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Editorial |

Disparities in Cystic Fibrosis Care and Outcome: Socioeconomic Status and Beyond FREE TO VIEW

Eitan Kerem, MD; Malena Cohen-Cymberknoh, MD
Author and Funding Information

CORRESPONDENCE TO: Eitan Kerem, MD, Department of Pediatrics, Hadassah-Hebrew University Medical Center, Mount Scopus, POB 24035 Jerusalem, Israel 91240


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(2):298-300. doi:10.1016/j.chest.2015.08.021
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The effect of socioeconomic status (SES) on health is well established. People with higher incomes, better jobs, and more years of schooling tend to be healthier and live longer whereas lower SES is linked to a number of factors that influence health status, including access to health care, health behaviors, lifestyle factors, chronic stress, and environmental and neighborhood exposures. Health disparities can result from limited access to clinical care owing to lack of availability on a national or local level, to limited family resources that restrict access to specialized services or therapy (financial of physical), or to lack of knowledge or education regarding how the disease should be treated, resulting in poor adherence to therapy.

Cystic fibrosis (CF) is the most common lethal autosomal recessive disease among white people although it is a pan-ethnic disease. Without appropriate treatment, affected individuals will not survive the first years of life. It is estimated that there are nearly 90,000 patients with CF worldwide, most of whom live in western Europe, North America, and Australia. Survival of patients with CF in these countries has improved significantly over the past decades as a result of better nutrition, frequent clinical evaluations and early identification and treatment of complications, including aggressive and intensive as well as expensive interventions by physicians and other health care workers who are specially trained in the management of CF. Several guidelines are available to assist CF caregivers in evaluating and monitoring patients, detecting complications, and preventing clinical deterioration. Despite this, disparities are found in the management and outcome of patients with CF.

The effect of poverty on the severity of CF has been reported for both developed and developing countries. In countries where health expenditure per capita is low, patients with CF experience more deprivation-related health disparities than all other individuals in the population. Children with CF who are from more disadvantaged populations show worse lung function, more impaired growth,, and higher rates of mortality than do children from more affluent areas. Results from a large multinational European study of demographic indicators in patients with CF have shown that in European Union (EU) countries with a high gross domestic product, most patients are now aged > 18 years whereas in countries not in the EU, the average age of patients is much lower. Because EU and non-EU countries have populations of similar size and similar distribution for severe genotypes causing CF, the reasons for this disparity is likely to be suboptimal therapy. Likewise, data from the European Cystic Fibrosis Society Patient Registry showed significant differences in the clinical status of patients with CF across European countries. It is conceivable that these disparities in disease outcome result from delayed diagnosis, lack of specialized care, and limited access to medications. Survival rates are different even between countries in western Europe, which suggests variations in quality of care among countries. This difference in survival, as mirrored in the level of FEV1 expressed as a percentage of predicted and weight expressed in z score, varied up to 20% in the same age group between countries. It is well known that within richer countries as well, children in affluent families have a better outcome. Using data from the US Cystic Fibrosis Foundation Registry, investigators reported a significantly higher risk of death, impaired growth, and reduced pulmonary function in children from lower-income vs higher-income families, and family income was independently associated with mortality.,, Parameters of low SES such as low income, limited education, or single caregiver were associated with suboptimal growth and low pulmonary function at baseline and over 24 months. In the United Kingdom, Barr and colleagues used death registration data in England and Wales to show that this socioeconomic divide in premature mortality for CF has persisted with no substantial narrowing for over 4 decades. Subsequently, Taylor-Robinson and colleagues showed that children with CF who are from more disadvantaged areas have worse growth and lung function compared with children from more affluent areas.

In their epidemiologic study of CF, Schechter and colleagues demonstrated that CF health outcomes are correlated with the SES spectrum, but these disparities were not explained by differential use of health services or prescription for long-term therapy. Furthermore, patients with lower SES were prescribed IV antibiotics more frequently, which suggests that SES-related disparities in CF health outcomes do not appear to be explained by less aggressive treatment of pulmonary exacerbations. Poverty and social class likely contribute to differences in health outcomes but could not be completely accounted for in the available data. Understanding the origins of these disparities may allow the development of interventions that will improve outcomes and reduce health inequality.

In this issue (see page 380), Buu and colleagues expended the spectrum of causes of health disparities by comparing differences in mortality rates and risk factors for worse lung disease between Hispanic and non-Hispanic patients with CF in California. The Hispanic population is the second largest ethnic group in the United States, characterized by younger age, less education, and less wealth, and with a very large immigrant component. It has been already shown that the population of Hispanic people with CF from the entire United States has an increased prevalence of important risk factors for morbidity and mortality. Here, Buu and colleagues retrospectively analyzed the Cystic Fibrosis Foundation Patient Registry data of California residents who were diagnosed in or after 1991 and who were aged <18 years. They found that patients of Hispanic ethnic origin who had CF had a mortality rate 2.8 times higher than that of non-Hispanic patients. Furthermore, lung function measured by FEV1 was significantly lower for Hispanic patients than for non-Hispanic ones at age 6 years. However, the more severe disease among Hispanic patients with CF was independent of biological, diagnostic, clinical, and social factors that were previously shown to be associated with increased mortality. Furthermore, no difference was observed in access and use of CF center care, age at diagnosis, and nutritional status. The question thus remains, what causes the poorer outcome for Hispanic patients with CF? One reason could be modifier gene(s); however, this is unlikely since Hispanic people are not a homogeneous closed population. Hence, poverty and social class contributed to differences in health outcomes but could not be fully accounted for in the data. Since there was no difference in accessibility to CF centers and medicine, there must be additional contributing factors associated with ethnicity, such as adherence to treatment regimens, self-management, culture, health literacy, and English proficiency,, that contribute to acceleration of the disease process.

Significant ethnic differences in prognosis and clinical status have been reported. In the United States, patients of black descent have significantly poorer nutritional status and pulmonary function than their Caucasian counterparts. In the Western Cape region in South Africa, black children became infected with Pseudomonas aeruginosa significantly earlier than those of European descent and had a worse 5-year survival rate. Although socioeconomic factors were implicated in both studies, ethnicity and underrecognition were also linked. Since routine CF home management requires a complex, time-consuming treatment regimen, it is important to acknowledge that there will be certain sociocultural challenges in managing and interacting with patients from different ethnic, cultural, or spiritual backgrounds. To include a possible impact of cultural beliefs on adherence rates, caregivers need to make adjustments and spend more time ensuring that explanations about treatment rationales are properly conveyed and understood. Therefore, preventive and management strategies, as suggested by Buu and colleagues, may be both cost-effective and more culturally appropriate for Hispanic patients with CF and their families.

References

Cohen-Cymberknoh M. .Shoseyov D. .Kerem E. . Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am J Respir Crit Care Med. 2011;183:1463-1471 [PubMed]journal. [CrossRef] [PubMed]
 
Taylor-Robinson D.C. .Smyth R.L. .Diggle P.J. .Whitehead M. . The effect of social deprivation on clinical outcomes and the use of treatments in the UK cystic fibrosis population: a longitudinal study. Lancet Respir Med. 2013;1:121-128 [PubMed]journal. [CrossRef] [PubMed]
 
Westwood A.T. .Ireland J.D. . Children with cystic fibrosis in South Africa: an improving nutritional picture. J Trop Pediatr. 2000;46:119-121 [PubMed]journal. [CrossRef] [PubMed]
 
  :- [PubMed]journal
 
Buu M.C. .Sanders L.M. .Mayo J.A. .Milla C.E. .Wise P.H. . Assessing differences in mortality rates and risk factors between Hispanic and non-Hispanic patients with cystic fibrosis in California. Chest. 2016;149:380-389 [PubMed]journal
 
McCormick J. .Mehta G. .Olesen H.V. .Viviani L. .Macek M. Jr..Mehta A. . Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet. 2010;375:1007-1013 [PubMed]journal. [CrossRef] [PubMed]
 
Schechter M.S. .Margolis P.A. . Relationship between socioeconomic status and disease severity in cystic fibrosis. J Pediatr. 1998;132:260-264 [PubMed]journal. [CrossRef] [PubMed]
 
Schechter M.S. .Shelton B.J. .Margolis P.A. .Fitzsimmons S.C. . The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States. Am J Respir Crit Care Med. 2001;163:1331-1337 [PubMed]journal. [CrossRef] [PubMed]
 
O’Connor G.T. .Quinton H.B. .Kneeland T. .et al Median household income and mortality rate in cystic fibrosis. Pediatrics. 2003;111:e333-e339 [PubMed]journal. [CrossRef] [PubMed]
 
Balmer D.F. .Schall J.I. .Stallings V.A. . Social disadvantage predicts growth outcomes in preadolescent children with cystic fibrosis. J Cyst Fibros. 2008;7:543-550 [PubMed]journal. [CrossRef] [PubMed]
 
Barr H.L. .Britton J. .Smyth A.R. .Fogarty A.W. . Association between socioeconomic status, sex, and age at death from cystic fibrosis in England and Wales (1959 to 2008): cross sectional study. BMJ. 2011;343:d4662- [PubMed]journal. [CrossRef] [PubMed]
 
Schechter MS, McColley SA, Silva S, Haselkorn T, Konstan MW, Wagener JS. Association of socioeconomic status with the use of chronic therapies and healthcare utilization in children with cystic fibrosis.J Pediatr. 155(5):634-639.e1-e4.
 
Watts K.D. .Seshadri R. .Sullivan C. .McColley S.A. . Increased prevalence of risk factors for morbidity and mortality in the US Hispanic CF population. Pediatr Pulmonol. 2009;44:594-601 [PubMed]journal. [CrossRef] [PubMed]
 
Watts K.D. .Schechter M.S. . Origins of outcome disparities in pediatric respiratory disease. Pediatr Ann. 2010;39:793-798 [PubMed]journal. [CrossRef] [PubMed]
 
Farrell M.H. .Kuruvilla P. . Assessment of parental understanding by pediatric residents during counseling after newborn genetic screening. Arch Pediatr Adolesc Med. 2008;162:199-204 [PubMed]journal. [CrossRef] [PubMed]
 
Hamosh A. .FitzSimmons S.C. .Macek M. Jr..Knowles M.R. .Rosenstein B.J. .Cutting G.R. . Comparison of the clinical manifestations of cystic fibrosis in black and white patients. J Pediatr. 1998;132:255-259 [PubMed]journal. [CrossRef] [PubMed]
 
Westwood A.T. . The prognosis of cystic fibrosis in the Western Cape region of South Africa. J Paediatr Child Health. 1996;32:323-326 [PubMed]journal. [CrossRef] [PubMed]
 

Figures

Tables

References

Cohen-Cymberknoh M. .Shoseyov D. .Kerem E. . Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am J Respir Crit Care Med. 2011;183:1463-1471 [PubMed]journal. [CrossRef] [PubMed]
 
Taylor-Robinson D.C. .Smyth R.L. .Diggle P.J. .Whitehead M. . The effect of social deprivation on clinical outcomes and the use of treatments in the UK cystic fibrosis population: a longitudinal study. Lancet Respir Med. 2013;1:121-128 [PubMed]journal. [CrossRef] [PubMed]
 
Westwood A.T. .Ireland J.D. . Children with cystic fibrosis in South Africa: an improving nutritional picture. J Trop Pediatr. 2000;46:119-121 [PubMed]journal. [CrossRef] [PubMed]
 
  :- [PubMed]journal
 
Buu M.C. .Sanders L.M. .Mayo J.A. .Milla C.E. .Wise P.H. . Assessing differences in mortality rates and risk factors between Hispanic and non-Hispanic patients with cystic fibrosis in California. Chest. 2016;149:380-389 [PubMed]journal
 
McCormick J. .Mehta G. .Olesen H.V. .Viviani L. .Macek M. Jr..Mehta A. . Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet. 2010;375:1007-1013 [PubMed]journal. [CrossRef] [PubMed]
 
Schechter M.S. .Margolis P.A. . Relationship between socioeconomic status and disease severity in cystic fibrosis. J Pediatr. 1998;132:260-264 [PubMed]journal. [CrossRef] [PubMed]
 
Schechter M.S. .Shelton B.J. .Margolis P.A. .Fitzsimmons S.C. . The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States. Am J Respir Crit Care Med. 2001;163:1331-1337 [PubMed]journal. [CrossRef] [PubMed]
 
O’Connor G.T. .Quinton H.B. .Kneeland T. .et al Median household income and mortality rate in cystic fibrosis. Pediatrics. 2003;111:e333-e339 [PubMed]journal. [CrossRef] [PubMed]
 
Balmer D.F. .Schall J.I. .Stallings V.A. . Social disadvantage predicts growth outcomes in preadolescent children with cystic fibrosis. J Cyst Fibros. 2008;7:543-550 [PubMed]journal. [CrossRef] [PubMed]
 
Barr H.L. .Britton J. .Smyth A.R. .Fogarty A.W. . Association between socioeconomic status, sex, and age at death from cystic fibrosis in England and Wales (1959 to 2008): cross sectional study. BMJ. 2011;343:d4662- [PubMed]journal. [CrossRef] [PubMed]
 
Schechter MS, McColley SA, Silva S, Haselkorn T, Konstan MW, Wagener JS. Association of socioeconomic status with the use of chronic therapies and healthcare utilization in children with cystic fibrosis.J Pediatr. 155(5):634-639.e1-e4.
 
Watts K.D. .Seshadri R. .Sullivan C. .McColley S.A. . Increased prevalence of risk factors for morbidity and mortality in the US Hispanic CF population. Pediatr Pulmonol. 2009;44:594-601 [PubMed]journal. [CrossRef] [PubMed]
 
Watts K.D. .Schechter M.S. . Origins of outcome disparities in pediatric respiratory disease. Pediatr Ann. 2010;39:793-798 [PubMed]journal. [CrossRef] [PubMed]
 
Farrell M.H. .Kuruvilla P. . Assessment of parental understanding by pediatric residents during counseling after newborn genetic screening. Arch Pediatr Adolesc Med. 2008;162:199-204 [PubMed]journal. [CrossRef] [PubMed]
 
Hamosh A. .FitzSimmons S.C. .Macek M. Jr..Knowles M.R. .Rosenstein B.J. .Cutting G.R. . Comparison of the clinical manifestations of cystic fibrosis in black and white patients. J Pediatr. 1998;132:255-259 [PubMed]journal. [CrossRef] [PubMed]
 
Westwood A.T. . The prognosis of cystic fibrosis in the Western Cape region of South Africa. J Paediatr Child Health. 1996;32:323-326 [PubMed]journal. [CrossRef] [PubMed]
 
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