Nancy W. Berk
Mary L. Marazita
One of the most common congenital anomalies, cleft lip with or without cleft palate (CL/P) is also one of the most common visible birth defects, occurring in 1 of every 500 to 1000 live births worldwide (Murray, 1995). While the severity of this condition varies, multidisciplinary treatment is often warranted. This may include craniofacial surgery, specialized dental and orthognathic treatment, speech and hearing intervention, and educational, psychological, and social assessment and intervention. The National Institute of Dental and Craniofacial Research (2000) estimates that in the United States the annual health cost for craniofacial anomalies approaches $1 billion and reflects the “comprehensive and time-consuming habilitation required to enable each child born with such birth defects the opportunity to pursue a productive life.”
The preceding statement recognizes the inherent right of each individual to pursue a life that is free from the debilitating physical and social effects of a birth defect. The economic cost of CL/P is multidimensional. It is felt by the patient, his or her family, and society. It is highlighted by fiscal burden for the patient and family and can represent lost potential and productivity at the individual, family, and societal levels, which is often forgotten by those focused on dollars, budgets, and reimbursement.
Ideally, this topic should be examined within the rigor of standard health economic paradigms. However, the absence of economic data on clefting prohibits us from assessing the precise fiscal impact of CL/P. The purpose of this chapter then is to elucidate, for the health professional, the myriad costs associated with this condition and to serve as a springboard for future research that would enable health professionals, including health economists, to better determine the magnitude of these costs. The term cost will be used throughout this chapter as a global lay construct reflecting price, loss, and sacrifice. This chapter first addresses the direct financial costs associated with CL/P, with an illustration of early childhood treatment costs in a patient cohort (age 0-6). Next, physical costs are addressed with respect to the morbidity and mortality seen in the CL/P population, including families. Finally, psychosocial costs are reviewed, including those related to health beliefs and behaviors, the impact of facial differences, social competence, and psychosocial interventions.
These categories (financial, physical, and psychosocial) provide a means of conceptualizing the many costs associated with clefting. They are not, however, mutually exclusive. For example, psychosocial costs, such as social anxiety, may influence job opportunities for a patient, in turn impacting his or her income and financial resources. This chapter provides a foundation for understanding these costs and their interrelated nature, as well as the costs associated with the inability or unwillingness to access specialized care.
Financial Costs
While there are little data on the medical costs of CL/P, it is well accepted that the medical costs for those with chronic health conditions, including those associated with genetic disorders, exceed costs for those in good health. Variability of medical costs has been associated with disease severity for genetic disorders such as cystic fibrosis, even mild severity being associated with substantial cost (Lieu et al, 1999). In a study of 1993 Medicaid claims data for 310,977 children with one of eight selected chronic conditions, Ireys et al. (1997) found medical care for these children to be 2.5 to 2.0 times more expensive than medical care for children in general. These costs were found to be conditiondependent. While CL/P was not included among these eight conditions, such findings illustrate that medical care costs are burdensome for those with chronic health problems.
While many have acknowledged the overwhelming financial burden associated with CL/P, few have directly assessed the associated financial cost. In perhaps the most frequently cited study of financial cost of CL/P, Waitzman et al. (1994) reviewed the total cost per case based on samples in California, that acknowledging direct and indirect costs. The authors calculated this to range from $29,000 to $246,000. Their conservative estimate of the cost per case, which has been widely quoted in the cleft literature, is $92,000. Waitzman et al. (1994) noted that the “high total cost of cleft lip/palate reflects its high incidence coupled with relatively high frequencies of first-year mortality and activity limitation” (p. 200). This number, while assessing indirect costs, did not include occupational costs to parents or psychosocial costs to the patient, such as pain or anxiety. Thus, this may be an extremely low estimate of the fiscal burden of this birth defect on the patient, family, and society. Direct financial costs include surgeries, dental care, speech and language intervention, audiological assessment, and psychological and educational assessment and intervention. Aspects of the financial impact on the family that are often neglected or unassessed include parking, transportation, and other travel expenses related to patient treatment. Insurance concerns and restrictions, particularly with respect to the identification of CL/P as a pre-existing condition, may impact parental occupational opportunities and advancement (Strauss, 1994).
Also, CL/P is associated with significant costs beyond birth, including nonmedical direct costs such as special education services (Waitzman et al., 1994). For example, children with CL/P are at risk for learning disabilities and other developmental problems (Broder et al., 1998; Kapp-Simon and Krueckeberg, 2000). In comparison to unaffected peers, CL/P children have higher rates of learning disabilities (Richman et al., 1988; Eliason, 1990; Broder et al., 1998). If the educational needs of these at-risk children are routinely assessed and addressed, the financial cost of care rises at the point of service (e.g., educational testing and intervention can be costly). However, such intervention may prove to defray part of the societal costs and psychosocial impact of CL/P by maximizing educational experiences and promoting the patient's academic success and future occupational opportunities and productivity.
As an illustration of some of the costs associated with cleft lip (CL) and cleft palate (CP), we have summarized the average number of services provided within the University of Pittsburgh Cleft Palate-Craniofacial Center (CPCC), as well as average fees charged, for the first 5 years of life for selected patients from the 1994 birth cohort. Also presented are numbers and fees charged for plastic surgery procedures outside of the CPCC. These numbers do not fully reflect all costs, particularly those associated with dental procedures such as orthodontia and oral surgery. While these specialties are represented within the CPCC, cost assessment and estimates are difficult to track for most patients. Treatments and related billing are often conducted outside of the CPCC by either the affiliated specialty department or specialists. In some cases, patient reluctance to travel long distances for dental procedures has resulted in the seeking of specialty care outside of the team network. This sample may not be representative of all CL/P patients. While the cleft severity of this sample resembles that seen in national birth registry data, socioeconomic status is somewhat lower. This difference would not impact patient treatment needs but could potentially influence parental treatment-seeking behavior. Traditionally, attrition secondary to low socioeconomic status is rare in this group, given the child's obvious medical needs until at least age 6. Nonetheless, these data should be interpreted cautiously with these limitations in mind.
TABLE 36.1 summarizes the selected patients. Included are patients with nonsyndromic anomalies who were born in 1994, began as patients with the CPCC at birth, and have remained continuously under the care of the CPCC until May of 2000. This cohort was chosen to track patients who had received comprehensive team care at the CPCC rather than patients who may have been seen for isolated visits (e.g., second opin ions, lost to follow-up secondary relocation). As most CL/P children receive care up to age 6 regardless of severity, this cohort enabled us to examine the early financial impact of clefting experienced by nearly all families to some extent. There were 38 such patients, six with CL, 21 with cleft lip with cleft palate (CLP), and 11 with CP. The usual male preponderance was seen for the CL and CLP categories, and the usual slight female preponderance was seen for CP (see Chapter 12 for more details).
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TABLE 36.1. Patients of the University of Pittburgh Cleft Palate-Craniofacial Center (CPCC) from 1994 Birth Cohort Who Are Currently Active |
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TABLE 36.2. Patients from the 1994 Birth Cohort of the University of Pittsburgh Cleft Palate-Craniofacial Center (CPCC) Receiving Services over the First 5 Years of Life |
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TABLE 36.2 summarizes the total number of services provided by the CPCC over the first 5 years of life for the 1994 birth cohort and the total number of plastic surgery procedures. Presented are the minima, maxima, and averages by cleft type. The specialties providing services within the CPCC include audiology; pediatrics; genetics; ear, nose, and throat (ENT), speech/language, dental (consults), plastic surgery (consults), psychology, and family support. Also summarized is the care received outside of the CPCC and treatment planning after full team staffing of individual cases (see Section II of this book for a full description of multidisciplinary care of CL/P patients; also refer to the American Cleft Palate-Craniofacial Association website http://www.cleftline.org). Examples of the plastic surgery procedures provided to this cohort during the first 5 years of life include lip repair, primary nasal reconstruction, posterior and anterior palatal repair, and closure of fistulae (see Chapter 26 for more details on surgery). Not surprisingly, the CLP members of the birth cohort had the highest average number of CPCC services and plastic surgery procedures (about 1.5 times the average for CL or CP individuals and about 4 times the average plastic surgery procedures).
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TABLE 36.3. Fees Billed to Patients from the 1994 Birth Cohort of the University of Pittsburgh Cleft Palate-Craniofacial Center (CPCC) for Services over the First 5 Years of Life |
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TABLE 36.3 summarizes the fees billed to these patients. Presented are the minima, maxima, and average fees billed during the first 5 years of life for CPCC services and plastic surgery procedures. These figures represent the fees billed, not those collected. For the purpose of this chapter, billable fees are presented since these are based on the cost of providing the services and, therefore, represent a more accurate reflection of the “costs” of having a cleft than would collections. Again, not surprisingly, CLP individuals have the highest average fees billed over the 5-year period (about 1.5 times the average for CL or CP individuals and about 4 times the average plastic surgery fees).
TABLE 36.4 presents the categories of service provided by the CPCC for the first 5 years of life. Presented are average numbers of services and average fees billed for each category, by each cleft type. These numbers highlight the direct medical costs faced by those affected by CL/P. However, they capture only a portion of that burden in that costs are often incurred beyond those charged by the multidisciplinary center and the surgeon. Likewise, costs extend far beyond the first 5 years of life.
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TABLE 36.4. Categories of Service Provided to Patients of the 1994 Birth Cohort of the University of Pittsburgh Cleft Palate-Craniofacial Center (CPCC) over the First 5 Years of Life |
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Physical Costs
In addition to the financial costs of having an oral cleft are other equally significant costs. There are several morbidity factors associated with having a cleft, as well as increased risk of neonatal mortality. The impact on the family includes problems associated with low birth weight, fetal wastage, and risk of recurrence.
Individual
The two major factors that have been consistently shown to affect the individual with a cleft are associated malformations and neonatal mortality. There is also some evidence that cleft individuals are at risk for low birth weight (Emanuel et al., 1973; Cooper et al., 2000). Table 36.5 presents the percentages of CL, CLP, CL/P, and CP individuals having additional malformations from several large, population-based studies throughout the world. A consistent finding in each of these studies was that the frequency of associated anomalies is higher for CP than for CL/P cases. The percentage of CP individuals having associated anomalies ranges from 15.1% in a large series from Denmark (Christensen, 1999) to 55% in a patient population in California (Jones, 1988). The percentage for CL/P ranges from 6.4% in Denmark (Christensen, 1999) to 29.2% in New York (Druschel et al., 1996). The wide range in the reported percentages from study to study is probably due in part to differences in the definition of associated anomalies, particularly in whether major and minor anomalies are included.
TABLE 36.6 presents selected population-based studies that tested whether cleft cases had higher neonatal mortality (death during the first year of life) than controls. A consistent finding across these studies was that CL/P and CP individuals with additional associated anomalies were significantly more likely to die during the first year of life than were controls. The results for individuals with isolated CP were also consistent across studies in that they were not more likely to die during the first year of life than controls. However, the results for isolated CL/P were not consistent, with two studies finding a significantly increased risk for cases vs. controls and two studies not finding a statistically significant increased risk.
Furthermore, two studies investigated low birth weight in CL/P and CP individuals vs. controls. Druschel et al. (1996) reported that both isolated CP and CL/P had slightly higher rates of low birth weight [<2500 g, risk ratio = 1.3, 95% confidence interval (CI) 1.0-1.7 for CP; risk ratio = 1.3, 95% CI 1.0-1.5 for CL/P) than did individuals with no anomalies but that CP and CL/P individuals with other anomalies had greatly increased rates of low birth weight (<2500 g, risk ratio = 4.4, 95% C.I. 3.8-5.1 for CP; risk ratio = 4.5, 95% CI 3.9-5.2 for CL/P). Menegotto and Salzano (1990) investigated birth weight in isolated CP and CL/P cases vs. controls and found that CP individuals did not have a significantly lower birth weight than controls but that CL/P individuals did (p < 0.001).
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TABLE 36.5. Percentages of Cases Having One or More Other Malformations* |
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The associated anomalies, neonatal mortality, and low birth weight have concomitant financial and psychosocial costs. While early mortality may reduce medical costs for families, one might argue that other costs prove to be equally as devastating, particularly from a psychosocial perspective (e.g., psychological trauma, its impact, and subsequent intervention needs). Unfortunately, such costs have been neither well characterized nor well documented in this population.
Family
In addition to the morbidity and neonatal mortality costs to the individual are the costs to the families of affected individuals. In other words, the concomitant costs associated with oral clefting are disproportionately borne by the families in which there is a cleft individual.
First, the risk of having an additional affected family member (recurrence risk) is significantly higher than the population risk of having a cleft for every degree of relationship (Murray, 1995; Christensen, 1999). Therefore, the financial costs of having an individual with a cleft are magnified within an affected individual's family because there is likely to be an additional affected family member.
Furthermore, that there may be additional noncleft morbidity in families of individuals with a cleft. Although not a consistent finding across all studies, multiple studies have found an increase in fetal mortality in the sibships of cleft individuals (Dronamraju and Bixler, 1984; Menegotto and Salzano, 1990). Also, there may be an increased risk of cancer in the families of cleft individuals. Two genetic loci involved with neoplasms have shown positive linkage or association with nonsyndromic CL/P. Retinoic acid receptor-a (KARA, 17q21) has shown positive association with CL/P (see Chapter 20), is involved in the teratogenicity of vitamin A, and is in the same chromosomal region as a major breast cancer gene (BRCA1). BCL3 (19ql3) is a proto-oncogene that has shown both positive association and linkage with nonsyndromic CL/P (see Chapter 21). Yang et al. (1994) also showed that there were much higher rates of cancer in the relatives of CL/P probands vs. published population rates. However, this finding was not confirmed by Steinwachs et al. (2000).
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TABLE 36.6. Risks of Dying in the First Year of Life for Cleft Lip with or without Cleft Palate (CL/P) and Cleft Palate (CP) |
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As summarized earlier, a high proportion of CL/P and CP individuals have additional associated anomalies (estimates range from 6.4% to 29.2% for CL/P and from 15.1% to 55% for CP). A significant proportion of cleft individuals with associated anomalies have mendelian syndromes with variable expressivity; therefore, some of their relatives may have findings other than a cleft. For example, Stickler's syndrome is the most common syndrome that may include CP; other associated features that may occur alone in carriers of the gene include high myopia and/or detached retina and joint disorders.
In addition to the disproportionate burden borne by families of cleft individuals, those racial and ethnic groups with high birth prevalence of clefts face an increased burden from the costs of clefts secondary to the higher prevalence. As reviewed by Wyszynski et al. (1996), Native Americans have the highest reported birth prevalence of CL/P (3.6/1000 live births), followed by Asians (2.1/1000 in Japanese, 1.3-1.7/1000 in Chinese), Caucasians (1/1000), and individuals of African descent (0.3/1000).
Psychosocial Costs
Differences can be stigmatizing. For the individual with CL/P, differences often involve varied degrees of facial scarring, dental and orthognathic features such as missing teeth, and speech and language limitations. Syndromic clefts may be associated with a myriad of visible differences, from minor structural ones to major differences in symmetry. The psychosocial implications of CL/P have been acknowledged and examined (Kapp-Simon et al., 1992; Richman and Eliason, 1993), but the costs are rarely assessed. This may be due in part to the lack of available outcome data. Some psychosocial aspects of CL/P (e.g., learning disabilities) have been noted in financial cost studies because of their commonality and the acknowledged fiscal burden. Other aspects that may be more difficult to assess or have more variability among patients and families are reviewed below.
Psychosocial costs often translate into economic costs for the individual, the family, and society. This may occur in a variety of ways. For example, psychosocial factors associated with CL/P may warrant specific treatment that is associated with a financial cost (e.g., psychotherapy). Indirect costs may occur via opportunistic losses related to societal, individual, or familial imposed restrictions or limitations (e.g., vocational obstacles, relationship difficulties). Lack of treatment in any domain has the potential to contribute to psychosocial and financial burdens for the patient and family.
Costs Related to Health Behaviors and Beliefs
Individual
Chronically ill children who undergo frequent invasive medical procedures may be prone to anticipatory anxiety associated with future medical procedures (Jacobsen et al., 1990). Most research in this domain has involved pediatric cancer patients (cf., Redd et al., 1987; Jacobsen et al., 1990; Manne et al., 1992). The impact of early medical experiences on the lives of older CL/P patients has not been comprehensively examined. It is unclear how the early childhood experiences of these patients affect their attitudes toward preventive health behaviors, including medical and dental treatment seeking. However, certain health-related behaviors (e.g., smoking cessation, exercise) can have significant implications in the reduction of costs associated with illness prevention and detection.
Frequent medical appointments, hospitalizations, and associated medical problems (e.g., inner ear pathology) of CL/P children can result in excessive school absences. For some children, these absences may contribute to educational disadvantages. However, there is little research on how these health behaviors and problems impact the future of CL/P individuals from a cost perspective. It has been suggested that the academic performance of CL/P children is negatively influenced by speech, language, and hearing deficits as well as facial differences (Richman and Eliason, 1982). How this directly impacts career development and occupational outcomes is unknown.
Family
The economic burden of CL/P for the family extends beyond the price of surgeries, the cost of orthognathic intervention, or the costs of speech evaluation and treatment. The indirect costs for a parent include such things as lost work hours and opportunities (occupational and social) secondary to the treatment needs of the affected child. While families may be cognizant of these costs, members may also be reluctant to verbalize or elaborate upon these experiences.
Costs Related to Facial Esthetics
The impact of physical appearance, particularly facial attractiveness, on the reactions of others is well documented (cf., Berscheid and Gangestad, 1982; Reis and Hodgins, 1995). Individuals who are perceived by others as attractive are more likely to receive preferential treatment in educational, occupational, legal, and other social settings. Because the majority of research on attractiveness involves unaffected individuals ranging from attractive to unattractive, it cannot be assumed that conclusions based on these studies are generalizable to CL/P populations (Reis and Hodgins, 1995).
Certainly, when excluding the specific visible characteristics of the craniofacial anomaly, CL/P patients, like unaffected individuals, span a range of attractiveness. How the interaction between patient attractiveness and severity of the anomaly influences perceived attractiveness is not known. We are currently examining the contributions of facial attractiveness, severity of the anomaly, and auditory attractiveness in predicting perceived attractiveness.
Individual
Berscheid and Gangestad (1982) noted the following:
The social psychological effects of physical attractiveness are pervasive, strong, and generally uniform in nature. They are such that the physically attractive, whether male or female, old or young, black or white, or of high or low socioeconomic status, receive preferential social treatment in virtually every social situation examined thus far. (p. 290)
Highly attractive individuals have been found to elicit more favorable social impressions, responses, and interactions (Berscheid et al., 1971; Bull and Rumsey, 1988; Feingold, 1992). Individuals with craniofacial differences are more prone than unaffected individuals to unpleasant social responses from others, particularly strangers (Reis and Hodgins, 1995). Individuals with physical deformities are more prone to being avoided by others who may be uncomfortable with, or insensitive to, the anomaly.
Reactions from others and perceived reactions can influence an individual's behavior. Likewise, differential reactions from others may result in different opportunities and/or needs for individuals with visible differences. These experiential differences may translate to financial costs in areas such as career options and need for psychological intervention. It appears that adjustment in cleft adolescents may be related to an affected individual's self-perception of appearance, given that individuals who perceive themselves to be more attractive or less impaired have higher social competence scores (Tobiasen and Hiebert, 1993).
Teacher expectations are influenced by facial disfigurement (Richman, 1978), children with clefts being more likely to be inaccurately assessed with respect to their intellectual ability than their unaffected peers. While there is no direct evidence that this results in negative educational outcomes for the CL/P child, it does have strong implications with respect to teacher expectations, student advising, student opportunities, and educational/occupational/social outcomes for affected individuals.
Family
For most families, the initial reaction to the birth of a child with a cleft involves some degree of distress. This may be exacerbated by the fact that few children are diagnosed in utero, thus preventing any preparation (Berk et al., 1999). While learning of a birth defect or chronic health condition would normally elicit some level of anxiety, there are no data to suggest that the child's condition and treatment needs interfere with parent-child attachment or normal and positive interactions (Richman and Eliason, 1993). However, studies examining the early interaction of mothers and CL/P infants suggest that their interactions differ from those of unaffected infants and mothers. Specifically, affected infants (infants with CL/P and other craniofacial anomalies) and their mothers are less responsive and active in their interactions with one another (Field, 1995). The implications from this research with respect to long-term adjustment and subsequent costs are unknown.
Costs Related to Social Competence
Children and adults with CL/P are at higher risk for psychosocial adjustment problems in areas related to social competence than unaffected peers (Heller et al., 1981; Richman, 1983, 1998; Tobiasen and Hiebert, 1993; Tobiasen, 1995; Berk et al., 2001). Individuals with oral clefts may be more disadvantaged with respect to social affiliation and adaptation than unaffected adults.
A number of studies have revealed a tendency toward social inhibition and withdrawal in children, adolescents, and adults with oral clefts (Peter et al., 1975; Heller et al., 1981; Richman, 1983, 1997, 1998; Kapp-Simon et al., 1992; Tobiasen and Hiebert, 1993; Tobiasen, 1995; Kapp-Simon and McGuire, 1997). These disaffiliation behaviors associated with social anxiety can impair the development or maintenance of social relationships (Leary and Kowalski, 1995). This may have significant implications, given that social relationships, specifically certain forms of social support, may facilitate psychological adjustment and wellness (Wallston, et al., 1983; Cohen and Hoberman, 1983).
Withdrawal behaviors in children and adolescents with oral clefts may be mediated by other variables, particularly those associated with the severity of the craniofacial anomaly, perceived attractiveness, or perceived importance of facial attractiveness (Richman, 1983; Richman et al., 1985; Tobiasen and Hiebert, 1993).
Costs Related to Psychological Intervention
The need for, and benefits of, psychological intervention with CL/P individuals have been implied and acknowledged (Heller et al., 1985; Kapp-Simon et al., 1992; Kapp-Simon, 1995). In a survey of cleft/cranio-facial teams, Broder and Richman (1987) found that 80% of team directors identified mental health intervention as important for their patients. Of interest is that these same directors noted that fewer than 50% of patients received psychological services. It is unclear whether this is a reflection of underutilization, inaccessibility, or selective utilization (e.g., utilization by the most severely impaired).
Psychological intervention can enhance adjustment for individuals with CL/P. For example, Kapp-Simon et al. (1992) found social skills to be one of the best predictors of adjustment in adolescents with craniofacial anomalies. They suggested that social skill training may enhance adjustment in this population. Others have found family support groups, neuropsychological testing, and individual psychotherapy, while not warranted in all cases, to be clinically beneficial for some subsets of patients and families. Despite this general professional consensus, there are no data regarding the utilization of psychological services in this population. The percentage of CL/P individuals and families seeking treatment or evaluation is unclear. Costs for individual psychotherapy, like family therapy, differ significantly across practitioners and geographic areas. Third-party coverage is rarely substantial, rendering some families helpless to pursue a course of action they might otherwise seek. In addition, for some individuals, the stigma of seeking psychological and psychiatric services overrides the distress that warrants it. It is also unclear how many individuals refrain from reporting their utilization of psychological services based on this societal stigma.
Conclusion
The area of service utilization and costs in clefting has been explored only minimally. Nonetheless, it is clear that the known direct costs of specialty treatment for individuals with CL/P are significant. For the CL/P patient and family, the overwhelming burden is often the fiscal one, with lifetime costs estimated at approximately $92,000 with a range from $29,000 to $246, 000 (Waitzman et al., 1994).
Unfortunately, the unknown costs have the potential to equal or exceed these. The most poorly appreciated costs for the individual with CL/P appear to be the nonfinancial costs that directly and indirectly influence finances and future income. The least appreciated and rarely acknowledged burden involves physical and psychosocial costs to patient, family, and society. Physical costs include inequalities of morbidity and mortality that can be associated with clefts as well as ethnic and racial disparities in cleft prevalence.
Psychosocial costs include those associated with the execution of health behaviors, reactions to facial differences, and disparities in social competence (between affected and unaffected individuals). In addition, the inability to access or the unwillingness to utilize treatment has the potential to result in physical, psychosocial, and financial burden to the patient, the family, and society.
Health professionals must be cognizant of the burden facing the CL/P patient and family. This burden extends beyond direct medical costs and must be acknowledged by patients, families, and those who make decisions related to medical coverage/third-party reimbursement and government assistance. To enhance quality of life and maximize patient potential, we must identify at-risk patients and families and provide them with the strategies, resources, and tools to access intervention and support in all of the aforementioned domains.
References
Berk, NW, Cooper, ME, Liu, Y, Marazita, ML (2001). Social anxiety in Chinese adults with oral-facial clefts. Cleft Palate Craniofac J 38: 126–133.
Berk, NW, Marazita, ML, Cooper, ME (1999). Medical genetics on the cleft palate-craniofacial team: understanding parental preference. Cleft Palate Craniofac J 36: 30–35.
Berscheid, E, Dion, KK, Walster, E, Walster, GW (1971). Physical attractiveness and dating choice: a test of the matching hypothesis. J Exp Soc Psychol 7: 173–789.
Berscheid, E, Gangestad, S (1982). The social psychological implications of facial physical attractiveness. Clin Plast Surg 9: 289–296.
Broder, H, Richman, LC (1987). An examination of mental health services for cleft/craniofacial patients. Cleft Palate J 24: 158–162.
Broder, HL, Richman, LC, Matheson, P (1998). Learning disability, school achievement, and grade retention among children with cleft: a two-center study. Cleft Palate Craniofac J 35: 127–131.
Bull, R, Rumsey, N (1988). The Social Psychology of Facial Appearance. New York: Springer-Verlag.
Christensen, K (1999). The 20th century Danish facial cleft population—epidemiological and genetic-epidemiological studies. Cleft Palate Craniofac J 36: 96–104.
Cohen, S, Hoberman, HM (1983). Positive events and social supports as buffers of life change stress. J Appl Soc Psychol 13: 99–125.
Cooper, ME, Stone, RA, Liu, Y, et al. (2000). Descriptive epidemiology of nonsyndromic cleft lip with or without cleft palate in Shanghai, China, from 1980 to 1989. Cleft Palate Craniofac J 37: 274–280.
Cornel, MC, Spreen, JA, Meijer, I, et al. (1992). Some epidemiological data on oral clefts in the northern Netherlands, 1981–1988. J Craniomaxillofac Surg 20: 147–152.
Dronamraju, KR, Bixler, D (1984). Fetal mortality in oral cleft families (V): studies of sporadic vs familial and pure vs syndromic clefts. Clin Genet 25: 314–317.
Druschel, CM, Hughes, JP, Olsen, CL (1996). First year-of-life mortality among infants with oral clefts: New York State, 1983–1990. Cleft Palate Craniofac J 33: 400–405.
Eliason, MJ (1990). Neuropsychological perspectives of cleft lip and palate. In: Multidisciplinary Management of Cleft and Palate, edited by J Bardach and HL Morris. Philadelphia: Saunders, pp. 825–831.
Emanuel, I, Culver, BH, Erickson, JD, et al. (1973). The further epidemiological differentiation of cleft lip and palate: a population study of clefts in King County, Washington, 1956–1965. Teratology 7: 271–281.
Feingold, A (1992). Good-looking people are not what we think. PsycholBull 111: 304–341.
Field, T (1995). Early interaction of infants with craniofacial anomalies. In: Craniofacial Anomalies. Psychological Perspectives, edited by RA Eder. New York: Springer-Verlag, pp. 99–110.
Heller, A, Rafman, S, Zvagulis, I, Pless, IB (1985). Birth defects and psychosocial adjustment. American J of Diseases of Children 139: 257–263.
Heller, A, Tidmarsh, W, Pless, IB (1981). The psychosocial functioning of young adults born with cleft lip or palate. Clin Pediatr 20: 459–465.
Hujoel, PP, Bollen, AM, Mueller, BA (1992). First-year mortality among infants with facial clefts. Cleft Palate Craniofac J 29: 451–455.
Ireys, HT, Anderson, GF, Shaffer, TJ, Neff, JM (1997). Expenditures for care of children with chronic illnesses enrolled in the Washington State Medicaid program, fiscal year 1993. Pediatrics 100: 197–204.
Jacobsen, PB, Manne, SL, Gorfinkle, K, et al. (1990). Analysis of child and parent behavior during painful medical procedures. Health Psychol 9: 559–576.
Jones, MC (1988). Etiology of facial clefts: prospective evaluation of 428 patients. Cleft Palate J 25: 16–20.
Kapp-Simon, KA (1995). Psychological interventions for the adolescent with cleft lip and palate. Cleft Palate Craniofac J 32: 104–108.
Kapp-Simon, KA, Krueckeberg, S (2000). Mental development in infants with cleft lip and/or palate. Cleft Palate Craniofac J 37: 65–70.
Kapp-Simon, KA, McGuire, DE (1997). Observed social interaction patterns in adolescents with and without craniofacial conditions. Cleft Palate Craniofac J 34: 380–384.
Kapp-Simon, KA, Simon, DJ, Kristovich, S (1992). Self-perception, social skills, adjustment, and inhibition in young adolescents with craniofacial anomalies. Cleft Palate Craniofac J 29: 352–356.
Kumar, P, Hussain, MT, Cardoso, E, et al. (1991). Facial clefts in Saudi Arabia: an epidemiologic analysis in 179 patients. Plast Reconstr Surg 88: 955–958.
Leary, MR, Kowalski, RM (1995). Social Anxiety. New York: Guilford.
Lieu, TA, Ray, GT, Farmer, G, Shay, G (1999). The cost of medical care for patients with cystic fibrosis in a health maintenance organization. Pediatrics 103: e72.
Manne, SL, Bakeman, R, Jacobsen, PB, et al. (1992). Adult-child interaction during invasive medical procedures. Health Psychol 11: 241–249.
Menegotto, BG, Salzano, FM (1990). New study on the relationship between oral clefts and fetal loss. Am J Med Genet 37: 539–542.
Menegotto, BG, Salzano, FM (1991). Epidemiology of oral clefts in a large South American sample. Cleft Palate Craniofac J 28: 373–376.
Milan, M, Astolfi, G, Volpato, S, et al. (1994). 766 cases of oral cleft in Italy. Eur J Epidemiol 10: 317–324.
Murray, JC (1995). Face facts: genes, environment, and clefts. Am J Hum Genet 57: 227–232.
Murray, JC, Daack-Hirsch, S, Buetow, KH, et al. (1997). Clinical and epidemiologic studies of the cleft lip and palate in the Philippines. Cleft Palate Craniofac J 34: 7–10.
National Institute of Dental and Craniofacial Research (2000). http://www.nidcr.nih.gov, p. 1.
Peter, JP, Chinsky, RR, Fisher, MJ (1975). Sociological aspects of cleft palate adults: IV. Social integration. Cleft Palate J 12: 304–310.
Redd, WH, Jacobsen, PB, Die-Trill, M, et al. (1987). Cognitive/attentional distraction in the control of conditioned nausea in pediatric cancer patients receiving chemotherapy. J Consult Clin Psychol 55: 391–395.
Reis, HT, Hodgins, HS (1995). Reactions to craniofacial disfigurement: lessons from the physical attractiveness and stigma literatures. In: Craniofacial Anomalies. Psychological Perspectives, edited by RA Eder. New York: Springer-Verlag, pp. 177–198.
Richman, L (1978). The effects of facial disfigurement on teachers' perception of ability in cleft palate children. Cleft Palate J 25: 155–160.
Richman, L (1998). Fearful shyness versus solitary passivity in socially inhibited children with cleft. Presented at the annual meeting of the American Cleft Palate-Craniofacial Association, Baltimore, Maryland, April 24, 1998.
Richman, LC (1983). Self-reported social, speech, and facial concerns and personality adjustment of adolescents with cleft lip and palate. Cleft Palate J 20: 108–112.
Richman, LC (1997). Facial and speech relationships to behavior of children with clefts across three age levels. Cleft Palate Craniofac J 34: 390–395.
Richman, LC, Eliason, M (1982). Psychological characteristics of children with cleft lip and palate: intellectual, achievement, behavioral and personality variables. Cleft Palate J 19: 249–257.
Richman, LC, Eliason, MJ (1993). Psychological characteristics associated with cleft palate. In: Cleft Palate: Interdisciplinary issues and treatment, edited by KT Moller and CD Starr. Austin, TX: Pro-Ed, pp. 357–380.
Richman, LC, Eliason, MJ, Lindgren, SD (1988). Reading disability in children with clefts. Cleft Palate J 25: 21–25.
Richman, LC, Holmes, CS, Eliason, MJ (1985). Adolescents with cleft lip and palate: self-perceptions of appearance and behavior related to personality adjustment. Cleft Palate J 22: 93–96.
Steinwachs, EF, Amos, C, Johnston, D, et al. (2000). Nonsyndromic cleft lip and cleft palate is not associated with cancer or other birth defects. Am J Med Genet 90: 17–24.
Strauss, RP (1994). Health policy and craniofacial care: issues in resource allocation. Cleft Palate Craniofac J 31: 78–80.
Tobiasen, JM (1995). Social psychological model of craniofacial anomalies: example of cleft lip and palate. In: Craniofacial Anomalies. Psychological Perspectives, edited by RA Eder. New York: Springer-Verlag, pp. 233–257.
Tobiasen, JM, Hiebert, JM (1993). Clefting and psychosocial adjustment: influence of facial aesthetics. Clin Plast Surg 20: 623–631.
Waitzman, NJ, Romano, PS, Scheffler, RM (1994). Estimates of economic costs of birth defects. Inquiry 33: 188–205.
Wallston, BS, Alagna, SW, DeVellis, BM, DeVellis, RF (1983). Social support and physical health. Health Psychol 2: 367–391.
Wyszynski, DF, Beaty, TH, Maestri, NE (1996). Genetics of nonsyndromic oral clefts revisited. Cleft Palate Craniofac J 33: 406–417.
Yang, P, Mitchell, E, Marazita, ML, et al. (1994). Do cancers aggregate in families ascertained through probands with cleft lip and/or cleft palate? Am J Hum Genet 55: A97.