Kawasaki Disease: Early Detection Could Save your Child's Life

Kawasaki Strawberry Tongue 
The leading cause of childhood acquired heart disease in North America is KAWASAKI DISEASE. Know the symptoms of this common childhood illness to detect KD early and prevent the chance of heart attack. 

What is Kawasaki Disease?

Kawasaki Disease (KD) is an autoimmune disease that causes inflammation in the walls of the arteries throughout the body, known as vasculitis. Most importantly, it can affect the medium-sized coronary arteries that supply oxygenated blood to the heart. KD is also referred to as 'Mucocutaneous Lymph Node Syndrome' as it also affects the lymphatic system including lymph nodes, skin, mucous membranes in the mouth, nose and throat (MayoClinic.org).

It is most common in infants and young children under the age of five, usually of Asian decent. KD is considered the leading cause of childhood acquired heart disease in developed countries. Although the cause is unknown and doctors believe it is not contagious, it is hypothesized that a virus or other microbial agent may be the root cause (kdfoundation.org).

The acute stage involves hyperactivity of the immune system. In response to an unknown trigger, immunoregulatory abnormalities can be found as well as an increase in cytokine secretions which target the endothelial cells of the arteries, which produce antigens. Antibodies are produced in reaction to these antigens which target the vascular endothelium resulting in various events that lead to vascular damage (Taulbert, Shulman, 1999).

 

Know the Signs and Symptoms:

KD can be diagnosed based on these principle clinical findings, the main symptom being a long-term fever:

  • Fever that lasts for five days or more (peak 102-104˚F or higher)
  • Red bloodshot eyes (conjunctivitis) , without drainage or crusting
  • Rash, often worse in the groin area
  • Bright red, swollen, cracked lips, "strawberry" tongue, which appears with shiny bright red spots
  • Swollen hands and feet and redness of the palms and soles of the feet
  • Swollen lymph nodes in the neck

Organs Affected

Kawasaki is classified by vasculitis, making the cardiovascular system the main target. The heart is the main organ affected as myocarditis occurs in the acute phase of 60% of patients and coronary aneurysms pose the greatest risk. The second major system affected is the lymphatic system, causing swelling in the mucous membranes and lymph nodes. The immune system is affected as one form of diagnosis is marked by increased number of white blood cells. The gastrointestinal tract is affected as diarrhea and abdominal pain are common symptoms. The liver is affected as 40% patients experience mild hepatic dysfunction. The neurological system is affected as 90% cases show extreme irritability (hearing loss, cranial nerve VII palsy and seizures pose a rare risk). Urethritis and enlarged kidneys may be seen. Inflammation of the joints can lead to arthritis in the subacute phase and inflammation of the lungs may also occur (Lang, 2001).

Disease Progression

Acute Phase: Approximately 7-14 days. Mainly the mucocutaneous changes and the lymph system are most evident. Edema and inflammation of hands and feet develop at the end of this stage.

Sub Acute Phase: Week 4-6. Fever has abated and peeling of the skin on hands and feet begin. Coronary aneurysms develop at this stage. Arthritic symptoms of joints and lasting irritability occur.

Long-Term Complications

Coronary artery aneurysms, calcification and stenosis. Atherosclerosis, stroke (due to scaring and thickening of the arteries), aorta root dilation, scarring of the heart and dysrhthmia (Gordon, Kahn, Burns 2009).

2. Causative Factors

a) Contributory Causes: Diet

In the functional medicine community, a leaky gut has been associated with the exasperation of autoimmune disease symptoms. The lining of the small intestines has a selective permeability which is integral in moving pathogens out through excretion and releasing nutrients into the bloodstream. If the lining becomes inflamed and damaged by harmful bacteria or irritating food substances such as gluten, it becomes more permeable. 'Holes' in the gut lining will prematurely leak large food molecules into the bloodstream, causing the immune system to react and attack them as a foreign invaders. If the immune system is up-regulated due to constant attacks, it may begin to identity and attack self tissue as foreign as well (autoimmunity). This is the basis behind the leaky gut and autoimmunity connection. Dr. Terry Wahls has a theory that autoimmune disease can be cured by a very strict diet which is focused on the increased production and regeneration of mitochondria through nutrient dense foods. Her diet removes common allergens such as grains, legumes and dairy to reduce inflammation. It is very high in anti-inflammatory fats from nuts and seeds, fish and liver. It is considered a version of the paleo diet, as nutrient dense organ meats play a big role in providing certain nutrients which regenerate the mitochondria. Lastly, an immensely large amount of anti-oxidant rich fruits and vegetables are consumed to further reduce damage on cells. (Wahls, 2014)

b) Environmental

A recent theory is held that there may be a strong environmental contributing factor to the development of this disease in susceptible patients. 'The Windborne Theory' was initiated by the discovery of a consistent pattern between seasonal shifts in winds coming from Central Asia and fluctuations in the numbers of KD cases in Japan, Hawaii and San Diego. The wind is thought to carry certain fungus and bacteria which can sustain the temperature and travel time across the ocean, although the specific infectious agent is yet to be identified.

c) Infectious Cause:

Because cases of the disorder tend to cluster geographically and by season, researchers have suggested that an infection may be involved. However, no infectious agent (such as a virus or bacteria) has been identified.

d) Predisposing Cause:

Genetic:

KD was originally discovered in Japan by Dr. Tomisaku Kawasaki, and today the Japanese see ten times more yearly diagnoses than the US, with 10,000 new cases per year in Japan (Gordon, Kahn, Burns 2009). A recent study analyzing 2,544 Japanese KD patients found that there is a susceptibility gene called ITPKC which has a significantly higher rate in those of Japanese decent, that can be attributed to pathogensis of this disorder (Onouchi, Fukazawa, Yamamura 2016). The ITPKC gene provides instructions for the production of the enzyme inositol 1,4,5-trisphosphate 3-kinase C. This enzyme limits the activity of T cells in the immune system. (T cells are attributed to identifying foreign substances which defends the body against infection). Inositol is important because it reduces overproduction of T cells which can lead to excess cytokines (immune proteins) which lead to excess inflammation and subsequent tissue damage. If there is a variant or defect in this enzyme inositol as seen in a variation of the ITPKC gene, there may be an interference with the body's ability to reduce T cell activity, which can lead to inflammation and damage of blood vessels and result in the symptoms of Kawasaki. This is one of many suspected gene variations and causes of KD (US National Library of Medicine).

Hereditary:

There is hereditary link in the prevalence of KD. This familial predisposition can be passed on through family members, and it is considered that children of parents who have had KD are twice as likely at risk of developing the disease. Those with a sibling affected with KD are ten times as likely in developing the disease. (US National Library of Medicine).