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Physical and Psychosocial Health in Children Who Have Had Kawasaki Disease

^* From the Department of Cardiology, Children’s Hospital, Boston, Massachusetts; and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts

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	ABSTRACT

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Objective. The purpose of this study was to examine the physical and psychosocial well-being of children who have had Kawasaki disease (KD), including the influence of coronary artery status on health and health perceptions.

Methods. The Child Health Questionnaire (CHQ) measures overall physical and psychosocial well-being in children 5 to 18 years. To study the long-term impact of KD on overall health status, we mailed the CHQ to patients without a history of coronary artery abnormalities (normal group), with regressed aneurysms (regressed group), with current coronary aneurysms <8 mm (mild-moderate aneurysm group), and with giant aneurysms 8 mm (giant group).

Results. Of 201 questionnaires mailed, 174 were delivered and 110 (63%) were completed. Median age (range) at completion was 10.5 years (5.1–17.9 years) and at illness onset was 3.1 years (0.2–12 years). There were no significant differences in psychosocial summary scores in any of the Kawasaki groups when compared with the US population sample. Physical summary scores were also similar to the US population sample in the normal coronary, mild-moderate aneurysm, and regressed aneurysm groups. However, the giant aneurysm group had significantly lower physical summary scores compared with the US population sample. Among subscales, general health perceptions in the KD groups were lower than in the US population sample, reaching statistical significance in all but the mild to moderate aneurysm group. In addition, parents whose children have had KD reported a higher proportion of anxiety issues, allergies, and orthopedic/bone/joint issues in their children than did the general US population sample. We did not find any difference in the incidence of attentional, behavioral, or learning issues when compared with the US population sample.

Conclusions. KD patients without coronary artery aneurysms were similar to the general population in their general physical and psychosocial health. However, the parents of children in all KD groups reported lower general health perceptions than parents in the US population sample, suggesting that long-term concerns about their children’s health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores.

Key Words: Kawasaki disease • Child Health Questionnaire • functional health status • quality of life

Abbreviations: KD, Kawasaki disease • CHQ, Child Health Questionnaire • JRA, juvenile rheumatoid arthritis • ADHD, attention-deficit/hyperactivity disorder

	INTRODUCTION

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Kawasaki disease (KD) is an acute, systemic vasculitis of unknown cause. First described in Japan in 1967 by Dr Tomasiko Kawasaki,¹ this syndrome has become the leading cause of acquired heart disease in children in the United States. Without treatment, 20% to 25% of patients develop dilation or aneurysms of their coronary arteries.² Treatment with intravenous globulin within the first 10 days of illness significantly decreases the incidence of coronary aneurysms to <5%.³ Because it is a relatively newly described illness, the long-term effects of KD are still being delineated. Studies on the natural history of KD have focused primarily on the cardiac complications resulting from this illness. However, the long-term impact of KD on a child’s general health status has not been well documented.

The purpose of this study was to examine the physical and psychosocial well-being of children who have had KD, including the influence of coronary artery status on health and health perceptions. For this purpose, we selected a comprehensive tool, the Child Health Questionnaire (CHQ), which is designed to measure both physical and psychosocial functioning in children. The CHQ provides a profile of 12 individual health concepts, as well as overall summary measures of physical and psychosocial functioning and well-being. It is a parent-completed tool that has been validated in multiple settings for children between 5 and 18 years of age.⁴

	METHODS

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Subjects
The Department of Cardiology at Children’s Hospital Boston maintains a prospective database for all patients who receive a diagnosis of KD at this institution. Patients were eligible for this study when they 1) had received a diagnosis of KD between 1984 and 1998, 2) were evaluated by 2-dimensional echocardiography at our institution on at least 1 occasion, and 3) were between the ages of 5 and 18 years at the time of the study. Institutional review board approval was given, and informed consent was obtained from subjects or their parents according to the guidelines of the Institutional Committee on Clinical Investigation.

Method of Patient Classification
Each patient was assigned to 1 of 4 groups according to coronary status on all available echocardiograms. We classified as abnormal those coronary segments either with dimensions at least 3 standard deviations above the mean for body surface area (ie, coronary artery z score 3) or that fulfilled the Japanese Ministry of Health Criteria for coronary artery aneurysms.⁵ The 4 groups were 1) normal, those whose coronary measurements were normal on all echocardiograms; 2) regressed aneurysms, those with previous coronary dilation whose most recent coronary artery dimensions all were within normal limits; 3) mild to moderate aneurysms, those with 1 or more dilated coronary arteries at the most recent echocardiogram, none of which ever exceeded a dimension of 8 mm; and 4) giant aneurysms, those with at least 1 aneurysm that had ever measured 8 mm.

CHQ
The CHQ PF-50 is a 50-item, parent-completed questionnaire designed to measure the physical and psychosocial well-being of children. The CHQ was constructed for use in assessment of children with and without chronic conditions. Normative data have been obtained as part of a large national study and consist of parents of 391 noninstitutionalized children between the ages of 5 and 18 years. Parent ethnicity, gender, education, and work status have been collected and are available for comparison. In addition to the US population sample, the CHQ has been benchmarked for children with asthma, juvenile rheumatoid arthritis (JRA), attention-deficit/hyperactivity disorder (ADHD), and epilepsy. Two overall summary scores are generated for each patient: a physical summary score and a psychosocial summary score. Higher scores indicate better physical and psychosocial functioning. The CHQ PF-50 measures components of physical and psychosocial functioning in 12 categories (subscales). These subscales contribute to the 2 overall summary scores. The subscales that contribute most heavily to the physical summary score are physical functioning, role/social limitations (physical), bodily pain/discomfort, and general health. The subscales that contribute most heavily to the psychosocial summary score are mental health, role/social limitations (emotional and behavioral), behavior, and self-esteem. Parent impact/emotional, parental impact/time, family activities, and family cohesion contribute to both summary scores. In addition, parents are asked to indicate on a checklist whether their child has ever been told that he or she has specified chronic medical conditions or diagnoses.⁴

Data Collection
We performed a cross-sectional survey of patients with KD using the CHQ PF-50 instrument. We mailed the questionnaire to the parents of all children in the regressed aneurysm, mild to moderate aneurysm, and giant aneurysm groups and to a stratified random sample of 150 patients from the normal group. Stratification was based on gender, age at illness, and length of time since the onset of KD. A sample size of 150 was chosen to provide 80% power to detect a 5-point difference in mean scores between our group and the normative sample for the CHQ subscale with the largest standard deviation among scores. When the questionnaire was not returned within 30 days, a second questionnaire was mailed. When questionnaires were undeliverable, we attempted to verify the patients’ addresses and the questionnaires were mailed again.

Statistical Analysis
Mean physical summary scores, psychosocial summary scores, and individual subscale scores for each of the 4 coronary status groups were compared with the corresponding means of the US population sample using the 2-sample t test; no adjustment was made for multiple comparisons. Differences in subscale scores among the 4 groups were evaluated using the Kruskal-Wallis test. Linear regression analysis was used to explore the relationship between summary scores and coronary status group and other patient characteristics, including comorbid conditions. A 5-point difference in means was considered clinically meaningful.⁴ The proportions of patients who reported various chronic medical conditions were compared with the US population sample using the Fisher exact test. Mean summary and subscale scores for patients in the normal coronary group were also compared with the corresponding means for children with asthma, JRA, ADHD, and epilepsy using the 2-sample t test. To help evaluate the role of selection bias, we compared the characteristics of patients who returned the questionnaire with those who did not; we used the Fisher exact test for categorical variables and the Wilcoxon rank-sum test for continuous ones. P .05 was considered statistically significant.

	RESULTS

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Patient Sample
Of the 641 patients in our institutional database who had KD between 1984 and 1998, 492 met the eligibility criteria for our study. Of these, we identified abnormal coronary arteries in 51 children: 20 in the regressed aneurysm group, 18 in the mild to moderate aneurysm group, and 13 in the giant aneurysm group. Questionnaires were sent to all patients in these 3 groups. A total of 441 patients were in the normal coronary group, from which 150 were randomly selected to receive the survey. Of 201 total questionnaires mailed, 27 (13%) were undeliverable, reflecting patients lost to follow-up. Of the remaining 174 questionnaires, 110 (63%) were completed and returned, and 1 parent declined. Two children were excluded post hoc because of severe preexisting developmental delay. Questionnaires were returned for 81 (54%) of 150 children in the normal group, 9 (45%) of 20 with regressed aneurysms, 9 (50%) of 18 with mild to moderate aneurysms, and 9 (69%) of 13 with giant aneurysms. Compared with study participants, patients for whom the questionnaires were not returned did not differ significantly in terms of gender, coronary status, age at illness, or time since onset of illness.

Among the children for whom the questionnaires were returned, the male:female ratio was 1.4:1. The median age at the time of questionnaire completion was 10.5 years (range: 5.1–17.9 years) and at onset of illness was 3.1 years (range: 0.2–12.0 years). The median interval between the onset of illness and survey completion was 6.5 years (range: 0.2–15.8 years). The distribution of the parent’s race/ethnicity was 77% white, 6% black, 7% Hispanic, 6% Asian American, 2% other, and 1% unknown.

Summary Scores
The mean summary scores of patients in each coronary artery group are compared with those in the US population sample in Table 1. Children in the normal, regressed aneurysm, and mild to moderate aneurysm groups each had mean psychosocial summary and physical summary scores similar to those of the US population sample. Patients with giant aneurysms had a similar mean psychosocial summary score but a lower mean physical summary score compared with the US population sample (47.3 ± 9.7 vs 53.0 ± 8.8; P = .05). Within the combined KD groups, mean physical and psychosocial summary scores were not significantly associated with patient gender, age at illness, time since the onset of illness, gender of the parent completing the questionnaire, or the parent’s level of education, adjusting for coronary status.

Chronic Conditions
A checklist at the conclusion of the CHQ PF-50 inquired of parents whether they have ever been told by a teacher, school official, doctor, nurse, or other health professional that their child has certain medical conditions. Compared with the US population sample, parents of children with KD (all coronary groups combined) reported a higher prevalence of chronic allergies or sinus trouble (26.9% vs 17.6%; P = .04); orthopedic, bone, and joint problems (11.1% vs 3.4%; P = .004); and anxiety (13.9% vs 5.2%; P = .005; Table 2). The prevalence of each of these 3 conditions was also significantly higher for the normal coronary group alone relative to the US population sample.

	DISCUSSION

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During the acute phase of KD, parents are confronted with the possibility that their child could acquire lifelong cardiac disease. In a matter of weeks, the prognosis becomes clear as children either recover without detectable coronary dilation or develop coronary artery sequelae of varying severity. For those who develop coronary aneurysms, the severity of coronary involvement, most powerfully determined by coronary artery dimension, determines the subsequent frequency of follow-up visits, testing, medication use, and long-term cardiac morbidity with its associated interventions, such as interventional catheterization or cardiac surgery.⁶ Thus, whereas all parents face similar fears in the earliest days of KD, their experiences differ subsequently depending on the status of their child’s coronary arteries. KD thus presents an interesting paradigm in which to examine the effects of an acute life-threatening illness on parental perceptions as a function of the child’s subsequent outcome. The current report comprises the first description of general health status and quality of life in patients with KD.

The finding of worse physical health status among children with giant aneurysms is consistent with the documented natural history of patients in this most severely affected group. Virtually all early and mid-term cardiac morbidity and mortality occurs in children with giant aneurysms. In large aneurysms, thrombosis is promoted both by sluggish blood flow within the massively dilated vascular space and by the frequent development of stenotic lesions at the proximal or distal ends of the aneurysms. Indeed, the principal cause of death in KD is myocardial infarction caused by thrombotic occlusion of a giant aneurysm of the coronary artery, sometimes related to associated stenosis.⁷ Children with giant aneurysms are generally maintained on warfarin and aspirin, undergo testing with a stress-imaging modality at least annually, and undergo at least 1 cardiac catheterization. Their parents are taught cardiopulmonary resuscitation, schools are informed about which signs and symptoms warrant immediate medical attention, and older children are instructed to tell their parents or teachers if they have symptoms of angina. In our sample of 9 children with giant aneurysms, 2 had undergone coronary artery bypass procedures. The worse physical health scores of children in the giant aneurysm group thus affirm the validity of the CHQ PF-50 for assessment of children with heart disease.

In our study, we found that patients who had KD and in whom coronary artery dilation had never been detected were in fact similar to a sample from the general US population with respect to general health status, as reflected by their summary scores on overall physical and psychosocial well-being. Similarly, patients with mild to moderate coronary aneurysms and those with regressed aneurysms had normal physical and psychosocial health status. In contrast, those with giant aneurysms had significantly lower overall physical health status than the US population sample, although their psychosocial status was similar. Although these summary measures were similar to those in the US population sample among all but the giant aneurysm group, parents in all 4 coronary status groups—normal coronary, regressed aneurysms, mild to moderate aneurysms, and giant aneurysms—had more concerns about their child’s general health as evidenced by lower scores on the general health perceptions subscale score, reaching statistical significance in all but the mild to moderate aneurysm group. These concerns about their child’s health were evident, although our survey was performed at a median of 6.5 years after the acute illness. This subscale asked questions as to whether parents believe that their child is as healthy as other children, whether their child has ever had a serious illness, and whether they are concerned about their child’s present and future health. Lower general health perceptions among parents of all children who have had KD may reflect the impact of having a child who has had a potentially serious illness with no known cause and with little long-term data beyond 2 decades. These findings were consistent with results from King et al,⁸ who reported that 34% of parents questioned believed that Kawasaki disease had a lasting effect on their child. These parents verbalized concerns that their child seemed more "sickly" than other children.

Last, we found that children with KD had a greater frequency of some comorbid conditions compared with the US population sample. Specifically, a higher proportion of KD children had chronic allergies or sinus trouble. This finding is consistent with earlier work reporting an increased prevalence of preexisting eczema among children who develop KD⁹ because children with eczema tend to have more allergies in general. Parents of children with KD also reported a higher frequency of orthopedic, bone, and joint problems than parents of the US population sample. Approximately one third of patients with KD have a temporary arthritis in the first 2 months of their disease. Because this questionnaire does not specify the time period during which symptoms occurred, we are unable to distinguish whether the KD sample had more orthopedic or rheumatologic problems than normal after convalescence from their illness. Parents of children with KD reported more anxiety disorders in their children than reported by the US population sample. It is possible that a greater prevalence of reported anxiety may relate to the stresses of having had a severe childhood illness or the effect of repeated medical evaluations. However, it is also possible that the higher likelihood of anxiety disorders among children with KD reflects a selection bias among the families that chose to respond. Finally, we did not find an increased incidence of reported attentional deficits or learning or behavioral problems in the KD sample when compared with the US population sample. This is consistent with findings from King et al,⁸ who reported normal cognitive and academic ability in a sample of 32 patients with KD when compared with national norms. However, in contrast to his findings that suggested the potential for mild behavioral difficulties, our sample did not report more attentional or behavioral issues than those found in the normative population.⁸ In King’s study, the teacher’s report of behavior was similar in cases and sibling controls, although parents perceived their child who had had KD as having more behavior problems than their siblings.

Our study should be viewed in light of certain limitations. Parents who responded to the questionnaire may have been more concerned about their children than those who did not participate. Thus, study results may be affected by selection bias. Our sample was drawn from a single institution, so results may not be generalizable to patients with KD followed by other centers. Because parents rather than the patients themselves completed the health status questionnaire in the present study, it is possible that the health perceptions of the children may have differed from those of their parents. In the pediatric population, parent proxy tools are generally accepted as being a reliable measure of child health status.¹⁰ An additional study limitation is that the CHQ is a generic instrument applicable to a broad spectrum of children and adolescents and, like other pediatric measures of quality of life in children, does not have a disease-specific module for children with heart disease. Finally, the small number of children in the 3 groups with coronary abnormalities limited our power to detect smaller differences from the US population sample.

	CONCLUSION

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Using the CHQ PF-50 to assess health-related quality of life, we found that the overall physical and psychosocial health status in children who have had KD is similar to that in the US population sample with the exception that children with giant coronary aneurysms have worse physical health status. These results provide reassurance that the general health status of children who have had KD is excellent except among those few who are severely affected. Regardless of their child’s coronary artery status, parents had more concerns about their child’s general health than those in the US population sample. Lower general health perceptions among parents of all children who have had KD, even in the absence of coronary artery sequelae, may reflect the impact of a potentially life-threatening illness on a parent’s assessment of their child’s vulnerability. In addition, because most centers follow all patients with KD with echocardiograms, lipid profiles, and cardiac evaluations intermittently for many years after the actual illness, families may perceive mixed messages about their child’s health. Additional long-term studies are necessary to identify ways to support families and to minimize the effects of cardiac nondisease in children without coronary sequelae.

	ACKNOWLEDGMENTS

 
This study was supported in part by the Kobren Fund.

We thank Donna Donati and Donna Duva for data coordination and Kathleen M. Alexander for manuscript preparation.

	FOOTNOTES

 
Received for publication May 7, 2002; Accepted Sep 11, 2002.

Reprint requests to (A.L.B.) Department of Cardiology, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail: annette.baker{at}tch.harvard.edu

	REFERENCES

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3. Newburger JW, Takahashi M, Burns JC, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med.1986; 315 :341 –347[Abstract]
4. Landgraf JM, Abetz L, Ware JE Jr. Child Health Questionnaire (CHQ). 1st ed. Boston, MA: Health Institute, New England Medical Center; 1996
5. Research Committee on Kawasaki Disease. Report of Subcommittee on Standardization of Diagnostic Criteria and Reporting of Coronary Artery Lesions in Kawasaki Disease. Tokyo, Japan: Ministry of Health and Welfare; 1984
6. Dajani AS, Taubert KA, Takahashi M, et al. Guidelines for long-term management of patients with Kawasaki disease. Report from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation.1994; 89 :916 –922[Abstract/Free Full Text]
7. Kato H, Ichinose E, Kawasaki T. Myocardial infarction in Kawasaki disease: clinical analyses in 195 cases. J Pediatr.1986; 108 :923 –927[CrossRef][ISI][Medline]
8. King WJ, Schlieper A, Birdi N, Cappelli M, Korneluk Y, Rowe PC. The effect of Kawasaki disease on cognition and behavior. Arch Pediatr Adolesc Med.2000; 154 :463 –468[Abstract/Free Full Text]
9. Brosius CL, Newburger JW, Burns JC, Hojnowski-Diaz P, Zierler S, Leung DY. Increased prevalence of atopic dermatitis in Kawasaki disease. Pediatr Infect Dis J.1988; 7 :863 –866[ISI][Medline]
10. Parsons SK, Brown AP. Evaluation of quality of life of childhood cancer survivors: a methodological conundrum. Med Pediatr Oncol.1998; (suppl 1) :46 –53

This Article Abstract Full Text (PDF) P3Rs: Submit a response Alert me when this article is cited Alert me when P3Rs are posted Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Citing Articles Citing Articles via CrossRef Citing Articles via ISI Web of Science (3) Citing Articles via Google Scholar Google Scholar Articles by Baker, A. L. Articles by Jenkins, K. J. Search for Related Content PubMed PubMed Citation Articles by Baker, A. L. Articles by Jenkins, K. J. Related Collections Infectious Disease & Immunity Related AAP Red Book topics: Kawasaki Disease (Mucocutaneous...

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