Childhood threat -- Kawasaki disease strikes mostly those under 5; cause is unknown
By: CAMERON LEIGH JAMES - For the North County Times | ∞
Beth Avant and her son Lucas, 3, are seen in the backyard of their Encinitas home recently. Lucas has Kawasaki disease but luckily was diagnosed early. The symptoms mimic those of a viral infection and can lead to delays in treatment.
ROBERT BENSON For the North County Times
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In October 1998, three days after Tony and Mijian Oleas and their 1-year-old son, Michael, returned home from a Hawaiian vacation, Michael developed a persistent fever.
On the second day, when a rash developed, the couple's pediatrician said not to worry. By the fourth day, the whites of Michael's eyes were bloodshot, his lips were cherry red, his tongue looked like a strawberry, and his fever was hovering between 102 and 104. A second trip to the pediatrician yielded no diagnosis.
"My wife called me at work and told me she thought our son was dying," said Oleas, a Poway businessman of Filipino descent.
On the sixth day, Michael's feet began to swell and his body turned red. The next day, Mijian insisted they take Michael to Children's Hospital, where they finally got an answer.
Michael had Kawasaki disease.
"They had to admit him because he had a rare disease and ran the risk of having a heart attack," said Oleas, whose wife is of Korean descent. "That was the single scariest event in my life."
According to the American Heart Association, of the 4,000 cases of Kawasaki disease diagnosed annually in the United States, 80 percent are under age 5, but babies and older children can get it, too. Primarily a childhood disease, it occurs more often in boys than girls. Children of Asian descent are particularly susceptible; however, it crosses ethnic lines.
Kawasaki disease is named for Tomisaku Kawasaki, a Japanese pediatrician who first wrote about it in 1967. It is characterized by inflammation of blood vessels throughout the body.
Red eyes, red rash
"It acts like an immune reaction but gets confused with viral infections, because it mimics symptoms of other viruses: red eyes, fever and rash," said Dr. Jane Burns, a professor and chief of the Division of Allergy, Immunology & Rheumatology in the Department of Pediatrics at UC San Diego School of Medicine.
Symptoms include rapid onset of persistent fever; rash; swollen hands and feet; red and irritated eyes; swollen and irritated lips, mouth and throat; and swollen lymph nodes in the neck.
Fewer than 1 percent of those who get the illness die, but long-term complications, including damage to coronary arteries, can occur.
Approximately 25 percent of patients develop aneurysms, a ballooning of the coronary artery walls, which weaken as a result of the weakened immune system. Kawasaki disease is the No. 1 cause of acquired heart disease in children.
The cause is unknown, so there is no diagnostic test and no means of prevention. Some research suggests that an infection or environmental agent can trigger the illness in individuals who are genetically susceptible.
Hot spots
Burns is director of the Kawasaki Disease Research Center at Rady Children's Hospital. She has studied the illness since 1978. She follows 850 patients who have been diagnosed with Kawasaki disease.
According to her, reported cases in San Diego County peak in March and again in June and July, and there are several geographic hot spots.
"We don't know why we see higher numbers in Chula Vista, Mira Mesa and Oceanside," she said. "It's like the agent blows into town and picks off the susceptible. It appears like an infectious disease, but it doesn't look like a contagious disease." Diagnosing Kawasaki disease is difficult, she said, because all symptoms may not be present simultaneously.
When Kawasaki patients are admitted to the hospital, a high dose of immune globulin (a biological product that comes from donor blood and contains antibodies) is administered intravenously. An echocardiogram is performed to determine whether the heart has been damaged. Intravenous immune globulin, also called IVIG, was first given to Kawasaki patients in Japan in 1983. In the U.S., IVIG has been used since 1986, but why it works is a mystery.
"We don't know why it works. Somehow, it turns off the immune system," said Burns. "The goal of treatment is to stop the inflammation."
Speedier diagnosis
Beth Avant of Encinitas was luckier to have a speedier diagnosis than the Oleas family. Her 9-month-old son, Lucas, was admitted to Children's Hospital in December 2004, three days after symptoms of a persistent fever, rash, bleeding lips and bloodshot eyes were diagnosed as Kawasaki disease.
"We walked in the door and the nurse identified it immediately. We were extremely lucky," she said. "They started the IVIG that night. The next morning he was up and bouncing around."
Not all children respond to the IVIG treatment, but most patients who receive timely treatment make a full recovery within six to eight weeks. The long-term prognosis for treating Kawasaki disease with IVIG is unknown.
"It's good as far as we know. It is still a mystery as to whether there are long-term effects," said Burns. "We're not seeing any problems with the blood thus far. These kids are leading normal lives."
Family link
Recurrence is extremely rare, but Kawasaki can run in families. The Oleas' daughter, Audrey, got Kawasaki when she was 4 months old.
"A younger sibling of a child or one that isn't born yet has a tenfold increased risk," said Burns. "So parents should notify family members, cousins, uncles that the gene is in the family."
Awareness among the medical community and general public is critical to early diagnosis and treatment.
"If a child isn't diagnosed and treated within the 10 days, statistically, they have a greater chance of developing the heart problems," said Oleas, who is on the Kawasaki Disease Foundation board of directors. The nonprofit organization is dedicated to raising awareness and supporting families coping with the illness.
Last March, 18-month-old Fallbrook resident Amanda Marquardt had a persistent fever of 105 degrees. Within 48 hours, she became lethargic, and her lips and eyes turned bright red. The next day, her pediatrician diagnosed her with roseola, and recommended Motrin and Tylenol to reduce the fever.
"Later that day, I knew it wasn't the flu," said Marquardt's mother, Julie.
Amanda's tongue had turned bright red, and by the next morning, she'd broken out in a rash.
"She looked like she had the mumps. She had welts from head to toe."
Amanda was admitted to Children's Hospital with Kawasaki disease. An echocardiogram indicated her arteries were enlarging. She received two doses of IVIG and spent eight days in the hospital.
"It's been about a year, and everything looks good," said Marquardt.
Cameron Leigh James is a freelance writer.
Early symptoms of Kawasaki disease:
- Presence of a spiking, remittent fever that lasts several days
- Rash, often worse in the groin area, which appears within five days of onset of fever
- 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
- Redness of the palms and soles of feet
- Swollen lymph nodes
During the first and second week of the illness, other symptoms may appear, such as:
- Skin on fingertips and toes starts to peel
- Temporary arthritis that causes joint pain throughout the body
- Enlarged gallbladder
- Temporary hearing loss, abdominal pain, vomiting and diarrhea
It's important to note all symptoms, because all symptoms may not present simultaneously.
- Source: Kawasaki Disease Foundation: www.KDfoundation.org
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Gina wrote on Jun 11, 2007 12:35 PM:Cameron James has done an immeasurable service to the public by writing an article on this disease about which so many parents are unaware. Please keep up the good work; it may save lives!
Mom wrote on Jun 12, 2007 6:43 AM:My son was diagnosed with Kawasaki when he was 5 years old. He received treatment on day 7. One of the things the article didn't mention was the way the skin on the bottoms of his feet and the palms of his hands died. For days after he was released from the hospital we were cutting sheets of skin that was hanging off his hands and feet. Today he is a 6'6", 270 lb teenager who has had no lasting effects from the disease.
Susie wrote on Jun 13, 2007 9:29 AM:Education plays such a vital role in being able to quickly diagnose potentially life threatening diseases. Cameron James did an excellent job in bringing this important information about Kawasaki disease to the public and especially to the parents of young children.
mary wrote on Jun 13, 2007 5:36 PM:My 5 year old son Erik was diagnosed on the ninth day of illness May 22, 2007 and was treated by Dr. Burns at Children's Hospital. The scary thing is that 3 doctors all said not to worry (including my primary doctor). But my primary doctor did agree to refer him to Dr. Burns to ease my mind. I never heard of KD, until a Dr. at Children's ED mentioned the possibility on day 3 of fever. We parents have to be advocates and follow our gut instincts. It's just a simple blood test that show high level of inflammation, high SED rate and high platelet count. Thank you Cameron James for bringing this to parents attention.
Cheryl wrote on Jun 15, 2007 3:20 PM:My son, Gavin, was diagnosed with Kawasaki's Disease when he was about 1 1/2 yrs old. I'm glad his pediatrician was concerned enough about is symptoms to contact Children's Hospital and have him admitted. He was treated with IVIG and his follow up EKG was normal. Last year my daughter (@ about the same age as my son) was also diagnosed with KD. Luckily, I recognized the symptoms (high fever w/out relief from OTC meds; unconsolable; rash on trunk,groin , palms of her hands and soles of her feet; bright red lips and strawberry tongue) but I had to go through 3 physicians to convince them (this was NOT @ Children's Hospital.) One of them told me that KD is almost non-existant. One told me he thought she had Hand, Foot & Mouth Disease. One, even after my daughter had been admitted, wanted to hold off on the IVIG because it was EXPENSIVE (even giving me the dollar amount) and they still weren't convinced she had KD. I insisted they give it to her, I didn't care what is cost, besides...I WORKED FOR THAT HOSPITAL!!! A couple days later the rash was gone, but her lips were raw and her hands and feet had began to peel. Her follow up EKG, like her brother's, was normal. I want other parents to be aware that Kawasaki's Disease is very much EXISTANT. Always trust your gut, especially when it comes to the welfare of your children.
Mimi wrote on Sep 10, 2007 7:58 PM:My son had extreme fever for 7 days without responding to the Panadol, Nurofen and antibiotics his GP prescribed. On day 8, I brought him to another GP who told me to bring him to the hospital immediately because he thought something was not right. I brought him to a private hospital thinking he would be given the best of care. The admitting doctor at the ER told me it was a case of KD. The pediatrician told me it was a case of suspected KD. During these times, he developed the full symptoms of KD, ie. red eyes, extreme high fevers, red and tender lips that began to crack and bleed, mouth ulcers, diarrhea and peeling of hands and feet. And yet they kept him there for another 7 days after all these blood and urine tests they carried out. The ped. even told me it was not KD as they suspected. Not until I insisted that my baby be transferred to another hospital that they reluctantly discharged us. At the public hospital, he received immediate IVIG and Aspirin when his echo. result found he already had aneurysm of the heart. He is now recovering but I am so anguished about the action or inaction of those I trust. I wish I had been made aware of this disease before. I have now spread the info of this disease to all those I know with little children. I now pray to God everyday to help my son get better and become a normal healthy person that he deserves to be.
Mandy wrote on Apr 24, 2008 6:23 AM:My son's GP knew he had KD before his fever of 104.5 even broke...but the doctors at the children's hospital did not believe her. He was diagnosed on day 16 after 9 trips to the hospital and was having a relapse. His first echocardiogram on day 17 was clear, but apparently that is normal and the one on May 27th will show the real story. I pray that everything will work out in my son's favour...he is only 3.5 years and loves all sports and is very high energy.
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