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Kawasaki Disease

  • If recognized and treated early, your child can begin to feel better in a few days, with a low likelihood of long-term heart issues. Children who receive treatment within the first ten days of illness have a less than 4 percent risk of coronary artery damage.

    If your child has been diagnosed with Kawasaki disease, your understanding of the condition will help you and your family to cope with this illness.

    Kawasaki disease (mucocutaneous lymph node syndrome) is an acute systemic illness characterized by inflammation of the blood vessels (vasculitis). It begins with the onset of a high fever for at least four days, along with other signs and symptoms that develop over the course of approximately one to two weeks.

    • Inflammation from Kawasaki disease affects the blood vessels of many tissues in the body—including the hands, feet, whites of the eyes, mouth, lips, throat, lymph nodes and skin. The illness lasts for six to eight weeks, and more commonly affects children under age 5 years (75 percent). Boys are 1.5 times as likely to develop Kawasaki disease as girls.
       
    • Without treatment, approximately 20 percent of patients develop damage to their coronary arteries (the blood vessels that supply the heart with oxygen), resulting in those vessels’ enlargement (dilation or aneurysm formation).
       
    • If recognized and treated early, your child can begin to feel better in a few days, with a low likelihood of long-term heart issues. Children who receive treatment within the first ten days of illness have a less than 4 percent risk of coronary artery damage.
       
    • Because symptoms often resemble those of other childhood diseases and there’s no definitive test available, the diagnosis of Kawasaki disease is made by an experienced clinician on the basis of clinical symptoms and laboratory findings.


    How Boston Children’s Hospital approaches Kawasaki disease

    The experienced team members of Boston Children’s Hospital Kawasaki Disease Program provide coordinated care for children with Kawasaki disease—both during the illness’s acute phase and in short- and long-term follow up.

    Each year, our multidisciplinary program—staffed by expert cardiologists, rheumatologists and a pediatric nurse practitioner—treats more than 65 new patients who have confirmed or suspected Kawasaki disease. We follow more than 1,500 children and young adults who’ve had Kawasaki disease in the program’s outpatient Heart Care Center.

    The Kawasaki Disease Program at Boston Children’s serves as a national and international resource for parents and physicians. We provide families with a wealth of information, and support—and we help pediatricians and other specialists to confirm and treat this hard-to-diagnose illness.

    A member of Boston Children’s Kawasaki Disease Program is on call for you and your pediatrician 24 hours a day, seven days a week to help make a diagnosis, provide appropriate treatment, and offer support for your family. With our compassionate, family-centered approach, you and your child are in the best possible hands.

    Kawasaki disease: Reviewed by Jane Newburger, MD
    © Boston Children’s Hospital, 2011

  • It’s natural for you to be concerned right now about your child’s health—Kawasaki disease can be alarming for you and uncomfortable for your child. However, most children recover without significant heart issues. And you can be assured that at Boston Children's Hospital, your child is in expert hands.

    What is Kawasaki disease?

    First described by T. Kawasaki, MD, in Japan in 1967, Kawasaki disease is the most common form of acquired (rather than congenital) heart disease in the United States and Japan. The disease is most common in children under age 5 years (75 percent), although children of any age can develop it. Boys are 1.5 times as likely as girls to develop the disease.

    Kawasaki disease is an acute systemic illness characterized by inflammation of the blood vessels (vasculitis). It begins with the onset of a high fever, along with other signs and symptoms that develop over the course of approximately one to two weeks. The total duration of illness (before the child is completely back to normal) is six to eight weeks.

    The inflammation from Kawasaki disease affects the blood vessels of many tissues in the body—with symptoms that include changes in the hands, feet, whites of the eyes, mouth, lips, throat, lymph nodes and skin. Affected children have a high fever for at least four days before diagnosis.

    If recognized and treated early, your child can begin to feel better in a few days, with a low likelihood of long-term heart issues. In rare cases (about 4 percent) even with treatment, Kawasaki disease can result in a coronary artery aneurysm. There’s no definitive test for the disease, and its symptoms often resemble those of other childhood diseases.

    What are the signs and symptoms of Kawasaki disease?

    The signs and symptoms of Kawasaki disease often resemble other conditions or medical problems, so always consult your child’s physician for a diagnosis. Your child’s doctor will be looking for typical signs and symptoms—starting with a persistent high fever (101 degrees F to 104 degrees F) for at least four days, along with four out of five of the following:

    • changes in extremities
      • red, swollen palms of hands and soles of feet
      • peeling skin around the fingertips, hands or feet (occurs later in the illness)
    • non-specific rash on the body, often accentuated in the groin area
    • bloodshot eyes (no discharge)
    • redness in the lips, mouth and tongue
      • dry, red, cracked lips
      • inflamed, red mouth
      • swollen, red tongue (“strawberry tongue”)
    • swollen lymph node(s) in the neck (more than 1.5 cm), usually on one side

    Photos 1 and 2 from Circulation. 2001;103:335. © 2001 American Heart Association, Inc. Used with permission.

    It is possible to have Kawasaki disease without showing all of these signs. Such cases are called “incomplete” (or atypical) Kawasaki disease. Infants younger than 6 months are most likely to have incomplete Kawasaki disease, and infants have the highest risk for developing coronary artery damage.

    Other symptoms that may develop include:

    • arthritis-like symptoms (joint pain and swelling of the joints)
    • extreme irritability
    • diarrhea
    • vomiting
    • abdominal pain
    • enlarged liver or gallbladder
    • cough and respiratory symptoms

    Who’s affected by Kawasaki disease?

    Kawasaki disease occurs more often in Japan than in any other country. Children of Asian or Asian-American heritage have a higher risk of Kawasaki disease regardless of where they live—although Kawasaki disease can occur in any racial or ethnic group.

    The majority (75 percent) of children who develop Kawasaki disease are age 5 years or younger, although children of any age can get the disease. The average age of a child with the illness is approximately 2 years, and boys are about 1.5 times as likely to develop Kawasaki disease as girls.

    Researchers continue to explore the genetic factors that put some children at higher risk for Kawasaki disease, since:

    • Children of Japanese origin, no matter where in the world they live, are more likely to get Kawasaki disease.
    • Brothers and sisters of children with Kawasaki disease are slightly more likely than other neighborhood children to develop the illness at a later time.
    • Kawasaki disease has been reported in the children of parents who themselves had been affected in childhood.

    Who should be treated for Kawasaki disease?

    Any child with Kawasaki disease in the acute phase should receive treatment—ideally within the first seven to 10 days of the illness. Children in whom the diagnosis of Kawasaki disease is made after the tenth day of illness should be treated if they still have fever, or if they have coronary aneurysms together with ongoing laboratory tests suggesting inflammation.

    Why do you treat Kawasaki disease?

    Approximately 20 percent of untreated children with Kawasaki disease develop abnormalities in their coronary arteries (the blood vessels that supply the heart with oxygen), resulting in those vessels’ enlargement/aneurysm formation (dilation of the blood vessel). The goal of treatment in Kawasaki disease is to decrease the risk of the child developing coronary enlargement/aneurysm(s).

    In most cases, treatment reduces the duration of fever, as well as the risk of developing coronary abnormalities. Rarely (in less than 5 percent of cases), even with treatment, Kawasaki disease can weaken the wall of one or more of the coronary arteries, resulting in an aneurysm.

    What are the phases of Kawasaki disease?

    There are three phases of Kawasaki disease:

    • acute phase:The acute phase comes on rapidly with the abrupt onset of a high fever that lasts for at least four days. In this phase, treatment with IVIG (intravenous immunoglobulin) is the standard of care, given in the hospital as soon as a diagnosis is made. Besides fever, other signs and symptoms that may come and go during your child’s illness include:
      • swelling or redness of the hands and feet
      • rash
      • bloodshot eyes
      • red, cracked lips, “strawberry tongue”
      • enlarged lymph node in the neck
      • irritability
    • subacute phase: During the subacute phase (between acute and chronic), your child’s fever will go down, but the disease will continue to affect other systems. Lingering symptoms and laboratory findings may include:
      • arthritis in his small or large joints
      • continued irritability
      • peeling skin on hands and feet
      • elevated platelet counts
      • anemia
    • convalescent phase: In the convalescent phase (the gradual return to health and strength), most findings on your child’s physical exam will have returned to normal, but his blood work will show resolving inflammation.

    Irritability is a feature of Kawasaki disease throughout the course of a child’s illness. It may be the last symptom to resolve.

    How is Kawasaki disease diagnosed?

    One challenge in diagnosing Kawasaki disease is distinguishing it from other childhood diseases that have similar symptoms, such as some viral infections, including measles, adenovirus, enterovirus and Epstein-Barr virus—as well as illnesses caused by bacterial toxins, such as scarlet fever, toxic shock syndrome or bacterial infection of the lymph nodes in the neck.

    A prolonged high fever (at least four days), along with several other symptoms, must be present for your child’s doctor to consider Kawasaki disease as the cause of his illness, and other illnesses need to be ruled out. To arrive at a diagnosis, your child’s doctor will start by taking a full medical history and conducting a thorough physical exam. Diagnostic tests may include:

    • electrocardiogram (EECG or EKG): a test that records the electrical activity of the heart and shows abnormal rhythms (arrhythmias or dysrhythmias)
       
    • echocardiogram (cardiac ultrasound): a diagnostic tool that uses sound waves to produce a moving picture of the heart and heart valves in order to:
      • measure the coronary arteries
      • evaluate the structure and function of the heart muscle and heart valves
         
    • complete blood count (CBC): a measurement of the size, number and maturity of blood cells in a specific volume of blood; detects elevation in the numbers of white blood cells, which normally multiply in the presence of infection
       
    • erythrocyte sedimentation rate (ESR, “sed rate”) and/or c-reactive protein (CRP): non-specific markers of inflammation in the blood
       
    • urinalysis: testing of a urine sample for protein, red blood cells or white blood cells; helps rule out other diseases

    Observations and lab results that can support a diagnosis of Kawasaki disease include:

    • extreme irritability
    • inflammation of the joints (arthritis)
    • elevated white blood cell count
    • elevated liver function tests
    • signs of inflammation in the blood and urine
    • anemia

    It is possible to have Kawasaki disease without showing all of these signs. Such cases are called “incomplete” (or atypical) Kawasaki disease. Infants younger than 6 months are most likely to have incomplete Kawasaki disease, and infants have the highest risk for developing coronary artery injury.

    How is Kawasaki disease treated?

    The standard treatment for Kawasaki disease is IVIG (intravenous immunoglobulin), administered during the acute phase of the illness. IVIG has been shown to shorten the duration of the fever and decrease the risk of developing a coronary aneurysm. Because IVIG is given intravenously (through a vein), your child will need to stay in the hospital for at least one day, and often longer if IVIG is given.

    In addition, high doses of aspirin will help control your child’s fever until it has resolved, after which time the dose of aspirin is lowered. Lower-dose aspirin helps prevent blood clotting during this time.

    Most children respond to their first treatment with IVIG with reduced fever. If your child’s fever continues, he may need a second dose. Some children are resistant to IVIG (i.e., continue to have fever despite IVIG treatment) and may be treated with other therapies. The expert team in Boston Children’s Kawasaki Disease Program has wide experience treating children with IVIG-resistant Kawasaki disease.

    What are the risks to the heart from Kawasaki disease?

    A small but significant percentage (approximately 20 percent) of untreated children with the disease develop abnormalities in their coronary arteries (the blood vessels that supply the heart with oxygen), resulting in coronary artery dilation or aneurysms. Prompt treatment with IVIG reduces the risk of a child developing coronary complications to less than 5 percent.

    What causes Kawasaki disease?

    Despite decades of research, the cause of Kawasaki disease is still unknown. The disease is not contagious (spread from person to person). Outbreaks have been reported in waves within geographical areas, and the disease tends to occur more frequently in winter and early spring.

    Because no bacteria or virus has been proven to cause Kawasaki disease, some experts believe that Kawasaki disease is an immune reaction that children may have to a variety of infectious agents. Genetic susceptibility may play a role.

    How often does Kawasaki disease occur?

    Kawasaki disease is a major cause of heart disease in children. It has replaced acute rheumatic fever as the leading cause of acquired heart disease in children in the United States and Japan.

    Can Kawasaki disease be prevented?

    Currently, there’s no known way to prevent Kawasaki disease. Nothing you’ve done has caused your child’s condition, and there are no precautions you could have taken to prevent it.

    What is my child’s long-term outlook?

    Most children make a full recovery after their Kawasaki disease resolves. Any child who has had Kawasaki disease should receive periodic follow-up.

    Kawasaki disease was first described in the late 60’s, so information about the longer-term complications of the illness is restricted to the first few decades of life. Within this time frame, children who never developed coronary aneurysms at any time in their early illness have not developed future coronary artery disease causing clinical symptoms.

    However, we do like to make sure that children who’ve had Kawasaki disease do not have additional risk factors for coronary heart disease as they get older. Since the very long-term health risks from Kawasaki disease aren’t conclusively known, your child should be sure to eat a heart-healthy diet, exercise regularly and avoid smoking. At some point after the illness (about one year later), he should have a cholesterol profile checked as part of his routine well-child care.

    If your child developed an aneurysm as a result of Kawasaki disease, he’ll need more frequent cardiac follow-up. The frequency of follow-up and cardiac testing is determined individually for each patient, depending on the extent of coronary injury. Coronary arteries that are damaged may heal over time.

    In coronary arteries with large or giant coronary aneurysms, the healing process may result in tightness (stenosis) of the coronary artery, especially at the ends of the aneurysm. If a coronary artery blockage or stenosis occurs, the child is at risk for too little blood flow to the heart (myocardial ischemia) or a heart attack (myocardial infarction).

    Aneurysms may also occasionally occur in other arteries, such as those supplying the arms, legs or kidneys. These peripheral aneurysms rarely cause symptoms, and are generally seen only in patients who have giant coronary aneurysms.

    FAQ

    Q: What is Kawasaki disease?

    A: Kawasaki disease is an acute systemic illness characterized by inflammation of the blood vessels (vasculitis). It begins with the onset of a high fever for at least four days, along with other signs and symptoms that develop over the course of approximately one-two weeks. The inflammation from Kawasaki disease affects the blood vessels of many tissues in the body—including the hands, feet, whites of the eyes, mouth, lips, throat, lymph nodes and skin.

    Q: If my child has Kawasaki disease, will he be OK?

    A:If recognized and treated early, you child can begin to feel better in a few days, with a low likelihood of long-term heart issues. Without treatment, approximately 20 percent of patients develop damage to their coronary arteries (the blood vessels that supply the heart with oxygen), results in those vessels’ enlargement (dilation or aneurysm formation).

    Children who receive treatment with IVIG within the first ten days of illness have a less than 5 percent risk of coronary artery damage. Any child who’s had Kawasaki disease should receive periodic follow-up for the duration of time that his cardiologist advises.

    Q: How does Boston Children’s treat Kawasaki disease?

    A: The standard treatment for Kawasaki disease is IVIG (intravenous immunoglobulin), given during the acute phase of the illness. IVIG has been shown to shorten the duration of the fever and decrease the risk of developing a coronary aneurysm. Because IVIG is given intravenously (through a vein), your child will need to stay in the hospital for at least one day, and often longer.

    In addition, high doses of aspirin will help control your child’s fever until it has resolved, after which time the dose of aspirin is lowered. Lower-dose aspirin helps prevent blood clotting during this time.

    Most children respond to their first treatment with IVIG with reduced fever. If your child’s fever continues, he may need a second dose. Some children are resistant to IVIG (i.e., continue to have fever despite IVIG treatment) and may be treated with other therapies. The expert team in Children’s Kawasaki Disease Program has wide experience treating children with IVIG-resistant Kawasaki disease.

    Q: What are the signs and symptoms of Kawasaki disease?

    A: The signs and symptoms of Kawasaki disease often resemble other conditions or medical problems, so always consult your child's physician for a diagnosis. Your child’s doctor will be looking for typical signs and symptoms—starting with a persistent high fever (101 degrees F to 104 degrees F) for at least four days, along with four out of five of the following:

    • changes in extremities
      • red, swollen palms of hands and soles of feet
      • peeling skin around the fingertips, hands or feet (occurs later in the illness)
    • non-specific rash on the body, often accentuated in the groin area
    • bloodshot eyes (no discharge)
    • redness in the lips, mouth and tongue
      • dry, red, cracked lips
      • inflamed, red mouth
      • swollen, red tongue (“strawberry tongue”)
    • swollen lymph node(s) in the neck (more than 1.5 cm), usually on one side

    It is possible to have Kawasaki disease without showing all of these signs. Such cases are called “incomplete” (or atypical) Kawasaki disease. Infants younger than 6 months are most likely to have incomplete Kawasaki disease, and infants have the highest risk for developing coronary artery damage.

    Other symptoms may develop that include:

    • arthritis-like symptoms (joint pain and swelling of the joints)
    • extreme irritability
    • diarrhea
    • vomiting
    • abdominal pain
    • enlarged liver or gallbladder
    • cough and respiratory symptoms

    Q: If my child has Kawasaki disease, what should I ask my Boston Children’s doctor?

    A: Ask your doctor:

    • How will you diagnose my child?
    • What actions might you take after you reach a diagnosis?
    • What therapies are offered?
    • What should we expect when he comes home?
    • Will there be restrictions on my child’s activities?
    • Will there be long-term effects?

    Q: How is Kawasaki disease usually diagnosed?

    A: A prolonged high fever along with several other symptoms need to be present for your child’s doctor to consider Kawasaki disease as the cause for his illness, and other illnesses must be ruled out. To arrive at a diagnosis, your child’s doctor/practitioner will start by taking a full medical history and by conducting a thorough physical examination. Additional diagnostic tests may include:

    • electrocardiogram (ECG or EKG)
    • echocardiogram (cardiac ultrasound)
    • blood tests
    • urine tests

    Q: What should we do at home after Kawasaki disease?

    A:
    It’s important to monitor your child’s temperature after discharge and to contact your physician/practitioner if his fever returns. Low-dose aspirin is used to prevent clotting for approximately six to eight weeks after your child goes home—even longer if he’s had any heart or blood vessel complications, such as an aneurysm.

    Expect him to feel tired and irritable for a few weeks after he comes home. Irritability is often the last symptom to disappear. In addition, children may have peeling of the skin of their hands and feet (beginning at the tips). This generally occurs in the second week of illness and although it’s not painful, the new skin may be somewhat tender.

    Your child's lips may be cracked for a few weeks after he's been discharged, and his skin overall may be dry. You can apply Vaseline to his lips and an unscented lotion to his skin for hydration. Some children may have a temporary arthritis that can last for up to a few months.

    Q: What causes Kawasaki disease?

    A: Despite decades of research, the cause of Kawasaki disease is still unknown. The disease is not contagious (spread from person to person). Outbreaks have been reported in waves within geographical areas, and the disease tends to occur more frequently in winter and early spring.

    Because no bacteria or virus has been proven to cause Kawasaki disease, some experts believe that Kawasaki disease is an immune reaction that children may have to a variety of infectious agents. Genetic susceptibility may play a role.

    Q: Who gets Kawasaki disease?

    A. Kawasaki disease occurs more often in Japan than in any other country. Children of Asian or Asian-American heritage have a higher risk of Kawasaki disease regardless of where they live—although Kawasaki disease can occur in any racial or ethnic group.

    The majority (75 percent) of children who develop Kawasaki disease are age 5 years or younger, although children of any age can get the disease. The average age of a child with the illness is approximately 2 years, and boys are about 1.5 times as likely to develop Kawasaki disease as girls.

    Q: What is Boston Children’s experience treating Kawasaki disease?

    A: Boston Children’s serves as a national and international Kawasaki disease resource for parents and physicians. Our Kawasaki Disease Program—staffed by cardiologists, rheumatologists and a pediatric nurse practitioner—treats more than 65 new patients each year for definite or suspected Kawasaki disease, and we follow more than 1,500 children and young adults long-term.

    Q: What heart research and innovations are coming from Boston Children’s?

    A: There’s still a lot to learn about Kawasaki disease. Ongoing research in Kawasaki disease at Boston Children’s includes studies in several areas, such as:

    • searching for genetic factors that may play a role in the susceptibility of Kawasaki disease
    • finding a test to aid in the diagnosis of Kawasaki disease
    • advances in treatment options for children who don’t respond to conventional therapy
    • delineating the long-term sequelae of Kawasaki disease in patients with and without coronary aneurysms

    Causes

    Despite decades of research, the cause of Kawasaki disease is still unknown. The disease is not contagious (spread from person to person). Outbreaks have been reported in waves within geographical areas, and the disease tends to occur more frequently in winter and early spring. Because no bacteria or virus has been proven to cause Kawasaki disease, some experts believe that Kawasaki disease is an immune reaction that children may have to a variety of infectious agents. Genetic susceptibility may play a role.

    Signs and symptoms

    The signs and symptoms of Kawasaki disease often resemble other conditions or medical problems, so always consult your child's physician for a diagnosis. Your child’s doctor will be looking for typical signs and symptoms—starting with a persistent high fever for at least four days, along with four out of five of the following:

    • changes in extremities
      • red, swollen palms of hands and soles of feet
      • peeling skin around the fingertips, hands or feet (occurs later in the illness)
    • non-specific rash on the body, often accentuated in the groin area
    • bloodshot eyes (no discharge)
    • redness in the lips, mouth and tongue
      • dry, red, cracked lips
      • inflamed, red mouth
      • swollen, red tongue (“strawberry tongue”)
    • swollen lymph node(s) in the neck (more than 1.5 cm), usually on one side

    When to seek medical advice

    Call your health care provider if your baby or child has a persistent high fever for at least four days along with some or all of the diagnostic criteria described above.

    Questions to ask your doctor

    • How will you diagnose my child?
    • What actions might you take after you reach a diagnosis?
    • What therapies are offered?
    • Will my child be OK if he has Kawasaki disease?
    • Will there be restrictions on my child’s activities?
    • What should we expect when he comes home?
    • Will there be long-term effects?

    Who’s at risk

    Kawasaki disease occurs more often in Japan than in any other country. Children of Asian or Asian-American heritage have a higher risk of Kawasaki disease regardless of where they live—although Kawasaki disease can occur in any racial or ethnic group.

    The majority (75 percent) of children who develop Kawasaki disease are age 5 years or younger, although children of any age can get the disease. The average age of a child with the illness is approximately 2 years, and boys are about 1.5 times as likely to develop Kawasaki disease as girls.

    Researchers continue to explore the genetic factors that put some children at higher risk for Kawasaki disease, since:

    • Children of Japanese origin, no matter where in the world they live, are more likely to get Kawasaki disease.
    • Brothers and sisters of children with Kawasaki disease are slightly more likely than other neighborhood children to develop the illness at a later time.
    • Kawasaki disease has been reported in the children of parents who themselves had been affected in childhood.

    Complications

    A small but significant percentage (approximately 20 percent) of untreated children with the disease develop abnormalities in their coronary arteries (the blood vessels that supply the heart with oxygen), resulting in coronary artery dilation or aneurysms. Prompt treatment with IVIG reduces the risk of a child developing coronary complications to less than 5 percent.

    Long-term outlook

    Most children make a full recovery after their Kawasaki disease resolves. Any child who has had Kawasaki disease should receive periodic follow-up.

    Kawasaki disease was first described in the late 60’s, so information about the longer-term complications of the illness is restricted to the first few decades of life. Within this time frame, children who never developed coronary aneurysms at any time in their early illness have not developed future coronary artery disease causing clinical symptoms.

    However, we do like to make sure that children who’ve had Kawasaki disease do not have additional risk factors for coronary heart disease as they get older. Since the very long-term health risks from Kawasaki disease aren’t conclusively known, your child should be sure to eat a heart-healthy diet, exercise regularly and avoid smoking. At some point after the illness (about one year later), he should have a cholesterol profile checked as part of his routine well-child care.

    If your child developed an aneurysm as a result of Kawasaki disease, he’ll need more frequent cardiac follow-up. The frequency of follow-up and cardiac testing is determined individually for each patient, depending on the extent of coronary injury. Coronary arteries that are damaged may heal over time.

    In coronary arteries with large or giant coronary aneurysms, the healing process may result in tightness (stenosis) of the coronary artery, especially at the ends of the aneurysm. If a coronary artery blockage or stenosis occurs, the child is at risk for too little blood flow to the heart (myocardial ischemia) or a heart attack (myocardial infarction).

    Aneurysms may also occasionally occur in other arteries, such as those supplying the arms, legs or kidneys. These peripheral aneurysms rarely cause symptoms, and are generally seen only in patients who’ve giant coronary aneurysms.

    What you can do at home

    Expect your child to feel tired and irritable for a few weeks after he comes home. He may be able to go back to school/daycare after about a week, but may not feel 100 percent better until about six to eight weeks after his illness. Monitor your child’s temperature at home, and contact your health professional if the fever returns.

    Your child may be prescribed low-dose aspirin to prevent clotting for as long as six to eight weeks after he goes home. A child will need ongoing medication if he has coronary enlargement or aneurysms. Medications that affect clotting, such as aspirin, will cause easy bruising. While the child is on an anti-clotting medication, he should avoid activities that have a high risk of physical injury, and should take usual precautions such as wearing seat belts in the car and helmets on bicycles.

    Prevention

    Currently, there’s no known way to prevent Kawasaki disease. Nothing you’ve done has caused your child’s condition, and there are no precautions you could have taken to prevent it.

    Kawasaki disease glossary

    • acute phase: the first phase of Kawasaki disease, involving a high fever and vasculitis resulting in the typical signs and symptoms of  Kawasaki disease
       
    • aneurysm: a bulged out area of a weakened coronary artery wall. Blood clots can form in the ballooned area, blocking blood flow through the coronary artery.
       
    • blood clot: a congealed mass of blood that may block, or partially block, the flow of blood through an artery
       
    • cardiac/cardio-: pertaining to the heart
       
    • cardiac magnetic resonance imaging (MRI): a non-invasive diagnostic tool using 3-D imaging technology produced by magnets to accurately determine the blood flow, structure and functioning of your child’s heart
       
    • cardiac surgery: surgical procedure performed on the heart
       
    • cardiologist: doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants and children with heart problems.
       
    • complete blood count (CBC): a measurement of the size, number and maturity of different blood cells in a specific volume of blood; detects elevation in the numbers of white blood cells, which normally multiply in the presence of inflammation or infection
       
    • convalescent: gradually returning to health and strength
       
    • coronary arteries: blood vessels that supply the heart muscle with oxygen-rich blood
       
    • c-reactive protein (CRP): a non-specific marker of inflammation in the blood
       
    • diagnosis: medical determination of illness or disease based on history, physical examinations and advanced technology diagnostic testing tools
       
    • dilated: enlarged
       
    • echocardiogram (echo, cardiac ultrasound): a diagnostic tool that uses sound waves to produce a moving picture of the heart and heart valves in order to:
      • measure the coronary arteries
      • evaluate the structure and function of the heart and heart valves
         
    • electrocardiogram (ECG, EKG): a test that records the electrical activity of the heart and shows abnormal rhythms (arrhythmia)
       
    • erythrocyte sedimentation rate (ESR or “sed rate”): a non-specific marker of inflammation in the blood
       
    • incomplete (atypical) Kawasaki disease: a form of the disease in which not all of the typical diagnostic signs and symptoms show up. Infants younger than 6 months are most likely to have incomplete Kawasaki disease.
       
    • inflammation: the process that makes living tissue swell, become painful and turn red
       
    • IVIG (intravenous immunoglobulin): the standard medication for Kawasaki disease.
       
    • Kawasaki disease (KD, mucocutaneous lymph node syndrome): an acute illness characterized by a high, sustained fever and vasculitis; chiefly affects children
       
    • mucocutaneous lymph node syndrome: another term for Kawasaki disease
       
    • myocarditis: inflammation and irritation of the heart muscle
       
    • myocardium: the heart muscle
       
    • myointimal proliferation: the multiplying of cells lining the blood vessels
       
    • myopericarditis: inflammation and irritation of the heart muscle and the membrane covering the heart
       
    • pericardium: membrane covering the heart
       
    • stenosis (-es): blockage, blockages
       
    • symptoms: the presenting physical complaints for which a patient seeks medical attention
       
    • urinalysis: testing of a urine sample for protein, red blood cells or white blood cells
       
    • vasculitis: inflammation of the blood vessels

    For a more complete list of cardiovascular terms, visit Boston Children’s Cardiovascular glossary.

    For in-depth visual information on several of the conditions, diagnostic tools and procedures described above, visit our cardiovascular Multimedia library.

  • One challenge in diagnosing Kawasaki disease is distinguishing it from other childhood diseases that have similar symptoms, such as some viral infections, including measles, adenovirus, enterovirus and Epstein-Barr virus—as well as illnesses caused by bacterial toxins, such as scarlet fever, toxic shock syndrome or bacterial infection of the lymph nodes in the neck.

    A prolonged high fever (at least four days), along with several other symptoms, must be present for your child’s doctor/NP/PA to consider Kawasaki disease as the cause of his illness — and other illnesses need to be ruled out. To arrive at a diagnosis, your child’s doctor will start by taking a full medical history and conducting a thorough physical exam. Diagnostic tests may include:

    • electrocardiogram (ECG or EKG): a test that records the electrical activity of the heart and shows abnormal rhythms (arrhythmias or dysrhythmias)
    • echocardiogram (cardiac ultrasound): a diagnostic tool that uses sound waves to produce a moving picture of the heart and heart valves in order to: 
      • measure the coronary arteries
      • evaluate the structure and function of the heart muscle and heart valves      
    • complete blood count (CBC): a measurement of the size, number and maturity of blood cells in a specific volume of blood; detects elevation in the numbers of white blood cells, which normally multiply in the presence of infection   
    • erythrocyte sedimentation rate (ESR, “sed rate”) and/or c-reactive protein (CRP): non-specific markers of inflammation in the blood
    • urinalysis: testing of a urine sample for protein, red blood cells or white blood cells; helps rule out other diseases

    Observations and lab results that can support a diagnosis of Kawasaki disease include:

    • extreme irritability
    • inflammation of the joints (arthritis)
    • elevated white blood cell count
    • elevated liver function tests
    • signs of inflammation in the blood and urine
    • anemia

    Incomplete Kawasaki disease

    It is possible to have Kawasaki disease without showing all of these signs. Such cases are called “incomplete” (or atypical) Kawasaki disease. Infants younger than 6 months are most likely to have incomplete Kawasaki disease, and infants have the highest risk for developing coronary artery injury.

    Boston Children’s development of interventional catheterization 

    You’ll be comforted to know that Boston Children’s pioneered the use of interventional catheterization for many congenital heart defects and is a leader in the use of this procedure. 

    Heart surgery at Boston Children’s 

    Watch a fascinating webcast of heart surgery at Boston Children’s.

  • Treating your child for Kawasaki disease within seven to 10 days of the onset of illness will help reduce his risk of coronary complications. Treatment at Boston Children's Hospital aims to:

    • reduce fever and inflammation
    • control platelet activity
    • make your child more comfortable
    • protect the heart from damage

    The standard treatment for Kawasaki disease is IVIG (intravenous immunoglobulin), given during the acute phase of the illness. IVIG has been shown to shorten the duration of the fever and decrease the risk of developing a coronary aneurysm. Because IVIG is given intravenously (through a vein), your child will need to stay in the hospital while the IVIG is given and until his fever goes away.

    In addition, high doses of aspirin will help control your child's fever until it has resolved, after which time the dose of aspirin is lowered. Lower-dose aspirin helps prevent blood clotting during this time.

    Response vs. resistance to IVIG

    Most children respond with reduced fever to their first treatment with IVIG. If your child's fever continues, he may need a second dose. Some children are resistant to IVIG (i.e., continue to have fever despite IVIG treatment) and may be treated with additional therapies. The expert team at Boston Children's Kawasaki Disease Program has wide experience treating children with IVIG-resistant Kawasaki disease.

    At home: caring for your child after Kawasaki disease

    Expect your child to feel tired and irritable for a few weeks after he comes home. He may be able to go back to school/daycare after about a week but may not feel 100 percent better until about six to eight weeks after his illness. Monitor your child's temperature at home and contact your health professional if the fever returns.

    Your child may be prescribed low-dose aspirin to prevent clotting for as long as six to eight weeks after your child goes home. A child will need ongoing medication if he has coronary enlargement or aneurysms. Medications that affect clotting, such as aspirin, will cause easy bruising. While the child is on an anti-clotting medication, he should avoid activities that have a high risk of physical injury, and should take the usual precautions, such as wearing a seat belt in the car and a helmet when riding his bike.

    Follow-up care

    Most children make a full recovery after Kawasaki disease. Follow-up appointments will be scheduled approximately one to two weeks after treatment, and again four to six weeks after treatment. At these visits, your child will have a cardiac ultrasound (echocardiogram) to assess his coronary artery size and heart and valve function.

    At the same intervals, your child will also have blood work, which may include a complete blood count, platelet count, liver function tests and tests for inflammation in the blood.

    Following Kawasaki disease, your child should receive periodic follow-up. The frequency of follow-up and the testing that's ordered will be determined on an individual basis. The clinician will continue to monitor your child for coronary artery disease risk factors, such as high blood pressure and high cholesterol. Your child should be sure to eat a heart-healthy diet, exercise regularly and avoid smoking.

    As he grows: your child's long-term outlook after Kawasaki disease

    If your child has no coronary artery changes. Kawasaki disease was first described in the late 60's, so information about the longer-term complications of the illness is restricted to the first few decades of life. Within this time frame, children who never developed coronary aneurysms at any time in their early illness have not developed future coronary artery disease causing clinical symptoms.

    However, we do like to make sure that children who've had Kawasaki disease do not have additional risk factors for coronary heart disease as they get older. Since the very long-term health risks from Kawasaki disease aren't conclusively known, your child should be sure to eat a heart-healthy diet, exercise regularly and avoid smoking. At some point after the illness (about one year later), he should have a cholesterol profile checked as part of his routine well-child care.

    Research at Children's has also examined the psychosocial and physical functioning of children who've recovered from Kawasaki disease. We've found that children without aneurysms were similar in psychosocial and physical health to the general population.

    If your child developed aneurysms. If your child developed an aneurysm(s) as a result of Kawasaki disease, he'll need more frequent cardiac follow-up. The frequency of follow-up and cardiac testing is determined individually for each patient, depending on the extent of coronary injury.

    Coronary arteries that are damaged may heal over time. In coronary arteries with large or giant coronary aneurysms, the healing process may result in tightness (stenosis) of the coronary artery, especially at the ends of the aneurysm. If a coronary artery blockage or stenosis occurs, the child is at risk for too little blood flow to the heart (myocardial ischemia) or a heart attack (myocardial infarction).

    Testing is done at regular intervals to make sure that the heart muscle is getting enough blood flow. Testing may include periodic echocardiograms, stress tests, cardiac MRIs, cardiac CT scans and other imaging studies that assess heart function and blood flow to the heart. Cardiac catheterization may done in patients who still have significant coronary abnormalities one year after the initial illness, or if there are signs or symptoms that the heart isn't getting enough oxygen.

    Aneurysms may also occasionally occur in other arteries, such as those supplying the arms, legs or kidneys. These peripheral aneurysms rarely cause symptoms, and are generally seen only in patients who have giant coronary aneurysms.

    Coping and support

    At Boston Children's, we understand that a hospital visit can be difficult, and sometimes overwhelming. So, we offer many amenities to make your child's—and your own—hospital experience as pleasant as possible. Visit The Center for Families for all you need to know about:

    • getting to Boston Children's
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family

    In particular, we understand that you may have a lot of questions if your child is diagnosed with Kawasaki disease. How will it affect my child long term? What do we do next? We can connect you with a number of resources to help you and your family through this difficult time, including:

    • patient education: From the hospital to recuperation, our doctors and nurses will be on hand to walk you through your child's course and help answer any questions you may have. Upon your child's discharge, they'll also reach out to you by phone, continuing the care and support he received while at Boston Children's.
       
    • parent-to-parent: Want to talk with someone whose child has been treated for Kawasaki disease? We can often put you in touch with other families who've been through the same process that you and your child are facing, and who can share their experience.
       
    • faith-based support: If you're in need of spiritual support, we'll connect you with the Boston Children's chaplaincy. Our program includes nearly a dozen clergy— representing Protestant, Jewish, Muslim, Roman Catholic and other faith traditions—who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
       
    • social work: Our social workers and mental health clinicians have helped many families in your situation. We can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

    Boston Children's Heart Care Center

    The Heart Care Center at Boston Children's is one of the largest pediatric heart programs in the United States. Our staff of more than 80 pediatric cardiac specialists cares for thousands of children and adults with congenital and acquired heart defects each year, from simple to complex cases. We have experience treating rare heart problems—with results that are among the best in the world.

    Keep family and friends up-to-date during your child's treatment . . .

    … by creating a free Boston Children's Carepage

  • There’s still a lot to learn about Kawasaki disease. Ongoing research in Kawasaki disease at Boston Children's Hospital includes studies in several areas, such as:

    • searching for genetic factors that may play a role in the susceptibility of Kawasaki disease
    • finding a test to aid in the diagnosis of Kawasaki disease
    • advances in treatment options for children who don’t respond to conventional therapy, delineating the long-term sequelae of Kawasaki disease in patients with and without coronary aneurysms

    Genetic research in Kawasaki disease

    Boston Children’s investigators participate in a multi-center study aimed at increasing our understanding of why certain children become affected with Kawasaki disease. Experts believe that there may be genetic susceptibility in determining why some children get this illness. DNA is obtained from the child with Kawasaki disease and ideally from both biological parents.

    Developing a diagnostic test for Kawasaki disease

    By analyzing the urine of patients with Kawasaki disease, researchers are working to identify the “biomarkers”—the molecular telltale signs—that can serve as diagnostic and prognostic indicators for the condition. A quicker diagnosis of Kawasaki disease could lead to more efficient treatment, with the potential for fewer complications and less discomfort.

    Our Proteomics Center has excelled in developing fast, efficient and minimally invasive diagnostics by studying biomarkers for disease that are found in urine. Urine is easier to obtain than blood and has a narrower range of molecules and proteins to study. Recent innovations that diagnose Appendicitis early by finding these urinary biomarkers have encouraged the current study to find biomarkers for Kawasaki disease, which is funded by a one-year grant from Boston Children’s Translational Research Program.

    Alex Kentsis, MD, PhDHanno Steen, PhD, director of Boston Children’s Proteomics Center, and Susan Kim, MD, MMSc, rheumatologist and the clinical investigator enrolling patients for the study, will continue to build the molecular profile for Kawasaki disease by studying the urine of children with the condition, before and after treatment. They’ll compare these samples to urine samples from children with symptoms similar to Kawasaki disease, but stemming from a known, separate condition.

    Finding these markers may also help researchers characterize the pathophysiology of Kawasaki disease, which is still unclear to scientists. “If certain proteins are elevated in the urine, we may be able to determine where those proteins originate from, and possibly develop more effective treatment,” says Kim.

    Vascular health in Kawasaki disease


    The study examines the long-term effects of Kawasaki Disease on vascular health by comparing test results of 200 patients who’ve had KD to the test results of 50 age- and gender-matched healthy controls. We use three non-invasive techniques to study vessel reactivity: EndoPAT, carotid ultrasound and Millar tonometry. Each of these techniques provides insight into the health of the patient’s blood vessels, evaluating the stiffness of the vessels in different parts of the body, and the blood vessel reactivity.

    In addition to these tests, we gain information about the patient through questionnaires about exercise habits, eating habits, and personal and family medical history. Finally, we measure cholesterol levels and markers of inflammation in the blood. We hope to determine if there are long-term differences between those who’ve had Kawasaki disease and those who haven’t—and, if yes, what those differences are. We also hope to explore the factors that might lead to long-term effects based on the initial severity of the illness and the current condition of the coronary arteries in each patient.

    In addition to these studies, for which patients with a history of Kawasaki disease are  currently being recruited, Children’s investigators also participate in a number of studies analyzing the database of existing patients with Kawasaki disease (e.g., “retrospective” studies). Boston Children’s is a member of the Pediatric Heart Network (PHN), a group of researchers who’ve combined their efforts to study Kawasaki disease, among other heart conditions in young people.

    Learn more about Boston Children’s cardiac research and cardiology research.

    Boston Children’s heart research and innovations

    A significant amount of Boston Children’s groundbreaking cardiac research aims to refine and advance the open heart surgery and catheterization procedures that treat congenital heart defects in newborns and young children. Children’s Cardiac Surgery Research Laboratory is studying the mechanisms of heart disease and new treatments for children with congenital heart defects. 

  • Duncan's story

    What 4 1/2-year-old Duncan remembers about his eight-day stay at Boston Children's Hospital two years ago is that he ate a gummy worm, watched the movie Cars and met a dog. I remember everything else. And because I have a medical background, I unfortunately understood how sick he was at the time.

    Kawasaki disease causes inflammation of many tissues of the body, including the hands, feet, whites of the eyes, mouth, lips and throat. But the aspect of Kawasaki disease we were most concerned about was its potential effect on Duncan’s heart and blood vessels.

    What will always stand out in my mind is how Duncan's caregivers were able to make a terrifying situation not so terrible. Due to his "do everything the hard way" nature, Duncan became sick enough to end up in the Cardiac Intensive Care Unit (CICU) with an expert in this rare disease caring for him. He initially responded well to treatment, but by the third day, he had gained two pounds of fluid and was getting much worse.

    Dr. Jane Newburger came to see him, along with several students, doctors, and members of the Kawasaki team. While examining Duncan and talking to me, she also took the time to teach, passing around her stethoscope to everyone in the room to listen to “the best medical student gallop you'll ever hear.”

    That night, Duncan was moved to the CICU, had central and arterial lines placed, and was put on dopamine to improve the working of his heart. The many medications and other treatments he had there made a huge difference and I was soon taking him on rides in the go-kart, newly appreciative of how fortunate we both were. Several days and echocardiograms later, we were able to go home.

    Kawasaki disease could easily have left Duncan with serious, life-long heart problems, but two years later, he is as happy and healthy as any other 4-year-old. I look back on our time at Boston Children's, if not exactly fondly, with an immeasurable amount of gratitude to those who cared for Duncan.

    Thank you to those who enabled me to look beyond the rash, the fever, the endless medications and interventions, and made me believe that my baby would be totally fine. I never doubted that he was receiving the best care in the world. 

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