Frequently Asked Questions
What is Kawasaki Disease?
Kawasaki Disease is also called Kawasaki Syndrome or mucocutaneous lymph node syndrome. The illness was first described in Japan by Dr. Kawasaki in 1967.
It is an illness mainly affecting children under five. It is characterized by inflammation of the blood vessels (‘vasculitis’) and in particular damages the coronary arteries that supply blood to the heart.
Kawasaki Disease is a very little known condition in the UK, but it has extremely dangerous effects on children. Read some of the most frequently asked questions about it here.
About 1/3 of untreated children with KD suffer heart damage, making it the most common cause of heart disease acquired in childhood in many countries, including the UK.
Kawasaki Disease is the leading cause of acquired heart disease in children.
What are the causes of KD?
The causes of Kawasaki Disease are not yet known, but recent research showed a possible relationship between genetic factors and an infection (virus or bacteria) that could trigger the KD. Because of hereditary tendency, KD occurs more frequently in both Japanese and Korean children and among siblings. For the same reason, the risk of developing KD is high in the children of KD patients.
Who gets KD?
Children under the age of 11. 80% of patients are under the age of 5.
Patients usually begin with a high fever lasting at least five days. Symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart. The most important aspect of the disease is how it effects the heart. The disease can cause inflammation of blood vessels in the coronary arteries, which can lead to aneurysms.
If your child has the following symptoms for more than 4 days ask the doctor for a heart scan by a Pediatrician.
A High Fever
Red Bloodshot eyes
An Angry Rash
Red Cracked lips and strawberry tongue
Swollen lymph nodes in the neck
Peeling feet and hands
How is Kawasaki Disease diagnosed?
Diagnosis is usually based on an evaluation of classic symptoms. Possible diagnostic tests include blood studies (complete blood count and blood sedimentation rate), electrocardiogram, echocardiogram, chest x-ray and urinalysis.
After observing the presence of the typical symptoms of KD, is necessary to make other tests in order to make a correct diagnosis of KD.
The aim of these tests is ruling out other conditions that could be causing their symptoms. Possible conditions your child could have include:
Measles: a highly infectious viral illness that causes a fever, rash, and conjunctivitis.
Scarlet fever: a bacterial infection characterized by a pink rash. Treatment with penicillin for 24 hours generally clarifies the diagnosis; if the patient fails to improve on this therapy, KD should once again be considered.
Toxic shock syndrome: a bacterial infection that can share some clinical features with severe KD.
Drug reaction: a very severe allergic reaction to medication can sometimes be confused with KD because it causes a rash.
Viral illness: such as virus infection, can share clinical features of KD. Children with adenovirus infection usually present with exudative pharyngitis and conjunctivitis.
Viral Meningitis: an infection of the protective membranes that surround the brain and spinal cord
Lupus: an autoimmune condition that can cause a range of symptoms, including fatigue, joint pain, and a rash.
To confirm the diagnosis of KD, several tests need to be done:
Urine sample: used to check white blood cells in the urine
Blood tests: used to analyze white blood cell and platelet count and other signs of inflammation.
Echocardiography: to check every possible damage to the blood vessel.
What is the prognosis?
With early recognition and treatment, full recovery can be expected. However, 2% of patients die from complications of coronary blood vessel inflammation. Patients who have had Kawasaki Disease should have an echocardiogram every 1-2 years to screen for heart problems.
What is the typical treatment for Kawasaki Disease?
The most important goal to achieve is treating your child as soon as possible, ideally when they have still a fever. They will need to stay in the hospital during the treatment period.
The “gold standard” treatment consists of an infusion of gamma globulin (lVlG) and high doses of Aspirin.
The IVIG is a solution of antibodies taken from healthy donors that are injected directly into a vein. Its function is reducing the fever and the risk of heart problems. After your child is given IVIG, their symptoms should improve within 36 hours. If their fever doesn't improve after 36 hours, they may be given a second dose of IVIG
The Aspirin is used to reduce fever (high temperature) and ease pain and discomfort. It has an anti-inflammatory effect at high-doses and it prevents blood clots forming (antiplatelet) at low doses.
The dose of aspirin your child is prescribed and how long they need to take it for depends on their symptoms. It is likely that your child will take high-dose aspirin until their fever subsides and could be prescribed low-dose aspirin until 6 to 8 weeks after the start of their symptoms.
The reason is reducing blood clots if there are problems developing in the blood vessels.
Some times the use of Corticosteroids could be recommended when IVIG hasn't been effective or if your child might have a high risk of heart problems. The Corticosteroids are a type of medication that contain hormones.
What happens if the Kawasaki Disease is not treated?
Without treatment, about 25% of children develop heart complications and the symptoms may be particularly uncomfortable.
IVIG within 10 days is necessary to reduce the risk of coronary artery damage. Without IVIG treatment, your child may trigger serious heart complications like a coronary aneurysm in which coronary arteries could become weak and lead to an enlargement of the blood vessel wall.
According to the research, the greatest risk of heart complications is for children under 1-year-old.
What is the typical recovery after the treatment?
Usually, the children go home after a few days and they will be prescribed a lower dose of Aspirin (ASA) to take at home for 6 weeks or longer if they have developed a coronary artery aneurysm. Its function is protecting the heart of your child by preventing clotting before the next echocardiogram.
Thanks to the treatment, your child should feel better in a few days after the first IVIG. However, he/she may continue to be tired, to experience joint pain and have a poor appetite. He/she needs a few weeks to recover their energy.
When your child is discharged from hospital, you should be given advice about how to care for them at home.
It’s important to check your child and call your doctor as soon as possible if signs of aspirin toxicity, return of fever or KD symptoms occur.
The use of Aspirin could lead to Reye’s syndrome, a really serious illness that could attack the blood, brain and the liver of your child, so it is important to carefully monitor them. If your child shows symptoms of the flu, you should stop the aspirin and immediately contact your child's heart doctor.
What is the follow-up procedure for the children with KD after hospital treatment?
Your child will be given a follow-up appointment and their heart will continue to be monitored.
In normal cases, it is necessary for an ultrasound scan of the heart (echocardiogram) after the diagnosis of KD and another one 4-8 weeks later. If your child developed coronary artery problems, he/she probably needs it and other heart tests more often than usual.
If your child develops any complications, follow-up treatment might be necessary and in case of an aneurysm, he/she may require: medications like anticoagulant drugs to prevent clots, surgery like Coronary artery angioplasty, stent placement and Coronary artery bypass graft.
What complications could be related to Kawasaki Disease?
Your child may develop heart complications after Kawasaki Disease such as inflammation of blood vessels or/and heart muscle, problems related to the heart valve and abnormal heart rhythm.
In particular, inflammation of the coronary arteries can lead to aneurysms that may trigger heart attack by resulting in blood clots. These children need long-term heart check-ups and less frequent assessments for the other parts of the body. In fact, KD might not only lead to heart problems but also other complications in terms of hydrops of the gall bladder and meningitis (inflammation of brain membranes).
However, your child can still lead a normal life and continue with daily tasks, which includes exercise. Some children with aneurysms might have some activity restrictions depending on the size of their aneurysm and on the medicines that he/she takes.
Can your child contract KD more than once?
It happens rarely in less of 2% of the cases. But if the symptoms reoccur, your child will need to see by a doctor.
Is it possible to prevent KD?
Today, the prevention of KD is impossible, but new research in terms of diagnosis and treatment is carried out every day.
Are there any vaccination restrictions after having had KD?
If children with KD plan to have vaccination, there are a few special requirements that are recommended.
For the measles-mumps-rebella (MMR) and measles-mumps-rebella-varicella (MMRV) or varicella vaccination (chicken pox), your child needs to wait at least 11 months after the treatment IVIG because the effectiveness of the vaccine can be lost if done previously. This is not the case with the flu vaccine.
Is Kawasaki Disease contagious?
There is no risk of contracting the disease from another patient, so KD is not contagious.
It is possible to get Kawasaki Disease in adult life?
Is really rare for an adult get KD. However, without a diagnosis, a child might have heart complications during adult life.
Given the current health concerns around the Covid-19, will it effect my child?
People with specific underlying health conditions such as Kawasaki Disease, the government urges them to shield themselves and stay at home as Dr Paul Johnstone, Director at Public Health England, said:
“The NHS are contacting the people who are most vulnerable to developing a very serious illness as a result of COVID-19 with specific advice to stay at home for at least 12 weeks”.
Can taking aspirin be part of the Kawasaki Disease treatment ?
Your child may prescribe aspirin if they have Kawasaki Disease. This is one of the few occasions where aspirin may be recommended for a child under 16 years old.
It’s used to treat Kawasaki Disease because:
It can ease pain and discomfort
It can help reduce a high temperature(fever)
A high dose, aspirin is an anti-inflammatory( it reduces swelling)
a low doses, aspirin is an antiplatelet( it prevents blood clots forming)
How many phases does the Kawasaki disease occurs?
The symptoms of Kawasaki disease usually develop in 3 phases over a 6-week period.
Phase 1: acute (weeks 1 to 2) High Temperature is usually of 38C or above, during this phase your child may develop rash , their fingers or toes may become red, hard or swollen, another symptoms may be their lips may be reds, dry or clacked up, this follows with their tongue may be red, swollen and covered in small lumps. Lastly your child may develop swollen lymph glands, this occurs in your child’s neck which may feel swollen lumps usually on one side.
Phase 2: sub-acute (weeks 2 to 4 ) During the sub-acute phase, your child’s symptoms will become less severe, but may last a while. The fever should subside.
Phase 3: Convalescent ( weeks 4 to 6 ) During this last phase you will notice a good improvement and all of signs of the illness should eventually disappear.
What happens once you get discharged from the hospital?
What happens once you get discharged from the hospital?
Once your child is discharged from hospital, you should be expecting some advice about how to care for them at home. Usually this means drinking plenty of fluids
Taking any medications that’s been prescribed for them with a follow up appointment mainly to monitor their heart rate.
Once everything has been tested and are no complications your child will be advice to stop taking aspirin.
Full recovery could take around 6 weeks,but may take longer in some situations.
Mary Glode, MD:
What is the Calcium score?
Calcium score is a screen test for aneurysm. A normal calcium score is zero.
Is it important to educate children about KD?
It is important to educate children about KD, as children with aneurysms need long term follow up, some of them even when adults. It is important to avoid children reaching adulthood and unknowingly having aneurysms.
Is there any association between KD and routine Vaccines?
Not for any existing vaccines. The COVID vaccine is currently only available for adults.
Why do some children get KD and others don’t (e.g. in a case of siblings or twins)?
Siblings are not genetically identical. Some will be genetically susceptible, others won’t.
How effective is steroid therapy for treating aneurysms?
They are used as a rescue therapy but not as a primary treatment. Current European trials are comparing immunoglobulin and immunoglobulin with steroids as a primary treatment
Could children with a history of KD be more susceptible to COVID?
It is currently unknown, but so far there is nothing to suggest that children with KD are more at risk of COVID, or that COVID can reactivate KD.
Where Does The Donated Money Go?
Currently, Kawasaki Disease Uk is working on using donated money to build a solid infrastructure within the charity by hiring long-awaited specialist team employees.
In doing so we hope to aid and expand our existing work of collating and sharing of important research files, as well as advocating for Kawasaki Disease by directly working with and distributing information leaflets and posters to local GPs , A&Es, and Primary schools in the local area in support of prompt diagnosis.
Dee Izmail ( the founder of Kawasaki Disease UK) plans to develop strategies to persuade the government to test all children under the age of 5 for Kawasaki Disease traits.
Kawasaki Disease UK hopes that with a solid internal infrastructure, the charity would be able to assist families globally who are unable to pay for life-saving immunoglobulin treatment.