I rang in the New Year at Seattle Children’s Hospital, squeezed into a slim hospital bed with my husband, our seven-year-old son sandwiched between us. We told stories and read Harry Potter and drank chocolate milk and thanked God he was getting better. Griffin fell ill a few days before Christmas and after a week of urgent care trips, questionable diagnoses, and no improvement, was diagnosed with Kawasaki Disease.
January 26th is Kawasaki Disease awareness day, and I want to join the effort to educate the public on this rare, mysterious, and often undiagnosed or misdiagnosed childhood disease. Below, you will find a brief overview of the disease itself followed by a more detailed account of our experience. The important takeaway here is that if your child is sick for several days with a high fever that won’t resolve, you should ask your pediatrician about Kawasaki. Don’t be fooled by the symptom list – Kawasaki can present atypically, and speedy diagnosis and treatment is crucial.
What is Kawasaki Disease? ( from the Kawasaki Disease Foundation website):
“Kawasaki disease (KD), also known as Kawasaki syndrome, is a serious illness characterized by inflammation of blood vessels throughout the body that primarily affects young children and infants. Kawasaki disease is the leading cause of acquired heart disease in children. Although about 80 percent of patients are under five years of age, older children and teenagers can also get KD, but this is uncommon. KD is more common in boys than girls, and the majority of cases are diagnosed in the winter and early spring. It is not contagious.
The disease is named after Tomisaku Kawasaki, a Japanese pediatrician who first described the illness in the medical literature in 1967. Although it is more prevalent among children of Asian and Pacific Island descent, KD affects people of all racial and ethnic groups. It is estimated that more than 4,200 children are diagnosed with Kawasaki Disease in the U.S. each year. The cause of KD is unknown, although an agent, like a virus, is suspected. There is no currently accepted scientific evidence that KD is caused by carpet cleaning or chemical exposure.
Kawasaki Disease is characterized by an inflammation of the blood vessels throughout the body. There is no specific test for KD; doctors make a clinical diagnosis based on a collection of symptoms and physical findings. Early symptoms of KD include:
- Fever that lasts for five or more days
- Rash, often worse in the groin area
- Red bloodshot eyes, without drainage or crusting
- Bright red, swollen, cracked lips, “strawberry” tongue, which appears with shiny bright red spots after the top coating sloughs off
- Swollen hands and feet and redness of the palms and soles of the feet
- Swollen lymph nodes in the neck
Understandably, children with these symptoms are extremely uncomfortable and irritable. Any parent whose child has persistent fever and any of these symptoms should take him or her to the doctor immediately.
Without treatment, about 25% of children develop heart disease involving the coronary arteries. Timely diagnosis and treatment (which usually includes intravenous gamma globulin) is highly effective in preventing coronary complications. Doctors continue to study the long-term outcome of children who do not appear to have coronary involvement. Other kinds of longer-term consequences (e.g., non-coronary) are extremely rare. There is no evidence that links KD with autism or a seizure disorder. A very small number of KD children might have a seizure in the early acute stage of KD when there are very high fevers, but there is no on-going or long term seizure prone condition.”
Our Story –
I usually avoid posting a lot of personal details online, but today is an exception. I remember well those first nights around Griffin’s diagnosis, when I poured over posts just like this, looking for any information that might bring clarity. I’m grateful for the other families who have posted their stories and hope that together we can increase awareness and possibly find some common denominators in our experiences.
The weekend before Christmas, Griffin woke with that glassy-eyed look that always comes with a cold. He’s pretty stoic about being sick, he has a high physical pain tolerance, and illness rarely slows him down, so his lethargy was unusual. The next day he ran a temperature and was complaining of neck pain, and we took him to the pediatrician, mainly to avoid an urgent care trip on Christmas Eve. Griffin had a 101 degree fever, and the doctor told us he had a flu-like illness, that we should give him lots of rest and keep him well hydrated. So off we went to the store to stock up on popsicles and Gatorade.
He seemed pretty mellow the next day, which was Christmas Eve, but did eat some popsicles , despite complaining that he felt like throwing up. He spent most of the afternoon in bed, declining to even watch movies, which, as any mother of a seven-year-old knows is unusual even for a sick kid.
On Christmas morning, we finally had to wake him at 9AM, because his younger brothers were going nuts with the waiting. He was warm to the touch, and we noticed that both his eyes were very red. Griffin moved out to the couch, where he lay, lethargic and unmoved by the sight of Christmas loot, refusing all liquids. We gave him his long-coveted lego set; he gave us a weak smile, but set it aside. And when he barely tore the wrapping of the next gift before asking if he could go back to bed, we knew something was seriously wrong. I took his temperature, and despite the children’s acetaminophen, his fever was back at 101, so off to urgent care we went.
At this point, I was concerned about appendicitis, as I had heard it can present in children as overall stomach achiness. Griffin still was complaining of neck and stomach pain, and now he began vomiting. The urgent care doctors checked him out, diagnosed him with gastroenteritis, and gave him IV fluids for dehydration. We read Neil Gaiman’s FORTUNATELY, THE MILK while waiting for the fluids – which was a bright spot of humor in an otherwise worrisome experience – and the doctors gave him some anti-nausea medication that seemed to help.
The next morning, we were relieved to see Griffin responding with increased energy and enthusiasm. He ate a few crackers, was back to downing popsicles and opened the rest of his Christmas gifts. But by afternoon he was declining again and stayed in bed for the rest of the day, getting up only to shift from bed to couch and back to bed. This seemed to be a recurring pattern that lasted the next few days. He would be a bit brighter in the morning, but by the afternoon and evening, his fever would be higher, the lethargy returned, and all he wanted to do was sleep.
The following day was even worse. He barely left his bedroom, slept most of the morning, and woke around noon with a 105 degree fever. My husband took him to the ER, where the doctors ran a bunch of tests and landed on a raging UTI. They gave him IV fluids and antibiotics, but it still didn’t sit well with them, so they recommended we transfer to Seattle Children’s Hospital.
Griffin and I took an ambulance ride across the water and soon found ourselves in Seattle Children’s ER where they ran more tests, checking for flu, viral illnesses, UTI, and many other things. They finally decided to admit us. The doctor told us that UTIs in boys who have never had them are rare, especially when he had no other UTI symptoms. Even if it was one, they wanted to make sure he got the antibiotics via IV, since he was having trouble keeping things down.
The next two days in the hospital were spent doing various tests, retelling Griffin’s story to many doctors and residents, and lots of waiting. On our first morning in the hospital, one of the doctors mentioned Kawasaki disease, but said it was on their long list, mainly because Griffin was atypical: he was outside the usual age range, and he only had two symptoms: prolonged fever and red eyes without discharge. Despite broad spectrum IV antibiotics, he continued to spike fevers most days and had no energy.
They did xrays to look for pneumonia and ultrasounds to double-check his appendix wasn’t inflamed and to look at his kidneys and bladder to rule out kidney infection. They took blood and ran IV fluids, and I was amazed at how well Griffin did with all the tests. He hadn’t been in the hospital before, except for a minor surgery he had at two-weeks old, so the whole experience was completely new for him – for all of us really. The IV insertion and blood draws seemed to be the main areas of concern for him. Other than that, he was enjoying ice-cream in the middle of the night and cartoons all day.
I had read online that Kawasaki Disease can sometimes cause cardiac damage and how an ultrasound of the heart is a non-invasive way to provide additional diagnostic information. When the resident came by on her midnight rounds, I asked her about this, and she said they were already considering an echocardiogram for the morning. They wanted to use it to rule out endocarditis, but they would also look for symptoms of Kawasaki.
An hour after the ultrasound, our room was filled with doctors. The cardiology team had looked at the results and seen significant dilation of two of Griffin’s cardiac arteries. Despite his lack of other symptoms, this is a conclusive diagnosis for Kawasaki. In fact, one of the cardiologists said that if he were to come in and be checked for something else, and they had seen his arteries, they would have thought: he has had Kawasaki in the past. It is unusual to see cardiac involvement so soon. At this point, it was only seven days after fever onset, five days after red eyes.
Even so, they immediately prepared him for the standard treatment for Kawasaki: IV immunoglobulin and high-dose aspirin. The IVIG therapy is pretty cool – someday he may even feel superheroish about it, since it basically gave Griffin’s body antibodies from donors’ blood. (THANK YOU PEOPLE WHO DONATE BLOOD!) I have OCD ways of thinking about medications, so I was very worried about a negative reaction, but he tolerated the medicine very well, and his acute symptoms cleared up almost immediately. Within an hour, this fever that had lingered for a week was gone, never to return. The next day, he was eating and drinking again, and, while he still wanted to stay in bed, I think that had more to do with the Spongebob Marathon on TV.
There were funny things as well. The way you never have enough pairs of socks or how glad I was for inane cartoons that made Griffin laugh again. We were fortunate that a good portion of our hospital stay we were roommate-free and had space to ourselves. The night of the IVIG treatment, however, we had our first roommate: a teenage boy, who, impossibly enough, sounded like he was in labor. I am not kidding. If you haven’t heard the sound of a man weeping and moaning for his mom, you really don’t want to. I have no idea what his illness was, but he sure needed something stronger than the Tylenol his nurse kept offering him. Sad-Moaning-Roommate-Man: I hope you are well now. Fortunately, Griffin slept through it all and we were able to transfer rooms in the morning. The rest of the time we had toddler or infant roommates, which was a much better fit for us.
After the IVIG treatment, we were so thankful that the acute phase of the illness was over. Seattle Children’s Hospital is one of the centers for Kawasaki research, and we couldn’t be at a better place for diagnosis and treatment of this disease. They spotted it our first day in the hospital and confirmed diagnosis in a day and a half. When I read stories of children who went undiagnosed for weeks, I am profoundly grateful for their experience and response. We stayed in the hospital for several days to make sure the fever didn’t return. It didn’t, and, after six days in the hospital, we were all very ready to be home.
Once the acute phase of Kawasaki is treated, there is a waiting period to see if there has been cardiac involvement. We already knew Griffin had some – his arteries were significantly dilated on the first echocardiogram – but we had received treatment within the optimal window (7-10 days post-fever onset), so we were hopeful that this would resolve on its own. The plan was to discharge us and have us return in a few weeks for a follow-up echocardiogram. Post treatment monitoring is super-important, because the scariest part of Kawasaki is what can happen to the heart.
When the inflammation from Kawasaki goes down, cardiac aneurysms can form, increasing the risk for clotting, i.e. heart attack. The doctor described it to us like this: the cardiac arteries are straight tubes. With the inflammation from Kawasaki, the arteries dilate, or grow. When the inflammation goes down, ideally, they would return to their pre-disease size. Sometimes, however, certain spots in the arteries stay inflamed, causing a balloon, or aneurysm, to form. When this happens, the blood doesn’t flow in a linear fashion. It will pool and eddy in the balloon area, increasing the likelihood for clots.
I have Factor V Leiden and compound heterozygous MTHFR mutation, genetic blood-clotting disorders, and I was concerned Griffin might have inherited these. If so, he would have an even greater tendency to clot. Unfortunately, the blood tests for these disorders takes almost a week, and upon discharge we had not received any results. So I asked for increased monitoring. The cardiologist agreed, and we set up our first follow-up echocardiogram for Monday, two days after hospital discharge.
The weekend at home was restful. Griffin was thrilled to see his brothers and vice versa. They had come to visit in the hospital and were enamored by the children’s play area, the cafeteria, and the unlimited TV access. All I have to say in sappy mom fashion is I am also so grateful to Nickelodeon. Nick, I might have mocked you before, but you got my son giggling after a week of glazed stares and made the hospital a positive experience for him, so I’ll forgive you for the atrocious fart-baby episode.
We arrived for our follow-up echocardiogram expecting little news, except the tiny hope that everything might be already resolving. Best-case scenario: the dilation would be gone. Worst-case scenario: the dilation would be unchanged and we would come back in a few weeks. Instead, the doctor informed us that the artery dilation had increased, that he saw evidence of aneurysm formation, and that Griffin would need to immediately go on blood thinners: warfarin and aspirin long-term and heparin for a few days until the warfarin could take effect.
I am familiar with these medications. I took a low-molecular-weight heparin during pregnancies, and a family member is on long-term warfarin. Even so, the thought of a seven-year-old on these medications, possibly forever, was overwhelming. Griffin apparently won a crappy lottery. 25% of untreated children with Kawasaki develop cardiac involvement. Most children who are treated within the window go on to recover completely. Less than 1% of treated children develop cardiac problems. And Griffin is one of those. The cardiologist told us he couldn’t offer us many statistics to help us know what to expect. 50-100 children diagnosed each year fall into this category, so they don’t really have a pool big enough to predict what might happen.
The aneurysms could resolve on their own in a year or two. They could stick around until his teens and resolve then. They could be a life-long condition. They could cause scarring that would keep him at a heightened risk of clotting. He could need an angiogram or stents or cardiac catheterization or some other invasive procedure. He could need nothing. But even so, blood thinners are the way to go, because they ensure that his risk of developing a clot is very low.
So we were readmitted to the hospital that afternoon for another six-day hospital stay. It was fairly uneventful compared to the first trip, mainly because he was only admitted for monitoring. First, to ensure the heparin was in the right range, and secondly, to monitor that the warfarin had thinned his blood into desirable levels. The most remarkable time of the entire stay was our final night, when we had an infant roommate. The baby wasn’t a problem – small cries, easily comforted. But her Favorite Swing had a creak in it. Back and forth. Creak. Squeak. Punctuated by regular IV beeping – something that hadn’t happened before. It was like an orchestra in there: IV pole beep. Creak. Squeak. I stumble up and buzz the nurse. Creak. Squeak. She comes in and turns off beeping. Creak. Squeak. IV pole beep. And repeat. Over and over again, broken only by blood draws and IV checks and no sleep at all. It’s a toss-up whether Sad Moaning Roommate might have been more welcome.
Anyway, here we are now, home for a week, adjusted to the rhythm of daily medication (spoonfuls of ice cream help the medicine go down in our house), weekly blood draws, and future echocardiograms looming overhead. I’m not one to post a lot of personal details online, but I remember too well those late nights in the hospital post-diagnosis, pouring over websites and blogs and forums and anywhere that could tell me a little more about Kawasaki, and so I’ve posted this here with the hope that it might help someone else.
If you are one of those desperate parents reading this in the hospital: I’m so sorry. I’m so sorry for the stress and strain of your last few weeks. For the fear and worry and breath-stealing moments in the middle of the night. For the difficulty of seeing your child in pain and knowing he or she is sick without knowing why. For the detachment and the way you have to push past emotions to do the next thing. For the hallowed halls of a children’s hospital, where the reality of frailty and sickness looms. For the way in which IVs and blood draws and blood pressure cuffs have become normal for your young child. For the way Kawasaki Disease has now become a part of your story.
And for you hospital staff: Thank you. You nurses and your unfailing positivity and patience with frightened children and their parents. You volunteers who stock the children’s room and come by dressed as clowns and with dogs and with gifts for sick children. You lab techs with your tricks to talk anxious children down and you IV experts with the worst job ever who still manage to do what needs done despite children’s cries. You residents and doctors, with your crazy hours and sleepless nights and the concern for your patients painted all over your faces. You who sweep the room floors clean and offer cheerful conversation that isn’t laden with medical terms. You ultrasound techs with your ability to find just the right movie. All of you who live and breathe the surreal rhythm of a children’s hospital: thank you.
And for you family and friends: I can’t even read your e-mails and notes and cards and gifts without tearing up. For you who brought food – unsolicited feasts that showed thoughtfulness and care. For you who sent gifts and notes to Griffin in the hospital and once we got home. For you who sent me gifts. For you who offered to care for our other boys. For you who took care of other responsibilities for us. For you who called and prayed and e-mailed and cried and worried with us. For all of this and more: I am so deeply grateful and awed by your show of grace and love. Thank you.
For more information on Kawasaki Disease: