Caregiver experiences with accessing sickle cell care and the use of telemedicine

Sickle cell disease (SCD) is the most common inherited hematologic disease in the world, disproportionately affecting African Americans in the United States [1]. One out of every 365 African American newborns is diagnosed with SCD, many of whom are born into low-income families [1, 2]. The complications of SCD are numerous, and include severe pain, acute chest syndrome, and stroke [3]. However, improvements in preventative therapies have increased the life span for patients with SCD, with more than 93% of children with SCD surviving to adulthood [4]. Improved patient outcomes though, are contingent on SCD patients receiving timely evidence-based medical care, including regular patient evaluations with hematologists, disease-specific education, psychosocial care, and genetic counseling [5‐7].

Despite the complexity of care required by SCD patients, many barriers prevent equitable access to healthcare [1, 8, 9]. Children with SCD experience longer than average emergency department (ED) wait times, difficulty with transportation, and insufficient availability of primary care providers [10]. Additionally, primary care providers report having inadequate SCD expertise, feeling uncomfortable with pain management, and other SCD-related care for prevention and treatment [11, 12]. Comprehensive Sickle Cell Centers are more commonly located in major metropolitan areas, creating additional challenges for families living in rural or under-resourced areas [13].

While previous studies have identified barriers to accessing care for adults with SCD, less is understood about the barriers that affect the pediatric SCD patient population [14]. Furthermore, there is limited data regarding barriers for those living in the Midwest, specifically in rural and medically under-resourced communities [1, 15, 16]. Telemedicine, the remote provision of medical care using real-time audio-visual consultation, is a valuable solution to addressing physician shortages and geographic barriers faced by patients and has proven successful for consultations in a variety of pediatric specialties [17]. However, information on the value of telemedicine exclusively for SCD patients is minimal in the existing literature, particularly for children with SCD [18‐20]. Additionally, attitudes of caregivers and patients with SCD regarding the use of technology, such as telemedicine, for addressing these barriers are not well established [21, 22].

Semi-structured interviews have proven to be a powerful tool for providers to gain a deeper understanding of patient experiences [23]. This study aims to increase the understanding of both facilitators and barriers to care, as well as perceptions of telemedicine by caregivers of pediatric SCD patients in a medically under-resourced area in the Midwest.

The lack of subspecialty centers for SCD compared to other genetic diseases has been well-documented [6, 38]. Fewer than 70% of children with SCD receive comprehensive subspecialty care, and only 20% experience effective care coordination between primary and subspecialty providers [39]. Although Crosby et al., have reported barriers to care experienced by individuals living with SCD, the effect of living in a largely rural midwestern state with medically under-resourced urban areas has not been well-described [15]. This qualitative study explored the experiences of caregivers in accessing healthcare for their child with SCD in rural and/or medically under-resourced areas of a midwestern state, as well as the use of telemedicine to overcome potential barriers.

The themes that emerged from the caregiver interviews in this study were consistent with barriers identified in previous research. However, these interviews revealed provider knowledge and bias as a novel, yet significant, barrier to receiving adequate SCD care. Caregivers often mentioned that they worry about how knowledgeable school personnel or a healthcare provider may be regarding SCD, and how this not only affects them personally, but also impacts their overall experience in the healthcare system. Caregivers expressed losing trust in their local community and hospitals because of this lack of knowledge, or worse, blatant bias (e.g., “…called drug seekers…”).

Trust of medical providers and the healthcare system is essential for ongoing care and shared decision-making in chronic diseases like SCD. It is especially important to build trust with patients with SCD and their caregivers, given that they generally comprise a particularly vulnerable population which has long been neglected and subject to racism in the healthcare system. Lack of provider knowledge or comfort with managing SCD not only increases this vulnerability and caregiver burden, but also furthers the development of medical mistrust. A focus on improving provider knowledge and addressing bias in the care of patients with SCD is essential to reducing barriers to accessing medical care [37]. Standardization of care through sickle cell-specific protocols, personalized pain plans, and broad provider education can address some of these concerns.

Another specific theme that caregivers frequently mentioned was related to long-term stress and mental health burden. Caregiver stress and the development or worsening of mental health has been well-documented, including in other chronic disease populations such as cystic fibrosis [35]. Additionally, caregiver perceptions of their child’s stress are associated with increased caregiver burden and worsening mental health [34]. Caregivers in our study reported worsening of their own mental health due to their child having a chronic disease, as well as the challenges associated with accessing care for their child. Thus, not only are they enduring the stress and burden of caring for a child with a lifelong illness, but also the structural inequities that lead to poorer access to care or quality of care adds to their burden and overall mental well-being.

To improve the access patients with SCD and their caregivers have to subspecialists with expertise in SCD, telemedicine has been piloted in similar rural and medically under-resourced communities [18]. The COVID-19 pandemic led to the mass adoption of the direct-to-consumer model of telemedicine, where the healthcare provider could deliver medical services directly to the patient in their home, often via a smartphone or tablet. This greatly increased the general population’s exposure to telemedicine, including individuals living with SCD. In our interviews, many of the caregivers had participated in some model of telemedicine. Interestingly, most who had experienced the direct-to-consumer model of telemedicine delivery thought a significant disadvantage was the lack of a hands-on physical exam. They viewed this disadvantage as significant enough that they would prefer to travel the distance to the subspecialty center to receive personalized care, despite the barriers that exist. The caregivers who had participated in the hub-and-spoke model of telemedicine for SCD care, did not report this as a disadvantage.

The barriers that caregivers of children with SCD experience in the Midwest have similarities to those previously reported in other parts of the U.S, and while telemedicine has been a useful adjunct to medical care during the COVID-19 pandemic, much still needs to be improved to provide adequate subspecialized care for the SCD population. Likely, there is value to leveraging multiple models of telemedicine (e.g., hub-and-spoke and direct-to-consumer) depending on the needs of the patient/caregiver, location, and disease severity. It may improve access to subspecialty care, but more importantly, similar to what has been done in pediatric diabetes care, these models need to be adapted to specifically serve the SCD population [40]. Further work evaluating adapted models for SCD care is needed to improve access to healthcare for this patient population.

Like all studies, this one has limitations that should be considered. The small, focused sample size representing a population in the Midwest can limit generalizability outside of the region. However, the sample size was adequate to achieve thematic saturation, demonstrating consistency in reported themes [41]. The study was also limited to caregivers of children and adolescents with SCD, and future studies evaluating perceptions and experiences of adolescent and young adult SCD patients living in rural or medically under-resourced areas would be of importance.

This qualitative study evaluates the experiences and perceptions of barriers to accessing SCD care for caregivers living in rural and medically under-resourced areas. While some reported themes overlapped with previous qualitative studies, those related to caregiver burden and provider knowledge or biases suggest barriers to care that may be unique to SCD patients, requiring interventions and models of care to be adapted for this specific population.