Sickle Cell Story Club: Implementation of a Clinic-Based Literacy Promotion Program

1 Introduction

Sickle cell disease (SCD) is the most commonly inherited blood disorder in the world, with one in every 350 African American/Black individuals born in the United States affected [1]. Characterized by a single mutation in hemoglobin, this progressive blood disorder can impact all organ systems including the brain. Common cerebrovascular concerns include both silent and overt strokes, and in some cases, severe vasculopathy (e.g., moyamoya disease) [2]. Roughly 20–37% of children with SCD will experience a silent stroke before the age of 14 years [3], highlighting both the early and progressive nature of the neurocognitive profile of youth with SCD.

The impact of this is significant, as children with SCD and confirmed silent strokes have significantly lower scores in reading and math when compared with those with normal magnetic resonance imaging (MRI) scans [4]. Areas of the brain dedicated to planning, organizing information, shifting attention (e.g., executive functioning) as well as visual attention, and verbal language skills are at particular risk for impact as evidenced by known patterns of deficits in youth with SCD [5]. While this demonstrates the role physiologic factors play in poor neurocognitive outcomes seen in children with SCD, recent studies suggest that slowed development in children with SCD is due to a combination of physiologic factors and environmental/demographic determinants [6, 7]. While the physiologic factors might be addressed via medical treatment, environmental factors can be moderated as well. There is a growing body of evidence exhibiting how reading and literacy in young children leads to better health outcomes, but the question remains how modulating this environmental factor might mitigate negative neurocognitive outcomes in children with SCD [8-10].

In the past decade, researchers have discovered that modest increases in book access and shared reading frequency lead to differences in pediatric brain function in specific locations that support other foundational literacy skills [11]. In other words, external factors in a child's life affect the optimization of brain pathways involved in reading. Families of low socioeconomic status are, thus, disproportionately impacted as book access and shared reading time are luxuries associated with financial stability. This reinforces the cycles between worse academic difficulties and poor health outcomes [11].

To address this discrepancy, several literacy promotion programs have been established. Reach Out and Read (ROR) is a program created by pediatricians who recognized the unique opportunity to promote reading during standard well-child visits during a critical stage of brain maturation (0–5 years) [12]. The role of the primary care physician (PCP) provides a platform to share with caregivers about why reading can be beneficial to a child's development, but this knowledge, has no practical benefit to families that do not have age-appropriate books in the home. ROR partners with PCPs across the country, providing families with children's books and parent education about the lasting emotional, linguistic, and literacy benefits of spending time together and reading aloud [13]. Additionally, PCPs are encouraged to model interactive reading time with the child, allowing parents an opportunity to engage during an office visit so the PCP might provide feedback. Dolly Parton's Imagination Library (DPIL) is a similar organization that promotes literacy by the distribution of age-appropriate books mailed directly to the child's home. Every month from birth to the age of 5 years, enrolled children receive an age-appropriate book resulting in a library of up to 60 books by the time they reach kindergarten [14].

Children enrolled in either the ROR or DPIL programs performed significantly better on the preliteracy section of the Kindergarten Readiness Assessment compared with children not enrolled in either program, suggesting that the programs support the preliteracy skills of at-risk children, improving their readiness to enter the school system and succeed academically [14]. Of note, a statistically significant association has been observed between enrollment in ROR and increased attendance of well-child visits [15]. While the books themselves may not be the reason for better visit attendance, it is more likely the whole-child approach to development serves to benefit the family-provider relationship and engagement in healthcare.

These programs, while impactful, have their limitations. Because enrollment in ROR is usually initiated in the primary care setting, children who do not see a pediatrician or who primarily see specialists (e.g., children with special healthcare needs) may not be reached by these literacy promotion initiatives. DPIL partners with local 501(c)3 organizations to enroll children in selected coverage areas, thereby limiting access only to areas with local partnerships. Additionally, both ROR and DPIL limit eligibility to children between the ages of zero and five. Representation in the chosen books is also limited. Only two books per year from DPIL are available in Spanish, and while they have a carefully elected committee to select the books distributed, DPIL does not share specific goals to include diverse representation in their selection of books. ROR, on the other hand, aims to provide books to kids that are linguistically and culturally diverse. However, their specific method of book selection and distribution, including whether materials are informed by the child's neurocognitive development, is unclear.

This study aimed to evaluate the feasibility and efficacy of implementing a diverse book ownership program, referred to as the Sickle Cell Story Club (SCSC), in an outpatient comprehensive SCD clinic. In the initial year, the program aimed to distribute one developmentally appropriate book per child seen during a comprehensive care appointment. It was anticipated that families would be receptive to receiving books as part of their visit, though we were uncertain about the level of impact on reading development given the limited instructional content associated with book distribution. We predicted that families of young children would be most receptive to the SCSC, consistent with early brain development. We anticipated that interest would vary throughout childhood, particularly in adolescence, given the variety of competing interests (e.g., technology, social skills, etc.).

2 Methods

3 Results

Between March 2021 and December 2023, a total of 1711 books were distributed to 386 patients. Children were between the ages of 11 days and 22 years (M = 8.23 years, SD = 6.30 years) at the time of their first book. Books were equally distributed to females (50.54%) and males (49.46). All genotypes were represented; Hb-SS (58.06%), Hb-SC (26.89%), Hb-Sβ+ (5.83%), Sβ0 (3.33%), and HbS-HPFH (0.83%). On average, patients received four books (range: 1–12 books). The majority of books were given to families by members of the psychosocial team (social work: 55.74%, psychology: 26.99%, school program: 9.60%, chaplaincy: 2.54%) compared with the medical team (providers: 1.93%, medical staff: 2.84%, research: 0.36%).

Most families initially reported having less than 20 books in their home (68.2%), with the majority reporting that they did not read as a family (47.5%) (Table 1). Of those who read at home, most were likely to spend less than 30 min per day reading together (23.9%). The majority of families did not utilize the library (70.3%). At their subsequent appointment, most families recalled receiving a book at their last visit (88.8%) and had read the book between visits (85.7%). Most families shared that they read the whole book (73.1%), whereas others noted that they read a few pages (9.6%) or about half (17.4%). When asked to reflect on the perceived reading level difficulty, most families thought the materials were “just right”(57.1%), though some noted that they were “too easy” (1.9%), “easy” (35.8%), or “hard” (5.2%).

After the initial 17 months, the majority of caregivers of young children (aged 0–5 years) endorsed reading as one of their child's favorite activities (67.3%), while others stated their child sometimes enjoys reading (29.6%) (Table 2). Regarding how often caregivers read aloud to their child, some stated they were not yet engaging in this activity (5.1%), some were not often doing this (9.2%), whereas the majority endorsed reading several times per week (50%) or at least once a day (35.7%). As a result of the SCSC, the majority of caregivers reported that they read a little more (50.5%) or a lot more (18.3%). No caregiver reported reading less as a result of the SCSC. Caregivers found the SCSC to be somewhat useful (16.1%), useful (34.4%), or very useful (49.5%) for their child's reading development.

Caregivers of children aged 6–12 years shared that the SCSC had either somewhat (22.1%) or completely (64.7%) positively impacted their child's reading confidence (Table 3). The majority of adolescents (aged 13–22 years) enjoyed getting a book as part of their appointment (80.4%) and liked the types of books they received (76.2%) (Table 4). Both caregivers of school children and adolescents agreed that the SCSC increased the frequency of reading behaviors as well as enjoyment toward reading. Regardless of age, nearly all families were likely to recommend the SCSC to another family (90.1%). Multiple families have returned to clinic and specifically requested the subsequent book in a chapter book or graphic novel series. One adolescent shared excitement about the access to diverse authors, noting that they had been unable to acquire these titles through their school library. One child exclaimed, “I loved Ada Twist! It's my favorite book!” when asked about the book they received at their previous visit. Many children and teens shared that they liked the book they received, sharing that they read it faster than other books or that it led them to their library to find books by the same author. Caregivers reflected on the frequency with which their child read the materials provided and stated, “You are so good at picking books that they actually like and will read.”

4 Discussion

Considering both disease sequelae and suboptimal access to high-quality education, patients with SCD are at a unique risk for differences in learning and attention [16]. Literacy is a particularly important area for intervention, given the ways in which language skills can be fostered in the home environment. Utilizing existing book ownership programs as inspiration, this project aimed to improve access to developmentally appropriate reading materials for patients as part of the standard of care during their comprehensive SCD clinic visits. Over 33 months, members of the multidisciplinary team were able to distribute over 1700 books to families, increasing their home libraries by approximately four books per child. Research exploring book ownership programs has demonstrated that a home library of at least 20 books may serve as a buffer for continued educational enrollment by upward of 3 years compared with peers. In our population, the majority of families noted having less than that benchmark initially, thus identifying a critical access gap that can be addressed during routine outpatient healthcare.

Given the ways in which the program was tailored to be culturally informed and accessible to patients being seen within their SCD visit, it was unsurprising that program feedback supported our hypothesis that families would find the SCSC to be acceptable and highly recommended to other families. While we suspected that caregivers of young children would find the SCSC to increase reading frequency and language development, we were surprised by the overwhelmingly positive uptake by our adolescent patients. Given the common interests found amongst digital platforms (e.g., social media, video games, etc.), the provision of physical books was deemed not only enjoyable but had a positive impact on reading engagement. It is certainly possible that the novelty of a book in the digital world was appealing, and we believe that the selection of books reflective of shared experiences and/or in formats less cumbersome to reluctant readers (e.g., graphic novels) likely explains these results.

While the initial intention of the SCSC was to increase access to books representative of our patient's lived experiences, the broader implications are important for all SCD Centers caring for pediatric patients. Compared with pediatric primary care, where ROR is primarily implemented, patients receiving regular SCD care will likely see their hematologist more frequently outside of the newborn period, often citing their hematologist as their primary physician [17]. Therefore, the distribution of books as part of standard SCD care allows for greater access points in supporting the home literacy environment. Given that language skills and reading development are persistent areas of neurocognitive concerns for patients with SCD, early and frequent strategies to mitigate these areas are within the realm of responsibility of the comprehensive SCD team.

In contrast to ROR, the distribution of books in the SCSC were done primarily by members of the psychosocial team rather than medical providers. This strategy supported the feasibility of the program, as the responsibility of book distribution was shared amongst multiple team members. Additionally, this allowed for the normalization of psychosocial team members in the routine SCD appointment, communicating the importance of physical, mental, emotional, and academic health as the standard of care. However, psychoeducation around incorporating a bedtime routine that included reading was still presented at the time of book distribution by the embedded psychologist when concerns related to sleep were identified in discussion, suggesting that anticipatory guidance can be provided across disciplines, in contrast to traditional ROR programming primarily delivered by the medical provider [18]. This is particularly salient given the known challenges with sleep hygiene, including sleep onset, maintenance, and waking in youth with SCD [19, 20]. Finally, the distribution of books from our embedded social worker fostered opportunities to build relationships outside of the typical resource needs or crises.

According to a recent policy statement from the american academy of pediatrics (AAP) [21], literacy promotion is imperative to child development, highlighting the unique role that pediatric primary care providers play in this critical developmental period. Merely the act of literacy promotion by pediatricians can be an evidence-based prevention strategy to support early brain and child development. Shared reading experiences are crucial to the parent–child relationship and serve as a buffer against the development of socio-emotional difficulties [22]. Consistent with the AAP policy statement [21], pediatricians are encouraged to promote the shared reading experience to cultivate language-rich interactions, ensure cognitive stimulation in the home, and nurture the parent–child relationship, ultimately bolstering child emotional development. Given the well-documented occurrence of emotional concerns in youth with SCD [23], and the impact of the parent–child relationship on coping [24], early promotion of emotion literacy skills is imperative for learning necessary coping strategies when faced with stressors. Additionally, ensuring a positive relationship between caregivers and their children with SCD is necessary for future shared healthcare decision-making and communication between the family and the medical team. As worse parental functioning (e.g., stress, mental, physical) has been associated with worse child functioning (pain, depressive symptoms, quality of life) in children with SCD [24], strategies for addressing this already vulnerable relationship are crucial.

It is important to note that SCSC launched approximately 1 year into the COVID-19 pandemic, a time in which the educational system had been impacted by abrupt changes to the learning environment (e.g., switch from in-person to virtual instruction). While commendable efforts were made to ensure minimal disruptions in learning, families have continued to report how this shift in instruction led to prolonged periods wherein children were expected to self-pace and ultimately lost out on critical years of education. Additionally, when reflecting on the access to community-based resources for children with SCD, particularly quality education metrics, we discovered that many of our families live in areas where school districts underperform compared with national averages, and rates of higher education attainment are lower [16]. This highlights the systemic inequities occurring at the State level that may be contributing to poor academic outcomes for children with SCD.

Additionally, the timing of the SCSC came shortly after the uprising of the Black Lives Matter movement which elucidated the pervasiveness of racism within the United States. Within children's literature, greater attention to the inequities in publishing revealed disproportionately fewer number of books written by Black authors or featuring Black characters. One of the best reading intervention strategies is to utilize materials with subject matter that is of interest to the student. Therefore, having access to books with characters that share likeness is imperative to supporting reading confidence. The deliberate selection of book materials featuring relatable characters within the SCSC is likely a contributing factor to the overall success and appreciation reported consistently by families.

While the SCSC is innovative in its approach, this study was not without limitations. While caregivers and adolescents both shared a perceived increase in the frequency and duration of time spent reading as well as a greater enjoyment in reading overall, quantitative measurement of these domains was not able to be assessed due to the limited parameters of the program. Future studies should aim to evaluate whether similar long-term effectiveness demonstrated in other book ownership programs (e.g., ROR) can be replicated with a program such as the SCSC. Additionally, we recognize that sustainability of the SCSC is contingent upon having a multidisciplinary care team and continuous funding.

Conflicts of Interest

Julia LaMotte and Seethal Jacob receive book royalties from A Kids Book About Co. The other authors have no conflicts of interest to disclose.