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A pilot study to explore knowledge, attitudes, and beliefs about sickle cell trait and disease

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Abstract INTRODUCTION: In the United States, newborn screening programs universally identify newborns with sickle cell disease (SCD) and heterozygote carriers (sickle cell trait [SCT]). Although there is a consensus to disclose SCT to parents, there
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  See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/40646886 A Pilot Study to Explore Knowledge, Attitudes,and Beliefs about Sickle Cell Trait and Disease  Article   in  Journal of the National Medical Association · November 2009 DOI: 10.1016/S0027-9684(15)31113-5 · Source: PubMed CITATIONS 27 READS 681 3 authors , including:Kruti AcharyaUniversity of Illinois at Chicago 16   PUBLICATIONS   133   CITATIONS   SEE PROFILE Lainie RossUniversity of Chicago 253   PUBLICATIONS   3,951   CITATIONS   SEE PROFILE All content following this page was uploaded by Lainie Ross on 28 November 2016.The user has requested enhancement of the downloaded file. All in-text references underlined in blueare linked to publications on ResearchGate, letting you access and read them immediately.  JOURNAL OF THE NATIONAL MEDICAL ASSOCIATIONVOL. 101, NO. 11, NOVEMBER 2009 1163 o r i g i n a l   c o m m u n i c a t i o n IntroductIon P opulation hemoglobinopathy screening pro-grams identify individuals with sickle cell disease (SCD), heterozygote carriers of sickle hemoglo- bin known as sickle cell trait (SCT), and those with oth-er hemoglobin variants. Individuals with SCD have an increased risk of morbidity and mortality secondary to infections, painful vaso-occlusive crises and strokes. In comparison, individuals with SCT are relatively asymp-tomatic but can develop vaso-occlusive sequelae in extreme conditions (ie, severe dehydration). 1,2  However, in general, the main focus of carrier identification is its reproductive implications. Screening for SCT began in the 1970s. 3,4  Early pro-grams focused on wide-scale community screening and retrospectively have been criticized because of the confu-sion caused by the misunderstandings of what it means to  be a carrier vs to be an affected individual. 3-6  Population screening for SCD in newborns became the standard of care after Gaston et al demonstrated in 1986 that penicil-lin could prevent severe morbidity and mortality in infants with SCD. 7  Whereas only a few states screened newborns for hemoglobinopathies in the early 1980s, by 1988, six-teen states had universal newborn screening hemoglobin-opathy programs and 14 had targeted screening programs or had a program in development. 8  By 1994, forty-two states were screening newborns for hemoglobinopathies, 9  with variable policies and practices regarding the disclo-sure of SCT detected in the newborn period. 10-15  In 1994, the Institute of Medicine report entitled  Assessing Genetic  Risk   argued effectively in favor of disclosure on the grounds that carrier information belonged to the child and his or her family. 9 Despite the consensus to disclose, there remains wide variability in how and by whom familial notification occurs. 16,17 Despite 20 years of newborn screening, there are no data that have examined the extent to which the knowl-edge of sickle cell carrier identification is correctly transmitted to children who are identified in the new- born period and what they are told about its health and reproductive implications. Understanding parental knowledge about sickle cell inheritance, its health, and reproductive implications; the stigma in the community Author Affiliations:  Departments of Medicine (Dr Ross, Carolyn and Mat-thew Bucksbaum Professor of Clinical Medical Ethics), Pediatrics (Dr Ross, professor; Dr Acharya, assistant professor), Surery (Dr Ross, professor), and the Collee (Dr Ross), MacLean Center for Clinical Medical Ethics (Dr Ross, associate director; Dr Acharya, faculty), University of Chicao (Ms Lan, research assistant), Chicao, Illinois. corresponding Author:  Kruti Acharya, MD, 950 E 61st St, SSC Room 207, Uni-versity of Chicao, Chicao IL 60637 (kacharya@peds.bsd.uchicao.edu). Funding/Support:  This work was funded in part by a rant from the Illinois Department of Public Health. Introduction:  In the United States, newborn screenin pro-rams universally identify newborns with sickle cell disease (SCD) and heterozyote carriers (sickle cell trait [SCT]). Althouh there is a consensus to disclose SCT to parents, there are limited empirical data about whether and how this information is transmitted to the carrier children.  Methods:  In-person questionnaires were administered to parents with SCT and parents of a child with either SCD or SCT to examine the knowlede, attitudes, beliefs, and dis-closure patterns about SCT of parents. Results:  Fifty-three adults were interviewed, half (27) of whom had a child with SCD. There was sinificant misunder-standin about sickle cell inheritance (mean score, 68%), but parents who have a child with SCD have better knowl-ede compared to those without a child with SCD (78% vs 58%, p  = .002). Respondents perceive minimal stima associ-ated with SCT. Unless there is an affected proband, individu-als with SCT rarely receive counselin or education outside of the family. Conclusions:  There is sinificant misinformation about what it means to be a carrier and its health and reproductive impli-cations. Formal professional counselin is rare, especially for those families without an affected proband. Strateies to increase the utilization of counselin and improve enetic literacy are necessary. Keywords:  sickle cell anemia 󰁮  knowlede, attitudes and beliefs 󰁮  stima, discrimination  J Natl Med Assoc.  2009;101:1163-1172 A Pilot Study to Explore Knowlede, Attitudes, and Beliefs about Sickle Cell Trait and Disease Kruti Acharya, MD; Colleen Walsh Lan, BA, BS; Lainie Friedman Ross, MD, PhD  1164  JOURNAL OF THE NATIONAL MEDICAL ASSOCIATIONVOL. 101, NO. 11, NOVEMBER 2009KNOWLEDgE, ATTITUDES, AND BELIEFS ABOUT SICKLE CELL towards carriers; and the use of formal genetic educa-tional resources by adults and children are important determinants in the correct transmission of this informa-tion. The aims of our study were to assess knowledge, attitudes, beliefs, and disclosure patterns about SCD and SCT among adults with SCT and parents of a child who has SCD or SCT. MethodS In-person interviews were conducted with a conve-nience sample of adults who themselves were sickle cell carriers or who had a child with SCT. Participants were either (1) biologic parents of a child with SCD who by definition are obligate sickle cell carriers (group A); (2)  parents of carrier infants identified through the newborn screening program, who were either sickle cell carriers, noncarriers, or had unknown sickle cell status (group B); or (3) carrier parents who just had a newborn but did not yet know their newborn’s hemoglobinopathy screen-ing results (group C). Individuals were excluded if they were aged less than 18 years old, non-English speaking, or had SCD themselves. Parents were recruited from (1) the University of Chicago sickle cell disease clinic, where their children are patients; (2) the Sickle Cell Disease Association of Illinois (SCDAI), a nonprofit organization dedicated to support and outreach for families with SCD and SCT; (3) a federally qualified health clinic affiliated with the University of Chicago; and (4) the University of Chi-cago postpartum inpatient unit. Respondents were recruited through flyers, in person, or by phone.A structured questionnaire was developed and piloted for the interview based on input from pediatricians, health care professionals interested in the intersection of genet-ics and public health, and community sickle cell advo-cates from SCDAI. These experts reviewed the question-naire for both face validity and understandability. The final questionnaire (Appendix) covered 10 top-ics, only 5 of which will be discussed in this manuscript: (1) respondents’ and children’s SCT status and disclo-sure patterns to children, (2) knowledge about SCD, (3) sources of knowledge, (4) health orientation to SCT, and (5) perceived stigma and experiences with discrimina-tion. We also collected and report demographic informa-tion, including gender, age, marital status, education, receipt of public assistance, age at birth of first child, the respondent’s sickle cell status, and the sickle cell status of all of their children.Intrafamilial disclosure focused on whether the par-ent had disclosed or planned to disclose the  parent’s  own status to the children, whether the parent had disclosed or planned to disclose the child’s  status to the child, and the age at which these disclosures would occur or had occurred. Parents were also asked the frequency of dis-cussions about sickle cell with each child, and whether each child had received counseling about sickle cell from someone other than a relative.Knowledge about SCD inheritance was measured using 10 true-false questions about sickle cell inheri-tance and the meaning of screening results. Most items were adapted from a survey administered to Nigerian college graduates. 18  Sources of information about sickle cell were selected from a list of 15 sources; respondents could choose as many as applied. Health orientation to SCT was measured using a Health Orientation scale (HOS) that consisted of 12  pairs of an adjective and its opposite (ie, good-bad) posi-tioned as anchors on a 5-point Likert scale. The HOS was first developed and evaluated by Wooldridge and Murray 19  in 1984 to examine perceptions of being sickle cell carriers by both carriers and noncarriers. We used the HOS to answer 2 separate questions: (1) How would you describe your own feelings when you consider that you have SCT? (2) How do you think most people feel about having SCT? The HOS was presented visually, and subjects were asked to complete each item after it was read aloud. Perceived stigma was measured by 8 items on a 5-point Likert scale that was adapted from a standardized stigma scale that had been developed by Sowell et al to examine stigma related to HIV. 20  Personal experience with discrimination related to SCT was assessed using 3 items about insurance and employment discrimination. All sections were presented to individuals in group A. Parents who were not carriers themselves were not asked about the timing and disclosure of the  parent’s  noncarrier status to their children. In addition, noncarrier parents were not administered the HOS and were not asked about  perceived stigma/discrimination. If the child was a non-carrier or had unknown sickle cell status, the parents were not asked about the timing and disclosure of the child’s  status. In all but the knowledge section, subjects were able to ask questions or ask for clarification.The University of Chicago institutional review board approved the project and waived written consent because the data were to be anonymous and the consent form would have been the only link to their identity. Rather,  participants gave oral consent to this minimal risk sur-vey, which was administered orally. Each question was read aloud to the subject by a trained research assistant or 1 of the authors. Subjects were able to skip questions or stop the interview at any time but were encouraged to guess on the knowledge true-false questions. Subjects were given $10 as a token of appreciation for participa-tion if the interview was done while they were waiting for a clinic visit. Participants who made a separate appointment to take the questionnaire received $25 plus transportation costs.Statistical analyses were conducted using SPSS for Windows version 16.0 (SPSS Inc, Chicago, Illinois.) First, descriptive statistics were generated for each sur-vey measure. For statistical analysis, we excluded  JOURNAL OF THE NATIONAL MEDICAL ASSOCIATIONVOL. 101, NO. 11, NOVEMBER 2009 1165 KNOWLEDgE, ATTITUDES, AND BELIEFS ABOUT SICKLE CELL responses that were left blank or marked as “not sure” (except for the knowledge true-false questions where a “not sure” or skipped question was combined with incorrect responses). Discussion frequency was catego-rized as either frequently (monthly or more) or not fre-quently (a few times or less). In the analysis about dis-closure in childhood, we coded answers with specific ages into 2 age groups (<13 years, and ≥ 13 years). We coded responses of “when they are able to have children and/or sex” as ≥ 13 years of age, but we were unable to code responses such as “when they can understand.” Responses to the stigma questions were averaged to obtain a stigma score for each individual. Answers of 4 respondents were excluded from the stigma/discrimina-tion portion of the survey because they answered “dis-agree” for all 8 stigma statements despite the fact that 2 questions were negatively framed for internal validity. Bivariate analyses using the c 2  test were used to com- pare the demographics of participants with the sickle cell status of their child. The c 2  test was also used to compare children with SCD and those with SCT with respect to the age at which the parents disclosed the child’s sickle cell status, the frequency of sickle cell discussions  between parent and child, and the receipt of nonfamilial counseling. Bivariate analyses were also conducted  between each possible source of information and whether the parent had or did not have a child with SCD. Mean values for HOS, knowledge, and stigma sections were compared with all demographic variables using an inde- pendent samples t   test for each variable. For questions in which 2 or more of the demographics were significant, a linear regression was performed with all individually sig-nificant variables to identify colinearity. Bivariate analy-ses of demographics using the c 2  test were performed to determine rates of correct responses for each individual knowledge question. Similarly, independent t   tests were used to compare HOS means between self and others for each individual adjective pair. reSultS Fifty-three subjects were interviewed: 27 from group A (with a child with SCD) and 26 from groups B and C (without a child with SCD). The mean age of respon-dents was 32 ± 9 years. All respondents self-identified as African American or non-Hispanic. The majority identi-fied a Christian tradition as their religion (n = 42 or 81%), but 10 (19%) had no religious affiliation. The majority of respondents were unmarried (n = 41 or 77%) women (n = 49 or 93%) living in households where at least 1 member was receiving some type of public assis-tance (n = 37 or 71%). One-fifth of respondents did not graduate high school (n = 11 or 21%) and 9% (n = 5) were college graduates. The average age at birth of a first child was 21 ± 6 years. Each of the designated groups (A,B, and C) were similar in demographic composition except that participants in group A were more likely to  be older and married, but there were no differences with respect to participant’s gender, age at birth of first child, education, having a religious affiliation, or receipt of  public assistance (data not shown). As illustrated in Table 1, the majority of our respon-dents were sickle cell carriers with approximately half having a child with SCD (n = 27 or 51%). Respondents most commonly learned their sickle cell status during  prenatal screening (36%) or by positive newborn screen-ing of their own child (26%). The majority of our sample tab 1.  Sickle Cell Trait Status   % a Do you know whether you have sickle trait?Yes, I have trait 47 89%Yes, I do not  have trait 3 6%No, I don’t know my status 3 6%When/how did you become aware of your sickle status?Prenancy screen 19 36%After my child had a positive newborn screenin 14 26%My own newborn screenin 6 11%Childhood testin, ae unknown (“My mom told me”) 4 8%Childhood screenin (9-12 y) 3 6%Other 4 8%Not aware of sickle cell status 3 6%Do you have a child with sickle cell disease?Yes 27 51%No 26 49%What is your child’s sickle status? At least 1 child with sickle cell disease 27 51%At least 1 child with sickle cell trait b  22 b  42% b No children with sickle cell disease or trait 13 25% a  Percentaes do not always add up to 100% due to roundin. b  9 families have both at least 1 child with sickle cell disease and at least 1 child with sickle trait.  1166  JOURNAL OF THE NATIONAL MEDICAL ASSOCIATIONVOL. 101, NO. 11, NOVEMBER 2009KNOWLEDgE, ATTITUDES, AND BELIEFS ABOUT SICKLE CELL had either a child with SCD or SCT, although 13 (25%) respondents (all from the University of Chicago post- partum inpatient unit) either did not have a child with SCD or SCT, or did not know their child’s sickle cell status.The 53 adults interviewed had 145 total children. Of those children, 31 had SCD, 27 had SCT, 75 were known to be healthy noncarriers, 7 were of unknown sickle cell status, and no responses were given about 5 children. Of the 47 parents with SCT only 1 parent stated that she had not told and would not tell any of her children that she had SCT, and another 4 parents did not respond to the ques-tion of whether they would tell their children their own status. Of the 58 children with SCD and SCT, 45 children (18 with SCT and 27 with SCD) had or would be told their own sickle cell status, whereas 13 children (9 with SCT and 4 with SCD) had not been told and their parents did not state whether or when they would do so. Thirty-eight of the 45 (84%) children learned or would learn their sickle cell status before the age of 13. Children with SCD were more likely to learn they had SCD before ado-lescence than those with trait (26/27, 96% vs 12/18, 67% respectively,  p  < .05). Five children with SCT learned or will learn in adolescence (28%), and 1 child with SCT and 1 child with SCD will learn “when they are old enough.” Children with SCD have more frequent discus-sions about sickle cell with their parents compared to children with SCT (24/27, 89% vs 6/21, 29% respec-tively,  p  < .001). Approximately half of children with SCD have received sickle cell counseling or education from someone other than a relative (13/24 or 54%), whereas only 1 child with SCT has received counseling or education beyond the family (1/19 or 5%;  p  < .01). There was significant misunderstanding about how SCD is inherited, with a mean score of 68% (Table 2). Only 12 individuals (12/53 or 23%) responded correctly to 9 or 10 of the 10 items. There was greater understand-ing among parents who had a child with SCD than those without a child with SCD (78% vs 58%,  p  = .002). No other demographic factor had significant impact on average knowledge scores (data not shown).Sources of information about SCD were diverse (Fig-ure 1). The most common sources of information for par-ents with a child with SCD were pediatricians (24/27 or 89%), SCD clinic staff (24/27 or 89%), and SCDAI employees (21/27 or 78%). For parents who did not have tab 2.  Knowlede of Sickle Cell genetics and Testin a  % c Asws A rspsci Wi Sik c disasA hay ci n = 53n = 27n = 26 Sickle cell disease can be transmitted by physical contact with an affected person (false). b 89%100%77%Sickle cell disease is a enetic condition (true). b 89%100%77%Over time, people with sickle cell trait can develop sickle cell disease (false).77%89%65%Sickle cell disease can be inherited only when both parents have trait (true).77%85%69%A positive sickle cell carrier test means (A)A. S/he definitely has the trait74%74%73%B. S/he is very likely to have the traitSickle cell disease can be inherited if one parent has the trait and the other parent does not (false).62%70%54%You can have sickle cell trait even if both of your parents do not (false). b 60%78%42%People with sickle cell trait have a mild form of sickle cell disease (false).59%60%58%A neative sickle cell carrier test means (B)A. S/he is very unlikely to have the trait.55%63%46%B. S/he definitely does not have the trait.Sickle cell disease can be inherited if one parent has sickle cell disease, even if the other parent does not have the trait (false). b 40%59%19%Averae percent correct on all 10 questions. b 68%78%58% a  The answer “not sure” was coded as an incorrect response b   p < .05 between parents who have a child with sickle cell disease and those with all healthy children.
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