Kawasaki - the disease, not the motorcycle

[This post was originally published about a year ago on my old blog. I have edited for name changes and time passage.]

When someone says the word "Kawasaki," I'll bet you think of the motorcycle. I did too, until May, 2001. This is a long story. I never wrote it all down before, though it is something I have wanted to do. Now that I have this platform with whatever audience stops by, I thought I'd go ahead with it. For those of you that stick it out through the entire story, thanks. For those of you that don't want to plow through the whole thing, there is a rare disease called Kawasaki Disease that mainly affects children between the ages of 1 and 5 and is the main cause of acquired heart disease in children in the US. It's a rare and devastating illness, the first symptoms of which are a high persistent fever, not unlike a typical virus. If you have children, you may want to familiarize yourself with this illness.

One morning my 4-month-old daughter, Jo, woke up with a high fever (104.5 F). It was the Wednesday before Mother's Day, May 9, 2001. Tylenol would lower the fever, but 4 hours later it would come raging back. I took her to the doctor. The doctor that I usually see didn't have an open appointment, so I saw the doctor at the practice that I didn't like - the one who had told me that my 2-month-old (the same child, who obviously had chicken pox) didn't have chicken pox because... get this reason from a medical professional... she was too young to get chicken pox. Anyway, he sent us home with the diagnosis of a virus that would probably last around 5 days, keep using Tylenol for fever, push fluids, blah, blah, blah. Two days later, she wasn't eating and there was a HUGE swollen lymph node on the right side of her neck. So back to the doctor, and this time I got the one I liked. By that afternoon we were admitted into the children's hospital. And the nightmare began, because no one knew what was wrong.

IV antibiotics were begun immediately, because at the outset, some sort of infection was the doctors' best guess. But the antibiotics had no effect whatsoever. Specialists of every kind came to see us and tests of all kinds were run. The main doctor in charge of our case kept asking me if I had noticed redness in the whites of her eyes, which confused the hell out of me. It was the midnight spinal tap that finally did me in. We walked down the hall to the room where they do it, and the nurse said to me, "You don't want to be in there while they do this," and the doctor said, "It's better if you wait outside. We'll bring her right back to you." So I started walking back down the hall to the room, barely holding myself together. A nurse came up to me and put her arm around me while I walked, which pushed me completely over the edge, and I sobbed my way back to the room. (People being nice to me in tough situations always makes me cry, why is that?) She sat with me on the side of the little makeshift bed that the chair turns into until they brought Jo back to me. I had my head in my hands the entire time, and to this day I have no idea which nurse it was or what she looked like or if I ever saw her again during our stay. The spinal fluid was clear. The good news - not meningitis.

If you have never been in a situation where you don't know what is wrong and the experts can't seem to tell you either, let me assure you, there is no hell like it, especially when the one affected is your child. And our experts were some of the best. We were lucky enough to live near Philadelphia, so we had CHOP docs. You can't get much better than that. Yet they seemed flummoxed. Jo continued to deteriorate daily, and we honestly thought she was going to die.

As an aside, while all of this was happening, people were calling constantly for updates. I had not left the hospital since Jo was admitted, and just had my husband bring me clothes and toiletries. He stayed home with our oldest child, who was in school, while also moving his business out of a building that was being renovated. Flowers were sent, cards were sent, etc., but by far the nicest, most thoughtful and useful gift I received was from one of my sister-in-laws. She gave me her calling card number and said to use it for all the outgoing calls I would be making from the hospital room. At the time, my husband and I didn't have a calling card, and I wasn't about to leave the hospital to get one, so this was wonderful. I mainly used it to call her. When there were no more visitors for the day, and the floor was quiet, and the only lights were from the nurses' station in the hallway and the television, that's when I'd start to feel like I was going to lose it. So every night around 11:00, I'd call her. She'd talk to me for hours, never making me feel like I was imposing on her time, even though she had four kids and was going to have to get them up in the morning and off to school. We were on the phone every night until 2:00 or 3:00 in the morning. She knew I needed the distraction, and she provided it. She told me stories of when she used to teach really disturbed high school aged kids, and some of those stories were truly horrifying. She made me laugh (not at the school stories, other things) despite what was happening. She was my lifeline, and I'll always be grateful for it.

Finally, on Monday morning, the doctor came in and said she thought they had a diagnosis. It might be Kawasaki Disease. It was such a relief having a name to put to it, even if it was just a possibility. And Jo had woken up with red eyes! I said to the doctor, "The whites of her eyes are red. Why did you keep asking me about that?" She said it was another symptom of the disease. The other doctors on the team (yes, an entire team of various specialists had been created to try to determine what was wrong with my child) wanted to wait another day before beginning treatment to be sure (this is a disease that can only be diagnosed by the appearance of a myriad of symptoms, all of which were not observable in Jo as yet), but she felt this was the correct diagnosis, in any case the treatment wouldn't hurt Jo, the treatment should be begun ASAP if the diagnosis is correct and the treatment takes 12 hours or so to prepare. It was up to my husband and me to make the call.

I called my husband at work and had him look up and print everything he could find about Kawasaki Disease. While I waited for the information, I called my mother, who had decided to come up from SC to help out with my oldest while I stayed in the hospital. At the time, she worked for a cardiologist, and when she told him she had to take some time off because her grandbaby might have Kawasaki Disease, his response was, "Oh, shit." Not the news you want to hear from someone in the know. Shortly thereafter, my husband called back with info about the disease. I found out that no cause was known. It may be genetic, environmental, viral or some combination. There was no definitive way to test for it, only the appearance of several symptoms could be used to diagnose. It was so rare that some pediatricians had never even heard of it. Kids would get it, recover, and then die from heart attack, because the disease itself isn't what kills, it's the aneurysms in the coronary arteries the disease leaves behind. It's most common in Asians, most common in boys and most common between the ages of 1 and 5. My little white baby girl was the rarest of the rare. But everything fit. I was convinced, my husband was convinced. I called the doctor in and told her we wanted to begin treatment immediately.

That night, the intravenous immunoglobulin (IVIG) was begun. High-dose aspirin therapy was also begun. By morning, Jo was improving, so the "wait and see" part began. My mother arrived. I was ensconced in hospital life. Time of day, weather, etc. was all irrelevant. I took showers in a room down the hall, while a nurse babysat. Yes, babysat. There was a new nurse on the floor and she would come stay in the room with Jo so I could get a shower. She was a really sweet girl, and somehow I found out that she loved YooHoo (the chocolate drink, for anyone who is wondering), which is all that I really remember about her now. Anyway, by Wednesday, Jo was still not completely better. The result of the treatment is usually immediate, and when it's not, the treatment sometimes has to be repeated. So since the first treatment had resulted in obvious improvement, it was decided that the diagnosis had been correct and the decision was made to repeat the treatment.

Once the second treatment was complete, Jo continued to improve. Since the outcome of this ordeal now looked positive, I took a picture (see left) for Jo to look at when she was older. At some point around the 7th or 8th day, I left my mother with Jo and actually left the hospital to try to find a gift for the staff, mainly the nurses. They had been wonderful, and I wanted them to know they were appreciated. I finally ended up at a fancy little Italian bakery, where I walked in and asked for a tray of cookies. The lady behind the counter said that trays had to be ordered in advance and she didn't have anything extra today. I said okay and was turning to leave, when she saw the hospital bracelet (which the hospital makes the parent wear as well, for security reasons) on my arm and asked me about it. I told her the story and she ripped the order slip off a big tray of cookies and sold it to me, saying she would redo the tray for the folks who ordered it, because they weren't expected in to pick it up for several hours. The nurses were thrilled with the cookies.

We were able to leave the hospital after a 10-day stay, once Jo's eating had returned to normal. I cleaned out the room that Jo and I had lived in for 10 days, wrote a note to the staff thanking them for all they had done and left it with a small picture of Jo, to whom the nurses had really taken a liking, along with a 6-pack of YooHoo for the nurse who babysat for me, and took my now healthy and alive child back home.

Jo had to be on high-dose aspirin therapy for a while and low-dose aspirin therapy for many months after, because the disease causes an extremely high blood platelet count, which can cause clots that can break free and result in death. The high platelet count is a lingering effect and can take quite a long time to return to normal. Now besides the worry over Reye Syndrome if a child gets the flu or something while on the aspirin therapy, I had the added problem of getting aspirin tablets into an infant. I searched for liquid children's aspirin to no avail, so I had to continue what had been done in the hospital (which I'm proud to say that I came up with, not that it took a rocket scientist, when a nurse came in with aspirin tablets and couldn't figure out how to get a 4-month-old to take them). So three times a day for many months, I crushed aspirin into a little bit of water, and used a syringe to give it to her.

Now seven years later, Jo is a happy and healthy little girl. When this first happened, we thought that Jo would have to be followed by a cardiologist for the rest of her life, but I'm happy to report that last year we had our last cardiologist appointment. The doctor told us that the new medical wisdom was that the risk of cardiac problems for children who had never shown cardiac involvement within a few years of the disease was no different than if they had never had the disease. I have to admit to a very difficult time believing this, and I have had moments of panic on one or two occasions when Jo said that her chest hurt (turned out to be from falling or bumping into something). The doctor did say to call if Jo ever complained of chest pain, which gives further credence to my reluctance to believe her heart is just as strong and healthy as those of my other kids. But we were and are lucky. Some parents lose their children because it is either not diagnosed correctly or not diagnosed in time. Some kids are left with aneurysms that dictate their lives - no sports, no excitement, no amusement park rides, etc. Jo plays soccer, is a red-belt in Tae Kwon Do and wants to run cross country when she's older. Conventional wisdom seems to be that the treatment needs to be begun within 5 days of the onset of the fever to avoid cardiac involvement. We got in just under the wire.


For more information, family stories and resources, please visit the Kawasaki Disease Foundation website.


Also, check out this website by a teen who had KD.

Photo credits: Got the motorcycle picture here. The calling card picture cam from the Sam's Club website.