Sickle Cell Anemia Case Study Nursing



Individuals with sickle cell disease (SCD) have reported being stigmatized when they seek care for pain. Nurse attitudes contribute to stigmatization and may affect patients' response to sickle cell cues, care-seeking, and ultimately patient outcomes.


The purpose of this study was to determine if there are significant differences in nurse attitudes towards patients with SCD by worksite- medical-surgical units compared to emergency departments/intensive care units (ED/ICU).


The study used a cross-sectional, descriptive comparative design.


The sample consisted of 77 nurses (36 nurses from the ED/ICU and 41 nurses from medical-surgical units) who completed an anonymous online survey.

Results and Conclusions

There were no significant differences in attitudes by worksite with both sites having high levels of negative attitudes towards patients with SCD. Findings suggest that nurses from both worksites need additional education about SCD and care of this vulnerable, patient population.

Keywords: Attitudes, stigma, sickle cell


Many individuals with chronic illnesses are living longer lives. The role of self-care in these disease states has been increasingly studied in recent years given its importance to managing the disease process over time. Chronic disease management involves self-care which is highly individualized. In order to successfully perform self-care behaviors, individuals must be able to recognize and interpret evolving problems that are unique to their bodies (cue recognition), then use specific strategies such as seeking medical help to address those problems (cue response). Cue recognition and response are important for individuals living with sickle cell disease (SCD). Negative nurse attitudes may affect patients' response to sickle cell cues, thus may affect clinical care and ultimately patient outcomes. It is important to understand nurse attitudes towards patients with SCD and to determine if they differ by worksite. The limited studies that have reported nurse attitudes towards patients with SCD have either focused on the ED or have not specified a worksite. The purpose of this study was to determine if there are significant differences in nurse attitudes towards patients with SCD by worksite- medical-surgical units compared to emergency departments/intensive care units (ED/ICU).

Background: Challenges of Care-Seeking for SCD

Sickle cell disease is the most common genetic disorder in the United States (Pack-Mabien & Haynes, 2009). It is an inherited blood condition that results in a genetic defect in the hemoglobin structure (Creary, 2007), leading to the classic sign of sickle shaped red blood cells. The sickled red blood cells cause hemolysis which leads to anemia and other complications that can affect every body system. They may also lead to irreversible damage (Zack-Williams, 2007). The clinical manifestations of SCD are primarily caused by two mechanisms: hemolysis and vaso-occlusion. Hemolysis is the most recognizable sign of SCD. The average hemoglobin of an individual with SCD is 6-9 gm/dl (Howard & Oteng-Ntim, 2012; MacMullen & Dulski, 2011), as compared to the norm of 13.8 to 17.2 gm/dL for males and 12.1 to 15.1 gm/dL for females (Medline Plus, 2014). Another major clinical feature, vaso-occlusion, results in acute pain. These pain episodes have been characterized as sickle cell crises or pain crises. In a critical reappraisal of sickle cell pain, Ballas, Gupta, and Adams-Graves (2012) agree with Diggs' (1956) description of sickle cell pain as typically being sudden onset in the low back, or one or more joints or extremities. It can be confined to one area or it can migrate and the pain is often continuous and throbbing. These crises are the primary reason for health care utilization and they often result in hospitalizations (Lattimer et al., 2010).

Care for sickle cell crises is typically sought after strategies at home have been exhausted with no relief and the pain has reached unbearable levels (Jenerette, Brewer, & Ataga, 2013). Individuals who present to the hospital in sickle cell crises are often stabilized in the emergency department with fluids, oxygen, and pain medication then discharged; however, discharge does not necessarily indicate resolution of the crisis (Ballas, Gupta & Adams-Graves, 2012). More severe cases or cases worsened by delayed analgesia result in admission for further symptom management and/or treatment of the underlying problem (Ballas, 2011). Beyond the emergency department (ED), inpatient assignment, nursing unit or intensive care unit, is based on patient status and bed availability. Inpatients with SCD are often assigned to medical-surgical units because hospitalists are increasingly more likely to manage inpatient admissions of adults with SCD (Smith, Jordan, & Hassell, 2011); therefore, medical-surgical nurses are seeing an increased number of patients with SCD (Jenerette, Brewer, & Moura, in press).

Regardless of the ED, intensive care, or inpatient unit, nurses are the chief providers of direct clinical care and interact most often with patients. They are essential for pain management, health education, and prevention of subsequent sickle cell crises (Valente et al., 2010). Nurses not only communicate patient status and concerns to the primary care provider, but they also offer recommendations based on nursing judgment and implement prescribed therapy. In doing so, nurses assume a large percentage of the responsibility to serve as patient advocates. Attitude can serve as a barrier between a nurse and a patient. Prior to entering nursing school, many nurses develop negative attitudes about pain and the use of opioids for pain management as a result of their ethnic background, values, family, church, and community (Pack-Mabien, Labbe, Herbert, & Haynes, 2001). Having preconceived notions about pain can result in imprecise pain assessments and thus insufficient treatment of pain.

The pain management process can be difficult for all involved because individuals with chronic pain may not show visible signs of pain. Nurses must rely on the patient's subjective description of pain to guide assessment and treatment. Thus, the mantra that pain is “whatever the experiencing person says it is, existing whenever the experiencing person says it does” (McCaffery, 1968, p.95) ought to guide practice. This does not always occur. Persons with SCD report being discriminated against, being stigmatized by healthcare providers, feeling as though their complaints are ignored, and just being poorly treated when they access the health care system (Jacob, 2001; Jenerette & Brewer, 2010; Todd, Green, Bonham, Haywood, & Ivy, 2006).

Vaso-occlusion, a cause for pain in SCD, may lead to other complications such as infection, acute chest syndrome, stroke, renal dysfunction, retinopathy, avascular necrosis, and cholelithiasis (Howard & Oteng-Ntim, 2012; MacMullen et al., 2011). In addition, an increased frequency of pain episodes has been associated with a higher risk of early death (Reddin, Cerrentano, & Tanabe, 2011). Consequently, timely evaluation and treatment is imperative. On the contrary, patients with SCD report long delays in receiving pain meds, insufficient treatment of pain, allegations of being a drug seeker, and that providers lack an understanding of SCD (Lattimer et al., 2010). Nurses were reluctant to administer high doses of opioids to patients with SCD experiencing an acute pain crisis because they felt they were contributing to the patient's addiction (Khattab, Rawlings, & Ali, 2005). It is believed that patients with SCD become dependent on pain medications or that they should have a lower pain threshold. Nurses who were younger (less than 39 years old), had less education (lower than a Master's degree), and had less than 10 years of active nursing experience (often between zero and 5 years) were more likely to believe that drug addiction frequently develops in the management of sickle cell pain and should be a primary concern in the care of patients with SCD (Pack-Mabien et al., 2001). These nurses were also most likely to believe that most patients with SCD are drug addicts. In fact, nurses reported that patients with SCD require too much time and that they can, at times, look too healthy to be sick (Valente et al., 2010). Unlike cancer and postoperative pain, there appears to be a lack of sympathy for those with SCD (Pack-Mabien et al., 2001).

The Theory of Self-Care Management for Sickle Cell Disease identifies lack of sickle cell crisis cue recognition/response as a vulnerability factor (along with complications, crises per year, and overprotection) that has a negative influence on health outcomes/health related quality of life (depressive symptoms, self-esteem, pain management experience, and health-related stigma). This relationship is positively mediated by self-care management resources which include assertive communication skills, coping behaviors, self-care ability, self-care actions, self-efficacy, and social support (Jenerette & Murdaugh, 2008). Negative nurse attitudes towards patients with SCD can affect the nurse-patient relationship and contribute to poor health outcomes as proposed in the Theory of Self-Care Management for Sickle Cell Disease. For example, when patients with SCD report lack of satisfaction with care-seeking experiences, they often delay care-seeking in the future by not responding appropriately to cues of an evolving pain crisis (Jenerette, Brewer, & Ataga, 2013).

Additionally, nurses are important conduits in the content and context of information that is relayed to the primary care provider, thus influencing the quality of care provided (Haywood et al., 2010) and patient outcomes. These attitudes are a major reason why patients limit or delay further care seeking when needed (Jenerette, Brewer, & Ataga, 2013). Patients then present in greater, uncontrolled pain and with more advanced issues when care is finally sought. This negatively affects the care experience and the cycle continues.



University institutional review board approval was obtained from The University of North Carolina at Chapel Hill prior to the start of the study. Additionally, the study was approved by the nurse councils at the two hospitals where the data were collected. In this descriptive comparative study, data were collected from July 2012 to December 2012.


A convenience sample of 77 nurses was recruited from the ED, intensive care units, or medical-surgical units from two hospitals. To be included, nurse managers agreed that their respective unit admitted patients with SCD.

Study Measures

The Qualtrics© online questionnaire consisted of demographics, the General Perceptions about Sickle Cell Patients Scale (Haywood, Jr. et al., 2010), three items adapted from the Sickle Cell Disease Health-Related Stigma Scale (SCD-HRSS; Jenerette, Brewer, Crandall, & Ataga, 2012) and an open-ended statement requesting any additional comments about nurse perceptions of patients with SCD.

Demographics Questionnaire

The demographic questionnaire requested information in order to describe the sample by age, sex, education, and practice.

Nurse Attitudes

Nurses' attitudes about patients with SCD were measured by the General Perceptions about Sickle Cell Patients Scale (Haywood, Jr. et al., 2010). This 17-item scales measures attitudes and beliefs towards adult sickle cell patients using Likert-type items. The scale is composed of four subscales that measure negative attitudes, positive attitudes, concern raising behaviors, and red flag behaviors. Each subscale score ranges from 0-100 with higher numbers indicating higher levels of the respective attitude. Internal consistency reliabilities have been reported to range from .76-.89, and construct validity was supported with expected correlations with the Medical Condition Regard Scale (Haywood, Jr. et al., 2010).

Stigma Items

The SCD-HRSS is a 30 item Likert scale that measures health related stigma perceived by individuals with SCD. The three adapted items focused on the cause of sickle cell pain, appropriate use of pain medication, and comparing SCD patients to other patients with medical conditions. The adaption allowed nurses to report agreement with these items. The Cronbach's alpha reliability coefficients for the total score and subscales of the SCD-HRSS have been reported to range from .69-.84, and construct validity was supported with expected correlations with the Beck Depression Inventory (Jenerette et al., 2012).


All eligible nurses received a link to the anonymous online survey from their respective nurse manager. Nurses provided informed consent by completing the survey. Nurses who provided their name and e-mail address, which were separated from study data, were eligible for a drawing for a $50 gift card at each of the two hospitals.

Statistical Analyses

Descriptive statistics, including means and standard deviations for continuous variables and proportions for categorical variables, were reported by worksite. Bivariate tests of the association of demographics and other characteristics variables with worksite were performed using t-tests, Fisher's Exact tests, or Cochran Mantel-Haenszel (CMH) tests of general association, as appropriate. Independent sample t-tests and analysis of variance (ANOVA) were employed to compare attitude and behavior outcomes across stigma categories and demographic characteristics. A post-hoc multiple comparison procedure using the Scheffé method was performed to assess pairwise differences among the levels of each factor with significant overall ANOVA F test. The p-values for all hypothesis tests were two-sided and statistical significance was set at p <0.05. All analyses were performed using the SAS software package, version 9.3 (SAS Institute, Cary, NC).



A total of 36 nurses from the ED/ICU and 41 nurses from medical-surgical units were included in this cross-sectional study. Demographic data and other nurse characteristics are summarized in Tables 1 and ​2. The vast majority of study participants were female (89.6%), white (82.6%), non-Hispanic (93.5%), and held a Bachelor of Science in Nursing (62.3%). The mean age for nurses in this sample was 38.2 years (SD 11.8). On average, study participants have been practicing nursing for 11.3 years (SD 11.36) and have been working in their current practice for 8.3 years (SD 8.28). Except for gender (p=0.044), the ED/ICU and medical-surgical groups were similar with regards to demographics and other characteristics.

Table 1

Categorical Baseline Demographic Variables and Other Characteristics of Nurses by Worksite

Table 2

Continuous Baseline Demographic Variables and Other Characteristics of Nurses by Worksite

Effect of Worksite

Independent sample t-tests were conducted to evaluate whether ED/ICU nurses and medical-surgical nurses demonstrated different attitudes and behaviors towards patients with SCD. The results of these tests are reported in Table 3. Overall, nurses working in the ED/ICU were more likely to show negative attitudes, concern-raising behaviors, and red-flag behaviors; they also had lower mean scores for positive attitudes. These differences, however, did not reach statistical significance (p=0.342 for negative attitudes; p=0.661 for positive attitudes; p=0.232 for concern-raising behaviors; p= 0.186 for red flag behaviors).

Table 3

Differences in Nurse Attitudes Toward SCD Patients by Worksite

Effect of Gender and Education

There was no effect of gender on negative attitudes [t(72) = -0.05, p=0.962], positive attitudes [t(74) = -0.82, p=0.413], concern-raising behaviors [t(75) = 0.13, p= 0.899], or on red flag behaviors [t(74) = -0.26, p= 0.793]. Likewise, mean scores across education levels were similar for negative attitudes [F(3,69) = 0.15, p=0.928], positive attitudes [F(3,72) = 1.01, p = 0.395], concern-raising behaviors [F(3,73) = 1.0, p=0.395], and red flag behaviors [F(3,72) = 1.05, p = 0.374].

Effect of Stigma on Nurse Attitudes

One-way analyses of variance (ANOVAs) were conducted to compare the effect of stigma on attitudes (positive, negative) and behaviors (concern-raising, red flags). Tables 4-​7 summarize the ANOVA results for each outcome of interest. These results revealed that the hypothesis of zero effect of the stigma item, “There is a real physical cause for sickle cell pain”, was supported. However, significant differences in attitudes and behaviors were found for the following two stigma items: “Patients use pain medication appropriately”, and “Patients with sickle cell do not complain about their illness any more than patients with other medical conditions”.

Table 4

Means, Standard Deviations, and ANOVAs for Effects of Stigma Variables on Negative Attitudes

Table 7

Means, Standard Deviations, and ANOVAs for Effects of Stigma Variables on Red Flag Behaviors

Post hoc comparisons using the Scheffé procedure indicated that nurses who agreed that “Patients use pain medication appropriately” reported, on average, lower negative attitude scores, lower concern-raising behaviors scores, and higher positive attitude scores than those in either “disagree” or “strongly disagree” categories. Furthermore, nurses who either disagreed or strongly disagreed that “Patients with sickle cell do not complain about their illness any more than patients with other medical conditions” tend to achieve higher mean scores for negative attitudes, concern-raising behaviors, and red-flag behaviors.

Table 8 depicts select nurses' responses to the request for additional comments General Perceptions about Sickle Cell Patients Scale subscale scores are included.

Table 8

Nurses' Comments about Perceptions of Patients with SCD


Although other studies have reported clinicians' attitudes about patients with SCD (Glassberg et al., 2013; Haywood, Jr. et al., 2010; Ratanawongsa et al., 2009) this is the first known published study to explore differences in attitudes by nurse worksite. Additionally, the majority of previous studies focused on ED staff. A focus on the ED is important because many individuals with SCD frequent the ED. However, because care often continues beyond the ED and more patients are being admitted to medical-surgical units, it is important to understand attitudes of nurses in other areas.

Overall, responses to the questionnaire and comments suggest two major themes. First, the non-significant results comparing attitudes of ED/ICU nurses and medical-surgical nurses reveal that explorations about nurse attitudes need to extend beyond the ED. This is especially important because the attitudes most often exhibited by ED/ICU nurses and medical-surgical nurses were negative. This result is in contrast to a study by Ratanawongsa and colleagues (2009) that found that inpatient vs. emergency department providers and nurses vs. other providers had higher positive attitude scores.

Secondly, the survey responses and comments suggest that, in order to minimize the potential for stigmatization based on inaccurate perceptions, nurses need significant education regarding the care of patients with SCD. The comments provided by the nurses were illustrative of attitude scores and provide insight into potential foundations for stigmatizations of patients with SCD. Comments support the previously reported healthcare provider concerns about drug-seeking behaviors and addiction in patients with SCD (Khattab, Rawlings, & Ali, 2005; Lattimer et al., 2010). Nurses have a less negative attitude towards patients with SCD when they agree that the patients used pain medication appropriately. Nurses need to have a better understanding of the basic pathophysiology of SCD as well as the disease trajectory and complications. In order to better understand how patients with SCD use their medication and seek care, nurses need to understand addiction, dependence, and pseudo-addiction. Nurses would better understand care-seeking for the pain of SCD if they understood cue recognition and the factors that influence how individuals with SCD respond to cues of an evolving pain crisis.

Finally, nurses need to understand the coping mechanisms that may develop over a lifetime of living with a chronic disease that often involves both chronic and acute pain. Although there are potential objective indicators of pain such as increased heart rate, grimacing, etc., we know that pain is subjective. Nurses are taught that pain is what the patient says it is and occurs when the patients says it occurs (McCaffery, 1968). Due to coping mechanisms that have developed during the day-to-day management of SCD, patients may not present as expected demonstrating the typical signs of pain. Individuals who live with chronic and/or acute pain experience physiological and psychological changes that account for their pain behaviors and lack of visible signs of pain. Therefore, when a nurse perceives that patients with SCD are not in pain because they can talk on the telephone or pain scores do not match objective indicators, this can lead to stigmatization and delays in treatment. Nurses should never reach the conclusion that there is a better way to assess pain that does not include the patient's self-report.

In addition to more education regarding SCD and care of the patient with SCD (Valente et al., 2010), cultural competency training may be efficacious for nurses to recognize the ways that race, ethnicity, and culture may affect their attitudes and communication behaviors with SCD patients (Ratanawongsa et al., 2009). We know that the majority of individuals living with SCD in the US are African American and race does influence the provision of care.

Although this study revealed important information about nurse attitudes towards patients with SCD, there are some limitations that need to be considered. Even though the influence of the significant stigma variables on the reported outcomes (attitudes, behaviors) was in the direction expected, the observed statistical significance should be interpreted with caution due to sparse sample size (e.g. N=1) in some stigma categories. Additionally, the survey and comments were anonymous. There was no means to follow-up with respondents to clarify comments. Finally, the sample was not diverse as the majority of respondents were White females. Future studies should target larger, more diverse samples. Furthermore, perhaps a mixed-methods approach would provide more insight into nurses' attitudes towards patients with SCD in order to better inform future interventions.

Taken together, the results from both the survey and comments suggest that while the work setting (ED/ICU, medical-surgical) may not matter much, underlying stigma towards patients with SCD stigma may play a significant role in shaping nurses' attitudes and behaviors towards SCD patients. It is important to uncover the foundations that negatively influence the care of patients with SCD so that they can receive timely and appropriate care.


We thank the nurses who participated in this research. The project described was supported by Award Number UL1RR025747 from the National Center for Research Resources as grant 2KR321107 to the first author.


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Sickle-Cell Anemia
Sickle-cell anemia is a term that denotes a group of genetic disorders caused by sickle shaped hemoglobin. In patients with this disease the human red blood cells take a different shape upon deoxygenation because of polymerization of the abnormal sickle hemoglobin. This process causes damage to the red blood cell membrane and causes the red blood cells to get attached in blood vessels. This attachment or adherence of the red blood cells deprives the tissues of oxygen causing ischemia and infarction. The disease is chronic with periodic painful attacks, damage of internal organs with complications of strokes and subsequent shortened lifespan.Sickle-cell anemia is a specific form of sickle-cell disease caused by a homozygous mutation forming Hgb S. Other forms of sickle-cell disease include sickle-haemoglobin C disease, sickle beta-plus-thalassemia, sickle beta-zero-thalassemia and hemoglobin ss caused by heterozygous genes (Orah, 2000). Most children with SCD are healthy at birth and exhibit symptomslater after decrease in levels of fetal hemoglobin (HbF) and can be identified by routine screening procedures (SCDG, 1993; NSTF, 2000 and Pass, 2000). Every child with SCD should receive care coordinated only through an appropriate medical home (NSTF, 2000) and a paediatric nurse plays an important role in the care for SCD children.

Care for Sickle Cell Infants and Children
All infants with HbSS and Sßo thalassemia are usually given  penicillin V potassium prophylaxis, 125 mg orally, twice a day,initiated by 2 months of age (NHLBI, 2002; SCDG, 1993; Gaston, 1986 and AAP, 2000). Erythromycin prophylaxis may be used as an alternative for suspected penicillin allergy. Immunization of children with SCD includes the 7-valent pneumococcal conjugate and 23-valent pneumococcal polysaccharide vaccine and quadrivalent meningococcal polysaccharide vaccine (AAP, 2000; AAP, 2002; Overturf GD and AAP, 2000; AAP, 2000).Routine medical evaluations includes review of previous disease symptoms, laboratory findings, monitoring growth and development, detection of early signs of organ damage (Pass, 2000), evaluation of blood pressure (Pegelow, 1997),detection of splenomegaly, CNS complications, proliferative retinopathy, restrictive lung disease, pulmonary hypertension, cholelithiasis, proteinuria, avascular necrosis of the hip or shoulder, and leg ulcers.

SCD Complications and Nurse Care
Acute painful episodes are one of the most common reasons for the hospitalization of a child with SCD (Jakubik, 2000).Nursing interventions for such painful episodes include obtaining history of past and present management, pain assessment self-report, assessment of physiologic indicators especially blood pressure, behavioral indicators like activity level, ease of movement of affected area, frequency, administration of pharmacologic interventions like use of anti-inflammatory, use nonpharmacologic interventions like heat, massage or guided imagery, hydration and prevention of complications. The most common complication of an acute painful episode is acute chest syndrome that can be defined as any new infiltrate found on a chest x-ray (Lane, 1996). Nursing interventions in acute chest syndrome include adequate pain control, promoting patient mobility and incentive spirometry (Jakubik, 2000). Septicemia due to Streptococcus pneumoniae is a common cause of death in SCD children especially younger than 5 years old (Reid et al., 1995). This enhanced risk for such bacterial infections is attributed to splenic dysfunction. Nursing interventions includes prompt IV antibiotic and antipyretic administration, monitoring of vital signs for signs and symptoms of sepsis. Splenic sequestration is a problem unique to children with SCD and is a significant cause of morbidity and mortality (Kinney, 1990). Nursing interventions for splenic sequestration include intravenous fluid administration, PRBC administration, and monitoring of cardiovascular status. Administration of a standard 10 cc/kg PRBC transfusion increases the circulating volume of blood beyond what would normally be expected due to the introduction of the previously trapped RBCs (Zimmerman et al., 1997). Splenectomy should be done if a child has two or more sequestration episodes on account of the mortality associated with severe splenic sequestration. But unfortunately, children with sickle cell disease undergoing splenectomy develop acute chest syndrome easily because of their inability to defend infections and postoperative complications of general anesthesia. Nursing interventions include administration of broad spectrum antibiotics and vaccination against pneumococcus infection to prevent ACS (Wong et al, 1995).A laparoscopic, intracorporeal, splenic fragmentation technique is being employed to remove spleens in SCD children to avoid such complications (Phippen,1994). Assessing and treating recurrent and unpredictable pain in children with sickle cell disease is a complex process Armstrong, 1992)Aplastic crisis is another complication with an exacerbation of the patient's baseline anemia with a substantially decreased reticulocyte count, typically less than 1% (NHLBI, 2002; SCDGP,1993) caused by acute infection with human parvovirus B19, usually without the characteristicrash. Typical interventions include comparison of CBC and reticulocyte counts obtained during acute illness with baseline values and Red blood cell transfusions to prevent heart failure (Vichinsky, 2001).Acute neurologic symptoms and signs of stroke in SCD children include hemiparesis, aphasia or dysphasia, seizures, monoparesis, severe headache, cranial nerve palsy, stupor and coma (Frempong,1991). Initial interventions include CBC and reticulocyte counts and noncontrast computed tomography or magnetic resonance imaging to exclude hemorrhage (NHLBI, 2002). Alloimmunization can be prevented by Red blood cell minor antigen phenotype (Pegelow, 1997; Vichinsky, 2001; Rosse, 1990; Tahhan, 1994). Treatment includes anticonvulsants, other supportive care for seizures or increased intracranial pressure and a protocol of chronic transfusions. Acute initiation of transfusion is by partial exchange transfusion or erythrocytapheresis (Vichinsky, 2001). Transcranial Doppler ultrasonography aids identification of children at highest risk of stroke (Adams, 1997).Priapism is another complication common in children and adolescents with SCD. Priapism is a prolonged painful erection of the penis often occurring in the early hours of the day (Mantadakis, 1999).Priapism can occur either as stuttering episodes that last fewer than 2 to 4 hours or as severe episodes that last more than 2 to 4 hours and results in impotence. Interventions include hydration, analgesics, aspiration, irrigation and sometimes blood transfusions (Mantadakis, 2000).

The Psycho- Social Context of Nurse care of SCD Child
Conceptual models have been found to enhance the understanding and help the mediating factors such as professional knowledge, attitudes and beliefs, interpretation of objective data and resulting treatment decisions Armstrong, 1992. A Health Belief Model based on cognitive behavioural approach has been recently evaluated in working with clients diagnosed with sickle cell anemia (Scott, 1999).A recent study has identified disease-related risk factors and psychosocial resistance factors that impact adherence to prescribed acute care treatment for children with sickle cell disease in a sample of 24 children and has indicated moderate-to-high adherence(Barakat, 2004). Poorly coordinated and resourced haemoglobinopathy service has been identified as major problems for SCD care (Atkin, 1998). Sickle cell children with frequent episodes of pain crisis have been found to develop psychological problems like anxiety, depression, poor school performance, decreased participation in normal activities of daily living, and poor peer and family relationships (Jacob, 2001; 2006).

A paediatric nurse plays an important role in the care for SCD children especially during acute painful episodes, splenic sequestration and aplastic crisis. Poorly coordinated andresourced haemoglobinopathy service has been identified as major problems for SCD care.



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