Kawasaki Disease, Essay Example

Etiology

Kawasaki disease was first described by a Japanese pediatrician, Tomisaku Kawasaki, in 1967 (KD Foundation, 2010). KD “is an acute, systemic vasculitis of unknown cause affecting mainly neonates (infants) and young children (Paredes, Mondal, Brandao, and Chank, 2010).”  According to the KD Foundation (2010) an agent such as a virus is the suspected cause. The logic behind this reasoning is because of the nature of nationwide epidemics, seasonality of cases and the self-limited nature of the acute illness (Gordon, Kahn, and Burns, 2009). A search of the current medical literature does not reveal a known cause for KD. “KD can only be diagnosed clinically because there is no specific laboratory test for this condition. Most cases are diagnosed by exclusion of other diseases and when the clinical manifestations are compatible with the known diagnostic criteria for KD (Lin, Chang, Lu, Hu and Ke, 2010).”

Epidemiology

 Each year an estimated 4,200 children are diagnosed with KD (KD Foundation, 2010).  KD affects children of all racial groups though it is “prevalent among children of Asian and Pacific Island descent (KD Foundation, 2010).”  In Japan, the annual cases are more than doubled that of the U.S.  “The most recent national survey in Japan reported an annual increase of 10,041 and 10,434 new cases in 2005 and 2006, respectively (Fukazawa, 2010).” While there is currently there is no known way to prevent KD because its etiology is still not understood, “genetic influences on disease susceptibility and outcome have been identified, and the current paradigm proposes that KD results from exposure to a common agent that triggers the syndrome only in genetically susceptible hosts (Gordon, Kahn, and Burns, 2009).”

Pathophysiology

KD is a condition that mainly affects the heart. Autopsies of individuals who have died late after KD reveal calcified aneurysms, myointimal proliferation and organizing thrombus in the coronary arteries with recanalization (Gordon, Kahn, and Burns, 2009). An autopsy of a child who died 13 months after recovering from KD, from unrelated causes, showed that the “intima of the coronary artery was thickened, the internal elastic lamina was disrupted and smooth muscle cells were observed infiltrating into the intima (Gordon, Kahn, and Burns, 2009).” Other pathologic changes in the heart often caused by KD include fibrosis, degeneration of myocytes, hypertrophy and other myocardial abnormalities, including lymphocyte and plasma-cell infiltration (Gordon, Kahn, and Burns, 2009) .

Abdominal complications can also be present. In 2% to 3% of KD cases, intestinal pseudo-obstruction (IPO) occurs. Studies have revealed the findings characteristic of abdominal radiographs (including small bowel dilatation, an increased volume of gas, and thickening and thumb-printing of the small bowel walls indicate that such intestinal involvement may be due to vascular insufficiency (Lin, Chang, Lu, Hu, and Ke,2010).”

Signs and Symptoms

 An early symptom of KD is abdominal pain, including diarrhea, and these symptoms often can occur before the well-recognized clinical features. In the case reviewed by Lin, Chang, Lu, Hu, and Ke, (2010), an “abdominal plain film revealed multiple dilated intestinal loops (Lin, Chang, Lu, Hu, and Ke, 2010).” Abdominal symptoms are then followed by the recognized clinical symptoms, which include a fever that lasts more than 5 days, a rash in the groin area, red, bloodshot eyes that are not draining or crusting, bright, swollen, cracked lips and a strawberry tongue, which appears with shiny, bright red spots after the top coating sloughs off, swollen hands and feed and redness of the palms and soles of the feet and swollen lymph nodes in the neck (KD Foundation, 2010).” In their report,  Lin, Chang, Lu, Hu, and Ke (2010) warn that “Failure to recognize these symptoms may lead to a delay in treatment and thus increase the risk for the development of cardiac sequelae.”           

Medical Treatment

Treatment includes intravenous immunoglobulin (IVIG) and aspirin. When this treatment is administered before the 10^th day of illness, “the incidence of coronary involvement decreases to approximately 5% (Paredes, Mondal, Brandao and Chan, 2010).” Surgical treatments can include interventional catheter therapy or coronary artery bypass in cases where these types of procedures are necessary (Fukazawa, 2010).

Prognosis

The prognosis for KD depends on how early the disease is identified and when treatment is administered. According to  Gordon, Kahn, and Burns (2009) “up to 25% of untreated children will develop permanent damage to the coronary arteries with inflammatory cell infiltration of the arterial well, destruction of the internal elastic lamina, necrosis of smooth muscle cells, myointimal proliferation and subsequent aneurysm formation.”  With treatment, including IVIG, “it is estimated that up to two-thirds of arterial lesions (small to moderate-sized aneurysms) will regress to normal internal diameter in 1 or 2 years after disease onset, but regressions are less likely to occur after that point (Paredes, Mondal, Brandao and Chan, 2010).”

For KD patients who develop giant aneurysms, Warfarin is recommended in addition to aspirin therapy.  Anticoagulant therapy is also a recommended treatment in at both the acute stage of the disease and in long-term treatment since abnormal blood flow and abnormalities of the coagulation symptom can cause KD patients to be prone to coronary artery thrombosis and subsequent MI (Paredes, Mondal, Brandao and Chan, 2010).”

Summary

Occupational therapists who work with patients with KD will need to evaluate the patient and create an intervention that is specific to the client since some KD patients will have more significant heart damage than others. The process will also include an intervention and outcome monitoring by the occupational therapist on a long term basis (AOTA, 2008). The first step is to determine a probably outcome for the KD patient, which will be deduced from an extensive interview and review of the patients medical history.

Some of the possible after affects that children will suffer after being treated for KD are a loss of energy that can last for up to a month. The child might also have some psychological trauma from the hospital, procedures, IV and the disease itself. The child may also complain of joint pain, a common after effect of IVIG therapy. This pain should go away but the OT should be watchful to ensure that it does not persist longer than a month. It is important to recognize these symptoms and to treat them as they occur. Careful observation of the child is necessary to ensure that the healing process is occurring uninhibited and that trauma to the child is minimized.

Evidence has suggested that those who have KD during childhood may be at long term risk for cardiovascular disease (Fukazawa, 2010). An intervention can be planned to help prevent the possibility of cardiovascular disease in the future. A heart friendly diet can be created for the child and proper exercise routines should be established. By creating heart healthy habits for the child it can help to prevent cardiac complications in the future.

A video created by the “KD Foundation called Kawasaki Disease: A Parents Guide” is linked in the resources and is an excellent overview of the disease, its symptoms and its possible consequences for the child’s future.

References

American Occupational Therapy Association (2008) Occupational Therapy Practice Framework Domain and Process 2^nd Edition USA: AOTA Press

Fukazawa, R (2010) “Long-term Prognosis of Kawasaki Disease: Increased Cardiovascular Risk?” Current Opinion in Pediatircs 22(5), 587-592.

Gordon, John B., Kahn, Andrew M., Burns, Jane C. (2009) “When Children With Kawasaki Disease Grow Up” Journal of the American College of Cardiology 54(21), 1911-1920

Kawasaki Disease Foundation (2010) Web. Retrieved 12 November, 2010 from http://www.kdfoundation.org/

Kawasaki Disease: A Parents Guide KD Foundation Video Retrieved 12 November 2010 from  http://www.youtube.com/watch?v=5knlkzlU2-4&feature=email

Lin, YL, Chang, TJ, Lu, KC, Hu, WL, Ke, TY (2010) “Surgical Treatment of Kawasaki Disease with   Intestinal Pseudo-Obstruction” Indian Journal of Pediatrics Web. Retrieved 12 November 2010 from

Paredes, N., Mondal, T., Brandao, LR, Chank, AK (2010) “Management of Myocardial Infarction in Children with Kawasaki Disease” Blood Coagul Fibrinolysis 21(7), 620-631.