FEVER, FEVER, BURNING BRIGHT
It was a dark and stormy night…
Sherlock Holmes reclined in his chair, eyes glazed from days of inactivity . Our apartment door at 221B Baker Street burst open. A young and strikingly beautiful woman glanced about our sitting room. “Mr. Sherlock Holmes?” She looked at me. I pointed her to the lounging , lanky figure. “Help me, please Mr. Holmes,” she cried out, “my child has been burning up with for one week and three physician visits have yielded nothing. My daughter is sinking, I feel it. Please help us, if you are able.”
“If?” Holmes frowned and motioned the distraught woman to a chair and offered her a cup of tea. “Refresh yourself with a spot of Earl Grey, then let us proceed with the facts and without the histrionics, if you please.”
She took a sip of tea, a deep breath, and launched into her daughter’s story
“My 17 month old daughter was in the peak of health until one week ago today when she seemed less active then usual and had an axillary temperature of 101.5 degrees F. She seemed to rally the next morning, but by the evening, her fever returned at 103.3 degrees. She refused to eat dinner, but welcomed liquids. I gave her Tylenol with slight improvement in the fever. I took her to the clinic the next day and was told, after a thorough exam, that she had a virus and to keep using Tylenol or Motrin as needed. My baby had no nausea, no vomiting, no diarrhea, no rash, no exposure to animals, and no sick contacts. She stays with me throughout the day and night. I returned to the clinic on Monday, following the weekend, because she continued to have fevers on a daily basis. Once again, no source was found for the fever and we were assured that this virus would soon work its way out of her system.
48 hours later, today, on the 8th day of fever, I have come to you, Mr. Holmes, for I am truly at my wit’s end.
“Watson,” Holmes looked at me, “please spin your clinical magic and complete a physical examination on our tiny client.”
Temperature: 102.3 F
RR: 26 Pulse Ox 98% RA
General: active, alert, sociable, hydrated
Oral: mild redness oral pharynx
Neck: Supple, no LAD
Heart: no murmur
Skin: no rash except for 3 pinpoint red papules on back
Extremities: FROM at all joints, no redness, no edema or peeling
“I asked her about the red marks and the mother stated that perhaps they were flea bites from a neighbors cat that occasionally wondered into their yard. The patient is up-to-date on all immunizations and quite up to par on her growth charts and developmental milestones.”
“Admit this tiny person to the hospital immediately,” Holmes declared, “she has incomplete Kawasaki Disease.”
The mother fainted.
“Perhaps, Holmes,” I suggested, “you might want to use a bit more tact when talking to parents of small children.”
“What in the world for?” he seemed incredulous, “the world is a difficult and deadly place, Watson. The sooner a parent and child realize that this is a fact, the better.” Despite his harsh words, he ministered to the distressed mother with a gentleness that surprised me.
“My dear lady,” he spoke with more kindness, “allow me to share my logic with you and my hope for your daughter’s complete recovery. First, Kawasaki Disease is a challenge for the finest of medical detectives. We don’t understand what causes it and your daughter has a remarkable lack of clinical symptoms that clue us to the diagnosis. She has no conjunctivitis, no mucosal membrane involvement, no enlarged lymph nodes, no rash, and no hand and feet changes. She does however, have a compelling number of suggestive laboratory findings: the WBC count above 15,000, the low albumin, the sky-high sed rate, and a mild anemia. We must begin the high-dose aspirin now and the IV immunoglobulin to prevent devastating coronary aneurysms. We are beginning treatment on day 8 of her fever, which means we have every expectation of success in her case.
We started the IVIG 2mg/kg over 12 hours right away on our 17 month old female, along with aspirin 80mg/kg qid. She was afebrile within 8 hours, active, hydrated, and happy. We seemed to be on track. Then, at midnight on Day #9 of illness, she spiked a fever to 102 degrees F.
“Holmes,” I warned him, “I have bad news on our little client. She has spiked fevers, despite our diagnosis and treatment. Have we gone wrong in our reasoning? What if we missed meningitis? What if we missed mononucleosis or Typhus?
“Calm yourself, Watson, let us look at the facts and only the facts, rather than working ourselves into a lather over if, if, ifs. Let us consider meningitis, for it is true that we could be criticized for not doing a lumbar puncture on the day of admission. However, the cold fact is that a child with a week of fever due to bacterial meningitis would hardly be bouncing about the room like a proverbial Tigger, as our patient has done throughout this illness. And why would the WBC count be going down if we were missing a serious bacterial infection? And why in the world would her platelet count be climbing rather than falling due to marrow suppression in the face of overwhelming sepsis.
What about mononucleosis? Her age definitely portends against this diagnosis, but so does her lymphopenia. By the way, I had a monospot run just for you, and it was negative as was the smear review for atypical lymphocytes.
Typhus? A good thought, given the mother’s worry about flea bites from a vagabond kitten. However, the lack of rash, relative neutropenia, hyponatremia, thrombocytopenia, and hepatitis makes Typhus less likely.
But, a good clinician looks not just to eliminate the other possibilities, but to confirm what he believes to be the most likely cause of the illness. A little digging revealed that Kawasaki patients often have a lymphopenia, a platelet count that climbs, beginning on day 8 of illness, and a fever within 36 hours of the administration of IVIG. Our little client fit these criteria like a glove. Watson, we are still on the right track, have no fear.”
Our little client had no further fever spikes after that 36 hour mark. Her echocardiogram revealed clean coronaries, no ectasia, and no aneurysms. The high dose aspirin was stopped when she had been afebrile for 48 hours and low dose aspirin at 5 mg/kg was started. No repeat IVIG was needed, nor indicated.
She left the hospital a whole and happy little girl, with a very pleased mother and father.
We will continue the low dose aspirin until her sed rate and her platelet count drop into the normal range. We expect that should take 4-6 weeks, given her remarkable recovery both in fever and in her laboratory testing to date.
“Holmes,” I asked, “what about the UTI?”
“Elementary Watson,” he smiled, “true, true and unrelated. Imagine if she had an obviously abnormal urine on day #8 of the illness. We might have dismissed the issue as a mere UTI. Antibiotics would not have protected her heart and we would have missed our window to administer the IVIG and prevent lifelong debility or worse. No Watson, I believe that the Living God presents us with puzzle pieces that complete a perfect picture and protects us from distractions, if we but stay alert and awake. And now,” he took up his violin, “I will attempt to keep you awake you with a little atypical violin composition of my own.”