticking together (which they are supposed to do in order to help stop bleeding and form blood clots). By day 8, the next day, his platelet count was 750. If it kept going higher, he could be in danger from so many platelets clumping together, making the blood sludge in narrow vessels and potentially causing damage to major organs or a stroke. Matthew was about 26 pounds or so, and we were giving him 4 adult aspirin a day.

Kawasaki disease has yet another mysterious feature. It can cause coronary aneurysms in children. Usually only seen in older adults, these can be serious. They are weaknesses in the walls of the blood vessels that provide the heart muscle with blood. They form bulges in the vessel wall. If the weakened vessel breaks, part of the muscle will not continue working.

This disease is baffling, and reminds me that in past generations there were always those who wondered why they didn't know the cause of some illness even with their incredibly sophisticated 'modern' technology. The disease was only really identified (by Dr. Kawasaki in Japan) in the 1960s. With every technique of current molecular biology, no one has any idea of what causes this.

Because it has occasionally appeared to occur in clusters, it might be something infectious. But if it is, it's very hard to get. It's a rare disease. Maybe some people have a genetic predisposition to be susceptibility to it—it's more common in boys than girls and about 10 times more common in children of Japanese ancestry than in average American children. It's more common in children of other Asian descent than Caucasian children, but less common than in Japanese. Some people think that it's a lot more common than we know. Maybe those old people with aneurysms had unexplained fevers when they were kids, and nobody made anything of it at the time. But it weakened their vessel walls and 60 or 70 years later, they come to the attention of a cardiologist. Maybe adults are getting this disease too, but their symptoms are different. Maybe in adults they're just tired, feel run down, and keep forgetting where they put their keys....

Matthew never got all the symptoms of the disease. Since we don't know what causes it, there's no test for it. By the time I saw him on the evening of day 8, his eyes were very red. Within 12 hours of starting treatment, his fever was gone and he felt fine. Fortunately, the examination of his coronary arteries showed no aneurysms. Who knows how his disease might have progressed if treatment had been delayed by a few days? It's remotely possible he could develop heart-related problems later on, and he will be getting follow-up from the cardiologists. He seems to be fine for now, and he takes a chewable aspirin every day.

Don't ask your kid's doctor what it could be. Just try to find a doctor who knows that there are zebras out there, and who might recognize one at a distance.


  1. My son was diagnosed with Kawasaki's in 2006 at 2 years old. We were at the beach on vacation and he started with the fever and I took him to a walk in dr.'s office. He said he had hand foot and mouth. The next day he had a rash all over his abdomen and was vomiting. I took him back and the dr. looked perplexed and ordered an X-ray of his lungs! I called my husband who was packing up the car and we went home and saw his regular pediatrician. She said to finish the antibiotics and keep her updated on the fever. He was irritable and listless and you could not comfort him. It was very scary because he had had infections and fevers before but did not act like he was now. He seemed as though he could hardly walk. I called the doctor each day and she would ask about other symptoms, i.e. pink eyes, peeling skin on hands and feet. On the 5th day of high fevers she told me to bring him in and she said he had the strawberry tongue, pink eyes and he needed bloodwork. He could have Kawasaki's Disease and would need to see a cardiologist! I was trying to understand what she was saying and why would he need a heart doctor if he had a virus! We wound up that evening at Children's Hospital in DC where she sent us and told us they would be expecting our arrival and she had notified infectious diseases. She said do not leave until you get the treatment and I thought that was an odd thing to say. Of course we wouldn't leave without the treatment, that is why we are here. The ER dr. agreed with the diagnosis and ordered the IVIG. We went to a room and waited.....and waited.....and waited. Finally, I told my husband to see where the IVIG was. The shifts had changed and the new ER doctor was questioning the diagnosis!!!!!

  2. They said he didn't have all the symptoms. We were told they would have to confer with the cardiologist, pharmacist, and infectious diseases before they could give him the treatment. The reason-a nationwide shortage of the immunoglobulin (IVIG). The good news-they had it at the hospital. The bad news-they had to agree that he needed the liquid gold (as we later referred to it). I told the ER doctor that he had had all of the symptoms except the peeling feet and hands over the last five days and couldn't they be waning on their own? Isn't that what the brochure you gave us said would happen. But if not treated with IVIG he could have irreparable heart damage. I was scared they would deny the treatment and later discover they were wrong when it was too late and the damage was done. I was desperate. I called our pediatrician to ask her to plead our case and explain the symptoms she saw. Several doctors came and looked at him and finally a whole crew of people in white coats came in from infectious diseases. The head doctor said "You know we do not know a lot about Kawasaki's Disease. But what we do know if it is left untreated it could cause heart damage, and I don't think that is a risk we should take. I think we should give him the treatment." I could have hugged and kissed that man! We agreed he needed the treatment and thanked him profusely. They had to defrost(?) and prepare it. It was hooked up in about an hour and when done he was like a new child. He had his appetite back and was sitting up and alert! It was a miracle. I am forever thankful to our wonderful pediatrician Dr. Ogunrinde of Waldorf, MD and her knowledge to be able to correctly diagnose this disease. I am sure it goes undiagnosed a lot. I later understood her statement of do not leave without treatment! She told me that my son was the second time she had seen Kawasaki's in her career. The first was a little girl that she sent to the hospital and they did not treat her and sent home. The girl became sicker and she told the mother to take her back to the hospital and do not leave until she is treated! My son is caucasian (blonde hair blue eyes) with no Asian background. I know that it is more common in Asian boys.
    My son had to follow up with a few ultrasounds of his heart and thankfully shows no signs of any damage. And lastly, the day we took him home he developed peeling skin on his hands, the last and only symptom he had not had before. We called the hospital with that info.

  3. My almost 1 year old had a fever and was not himself. He wasn't taking his bottles and had no interest in solids. After 2 doctor visits the pedi decided we should go to the ER. The ER took chest x-rays and said it was pneumonia and they would admit him for overnight observation. Because of insurance we had to be transferred to our hospital which is a children's hospital. Six days later and lots of tests the infectious disease docs were out of theories and decided it could be Kawasaki. Since this was day 8 of the fever they wanted to treat him asap. He was back to his normal self within 6 hours of the start of treatment. They kept him in for 24 hours after then end of treatment for observation. So after 8 days in the hospital (and spending his 1st bday there) it was determined to be Kawasaki. What made it more difficult was because he didn't show all the symptoms. They stated that in a child so young they typically don't show all the symptoms.


Please let me know what you think. Do you know a child or situation like this?