The Kawasaki Disease Forum

Our 8 year old daughter was diagnosed with atypical, or incomplete, KD and was treated with IVIG and high doses of aspirin, followed by low-dose aspirin for ~8 weeks. She was subsequently diagnosed with eosinophilic esophagitis (EE). Below are some of the details:

Day 1, woke up not feeling well with a fever. By mid-afternoon was vomiting and vomited through the night. Also, started developing a red rash. The rash was extremely itchy and started with raised bumps that got red over time and her skin felt relatively rough. The rash is pretty much from neck to ankles.
Day 2, fever continued, rash was worse, given two previous episodes of strep this past spring that were accompanied by vomiting, we brought her to the ENT to do a rapid strep test (because the ENT was available quickly), which came back negative. ENT sent a sample out for a culture to verify. Our daughter was put on an antibiotic as a precaution given that the ENT thought the rash may be scarlet fever. Made an appt with pediatrician for the next day.
Day 3, pediatrician said that it appears to be textbook scarlet fever, said to keep her on the antibiotic and gave a very low dose of prednisone for itching. We only took 2 doses of that as it didn't appear to provide any relief.
Days 4 and 5 (weekend), fever continues and rash is extremely itchy. The fact that the fever is present has us concerned it was not scarlet fever.
Day 6, speak to pediatrician who finds out strep culture was negative and sends her for bloodwork. That evening, after a tub, the backs of her knees took on a purplish hue. Called pediatrician who said bring her to the ER as his bloodwork will not be back until the next day and he wants her platelets checked. Her eyes begin getting red. Took her to ER (affiliated with a medical school). A bunch of tests in ER (including chest x-ray, that indicates some minor pulmonary infiltrates). Elevated CRP. White blood cells in urine (but the culture that comes back later indicates no infection). ER doc admits her for observation and says "the attending may think i'm crazy, but this may be kawasaki and other symptoms may show up." Throughout that evening in the ER, her eyes get more and more red.
Day 7, more tests to rule other issues out. Keep her on antibiotics (higher doses, broad spectrum) as they want to make sure it is not bacterial. Sed rate start elevating. Was seen by infectious disease doc and cardiologist.
Day 8, we meet with the doctors and residents and all feel comfortable that the risks of overtreating (when not KD) is less than the risk of missing KD. Start IVIG and high doses of aspirin. She tolerated the IVIG well during infusion, but about 6 hours after it is complete, has severe head pain (aseptic meningitis) and nausea. Her hip starts really hurting. She previously had toxic hip syndrome. It is assumed it is related to KD or the virus that caused the KD and is characterized as reactive arthritis. Has an echocariogram and everything looks good.

- About 24 hours after finishing IVIG, her fever is below 100F. Her eyes were still red. Her fingers begin peeling. The rash is still there, but less pronounced.

- The next two weeks had ups and downs. Here hands peeled and her toes peeled. After being discharged from the hospital (at about day 10), she was exremely lethargic, her eyes remain red (worse later in the day). She consistently has a "temperature" but not a "fever" (stays below 100.4F). Her hip is so bad that she can't always make it to the bathroom in the nights. She sees a rheumatologist starts ibuprofen for the pain but then ends up on naprosyn. Has nausea relatively routinely that the doctors attribute to the NSAIDs or aspirin. They prescribe zofran. The eyes still being red has us very concerned -- the feeling was if the eyes remain red, she is not yet better and the doctors don't necessarily disagree (they expected her eyes to improve relatively quickly). Her markers of inflammation (CRP and ESR) start lowering, which is a good sign. We see a pediatric ophthalmologist to confirm no uveitis (everything looks ok). We and the doctors keep a close eye on her to figure out whether she needs more IVIG.

About 2 weeks from discharge she had another echocardiogram, which came back fine.

About 3 weeks from being discharged, she is nauseous and vomits for 24+ hours. We don't know if it is related to the KD, the treatment for the KD (e.g., naprosyn) or something altogether different. She is dehydrated. We bring her to the same ER. The continued red eyes still have the doctors puzzled. After another 5 days in the hospital, during which her hip issues worsened, she is diagnosed with EE. It is unclear whether the EE is independent from the KD or was exacerbated by the KD (or the KD treatment).

Here we are today (about two months out of the first hospitalization). Her energy level is vastly improved. The hip is mostly better. Her eyes are markedly improved, but we still see some pinkness from time to time. She has another echo schedule for tomorrow. She is tolerating her new EE diet (schedule for another upper GI scope in another 2 months or so).

Best regards,
CPB