Youth with Sickle Cell Disease: Genetic and Sexual Health Education Needs

Abstract

Objectives

Assess the need for and interest in a sexual health and sickle cell disease (SCD) inheritance educational program for youth with SCD.

Methods

Using a cross-sectional approach, qualitative data were collected during interviews of youth with SCD between ages 11–19 years from an urban hospital. Inductive and emergent coding was used to identify themes. Frequencies were recorded and analyzed.

Results

Thirty-five youth patients were approached; 20 (57%) consented and completed interviews. Half of the participants were adolescent girls. The mean age was 16.9±1.8 years (range 13–19). Of the 20 participants, 100% expressed interest in SCD genetic counseling and/or posed questions regarding SCD inheritance. Nineteen (95%) demonstrated deficits in sexual health knowledge and/or requested sexual health education. Seventeen (85%) actively engaged with educational materials. Twelve (60%) reported engaging in sexual activity, 7 (35%) in risky behaviors, and 3 (15%) reported past sexually transmitted infection (STI) diagnosis.

Conclusions

Youth with SCD demonstrated limited knowledge regarding sexual health and inheritance of SCD. Topics like information-seeking, limited knowledge, and need for social skills training provide evidence for the creation of an educational intervention for this vulnerable population. This program shows initial feasibility for youth with SCD.

Sickle cell disease (SCD) is an autosomal recessive blood disorder affecting approximately 100,000 individuals, primarily African Americans, in the United States (US).¹ This genetic condition causes multisystem morbidities including pain, infection, and stroke.^2–4 Sickle cell trait (SCT) is found in approximately 1 in 12 African Americans in the US.¹ Although individuals with SCT usually do not experience SCD symptoms, these individuals can pass SCT to their children, resulting in either SCT or SCD.^2,3 Despite universal newborn screening, the rate of SCD has not decreased over the past 20 years in the US.¹ Life expectancy for individuals with SCD has risen from 20 years in 1970 to approximately 40 years in 2005.⁵ This increase in life expectancy is thought to be due to newborn screening, prophylactic penicillin, and vaccinations.⁶

SCD Education

The majority of people with SCD will live into their childbearing years; therefore, individuals will make reproductive decisions about having a child and passing along SCD or SCT to their child.⁶ Limited knowledge among patients about the genetic heritability of SCD and SCT may decrease informed decision making when considering or planning pregnancy.^7–9 This limited comprehension regarding the genetic inheritance of SCD highlights the need for accurate and accessible education regarding the inheritance and reproductive implications of having a child when either parent has SCD or SCT.⁹

Sexual Health

There is a significant health disparity for African-American youth, who are disproportionately affected by sexually transmitted infections (STIs) through unprotected sexual activity.^10,11 In the city of St. Louis, MO, which represents the urban area of St. Louis and is separate from St. Louis County, the average rate of gonorrhea infection was nearly 18 times higher and the average rate of chlamydia infection was 13 times higher for African -American city residents compared to white city residents between 2006–2010 (904 per 100,000 vs 51 per 100,000 and 1766 per 100,000 vs 130 per 100,000).¹¹ In 2009, 15–24 year-olds accounted for 67% of gonorrhea and 73% of chlamydia cases in St. Louis, MO.¹¹

Missouri and African-American youth have higher teen pregnancy rates compared to national averages.^12,13 In addition, whereas African-American youth more frequently report using condoms during their last sexual intercourse, they also report risk behaviors, like early first sexual intercourse and 4 or more sexual partners, at higher rates when compared to their matched white and Hispanic peers.¹⁴ These risk behaviors are associated with unintended pregnancy, unsafe/unreliable contraception, and more sexual partners, potentially increasing STI exposure.^15–18

In Missouri public schools, HIV education is mandated, but comprehensive sex education is not.¹⁹ If education occurs, the curriculum must stress abstinence without requirements to be culturally relevant and unbiased.¹⁹ Prevention without accurate education is difficult and demonstrates a need for developing a program that will alleviate this striking public health challenge.

Risky Behaviors and Sickle Cell Disease

Youth with chronic conditions, like SCD, experience additional emotional, social, and physical threats during adolescence compared to their healthy peers.^20–22 For example, emotional support from peers may be limited, due to frequent school absences, and may contribute to the likelihood of engaging in risky behaviors as mechanism for increasing peer interaction.^20,21 Furthermore, due to the physical complications of SCD, youth with SCD may present with later onset of sexual activity compared with physically healthy peers, but they still engage in risky sexual behaviors.^22,23 Approximately 50% (60% of adolescent boys and 40% of adolescent girls) of youth 15–19 years with SCD report engaging in sexual activity.²² In a separate study of sexually active youth with SCD, 51% reported sexual activity before 15 years and 54% reported more than 3 lifetime partners.²³ This suggests that chronic conditions may not reduce youth engagement in risky behaviors.²³ Currently, these risky behaviors are rarely addressed by healthcare providers for youth with chronic conditions despite their repeated exposure to the healthcare system.²⁴

Given the disparities in sexual health and inheritance education, it is imperative to address this barrier by developing a risk-reduction program for this vulnerable population.²⁵ We set out to create a program to facilitate population health in youth with SCD, reducing these marked health disparities.²⁶ The primary goal of this project was to assess the interest and baseline behaviors, knowledge of sexual health, and awareness of the inheritance pattern of SCD among patients of an urban children’s hospital. A secondary goal was to measure the feasibility of an educational program by assessing acceptance by the patient population, patient interest, and potential for integrating an educational program into the existing hospital environment.²⁷ Feasibility was defined as a minimum of 50% participation by those recruited. We identified >50% participation to assess acceptance and interest as a feasibility measure to determine whether this intervention is viable for efficacy testing in the future.²⁷

METHODS

Participants

We used a cross-sectional approach for this qualitative investigation. Participants were recruited from the clinic population. We attempted to recruit all eligible patients who met our inclusion criteria, although most patients were recruited during weekday clinic hours. Patients were approached in an outpatient hematology clinic setting or during an inpatient hospital stay.

Inclusion criteria were: (1) diagnosis of SCD; (2) age 11–19 years; (3) informed consent/assent; and (4) ability to complete a 30-minute semi-structured interview based on self-report. Individuals were excluded it: (1) minors who were unable to obtain parent/guardian consent; and (2) inability to read or understand English. A $10 gift card was provided for participation.

After consent/assent, the parent/guardian completed a demographic form that included age, ethnicity, sex, SCD genotype, education/current grade level, employment, and insurance provider (public or private) as a measure of socioeconomic status. This additional data collection instrument was used to collect information to capture more complete demographic information of the child. The coordinator collected participants’ SCD genotype and insurance type from patient medical records and compared them to the self-reported demographic form.

Interviews

A semi-structured interview lasting approximately 20–40 minutes was conducted individually with youth by the study coordinator. The private interviews were adapted from The Sisters Informing Sisters about Topics on AIDS (SISTA) program developed by the Centers for Disease Control and Prevention (CDC).^28–30

Interviews began on a neutral subject to build rapport. The interviewer then explained the project using a standardized script, transitioning into the semi-structured interview (Table 1). Topics and questions discussed during the semi-structured interview covered sexual behaviors, knowledge, and interest in an educational program. Participants were encouraged to direct the interview toward the topics they found most interesting and relevant. As new topics emerged during the interviews, questions were added. For example, discussing sexual health education in school was not an initial question developed for the semi-structured interviews, but emerged through the interview process and was covered in all interviews. At the completion of the semi-structured interview, the interviewer provided educational materials sexual health and SCD inheritance. A genetic education diagram was provided and youth were given an explanation of the diagram and encouraged to ask questions.

Table 1.

Semi-structured Interview Topics and Questions Based on SISTA Questionnaire

Topic	Questions
Knowledge of Sex/ Sexual Behaviors	Do you know sex is? What is it?
Sexual Health/Sexual Health Education	What are condoms? Can you tell me about sex ed in school?
Risk-taking Behaviors	Have you used alcohol? When? Have you used drugs?
Knowledge of Sickle Cell Disease Genetics	What type of sickle cell disease do you have? Does your mom have sickle cell disease or trait?
Educational Programming	What would you like in a SCD genetic education program? A sexual health education program?

Data Analysis

Transcribed text from the semi-structured interviews and field notes were used for analysis. Our approach was based on principles from grounded theory using inductive and emergent coding strategies.³¹ This process involved continual evaluation and comparison throughout data collection and initial coding for our interpretation of the data. We started with open coding of the transcribed text, followed by thematic coding identifying common categories while constantly comparing texts. The research team assembled to finalize the codebook and explore the relationships among categories and data using axial coding strategies. Selective coding was used to describe detail, clarify, and finalize categories. Upon completion of coding, the team discussed the meaning and interpretation of coding categories.

Each interview had the same primary coder (AH) and used an additional secondary coder. A total of 3 secondary coders were used (JD, KS, DJ). A codebook was created and modified as needed. Few disagreements in coded text were identified, but the primary (AH) and secondary coder referenced the codebook to come to a consensus and resolve discrepancies. A code tie-breaker (AK) was used as needed. Field notes were used to clarify emotive force and other dynamics not captured in the transcriptions of the audio recordings. This approach was chosen because of its rigor and fit with the purpose of the project. Frequencies were determined from demographic data using SPSS (IBM Corporation, Armonk, NY, USA) and Microsoft Excel (Microsoft Corporation, Redmond, WA, USA).

RESULTS

Feasibility

Thirty-five patients were approached and all 20 (57%) participants consented if aged 18 years or older, or assented with parental consent, if aged less than 18 years. Recruiting in the outpatient clinic yielded approximately the same consent rate of 57% as those recruited in the inpatient setting (16 consented/assented of 28 approached inpatient; 4 consented/assented of 7 approached outpatient). Time constraints were the reason for most declines. As the age range of patients increased, the rates of participation increased. Three patients between 11–13 years were approached and one consented to participate. Eighteen and 19 year-olds were able to provide consent for themselves (10 of 12 patients 18–19 years approached for the study consented to participate). The overall rate of participation was 57%, indicating feasibility.

Youth Demographic Characteristics

Youth also completed a demographic form (Table 2). Mean age was 16.9 ± 1.8 years (range 13–19), and 50% were adolescent girls. Three youth (15%) did not know their type of SCD. All participants self-identified as African-American or African-American with another race/ethnicity. Approximately 75% of participants were enrolled public insurance (Medicaid, Medicare, and/or Social Security) indicating that the majority of participants demonstrated financial limitations sufficient to qualify for public insurance, which is consistent with the local and national pediatric SCD population.^32,33 Participants across all age ranges reported sexual activity.

Table 2.

Descriptive Characteristics of Youth Participants

Participants (N = 20)	Frequencies N (%)
Youth Participant Age in Years Mean ± Standard Deviation; (range)	16.9 ± 1.8 (13–19)
Sex of Youth Participants	10 (50%) adolescent girls
SCD Genotype	15 (75%) persons with HbSS 3 (15%) did not know genotype
Race/Ethnicity	20 (100%) self-identified African American/ African American with another race/ethnicity
Insurance	15 (75%) Medicaid
Reported Sexual Activity
13–15 years (N = 6)	2 (33%) (0 adolescent girls, 2 adolescent boys)
16–17 years (N = 4)	3 (75%) (2 adolescent girls, 1 adolescent boy)
18–19 years (N = 10)	7 (70%) (4 adolescent girls , 3 adolescent boys)

Reported Knowledge and Activities of Youth

Most youth (80%) reported sexual health education in school, but only 60% reported knowledge of HIV/AIDS. Half were able to verbalize contraception other than condoms. Twelve participants (60%) self-reported engaging in sexual activity and 3 (15%) reported past STI diagnosis. Thirty percent reported past alcohol use, and 10% reported past drug use, both considered risk behaviors.³⁴

Categories

Agreement between coders was approximately 80% (range 75%-85%). Through analysis, coders determined no new emergent categories, and therefore, developed category properties. Four primary categories were identified (Table 3).

Table 3.

Themes and Frequencies

		Frequency in each interview N (%)
Seeking Additional SCD Information
Incorrect or limited	Actively engages in SCD educational discussion, posed questions regarding SCD inheritance and/or accepted SCD educational information	20 (100%)
Knowledge of SCD inheritance	Verbalizes incorrect information, limited information, seeks clarification or does not know the inheritance pattern of SCD	14 (70%)
Sexual Health Education Needs and Interest
Sexual health education	Deficits in sexual health knowledge; request for sexual health education based on need for themselves or other youth with SCD	19 (95%)
Interest	Actively participating in sexual health education; interested in a sexual health education program	17 (85%)
Open to Learning	Demonstrates engaging in educational components of the semi-structured interview	17 (85%)
Developing Self Identity
Goals	Current goals and/or goals for future career or academic attainment	18 (90%)
Communication barriers	Difficulty verbalizing values and/or needs to partner	8 (40%)
Limited peer engagement	Does not socialize with peers outside of school; disease symptoms prevent peer interaction	7 (35%)
Risk behaviors	Engages in risky behaviors	7 (35%)

Seeking additional SCD information

Seeking additional SCD education was most commonly observed: 20 (100%) youth actively engaged in SCD educational discussion and accepted SCD educational information. Most participants shared that they had limited opportunities to learn about sickle cell disease and transmission.

‘And it’s nothing that I, I’ve obtained by myself… Learning about sickle cell, who has sickle cell. Especially transmission..’

(Adolescent boy, 18)

Some youth stated that they heard inheritance information previously, but that relearning the inheritance pattern was helpful.

‘Yeah, um…I think this is very important because I have been taught this plenty of times before but just to reiterate it and just to keep it in my mind so I can have it down pat. Like, off hand. I think that would be good.’

(Adolescent girl, 19)

Youth sharing incorrect information or limited information of the inheritance pattern of SCD was common as it was observed in 14 (70%) interviews. Many participants were interested in how sickle cell disease may play a part of family planning.

‘So if one parent got the full blown [SCD], is it still possible that the child can have it?’

(Adolescent girl, 17)

‘Um, it’s, if the mother and the father have the trait and they have sex or whatever then the baby will come out with the disease or if one of them, one person have the trait and the other one doesn’t then they son just has the trait or daughter or whatever.’

(Adolescent girl, 16)

Understanding the inheritance pattern within a personal context of future family planning was of interest to multiple participants. As one boy shared:

‘But, like I also have a question. Like, say, you know, like I have sickle cell…What if, my partner has the trait? Can the kids still?’

(Adolescent boy, 19)

For some youth, there was an emotional component to learning the inheritance pattern of SCD which was associated with their thoughts about their future family planning. During one interview, the participant indicated that she thought she would have a child with SCD, and therefore, she did not want to have any children because she did not want them to experience the health complications she had experienced. During the inheritance explanation, she learned that her child may not have SCD depending on her partner’s sickle cell status. This explanation was surprising to her:

Interviewer: ‘… If you have a partner who doesn’t have trait or disease then none of your children will have disease.’

Participant: ‘Really?’

Interviewer: ‘…Your children will only have trait.’

Participant: ‘Oh my gosh. Thank the Lord.’

(Adolescent girl, 18)

Youth also sought clarification on their parent’s trait status. Whereas they knew they had inherited SCD from their parents, there was still confusion surrounding the inheritance pattern. One participant shared that he did not believe his father had sickle cell disease or trait.

Interviewer: ‘Ok. And do you know how sickle cell disease is passed along?’

Participant: ‘Through a trait, through your DNA.’

Interviewer: ‘Um hum [yes]. And does your mom have sickle cell disease?’

Participant: ‘No she carries it. Just the trait.’

Interviewer: ‘All right. And how about your dad?’

Participant: ‘No.’

Interviewer: ‘Does he have trait?’

Participant: ‘No.’

(Adolescent boy, 15)

Sexual health education interest and needs

Nineteen (95%) youth indicated sexual health education needs by demonstrating deficits in sexual health knowledge or request for sexual health education based on need for themselves or other youth with SCD. One participant had incorrect knowledge of condoms. She believed condoms prevented pregnancy but increased the chances of contracting a STI. She specified that she learned about condoms at school. She stated:

‘Um, [condoms] protect against pregnancy and not, like, STDs… I think I’d rather, rather be pregnant than to have like HIV or syphilis or one of those 2.’

(Adolescent girl, 17)

Several youth discussed that they knew about STIs but they could remember the details about the STIs or transmission. HIV/AIDS knowledge was common, but most participants did not know about other STIs.

‘I’ve heard of some of them [STIs] but I can’t remember.’

(Adolescent boy, 15)

‘I didn’t know there were different kinds [of STIs]!

(Adolescent girl, 14)

‘Yes…Um… AIDS, HIV. That’s it. That’s all I know.’

(Adolescent boy, 18)

Youth also had limited knowledge of birth control. Whereas discussing her thoughts on decision making and sexual activity, one girl shared that she did not know if a contraceptive method (Depo-Provera shot) provided protection from STIs. She did indicate knowledge of using condoms to reduce risk in sexual activity:

‘Um… do shots [prevent STDs]? And um… if you have it, have safe sex using condoms. And, that’s all I got.’

(Adolescent girl, 13)

Some youth shared that they had knowledge of birth control but did not know about the different types:

Interviewer: ‘…so what kind of birth control have you heard of?’

Participant: ‘Um… I really don’t know. I just know birth control. I don’t know any specifics.’

(Adolescent boy, 19)

Seventeen (85%) demonstrated interest in a sexual health education program and actively participated in education during the semi-structured interviews. One boy shared that he was waiting for sexual health education in school but his school did not offer a comprehensive program.

‘Like, I mean, I always thought that why we don’t get sex ed? I always hear about people and other schools talking about how they got sex ed class. Why I can’t get it?’

(Adolescent boy, 16)

Youth also were interested in sexual health information topics and strategies to adopt healthy behaviors.

‘Uh, [learning that] ‘I could stop myself from doing that [getting an STI].’… So, I think that would, you know, absolutely help, 100%.’

(Adolescent boy, 16)

Open to learning

Participants thought that adolescents with SCD were curious to learn more information about SCD and sexual health but did not know whom to ask to learn more:

‘I mean like, you guys like, when you guys come to the hospital, that’d be helpful too. Especially um during the day here, on the ninth floor, cause like… A lotta families come through… Cause it could be like some teenagers out there who just like… want to know more [about sickle cell disease and sexual health], but they don’t know like how to get help or something like that. So yeah, I think that’d be cool.’

(Adolescent girl, 18)

Most youth actively engaged in the education discussion during the semi-structured interviews. Confusion around percentage and probability emerged during most discussions. As one participant stated:

Participant: ‘So 50% chance normal. 50% chance trait. 50% chance disease?…’

Interviewer: ‘[Interviewer providing information using visual diagram]…and if your partner doesn’t have any trait, normal hemoglobin. Then you’ll just pass along trait to the child.’

Participant: ‘Trait, trait, trait, trait.’

(Adolescent boy, 17)

Youth also asked questions, described the inheritance and were interested in how the results applied to their lives. As one participant demonstrates:

Interviewer: … can you have a child with sickle cell disease?

Participant: ‘Can you explain that more to me? If your partner had, uh, would they have trait or disease? Only if you partner is a carrier of sickle cell or in their family history has a history of sickle cell they could pass it along?’

(Adolescent boy, 18)

Developing self-identity

During our interviews, we observed questions and comments indicating our participants were in a period of developing their sense-of-self or self-identity.³⁵ These characteristics were observed through participants sharing goals, communication of values, social engagement, and risk behaviors.

Nineteen youth (95%) identified current and/or future career goals or academic attainment.

Participant: ‘Yup, no kids [until] after master’s degree.’

Interviewer: ‘…what are you thinking of studying at school?’

Participant: ‘Art… going into animation…’

(Adolescent girl, 13)

Interviewer: ‘So are you thinking of college next year, or…’

Participant: ‘Yes.’

Interviewer: ‘Oh wow. Are you in the midst of applications and all of that stuff?’

Participant: ‘Yeah.’

Interviewer: ‘Did you take the ACT this year?

Last year?’

Participant: ‘I’m taking it in December.’

(Adolescent girl, 17)

Eight (40%) reported difficulty with verbalizing their values and/or needs to an intimate partner. Youth shared that they may be aware of their personal values but that they did not have skills to communicate their values and/or needs.

‘I’m trying to use a condom and she say: “No you don’t need it.” So in my head, I’m like, “I do need a condom, what are you talking about?” Me being dumb. I went on and had sex with her without a condom. And, she got a baby right now.’

(Adolescent boy, 16)

As one girl shared, she wanted to ask a partner about STI status, but she did not know how to discuss this topic.

Interviewer: ‘Ok…if you thought your partner had an STD or something would you ask him to get tested? Have you asked before?’

Participant: ‘Um hum [yes] I mean, I’ve never had to ask. I’ve never thought it but I would.’

Interviewer: ‘Yeah, you would?’

Participant: ‘Um hum [yes]’

Interviewer: ‘So you don’t ask now?’

Participant: ‘I’ve never thought of it. I never thought to.’

(Adolescent girl, 18)

Seven (35%) youth reported not socializing with peers outside of school and indicated that disease symptoms influence their peer interaction.

‘Because when I found out and I went back to my friends and I was like, “Yeah I found out I had sickle cell.” It was all like, “Is that why you are short?” or “Can I catch it?” Or “Should I stay away from you?” And you know, it could hurt kids.’

(Adolescent boy, 15)

‘I just, my mom must know that I’m a house bound person. So she know that when I go out, you know, I’m ready to go home, I’m ready to come home.’

(Adolescent girl, 17)

Seven (35%) youth reported engaging in a variety of risk behaviors. As one boy shared, he engaged in multiple risky behaviors at one time:

Interviewer: ‘Ok. And … you had unprotected sex during that time because of using alcohol?’ Participant: ‘…yeah…’

(Adolescent boy, 16)

Another boy shared that he engaged in risky behaviors in the school setting:

Interviewer: ‘… and do you ever use drugs or alcohol? No, never in the past?’

Participant: ‘Yeah, I did. Once, but—’

Interviewer: ‘Okay. Was it drugs or alcohol?’

Participant: ‘Alcohol.’

Interviewer: ‘Okay, and it was just once?’

Participant: ‘Mm-hmm (yes).’

Interviewer: ‘Okay….and were you at a party, or how did…?’

Participant: ‘School.’

(Adolescent boy, 17)

Overall Education Preferences

Eleven (55%) youth preferred an informal discussion format for the educational program. Eight (40%) stated learning from the step-by-step SCD genetic inheritance diagram was preferable and 8 (40%) also preferred one-to-one discussion. Six (30%) stated an interest in an educational program using computers or technology.

DICUSSION

This study is one of the first to provide evidence of the baseline knowledge and behaviors of youth with SCD and their desire for sexual health and genetic inheritance education. Overwhelmingly, youth demonstrated limited knowledge in sexual health and genetic inheritance, but showed interest in an educational program designed specifically for their needs. Whereas other studies have examined important topics like reproductive decision making and genetic inheritance knowledge of individuals with SCD or SCT, few if any addressed sexual health and genetic knowledge and needs of youth with SCD.^36,37

Three prominent discoveries came from our analysis. The first is that all participants were seeking additional information regarding SCD inheritance within the context of their families. Some youth understood that they inherited SCD from their parents, yet they were not familiar with their probability of passing along SCD or SCT to their offspring. Moreover, none could self-generate the inheritance probability numbers for individuals with trait or disease. This demonstrates the limited knowledge and application of inheritance patterns, especially for their own future family planning. Developing materials that depicted their genotype and how they would pass the trait was supported and 85% demonstrated an increase in knowledge from this diagram. Beyond learning the inheritance pattern, there was an emotional component involved in learning about SCD inheritance and how it applies to their lives. Our participants were interested in learning about SCD inheritance for their own future family planning.

The second finding shows that our sample had limited knowledge of contraceptives and STIs. This may be a result of differing sexual health education programming available to youth with SCD in the St. Louis Metropolitan Area. Because there are not standardized curriculum requirements we do not have a clear understanding of what students are learning in schools. This may be negatively affecting our youth and overall population health.²⁶ Evidence of STIs and pregnancy rates may provide support of these observed differences within our sample population.^10–13 Another interesting finding was the reported sexual activity of youth aged 13–15 years. Whereas this is a small sample, additional research to investigate risk behaviors of young African Americans with a chronic disease is needed. This is important because education of risk reduction strategies should occur prior to sexual activity to prevent STIs and unintended pregnancies.

The third finding may be the most compelling. Youth are not just seeking information, but as they are developing their self-identities they are seeking out the practical application of skills based on their developing sense-of-self. Youth identified with certain values but were unable to verbalize their needs to their intimate partners. By providing a social skills based educational program, youth can practice to gain the confidence to use these skills in their daily lives. The World Health Organization defines sexual health as not just an absence of disease or dysfunction, but a positive and respectful approach to sexuality and sexual relationships free of coercion, discrimination, and violence.³⁸ Based on this definition we found that our participants benefited from a supportive, positive environment for discussing complex, sensitive topics during a period of self-identity development.

As with any feasibility study, we had limitations. This was a single center pilot study to assess the feasibility of recruiting youth to discuss a set of sensitive subjects. Despite the small sample size of 20, themes were well saturated, with no new codes emerging in the last several interviews. Although we met our primary goal of recruiting 50% of those approached, fewer than 50% of the youngest adolescents participated. The feasibility of recruiting persons 11–13 years of age should be explored further. The younger age group may not be as comfortable discussing sexual health, although their parents did support their participation as they consented for their child to enroll in the study. The study team may also need to reconsider the explanation of the study’s purpose in language geared to a less mature group. The American Academy of Pediatrics recommends providing age-appropriate sexuality health education starting in childhood and continuing into adolescence and adulthood in order to facilitate healthy sexuality.^39,40 Therefore, engaging youth 11–13 years in age appropriate education is consistent with expert recommendations.^39,40 Future studies may consider parental involvement in sexual health educational programs as there is a growing body of evidence to support this approach.^41–45 In our study, we decided to work directly with youth based on the social skills training theoretical framework and our aim to assess the feasibility of a hospital-based program. Based on indications of initial feasibility and the increasing evidence, educational programs involving parents may be considered in the future. In addition, responses during the semi-structured interviews were provided via self-report. Youth who did not provide information or engage in the interview may not have shared behaviors, limiting data collection. Participants also may have exaggerated behaviors. Building rapport at the beginning was used to facilitate honesty and trust. Furthermore, asking youth these questions is the only way to gather this information.

Future Directions

The next phase will be developing curriculum to meet the needs of patients with SCD. Community-based sexual risk-reduction programs using social skills training have been found to increase consistent condom use, reducing sexual risk behaviors.^28–30 To our knowledge, a hospital-based social skills training sexual health education intervention for youth with SCD has not been created. Based on our findings in this study, we created a working diagram to depict our approach (Figure 1). For youth with SCD, the interaction between sexual health, SCD inheritance and social skills training allows engagement in informed decision making. With a youth positive approach focusing on health, we hope to cultivate development of self-identity by providing the knowledge and skills for youth with SCD to make value consistent decisions.⁴⁸ Using this strong theoretical framework our curriculum will emphasizes relevance, social skills training, and health. ^{25,29,46–48}

Figure 1.

Diagram to Inform Intervention Development for Youth with SCD

Conclusion

Adolescents with SCD represent a vulnerable population.^{10,11,20–24,26} Our interviews show that our patient sample shares these risks and lack the knowledge and social skills to protect themselves from high-risk situations. Youth with SCD are developing their self-identity and have goals and expectations for their futures. They are working to learn and integrate SCD inheritance education into their self-identity and future planning which can be an emotional process. A customized educational program may support this process.