Introduction
Sickle cell anemia (SCA) is a disorder that belongs to a broader category of inherited sickle cell diseases (SCDs). Its primary characteristics include the irregular shape of blood cells and therefore affect oxygen distribution throughout the body. Normal red blood cells are round and flexible for effective movement through vessels (Shih & Cohen, 2020). The crescent or sickle-like shapes of blood cells present in individuals with SCA are rigid and cause a blockage within vessel passages, contributing to other health complications. Pediatric patients might have the same symptoms as adults have, but their treatment processes are more difficult due to their early age and continuous development. Long-term anemia, pain crises, acute chest syndrome, strokes, infections, and splenic sequestration are observed in severe cases. SCA management focuses on relieving symptoms and preventing complications by taking medications and blood transfusions (Shih & Cohen, 2020). Adherence to medication regimes is low, and the extent to which patients use prescribed medications may put them at risk for complications and high care costs. Thus, it is important to identify the methods to improve adherence like centered care and find what researchers think about its effectiveness.
Problem Statement
With ongoing decreases in infant mortality, the rates of children being born with either sickle cell anemia or traits are likely to grow. In addition, these children’s health conditions may be complicated by external factors such as weak health services and resources, low income, and poor nutrition. Thus, SCA presents several significant consequences that affect populations and health organizations. Many SCA pediatric patients must be hospitalized due to the necessity to identify and control symptoms such as acute anemia, pain crises, and bacterial infections (Shih & Cohen, 2020). However, these patients do not receive appropriate follow-ups in most cases because of insufficient care quality. As a result, mortality is heightened for the children who consider no more than one follow-up after hospitalization. This situation outlines a larger issue, while mortality may be decreased at birth; still, SCA treatment and follow-ups are ignored, and additional treatment can negatively affect the lives of children with the disorder.
Background
SCA is a genetic disorder that can be inherited early, regardless of other environmental or nutritional factors. When blood cells are sickled, they cannot complete their full functions and lead to the inability to carry oxygen and provide all body parts with the necessary number of critical substances (Shih & Cohen, 2020). It is not enough to treat the disease, and care providers focus on appropriate ways to analyze the present symptoms and predict complications. During the last several decades, treatment plans have been developed to avoid pain episodes and relieve symptoms in adults (Shih & Cohen, 2020). At the same time, several methods to mitigate the symptoms have been developed to help SCA children, considering their specific physiology and the inability to report on symptoms and changes immediately.
There are three major pharmacological approaches to be offered to pediatric patients. Hydroxyurea improves hematological characteristics, penicillin prevents infections, and iron chelators reduce toxicity levels (Shih & Cohen, 2020). Although these treatment steps are effective in 30-60% of cases, poor adherence to medications is related to increased hospital visits and poor life quality (Shih & Cohen, 2020). Therefore, it is recommended to pay attention to what children and their parents might know about medication adherence and predict adverse effects.
Medication adherence refers to the behavior of patients correctly taking prescribed medications and required intervals and times. In SCA cases, it is the primary factor that defines if a person can improve health outside of medical interventions or not. Poor adherence to certain medications usually results in worsening symptoms and provoking global pain, dehydration, increased heartbeat, and lightheadedness that may result in potentially lethal outcomes.
The patient-centered care approach is respectful and responsive to patients’ preferences, values, and needs. It is often implemented when individuals experience inadequate care because of cultural/gender/ethnic backgrounds and other personal characteristics. The impact of patient-centered care on medication adherence should be observed at several levels. At the individual, older patients improve their awareness of the disease; at the family level, parents reduce stressors and encourage family routine to support children; at the systems level, the medical staff supports interactions (Shih & Cohen, 2020). SCA pediatric patients need the best help, and it is the responsibility of parents and healthcare providers to offer effective options.
PICOT
The current research suggests that care barriers exist and become serious social and systemic challenges. Individuals are not able to find adequate healthcare providers in their initial confrontation with sickle cell anemia. The issue is further complicated when an adult is the primary caretaker of a child with sickle cell anemia. Such inability is followed by lacking availability of physicians who are both capable and aware of their patients’ diverse needs and values. The lack of patient-centered care creates a lack of communication, cooperation, and trust between patients and healthcare providers, leading to decreased medical adherence. Taking into consideration the current problem and the available perspectives, the theme of SCA in children can be discussed through the following PICOT question: “In children with sickle cell anemia, will the patient-centered care approach positively affect medication adherence compared to no interventions in five years?”.
Methods
To answer the offered PICOT question and contribute to understanding SCA in children, a systematic review search strategy was offered. The decision to work with several research databases was made. First, the study included the current findings from CINAHL Complete Library to identify several primary articles. Second, SAGE Journals and Wiley Online Library were chosen to identify several related sources on the topic. The BOOLEAN operators “AND” and “OR” were applied to connect such keywords as “sickle cell anemia,” “pediatric patients,” “medication adherence,” and “patient-centered care.”
In this case, the identification of inclusion and exclusion criteria is critical to remove unnecessary or ineffective sources. Inclusion criteria were related to the publication years and the context of the articles. The studies with cross-sectional designs about sickle cell anemia in pediatric patients published between January 2016 and December 2020 were chosen. The exclusion criteria were necessary to identify the articles that cannot bring the worth to the particular project. Thus, sources published other than in the English language or before 2016 were excluded. Also, no animal experiments, medication dosage projects, or genetic studies had to be present in the review.
More than 100 articles were found in the chosen databases. Most studies were focused on treatment for adult patients and general guidelines. Some articles considered the international context and addressed the situation in developing countries. Therefore, most sources were removed from the list, and five articles were finally chosen to discuss the topic and define the effects of patient-centered care on SCA patients’ medication adherence. The major characteristics of the studies were participants who were diagnosed with SCA and were older than 22 years. Interventions were related to medication adherence to define primary and secondary outcomes of the steps that helped pediatric patients in mitigating SCA symptoms.
Results
The SCA problem is not new in modern healthcare practice. Still, there is no unanimous opinion concerning its treatment because the reasons that cause it are different (Williams & Thein, 2018). Discussion of the characteristics of SCA, its causes, and effects improves understanding of the relationship between the medication used in such cases and the specifics of pediatric care (Williams & Thein, 2018). The results show that current literature on this topic focuses primarily on the impact of specific drugs such as hydroxyurea on children and the quality of pediatric care that may be changed and improved by following recommendations (Badawy et al., 2017). According to the study results, particular barriers to diagnosing SCA are determined by a low knowledge level in pediatric patients and their caregivers (Badawy et al., 2017). Improvement in the treatment of SCA is hindered by challenges such as the variability of the disease and lack of sufficient knowledge.
SCA Treatment in Children
Examination of patient characteristics to identify the scope of the problem is essential in attempts to learn who might require professional care and help. At this moment, about 100,000 Americans are diagnosed with the disease and report such symptoms as acute pain, chest syndrome, and organ damage (as cited in Badawy et al., 2017). The results show that younger African American female patients are at higher risk for SCA than other individuals (Badawy et al., 2017). Similar results are observed in Inoue et al.’s study (2016) to prove that the number of Blacks with SCA prevails in the population. Hydroxyurea is the main existing treatment for SCA, but it remains suboptimal in its implementation in pediatric care (Badawy et al., 2017). However, positive outcomes like symptoms reduction, improved quality of life, decreased health costs, and the nitric oxide donor are observed (Badawy et al., 2017; Inoue et al., 2016). Therefore, hydroxyurea is the primary medication used to treat SCA, and different exposure to this drug leads to variations in its effectiveness. Within the scope of patient-centered approach, the main attention of healthcare professionals – especially nurses – should focus on patient compliance and treatment adherence.
In case of pediatric patients, the priority lies in ensuring that the adult guardians of children with SCA are completely aware of the treatment process, its potential outcomes, and how to manage its side effects. The patient-centered approach relies heavily on the constant feedback provided by the patients in order to adjust and correct the treatment. Multidisciplinary teams should collaborate on the patient education to ensure the adherence to treatment and to help develop individual strategies of managing the disease after hospital discharge (Simpson et al., 2017). Nurses play the main role in this process, as they work directly with patients and their families, and establish unique connections with them.
Evaluation of treatment efficiency by the patient usually remains subjective, because it is influenced by a number of reasons that are not directly related to the result itself. These reasons often include specifically the medical staff’s approach to patient’s care – how professional, compassionate, and correct it is – which greatly affects the patient’s willingness to cooperate. Nurses should also consider the problem of different perception of the result of medical care by doctors and patients. Patients evaluate mainly the external manifestations of treatment, and therefore tend to underestimate the overall effectivity of care. Interestingly, this same fact may cause greater criticism from patients towards nurses in assessing satisfaction with medical care. In this case, patient-centered approach allows healthcare professionals receive continuous feedback from patients to address specific issues that may arise in the process of treatment.
Medical Adherence in Pediatrics
Although much attention is paid to the quality of patient education and cooperation with nurses in hospitals or after discharges, medication adherence remains a serious problem in modern health care (Crego et al., 2020; Inoue et al., 2016). Crego et al. (2020), discusses the consequences of lacking medication adherence and the underlying causes that remain prevalent in modern pediatric practices. According to Crego et al. (2020), hydroxyurea, penicillin, and iron chelators can increase life expectancy despite experiencing low adherence to pediatric interventions (Crego et al., 2020). Inoue et al. (2016), also report low medication adherence rates in SCA patients, including more than 80% in penicillin and similar hydroxyurea levels. Insufficient research in this area leads to a risk of bias toward hydroxyurea interventions (Inoue et al., 2016). At the same time, additional variables like cooperation with individuals, families, communities, and healthcare systems are discovered (Shih & Cohen, 2020). Addressing multiple domains within a particular care option may result in more prominent effects on medication adherence (Shih & Cohen, 2020). It is possible to hypothesize that the fundamental approaches and patient education are the initial barriers to increasing medication adherence among pediatric patients who are diagnosed with SCA.
Patient-Centered Care Approach
The introduction of medications to pediatric patients has certain positive and negative characteristics, but the main idea remains the same: patients cannot ignore pharmacological and non-pharmacological interventions. At the base of patient-centered care approach lies the ability of healthcare professionals to convince the patients and their families in the necessity of adhering to clinical recommendations and treatment. Thus, it is important to develop new, more individually-aligned care strategies and strengthen cooperation between parents, nurses, and other healthcare provider to help children diagnosed with SCA. Patient-centered care is highly promoted in various studies, including Riley et al. (2020), Shih and Cohen (2020), and Smaldone et al. (2019). Optimal healthcare experiences need to be shared among the populations, regardless of their ethnic or racial background to avoid complication and health crises in children.
Multicomponent interventions, including behavioral, educational, technological, and human support factors, are equally important in the assessment of the diagnosis (Shih & Cohen, 2020; Smaldone et al., 2019; Riley et al., 2020). For example, knowledge, self-efficacy, and self-monitoring are obligatory to improve medication adherence (Shih & Cohen, 2020). A great part of SCA treatment still occurs in the home setting, and both the patients and their adult caretakers require specific knowledge on how to properly manage the disease. Studies show that individuals with SCA and their families want to have access to treatment guidelines via technological means in order to improve their health outcomes and everyday life (Cronin et al., 2018). Thus, in the process of transforming care into more patient-centered models, existing nursing clinical protocols and guides need to be adapted for easier understanding by the patients and their caretakers.
Community healthcare professionals also can play a significant role in educating and assisting pediatric patients with SCA. For example, 12 months were given to the participants of the HABIT intervention to observe if daily collaboration with community health workers could bring positive outcomes to young people’s lives (Smaldone et al., 2019). A literature review using the scholarly databases CINAHL and PubMed revealed that nursing education and the use of alternative medicine therapies can potentially help reduce vaso-occlusive crises in SCA patients (Riley et al., 2020). Patient-centered care is all about treating patients with dignity and respect and addressing people’s needs and personal characteristics in offering treatment options. The offered ideas and recommendations contribute to a better condition in pediatric care and an improved understanding of what SCA patients might expect.
Critical Barriers in Pediatric Care
There are many factors that might contribute to better medication adherence or reduce the quality of care and provoke new barriers. According studies by Badawy et al. (2017) and Inoue et al. (2016), adherence to hydroxyurea medication by SCA children remains a problematic issue in pediatric care. The most prevalent barriers could be a lack of outcome awareness, poor support staff, and non-agreement with recommendations despite counseling (Badawy et al., 2017). Moreover, a very specific bias is also prominent in cases of SCA complicated by a pain syndrome: patients who take opioid medication to alleviate the pain are stigmatized as “drug-seekers.” (Riley et al., 2020). Patients with vaso-occlusive crises have limited access to opioid drugs, resulting in the worsening of their condition (Riley et al., 2020). This kind of implicit bias in healthcare is especially prominent towards non-White population.
Another point that requires attention is the lack of education on the treatment processes and outcomes, potential effects of medication, and overall health literacy among patients. Sometimes, patients cannot understand what they do wrong or right because of their forgetfulness or limited knowledge about medications (Badawy et al., 2017). Therefore, self-efficacy is damaged and becomes another factor that contributes to low adherence (Badawy et al., 2017). There are cases when medication adherence is low because patients experience fears of side effects or the inability to achieve the desired effect, and this issue is commonly prevalent among children and their parents (Badawy et al., 2017). According to Inoue et al. (2016), communication between the clinic staff and involved manufacturers and technicians may be poor, resulting in equipment delays and ineffective patient support. Children who do not receive medication in time have higher risks of asthma development, which increases the morbidity of SCA by 81% (Black et al., 2020). Thus, staff might also face a number of prominent challenges with a lack of equipment, support, emerging issues, and little reimbursement.
Discussion
The results of this review prove that SCA patients have to follow specific treatment recommendations and obligations to manage the disease and predict the development of new symptoms and complications. In most pediatric cases, parents and healthcare providers need to take responsibility for medication adherence. Older children are able to control their prescriptions independently, but certain barriers to medication adherence emerge at any age. People might forget or neglect medications, and the patient-centered approach is recommended to remove some challenges and help patients achieve the best results. When nurses and other care providers focus on patients, regardless of their racial or ethnic characteristics, patient-doctor cooperation is improved and leads to consistency and self-efficacy, which are critical in SCA treatment.
References
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