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Health Status and Healthcare Use in a National Sample of Children with Sickle Cell Disease

      Background

      There is a paucity of population-based data describing health status and use of health services among children with sickle cell disease (SCD).

      Purpose

      This study provides estimates of co-occurring conditions, health impact and utilization, and barriers to care for a national sample of children with SCD.

      Methods

      Data were derived from the 1997–2005 National Health Interview Survey Child Sample Core. The study included 192 children aged 0–17 years with SCD whose race was reported as black or African-American, and 19,335 children without SCD of the same age and race. Parents or other knowledgeable adults reported on medical and developmental conditions, health status, and healthcare use and access.

      Results

      After adjusting for demographic characteristics, black children with SCD had higher odds of frequent severe headaches or migraines, intellectual disabilities, regular use of prescription medication, and fair or poor health status compared with black children without SCD. While healthcare and special education services use were generally higher for black children with SCD than for black children in the general population, those with SCD also had higher odds of reporting delays in accessing health care.

      Conclusions

      The health burden for children with SCD and their families is profound and may be exacerbated by barriers to accessing comprehensive medical care. Additional study of the extent of unmet needs for U.S. children with SCD is warranted.

      Background

      Sickle cell disease (SCD) is a group of autosomal recessive hemoglobinopathies characterized by the presence of sickle hemoglobin in the red blood cells. In the U.S., SCD is one of the most common genetic disorders, affecting approximately 1 in 350 black births and 70,000 Americans, regardless of race.
      • Bonds D.R.
      Three decades of innovation in the management of sickle cell disease: the road to understanding the sickle cell disease clinical phenotype.
      Children with SCD are at increased risk for severe morbidity such as hemolytic anemia, splenic dysfunction, pain crises, and bacterial infections.
      American Academy of Pediatrics
      Health supervision for children with sickle cell disease.
      Studies of neurologic complications in children with SCD have shown that the condition can be associated with cognitive effects such as deficits in short-term memory and difficulties in verbal tasks that might be related to deficits in auditory processing.
      • Ashley-Koch A.
      • Murphy C.
      • Khoury M.J.
      • Boyle C.A.
      Contribution of sickle cell disease to the occurrence of developmental disabilities: a population-based study.
      • Schatz J.
      • Finke R.L.
      • Kellett J.M.
      • Kramer J.H.
      Cognitive functioning in children with sickle cell disease: a meta-analysis.
      • Schatz J.
      • Finke R.L.
      • Kellett J.M.
      • Kramer J.H.
      Short-term memory in children with sickle cell disease: executive versus modality-specific processing deficits.
      • Schatz J.
      • McClellan C.B.
      Sickle cell disease as a neurodevelopmental disorder.
      • Steen R.G.
      • Hu X.J.
      • Elliott V.E.
      Kindergarten readiness skills in children with sickle cell disease: evidence of early neurocognitive damage?.
      • Steen R.G.
      • Miles M.A.
      • Helton K.J.
      • et al.
      Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit.
      Visual problems and sensorineural hearing deficits in adults and occasionally in children with SCD have also been reported.
      • Downes S.M.
      • Hambleton I.R.
      • Chuang E.L.
      • Lois N.
      • Serjeant G.R.
      • Bird A.C.
      Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study.
      • Friedman E.M.
      • Luban N.L.C.
      • Herer G.R.
      • Williams I.
      Sickle cell anemia and hearing.
      • Onakoya P.A.
      • Nwaorgu O.G.
      • Shokunbi W.A.
      Sensorineural hearing loss in adults with sickle cell anaemia.
      • Serjeant G.R.
      Sickle-cell disease.
      • Talbot J.F.
      • Bird A.C.
      • Serjeant G.R.
      • Hayes R.J.
      Sickle cell retinopathy in young children in Jamaica.
      • Talbot J.F.
      • Bird A.C.
      • Maude G.H.
      • Acheson R.W.
      • Moriarty B.J.
      • Serjeant G.R.
      Sickle cell retinopathy in Jamaican children: further observations from a cohort study.
      Children with SCD are at risk for neurocognitive impairments affecting their academic performance because of pathophysiologic effects on their central nervous system.
      • Day S.
      • Chismark E.
      The cognitive and academic impact of sickle cell disease.
      • Thompson R.J.
      • Gustafson K.E.
      • Bonner M.J.
      • Ware R.E.
      Neurocognitive development of young children with sickle cell disease through three years of age.
      Whereas the medical risks related to SCD have been described in the literature, population-based studies on health and functional status, healthcare use, and access to care for children with SCD are lacking. Findings from the few studies available indicate that low-income children with SCD have higher levels of hospitalization, emergency department visits, and home health care compared with children without SCD.
      • Raphael J.L.
      • Dietrich C.L.
      • Whitmire D.
      • Mahoney D.H.
      • Mueller B.U.
      • Giardino A.P.
      Healthcare utilization and expenditures for low income children with sickle cell disease.
      • Shankar S.M.
      • Arbogast P.G.
      • Mitchel E.
      • Cooper W.O.
      • Wang W.C.
      • Griffin M.R.
      Medical care utilization and mortality in sickle cell disease: a population-based study.
      Healthcare costs for children with SCD are also reported to be higher than those for children without the disease.
      • Bilenker J.H.
      • Weller W.E.
      • Shaffer T.J.
      • Dover G.J.
      • Anderson G.F.
      The costs of children with sickle cell anemia: preparing for managed care.
      • Kauf T.L.
      • Coates T.D.
      • Huazhi L.
      • Mody-Patel N.
      • Hartzema A.G.
      The cost of health care for children and adults with sickle cell disease.
      • Nietert P.J.
      • Abboud M.R.
      • Zoller J.S.
      • Silverstein M.D.
      Costs, charges, and reimbursements for persons with sickle cell disease.
      However, these studies were based on administrative databases for particular states or regions and therefore may not be generalizable to all children with SCD. Moreover, there is a lack of information describing the overall scope and prevalence of co-morbidities for children with SCD and how these factors affect the health burden for these children and their families.
      Data from the 1997–2005 National Health Interview Surveys (NHIS) were used to describe health status and health services use among black children aged 0–17 years with SCD. The impact of SCD on U.S. children was evaluated by examining (1) the prevalence of co-occurring medical and developmental conditions; (2) indicators of functional status and perceived health status; (3) use of healthcare and special educational services; and (4) barriers to accessing healthcare services for a national sample of children with and without SCD. It was hypothesized that black children with SCD would have higher levels of co-occurring conditions and healthcare utilization than their counterparts without SCD.

      Methods

      The data used for this study were derived from the NHIS, an annual, multistage probability sample survey of the civilian non-institutionalized population of the U.S.
      • Botman S.L.
      • Moore T.F.
      • Moriarity C.L.
      • Parsons V.L.
      Design and estimation for the National Health Interview Survey, 1995–2004.
      From each family selected to be interviewed, one child aged <18 years was randomly selected as the subject for the Sample Child Core questionnaire, which solicits information about children's health status, functional limitations, and access to medical care. A knowledgeable adult in each family, usually a parent or legal guardian, was selected to provide a proxy response for the sample child.
      The study population consisted of children aged 0–17 years who participated in the Sample Child Core portion of the NHIS from 1997 through 2005 and whose race was reported as black or African-American. Children of other races were excluded because of the low prevalence of SCD. Children with SCD were identified by an affirmative response from the knowledgeable adult when asked: Has a doctor or other health professional ever told you that [child's name] had sickle cell anemia? The total unweighted sample consisted of 19,527 children, of whom 192 had SCD; sample sizes were stable over the 9-year study period.
      For the co-occurring medical conditions queried for this study, parents were asked whether the following conditions had occurred during the 12 months preceding the interview: anemia, asthma attack, hay fever, respiratory allergy, food or digestive allergy, eczema or skin allergy, frequent diarrhea or colitis, frequent severe headaches or migraines, and three or more ear infections.
      The developmental conditions evaluated in this analysis included attention-deficit disorder and attention-deficit/hyperactivity disorder (ADD/ADHD; aged ≥2 years only); learning disability (aged ≥3 years only); intellectual disability; trouble hearing; trouble seeing; and other developmental delay. All other developmental conditions were excluded because of low frequency in the SCD group. With the exception of trouble hearing and seeing, all conditions were considered to be present if the knowledgeable adult answered in the affirmative when asked: Has a doctor or health professional ever told you that [child's name] had [condition]. Trouble hearing was positively identified if the respondent indicated that deaf, a lot, or a little trouble hearing best described the child's hearing without a hearing aid. For children aged >2 years, trouble seeing was positively identified if the respondent indicated that the child had any trouble seeing even when wearing glasses or contact lenses. For younger children, the use of glasses or contacts was omitted from the question. Although the survey questionnaire uses the phrase mental retardation, the term intellectual disability is preferred and was therefore used in this report.
      • Turnbull R.
      • Turnbull A.
      • Warren S.
      • Eidelman S.
      • Marchand P.
      Shakespeare redux, or Romeo and Juliet revisited: embedding a terminology and name change in a new agenda for the field of mental retardation.
      Health impact was assessed via questions about functional status that were found to be associated with developmental disabilities in a previous study
      • Boulet S.L.
      • Boyle C.A.
      • Schieve L.A.
      Health care use and health and functional impact of developmental disabilities among U.S. children, 1997–2005.
      and are similar to the criteria used in other studies for identifying children with special healthcare needs.
      • van Dyck P.C.
      • Kogan M.D.
      • McPherson M.G.
      • Weissman G.R.
      • Newacheck P.W.
      Prevalence and characteristics of children with special health care needs.
      A parent report of the child's health at the time of the survey compared with the child's health 12 months ago was assessed. Healthcare use during the past 12 months was evaluated according to the types of providers seen (medical specialist other than an obstetrician/gynecologist, psychiatrist, or ophthalmologist; mental health professional such as a psychiatrist, psychologist, or clinic social worker; allied health professional such as a physical, speech, respiratory, occupational, or audiology therapist; and optometrist, ophthalmologist, or eye doctor) and the frequency of visits to a doctor's office or clinic (excluding hospital and emergency room visits) and to a hospital emergency room. Receipt of special education or early intervention services was also examined.
      Barriers to accessing medical care were assessed with a series of questions addressing delays in receiving medical care during the past 12 months. Reasons for delay included not being able to get through on the telephone, not being able to get an appointment for the child soon enough, waiting too long in the doctor's office, not having transportation, and not being able to afford prescription medicine when it was needed.
      All estimates were weighted to reflect the national, non-institutionalized population of black children aged 0–17 years. SUDAAN was used to generate all estimates, SEs, and two-tailed chi-square tests. Because the unweighted sample sizes for children with SCD were small for some of the health outcomes, the relative SE (RSE) was calculated to assess the reliability of the estimates. The RSE is defined as the SE estimate divided by the estimate itself; estimates with RSEs from 30% through 50% have high variability and are flagged in the tables. Estimates with RSEs >50% were not presented. Odds ratios were calculated using multivariable logistic regression models adjusted for year of survey; gender; child's age at the time of the survey; maternal education; household income (% federal poverty level); health insurance; and region of residence. Given the high percentages of people with missing or unknown family income data, NHIS multiply imputed data were used for estimates of household income.
      • Schenker N.
      • Raghunathan T.E.
      • Chiu P.
      • Makuc D.M.
      • Zhang G.
      • Cohen A.J.
      Multiple imputation of family income and personal earnings in the National Health Interview Survey: methods and examples.
      Effect modification was assessed by comparing all associations across strata based on child's age, gender, and household income (data not shown). No remarkable differences were noted; however, sample sizes were small, and many estimates were imprecise.

      Results

      The average annual prevalence of reported SCD among black children aged 0–17 years in the 1997–2005 NHIS was 10.5 per 1000 (SE=0.9, 95% CI=8.8, 12.4), and there was no evidence of a secular trend (data not shown). Black children with SCD had a higher predominance of boys than did those without SCD (Table 1). They were also more likely than their counterparts without SCD to have a household income <100% of the federal poverty level and to receive Medicaid or State Children's Health Insurance Program. Compared with black children without SCD, those with SCD had a greater proportion of mothers with less than a high school education; however, the difference was not significant.
      Table 1Demographic characteristics of black children with and without sickle cell disease
      Children with SCD (n=192)Children without SCD (n=19,335)
      Unweighted nWeighted %Unweighted nWeighted %
      Year of survey
       1997–19995734.2666139.3
       2000–20027334.6651929.5
       2003–20056231.1615531.2
      Child gender
       Male10559.1
      p<0.05 for chi-square test of distribution of variable in children with SCD compared with children without SCD.
      971150.5
       Female8740.9962449.5
      Child age (years)
       <33216.6327816.1
       3–52612.7316316.3
       6–105631.4526228.4
       11–177839.3763239.2
      Maternal education
      Maternal education information missing for 17 children with SCD and 2373 children without SCD.
       <High school3628289419.4
       High school/GED6132.4552432.9
       >High school7839.6854447.6
      Household income (% federal poverty level)
       <1007447.8
      p<0.05 for chi-square test of distribution of variable in children with SCD compared with children without SCD.
      580734.7
       100 to <2004423.3505226.3
       ≥2007428.9847339
      Insurance
      Insurance information missing for eight children with SCD and 604 children without SCD.
       Medicaid/SCHIP9456.2
      p<0.05 for chi-square test of distribution of variable in children with SCD compared with children without SCD.
      674439.7
       Private insurance7132.6928246.7
       Other insurance64.19755.3
       No insurance137.117308.3
      Region
       Northeast2111.4350317.4
       Midwest5021.6372120
       South10660.310,16754.2
       West156.719448.4
      SCD, sickle cell disease; SCHIP, State Children's Health Insurance Program
      a Maternal education information missing for 17 children with SCD and 2373 children without SCD.
      b Insurance information missing for eight children with SCD and 604 children without SCD.
      low asterisk p<0.05 for chi-square test of distribution of variable in children with SCD compared with children without SCD.
      Black children with SCD were nearly seven times more likely to have anemia, as expected, and were more than twice as likely to have frequent severe headaches or migraines during the past 12 months compared with black children in the general population (Table 2). These associations remained after adjustment for sociodemographic characteristics. Black children with SCD also had moderately increased risks for hay fever; food or digestive allergy; and frequent diarrhea or colitis; however, the sample sizes among such affected children were small, and the CIs for these associations included 1.0.
      Table 2Medical and developmental conditions among black children with and without sickle cell disease
      Children with SCD, weighted %Children without SCD, weighted %AOR
      Models adjusted for year of survey, gender, child's age at time of survey, maternal education, household income, insurance, and region of residence.
      for SCD vs no SCD (95% CI)
      Medical conditions (past 12 months)
       Anemia13.7
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups.
      2.07.2 (4.1, 12.8)
       Asthma attack5.36.10.8 (0.4, 1.5)
       Hay fever12.58.41.5 (0.9, 2.6)
       Any respiratory allergy11.410.81.0 (0.6, 1.7)
       Food/digestive allergy5.0
      Relative SE 30%–50%
      3.31.8 (0.8, 4.1)
       Eczema/skin allergy14.110.81.2 (0.7, 2.2)
       Frequent diarrhea/colitis2.8
      Relative SE 30%–50%
      1.52.2 (0.8, 5.8)
       Frequent severe headaches/migraines
      Among children aged 3–17 years only
      18.8
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups.
      6.82.5 (1.4, 4,5)
       Three or more ear infections9.25.21.5 (0.7, 3.5)
      Developmental conditions
       ADD/ADHD
      Among children aged 2–7 years only
      8.6
      Relative SE 30%–50%
      5.51.1 (0.5, 2.3)
       Learning disability
      Among children aged 3–17 years only
      17.6
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups.
      8.71.3 (0.7, 2.4)
       Intellectual disability
      Survey question states: Has a doctor or health professional ever told you that [child's name] had mental retardation?
      2.7
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups.
      Relative SE 30%–50%
      1.03.3 (1.1, 9.5)
       Trouble hearing8.8
      Relative SE 30%–50%
      3.02.6 (1.3, 5.1)
       Trouble seeing4.8
      Relative SE 30%–50%
      2.91.9 (0. 8, 4.5)
       Other developmental delay7.2
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups.
      3.21.7 (0.8, 3.4)
      ADD/ADHD, attention-deficit disorder and attention-deficit/hyperactivity disorder; SCD, sickle cell disease
      a Models adjusted for year of survey, gender, child's age at time of survey, maternal education, household income, insurance, and region of residence.
      b Relative SE 30%–50%
      c Among children aged 3–17 years only
      d Among children aged 2–7 years only
      e Survey question states: Has a doctor or health professional ever told you that [child's name] had mental retardation?
      low asterisk p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups.
      Generally, there was a trend of higher prevalence for all developmental conditions among black children with SCD than for those without SCD (Table 2). Only ADD/ADHD, intellectual disability, trouble hearing, and other developmental delay were significantly different in the bivariate comparisons. With the exception of intellectual disability and trouble hearing, these associations were no longer significant in the adjusted models.
      Black children with SCD had considerably higher estimates than their counterparts without SCD for nearly all measures of health impact, and healthcare and special education service use assessed in this study (Table 3). As expected based on healthcare guidelines regarding penicillin prophylaxis for children with SCD,
      American Academy of Pediatrics
      Health supervision for children with sickle cell disease.
      more than 30% of black children with SCD had taken prescription medication regularly for a specific problem during the past year, and more than 13% of parents of children with SCD reported fair or poor health status recently. In addition, more than 20% had recently visited a medical specialist, an optometrist, ophthalmologist, or eye doctor, and had visited the emergency department more than once in the past year. Approximately 13% of black children with SCD received special education or early intervention services. The majority of these associations remained significant or approached significance in the adjusted models.
      Table 3Health status and healthcare use for black children with and without sickle cell disease
      Children with SCD, weighted %Children without SCD, weighted %AOR
      Models adjusted for year of survey, gender, child's age at time of survey, maternal education, household income, insurance, and region of residence
      for SCD vs no SCD (95% CI)
      Health impact
       Limited in ability to crawl, walk, run, or play
      Child is limited in ability and limitation has lasted or is expected to last >12 months
      6.0
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      Relative SE 30%–50%
      2.03.4 (1.6, 7.1)
       Needed special equipment4.3
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      Relative SE 30%–50%
      0.82.3 (0.6, 8.0)
       Has taken prescription medication for ≥3 months31.0
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      10.73.1 (2.0, 4.7)
       Reported health status fair or poor13.3
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      3.34.0 (2.2, 7.5)
      Healthcare and special education services use (past 12 months)
       Saw medical specialist23.0
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      8.43.2 (2.1, 5.0)
       Saw mental health professional11.6
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      5.32.1 (1.1, 4.0)
       Saw therapist
      Includes physical, speech, respiratory, or occupational therapist or audiologist
      8.8
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      4.41.9 (0.9, 3.8)
       Saw optometrist, ophthalmologist, or eye doctor23.017.81.3 (0.9, 1.9)
       More than nine office visits12.8
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      5.22.7 (1.6, 4.5)
       More than one visit to emergency department23.4
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      9.72.9 (1.9, 4.4)
       Had surgery or medical procedure4.8
      Child is limited in ability and limitation has lasted or is expected to last >12 months
      3.71.6 (0.8, 3.4)
       Received special educational or early intervention services13.2
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      6.21.8 (1.0, 3.2)
      Healthcare barriers (past 12 months)
       Could not get through on phone7.5
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      Relative SE 30%–50%
      1.83.0 (1.4, 6.6)
       Could not get appointment soon enough10.5
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      3.92.6 (1.3, 5.0)
       Waited too long in doctor's office8.7
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      Relative SE 30%–50%
      4.02.6 (1.3, 5.2)
       No transportation7.6
      p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      Relative SE 30%–50%
      3.31.5 (0.7, 3.5)
       Could not afford prescription medicine6.2
      Relative SE 30%–50%
      3.91.3 (0.6, 2.8)
      SCD, sickle cell disease
      a Models adjusted for year of survey, gender, child's age at time of survey, maternal education, household income, insurance, and region of residence
      b Child is limited in ability and limitation has lasted or is expected to last >12 months
      c Relative SE 30%–50%
      d Includes physical, speech, respiratory, or occupational therapist or audiologist
      low asterisk p<0.05 for chi-square test of distribution of variable in SCD and no-SCD groups
      Overall, black children with SCD had more problems accessing health services than black children in the general population. Notably, 11% of parents indicated that they could not get an appointment for their child soon enough, 9% reported waiting too long in the doctor's office, and 8% could not get through on the telephone. These associations remained after adjustment.

      Discussion

      The findings presented in this report indicate that, compared with the general population of black children in the U.S., those with SCD are more likely to be affected by certain co-occurring medical and developmental conditions, have concomitant functional limitations, and use more healthcare services. Although black children with SCD had more visits to doctors' offices, medical specialists, and emergency rooms than their counterparts without SCD, their parents reported more problems accessing available healthcare services, such as difficulty in setting up an appointment, excessive wait times before seeing a doctor, and difficulty in arranging transportation to the site of a healthcare provider. This finding is particularly noteworthy given that specialized comprehensive medical care for children with SCD has been shown to decrease morbidity and mortality during childhood.
      American Academy of Pediatrics
      Health supervision for children with sickle cell disease.
      The weighted prevalence of SCD in this analysis, 10.5 per 1000, is higher than estimates of the birth prevalence of SCD,
      • Bonds D.R.
      Three decades of innovation in the management of sickle cell disease: the road to understanding the sickle cell disease clinical phenotype.
      but it is consistent with an estimate from another national survey.
      • Miller J.E.
      • Gaboda D.
      • Davis D.
      Early childhood chronic illness: comparability of maternal reports and medical records.
      The reason for the discrepancy is not known but may be due to inaccurate classification of children with sickle cell trait, or a lack of definitions of terms, or lists of related symptoms, in the survey questionnaire. Low educational attainment has been found to be associated with decreased accuracy of maternal report of asthma
      • Miller J.E.
      • Gaboda D.
      • Davis D.
      Early childhood chronic illness: comparability of maternal reports and medical records.
      and may have contributed to the over-reporting of SCD in the present study. If sickle cell trait were misclassified as sickle cell disease, our findings may underestimate the true health burden for children with SCD.
      Increased risks for intellectual disability,
      • Ashley-Koch A.
      • Murphy C.
      • Khoury M.J.
      • Boyle C.A.
      Contribution of sickle cell disease to the occurrence of developmental disabilities: a population-based study.
      cognitive impairment,
      • Schatz J.
      • Finke R.L.
      • Kellett J.M.
      • Kramer J.H.
      Cognitive functioning in children with sickle cell disease: a meta-analysis.
      • Steen R.G.
      • Miles M.A.
      • Helton K.J.
      • et al.
      Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit.
      • Steen R.G.
      • Fineberg-Buchner C.
      • Hankins G.
      • Weiss L.
      • Prifitera A.
      • Mulhern R.K.
      Cognitive deficits in children with sickle cell disease.
      and vision
      • Ashley-Koch A.
      • Murphy C.
      • Khoury M.J.
      • Boyle C.A.
      Contribution of sickle cell disease to the occurrence of developmental disabilities: a population-based study.
      • Downes S.M.
      • Hambleton I.R.
      • Chuang E.L.
      • Lois N.
      • Serjeant G.R.
      • Bird A.C.
      Incidence and natural history of proliferative sickle cell retinopathy: observations from a cohort study.
      • Talbot J.F.
      • Bird A.C.
      • Serjeant G.R.
      • Hayes R.J.
      Sickle cell retinopathy in young children in Jamaica.
      and hearing impairment
      • Ashley-Koch A.
      • Murphy C.
      • Khoury M.J.
      • Boyle C.A.
      Contribution of sickle cell disease to the occurrence of developmental disabilities: a population-based study.
      • Friedman E.M.
      • Luban N.L.C.
      • Herer G.R.
      • Williams I.
      Sickle cell anemia and hearing.
      among children with SCD have been reported previously. The etiology of the neurologic complications associated with SCD is not well understood but may be related to overt stroke or silent cerebrovascular infarctions.
      • Armstrong F.D.
      • Thompson Jr, R.J.
      • Wang W.
      • et al.
      Cognitive functioning and brain magnetic resonance imaging in children with sickle cell disease Neuropsychology Committee of the Cooperative Study of Sickle Cell Disease.
      • Wang W.
      • Enos L.
      • Gallagher D.
      • et al.
      Neuropsychologic performance in school-aged children with sickle cell disease: a report from the Cooperative Study of Sickle Cell Disease.
      Some research indicates that cognitive deficits can be present in children with SCD who have never had an ischemic event.
      • Steen R.G.
      • Miles M.A.
      • Helton K.J.
      • et al.
      Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit.
      • Schatz J.
      • White D.A.
      • Moinuddin A.
      • Armstrong M.
      • DeBaun M.R.
      Lesion burden and cognitive morbidity in children with sickle cell disease.
      Similar to cognitive deficits, the cause of SCD-related hearing loss is not well understood. One possible explanation for sensorineural hearing loss among patients with SCD is impaired blood flow in the cochlear venous system.
      • Serjeant G.R.
      Sickle-cell disease.
      Individuals with SCD have an increased risk for proliferative retinopathy caused by occlusion of the small blood vessels in the peripheral retina.
      • Emerson G.G.
      • Lutty G.A.
      Effects of sickle cell disease on the eye: clinical features and treatment.
      Because this condition can lead to severe vision impairment if left untreated, the American Academy of Pediatrics has recommended screening children with SCD for proliferative retinopathy beginning at age 10 years.
      American Academy of Pediatrics
      Health supervision for children with sickle cell disease.
      Parents of children with SCD encountered more challenges in accessing healthcare services than parents of children without SCD, including long waits at doctors' offices and difficulty getting appointments soon enough when their child was sick. During SCD crises, these prolonged waiting times in outpatient settings might complicate a child's health conditions and result in more frequent and longer periods of hospitalization. Unfortunately, the NHIS does not include questions regarding the reasons for perceived delays in accessing healthcare services. Previous studies have identified several reasons for such gaps in healthcare coverage among children with special medical needs such as limited health insurance coverage, inadequate reimbursement, and complex authorization policies for managed care services.
      • Newacheck P.W.
      • McManus M.
      • Fox H.B.
      • Hung Y.-Y.
      • Halfon N.
      Access to health care for children with special health care needs.
      • Fox H.B.
      • Newacheck P.W.
      Private health insurance of chronically ill children.
      • McManus M.
      • Flint S.
      • Kelly R.
      The adequacy of physician reimbursement for pediatric care under Medicaid.
      • Fox H.B.
      • Wicks L.B.
      • Newacheck P.W.
      Health maintenance organizations and children with special health needs A suitable match?.
      Noll et al.
      • Noll R.B.
      • McKellop J.M.
      • Vannatta K.
      • Kalinyak K.
      Child-rearing practices of primary caregivers of children with sickle cell disease: the perspective of professionals and caregivers.
      reported that parents of children with SCD, in particular, may experience more frequent biases in hospitals and other settings.
      The socioeconomic condition of people with SCD may be a factor affecting their use of treatments and access to care. In addition to poor overall health, children from lower-income families have been shown to have health services marked by less continuity, more unmet needs, and fewer visits outside of the emergency room, even after controlling for confounders such as health insurance status.
      • Larson K.
      • Halfon N.
      Family income gradients in the health and health care access of U.S. children.
      Both SCD patients' socioeconomic condition and their distance from the nearest clinic have been found to be associated with their use of health services.
      • Haque A.
      • Telfair J.
      Socioeconomic distress and health status: the urban–rural dichotomy of services utilization for people with sickle cell disorder in North Carolina.
      In our study, nearly half of the children with SCD had household incomes below 100% of the federal poverty level, and 6.2% of parents of children with SCD reported that they were not able to afford prescription medicine in the 12 months before the interview. An evaluation of the socioeconomic characteristics of SCD patients and families during 1979–1981 yielded differences similar to the current report, with SCD patients having lower median incomes and lower educational attainment than their black counterparts without SCD,
      • Farber M.D.
      • Koshy M.
      • Kinney T.R.
      Cooperative Study of Sickle Cell Disease
      Cooperative study of sickle cell disease: demographic and socioeconomic characteristics of patients and families with sickle cell disease.
      thereby suggesting that such factors have not changed considerably between the two time periods.
      These findings are subject to potential limitations. Because the data are collected through household surveys that rely on parent-reported information and are not confirmed by medical records, the data may be subject to response and recall bias. Validity studies of the NHIS have indicated that children's medical conditions associated with chronic disability are likely to be underreported by parents.
      • Jabine T.B.
      Reporting chronic conditions in the National Health Interview Survey A review of findings from evaluation studies and methodological test.
      However, other studies have suggested that parent report of health service use was accurate for acute service use among younger children
      • Souza-Vazirani D.
      • Minkovitz C.S.
      • Strobino D.M.
      Validity of maternal report of acute health care use for children younger than 3 years.
      and for more severe health conditions.
      • Daly K.A.
      • Lindgren B.
      • Giebink G.S.
      Validity of parental report of a child's medical history in otitis media research.
      There is also some evidence that parent report of dissatisfaction with care and problems with ease of using healthcare services is positively associated with the severity of the child's condition.
      • Ngui E.M.
      • Flores G.
      Satisfaction with care and ease of using health care services among parents of children with special health care needs: the roles of race/ethnicity, insurance, language, and adequacy of family-centered care.
      Another possible problem is that the NHIS access-to-care and service-use measures are applicable to the general population of children but do not measure the use of special services that are essential for children with chronic conditions such as SCD.
      The NHIS survey does not collect data regarding SCD genotypes, which are associated with the severity and frequency of SCD complications. The survey question asked specifically about sickle cell anemia and may have been misinterpreted by respondents who did not know the difference between sickle cell anemia and sickle cell disease. Outcomes for children with SCD who had a stroke or other brain injury could not be compared with those who did not. Because the data are limited to non-institutionalized children, some outcomes may be underestimated. U.S. Census Bureau data for 2000 indicate that only 0.2% of children were living under formally authorized, supervised care or in the custody of institutions.
      • DeNavas-Walt C.
      • Proctor B.D.
      • Lee C.H.
      U.S. Census Bureau
      Current population reports, P60–229, income, poverty, and health insurance coverage in the U.S.: 2004.
      Finally, the small number of children with SCD in our study sample and the low prevalence of certain outcomes resulted in some point estimates with high variability. Nonetheless, these data are useful and the comparisons valid when the findings are interpreted in the context of the corresponding confidence limits.
      Our results highlight the notable association among SCD, risk for comorbid medical and developmental conditions, and use of health services in a large, recent, and nationally representative sample, and they provide a more comprehensive assessment of the health impact of SCD on children and their families than previous studies. This study underscores the importance of screening children with SCD for cognitive, hearing, and vision deficits and providing them with timely treatments. These findings also indicate that children with SCD have a disproportionate amount of difficulty in accessing necessary healthcare services and confirm reports by parents of children with SCD who experienced difficulties in obtaining effective treatment for pain episodes, ensuring adequate nutrition, and managing academic and social issues that affect their children.
      • Ievers-Landis C.E.
      • Brown R.T.
      • Drotar D.
      • Bunke V.
      • Lambert R.G.
      • Walker A.A.
      Situational analysis of parenting problems for caregivers of children with sickle cell syndromes.
      The majority of children with SCD live in low-income households, and, as a result, these families may face stressors in addition to chronic health concerns, such as limited access to quality health care, substandard housing, lower-quality daycare centers and schools, and neighborhood crime. These factors have been shown to have independent and combined effects on parenting stress, child development, and disease processes.
      • Evans G.W.
      The environment of childhood poverty.
      Additional studies are necessary to explore the extent of unmet health needs for children with SCD as well as the interaction among unmet needs, family functioning, and long-term health outcomes for this population of children.
      The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the CDC.
      No financial disclosures were reported by the authors of this paper.

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