Tag Archives: IVIG

My son’s brush with Kawasaki Syndrome

Five key symptoms aside from high persistent fever
(Photos taken from various sources on the internet)

SOME rare diseases that hit very young children come with ordinary signs and symptoms which when ignored could cause debilitating lifetime effects.

A few days before my five-year-old son, Khalil, was confined and diagnosed with a rare Kawasaki Syndrome in 2004, he developed a rash on his hands and feet.
I would have dismissed the signs as minor allergy. But the occurrence of abdominal pains, reddish eyes, dark red lips, and strawberry spots on Khalil’s tongue that accompany his swollen fingers made me suspect that he could be suffering from a condition worse than an allergy.

My son was full of energy despite the discomforts brought by these unusual symptoms.

I was nervous when I brought him to the pediatrician’s clinic anticipating the least serious diagnosis from the doctor.

When Dr. Annaliza Duran, a consultant at the FEU Hospital in Quezon City, saw my child she told me right away that something is wrong with the kid as she is familiar with his medical record since he was a baby.

“I don’t like his skin color (referring to Khalil’s pale yellowish skin at the time). I know when he is okay and when he is not,” Dr. Duran told me.

The then 39-year-old lady doctor initially said the symptoms are similar to disease-causing Streptococcus bacteria which are responsible for infections such as strep throat, scarlet fever, and some types of pneumonia.

Khalil’s grayish nailbed also indicate the poor entry of air in his lungs.
She recommended for my son’s confinement to find out what really ails him.

An X-ray made on Khalil at the hospital confirms her earlier diagnosis of pneumonia.

I thought that he would be sent home in a few days. But my worst fear happened on our third day at the hospital.

I received a phone call from Dr. Duran and slowly disclosed to me that aside from pneumonia, Khalil is also suffering from a rare Kawasaki disease.

I was stunned and could not control my tears when I heard what the doctor just said. I initially thought the disease was fatal and feel sorry for my son. I may have heard or read something about Kawasaki disease in the past, but never imagine one of my kids would develop it one day.

Even when doctors gave assurance that Kawasaki is not infectious, I still wonder how my child acquired such an ailment.

Doctors said there is no known cause of Kawasaki, but it could be associated to streptococcus bacteria.

Streptococcus bacteria are normally found in the human throat, respiratory tract, blood stream, and wounds. It is often airborne in hospitals, schools and other public places.

Dr. Duran and two other doctors, a consultant cardiologist and infectious disease expert also of FEU Hospital corroborated the impression of Kawasaki Disease based on clinical manifestations and laboratory analysis including CBC (complete blood count) and AFC (actual platelet count).

They said that most characteristics of Kawasaki were present on my child including the inflammation or swelling of hands, feet, whites of the eyes, mouth, lips and throat, fever, and swelling of the lymph nodes in the neck.

Khalil’s blood samples showed an increase in the number of white blood cells which normally multiply in the presence of an infection and elevated platelet levels.

He also had an elevated ESR or erythrocyte sedimentation rate. ESR is a measurement of how quickly red blood cells fall to the bottom of a test tube.
When swelling and inflammation are present, the blood’s proteins clump together and become heavier than normal. Thus, when measured, they fall and settle faster at the bottom of the test tube.

Doctors immediately prescribed an aspirin on my child to lessen the inflammation of many tissues that Kawasaki produces.

While the symptoms of Kawasaki run its course and resolve within a few weeks, it becomes a concern because the illness primarily affects the coronary or heart arteries which provide the heart muscle with an oxygen-rich blood supply.

The three doctors attending to my son recommended the immediate transfusion of Intravenous Immunoglobulin (IVIG) within 10 days to decrease the risk of heart and blood vessel complications.

Doctors said 10 to 15 percent of children afflicted with untreated Kawasaki developed heart problem.

“Medication is quite expensive, but we need to immediately transfuse IVIG the soonest possible time to avoid the setting in of possible complications particularly coronary heart disease,” Dr. Duran said.

Khalil needs to be transfused with 25 vials of 2.5 grams of IVIG. A vial costs a whopping P4,000 to P5,000.

On the ninth day (of the ailment), the medicine was transfused on my son for 21 uninterrupted hours.

My family and the doctors were praying that no side effects and rejection would occur during the course of transfusion.

Among the noted side effects of IVIG are fever, chill, and rashes.
We were thankful that Khali1 responded well to the medicine.

After ten long days, my son was finally given a clean bill of health by his doctors and was discharged of the hospital.

However, he needs to be regularly examined particularly by cardiologists to monitor if any heart problem has occurred after the illness.

I realized that no matter how well you keep your child away from dirt there is no guarantee that he is protected from acquiring certain types of diseases like the rare Kawasaki disease.

But with a timely consultation with your doctor, complications of specific ailments (that show ordinary signs and symptoms) could be prevented later on.


What is Kawasaki Syndrome?

Kawasaki syndrome (KS) was first recognized by Japanese physician Dr. Tomisaku Kawasaki in 1961.

It is a disease in young children with an unknown cause, giving rise to a rash, glandular swelling, and sometimes damage to the heart.

KS has replaced acute rheumatic fever as the leading cause of acquired heart disease in children in the United States (Asian or Asian American heritage) and Japan.

About 1,800 new cases are diagnosed in the US each year and the evidence is on the rise.

It is the most common form of vasculitis or inflammation of the blood vessel(s).
The heart, particularly the coronary arteries, is affected in as many as 20 percent of children with Kawasaki.

The disease can weaken the wall of one or more of the coronary arteries, causing it to bulge or balloon out. This weakened, ballooned area is called an aneurysm.

Blood clots can form in the ballooned area and possibly block the blood flow through the coronary artery. When this happens, the heart muscle will no longer receive an adequate supply of red blood, and the heart muscle can damaged.

The illness may cause the heart muscle (myocardium) to be irritated and inflamed, as well as the membrane covering the heart (pericardium). Irregular heart rhythms and heart valve problems may also occur with Kawasaki disease.

In most cases, the effects on the heart caused by KS are temporary, and heal within five or six weeks. However, coronary artery problems may sometimes persist for longer periods of time.

Early treatment of KS with aspirin and high-dose IVIG has proved to be effective in reducing clinical symptoms, as well as reducing the prevalence of coronary aneurysm.

The single-dose regimen for IVIG is now the standard recommended initial mode of therapy.

The disease was more prevalent in male children between 1 to 5 years old.
The most common symptoms of KS may include: moderate to high fever that rises and falls for up to three weeks; irritability; swollen lymph glands in the neck; spotty, bright red rash on the back, chest, abdomen, and/or groin; bloodshot eyes; sensitivity to light; swollen coated tongue; dry, red, cracked lips; dark red interior surfaces of the mouth; red swollen palms of hands and soles of feet; peeling skin around the nailbeds, hands, or feet; and swollen painful joints.

About 80 percent of all children have a full recovery after KS resolves.
Some heart problems may not be evident right away, so it is important to keep follow-up appointments with your child’s physician, even if your child is feeling well.

Meanwhile, the Department of Health said the country has no significant data of Kawasaki disease at present.

“Kawasaki is not infectious. It has unknown etiology (cause) and a not-reportable disease. But we have sporadic cases here in the Philippines,” said DOH medical officer Dr. Luningning Villa (she now handles the ASEAN Plus Three Emerging Infectious Diseases Programme).

My article came out in People’s Tonight in Nov. 2004