Although it’s more common among children of Asian and Pacific Island descent, it affects people of all racial and ethnic groups.
An estimated 4,200 children in the United States are diagnosed each year, and although the specific cause is not known, a virus is suspected.
As reported by the Kawasaki Disease Foundation, 80% of patients are younger than 5 yrs old, but although it’s also diagnosed in older children and teenagers, it’s less common.
However, It doesn’t usually affect children under the age of 6 months, which may be due to the protection they receive through their mother’s antibodies at a birth. It is not contagious.
A diagnosis of Kawasaki Disease (also called Kawasaki Syndrome) reveals inflammation in the walls of arteries throughout the body, including the coronary arteries, which supply blood to the heart muscle.
- It also affects the lymph nodes, the skin and mucous membranes inside the nose, mouth, and throat.
Inflammation itself is not always a bad thing. It’s part of the body’s immune response.
- It’s the body’s way of protecting itself from harmful substances. Without inflammation, infections, wounds and any tissue damage wouldn’t be able to heal.
One of its most serious and long-term effects, however, is on the heart.
We really just don’t know the cause of Kawasaki Disease, although there are multiple theories.
Symptoms resemble those of a virus or an infection, but no specific viral or bacterial cause has been identified.
The following are considered risk factors for Kawasaki disease:
- Age: It is diagnosed most frequently between the ages of 1-5
- Gender: Boys are more likely than girls to develop it
- Ethnic background: People of Asian ancestry, specifically Japanese or Chinese, and Black Americans are more susceptible to Kawasaki disease
- Genetics: If the parents had Kawasaki disease, their offspring may be more likely to have it, suggesting that it may be linked to an inherited gene
- Environment: In the northern hemisphere, from January through March, the rate is 40 percent higher than in August through October.
Signs and Symptoms
The signs and symptoms develop in three phases.
Phase 1 (known as the Acute Phase):
Symptoms appear from day 1 to 11. They show up suddenly and are usually intense.
- High Fever, which continues for at least 5 days and may reach 104 degrees ^F or 40 degrees C.
The fever does not respond to over-the-counter painkillers, such as ibuprofen or Tylenol.
- Conjunctivitis in both eyes, where the whites of the eyes become red, and the eyes may be itchy, watery, and sore
- A sore throat
- Swollen, chapped, and dry lips
- Swollen lymph glands and lumpiness in the neck
- A rash on the arms, legs, and body, and between the genitals and the anus
- A second rash on the palms of the hands and the soles of the feet, which may be accompanied by peeling skin
Phase 2 (Sub-acute phase)
Symptoms appear from days 12 to 21. They are less severe, but they may last longer. The body temperature should return to normal.
Symptoms may include:
- Peeling of the skin on toes and fingers
- Abdominal pain
- Joint pain
- Joint swelling
- Lack of appetite
Complications are more likely to occur during this phase and the child may experience more pain and be moody.
Phase 3 – Convalescence (Recovery)
This phase lasts from about day 22 to day 60.
- Symptoms improve, and the patient gradually recovers until all signs of the disease are gone.
The main concern is that Kawasaki can affect the vessels around the heart so the patient will need an echocardiogram for a thorough evaluation.
Diagnosis and treatment
To be diagnosed as having Kawasaki disease, there must be a fever for 5 or more days as well as four out of five main other findings:
- Changes in the lips or mouth
- Enlarged lymph nodes in the neck
- Rash on the body
- Changes on the hands or soles of feet
Currently, there is no one specific test to confirm Kawasaki disease.
Since the symptoms are similar to other childhood diseases, including the measles, scarlet fever, and juvenile arthritis, some tests may be ordered to eliminate those diseases.
A physician will consider the patient’s symptoms and perform a physical examination.
Blood tests and other tests can be ordered to evaluate:
- An elevated white blood cell count (found with an infection)
- Inflammation of the joints
- A high **sedimentation rate
**A sedimentation or “sed rate” blood test can reveal inflammatory activity in the body. While it’s not a stand-alone diagnostic tool, it can help the doctor diagnose or monitor the progress of an inflammatory disease.
- Mild anemia
- The presence of protein or white blood cells in the urine (which indicate infection)
An echocardiogram can check for damage to the heart and coronary arteries.
Other tests may include:
- Urine test: This can help indicate whether something else may be causing symptoms.
- Platelet count: Platelets are cells in the blood that clump together to help stop bleeding, and in Kawasaki disease, they are usually high.
- Erythrocyte Sedimentation Rate (ESR) test: A sample of red blood cells is placed into a test tube of liquid. If the time taken for the red blood cells to fall to the bottom is fast, this can indicate an inflammatory condition, such as Kawasaki.
- C-reactive protein (CRP) test: A high level of C-reactive protein in the blood, produced by the liver, can indicate an inflammation.
- Sodium test: Low sodium may be present.
- Albumin test: There may be low levels of albumin, a protein, in the blood.
Kawasaki disease can affect the heart.
The following tests may be ordered:
- Electrocardiogram (ECG/EKG): This device records electrical activity and heart rhythms.
- Echocardiogram: An ultrasound scan that checks the pumping action of the heart. Sound waves create a video image of the patient’s heart, and this helps the doctor see how well it is pumping.
Kawasaki disease is usually treated in the hospital, due to the risk of complications.
Some medications are used in treatment:
- Aspirin: Kawasaki disease leads to a very high blood platelet count, and a higher risk of clots forming in the bloodstream.
The Aspirin helps prevent blood clots and reduces the fever, rash, and joint inflammation. A high dose will normally be necessary.
The patient will be monitored for undesirable side effects. Aspirin therapy may continue for several weeks after recovery from symptoms.
- Intravenous immunoglobulin (IVIG): This decreases the risk of coronary **aneurysms.
Although heart problems are rare, it is crucial to monitor the heart.
If there are any indications of heart problems, the doctor may order follow-up tests, usually 6 to 8 weeks after the symptoms started.
- If the heart problems persist, the doctor may refer the patient to a Pediatric Cardiologist, a doctor specialized in diagnosing and treating childhood heart problems. And this is crucial for the child’s continuing health.
The following may be necessary:
- Anticoagulant medications, such as warfarin (Coumadin), heparin, or aspirin, to prevent blood clots
- Coronary artery angioplasty, a procedure which opens up an artery that has narrowed by inflating a small balloon inside the artery
➡ Alongside the angioplasty, a stent may be placed in a clogged artery to help prop it open, reducing the risk of it blocking again
If an aneurysm develops, the blood vessels leading to the heart become inflamed, causing a section of the artery wall to weaken and bulge outwards.
If an aneurysm does not heal itself, a blood clot can form, which raises the risk of a heart attack or internal bleeding if the aneurysm bursts.
Prompt treatment reduces the risk of complications, and most patients do not experience further problems.
Although heart problems are rare, The American Academy of Pediatrics notes that Kawasaki disease is the leading cause of acquired heart disease in infants and young children in the U.S. Consistent monitoring will be necessary.
Left untreated, Kawasaki disease can cause serious complications, in addition to an aneurysm.
Other complications include:
- Heart muscles and heart valves malfunction
- Myocarditis, an inflammation of the heart muscle (the myocardium)
- Pericarditis, an inflammation of the lining around the heart (the pericardium)
- Heart failure or heart attack
Although patients normally recover within a few weeks (even if there are heart or other complications), Kawasaki disease can affect other body systems.
Regular follow-up visits will be necessary, as well as an echocardiogram every one or two years to screen for heart problems.
Kawasaki Syndrome may not always be preventable, but it is treatable. Prompt action will spare our children from years of heart-related complications.
Stay informed, keep your loved ones safe. And, as always, please feel free to contact me by using the “Get In Touch” link on the top of any page, if you have any questions or need clarification on any points. You and your family’s health is important to me.