Noisy Water Review

The Psychosocial Effects of Sickle Cell Disease
in the African American Population

Terri Anderson

Abstract

Sickle cell disease (SCD) is a genetic abnormality that deforms the red blood cell into a sickle shape that does not travel easily through the circulatory system. People of African descent are the primary victims of this debilitating disease. Every aspect of the life of the client with SCD is greatly affected. Clients with SCD have expressed their concerns about the lack of education among healthcare providers and the community, lack of skill in treating SCD, and the stigmatization of the healthcare system toward clients with SCD. Clients repeatedly become labeled as drug seekers due to the predominant symptom of severe pain and are undertreated. Education of the healthcare team about the pain associated with SCD can enable the nurse to advocate for timely and effective pain relief for the client. The ER nurse can empower themself and others to develop an open and trusting relationship with the client afflicted with SCD and avoid the stigma of a drug seeker. From school age children to adults, clients with this disease are often isolated and mistreated. The school nurse and teachers are in a prime position to work together and with the parents to educate each other and the community about this disease and the best ways to support the families. The disruption in family life is significant for the client and the caregivers. Families are separated for extended periods of time for hospitalizations and treatments creating increased stress and caregiver role strain. An increase in education among health care providers who in turn can educate the community about SCD would influence positive outcomes for these clients from infancy to adulthood. Advocacy for these clients is lacking, and nurses are in a position to be the advocates for an increase of quality of care and community support.

The Psychosocial Effects of Sickle Cell Disease in the African American Population

Sickle cell disease (SCD) is one of the most common genetic diseases in the United States, affecting approximately 70,000 African-Americans occurring in about 1 of every 500 African-American births (Mann-Jiles & Morris, 2009). SCD is a chronic, autosomal recessive disorder identified by the predominance of the protein hemoglobin S (HbS) in red blood cells (Gold, Mahrer, Treadwell, Weissman, & Vichinsky, 2008). Hemoglobin is critical for the transport of oxygen throughout the body and is composed of two alpha globulin chains and two beta globulin chains. In sickle hemoglobin, the beta globulin differs from normal globulin by the substitution of valine for glutamic acid at the sixth position resulting in a crescent (sickle) shaped red blood cell (Gold, et al., 2008). Due to the odd shape of the red blood cell, it does not move easily through the blood vessels and obstructs vital blood flow to tissues and organs. After repeated cycles of deoxygenation and reoxygenation, sickled cells sustain permanent damage and are destroyed at a high rate, causing anemia (Gold, et al., 2008). The disease is characterized by chronic hemolytic anemia, increased susceptibility to infections, extensive organ damage, intermittent pain episodes, and chronic pain, all of which can range from mild to severe, often requiring emergency intervention (Mann-Jiles & Morris, 2009).

In addition to the debilitating physical symptoms and severe pain of SCD, the psychological and social effects of the disease have a significant impact on quality of life (Knight-Madden, Lewis, Tyson, Reid, & MooSang, 2011). The life of the person with SCD is continually interrupted and limited by physical symptoms, severe pain, hospitalizations, and treatments. The absences from work, school, and social functions create feelings of isolation and depression leading to negative coping behaviors (Mann-Jiles & Morris, 2009). This paper will examine the psychosocial impact of living with sickle cell disease and the nurse’s role in implementing interventions to achieve positive psychosocial outcomes and improve quality of life among the African American population.

Methods

Research was conducted through EBSCO host and the Health Source: Nursing Academic Addition database. The research term used in the EBSCO search engine was “sickle cell disease.” Research results were limited by the selection of peer reviewed journals and publication date of October 2007 through October 2012. Articles were chosen upon current research related to the psychosocial effects of sickle cell disease in the African American population. Articles chosen were of American peer-reviewed journals only.

The article by Graff, Hankins, Hardy, Hall and Roberts (2010), Exploring Parent-Sibling Communication in Families of Children with Sickle Cell Disease, reveals the findings of interviews with parent of children with SCD and the positive effects of communication within the family and members of the community. Their experiences of how providing care has affected their life relates to the topic of this paper.

The article by Bediako, Lattimer, Haywood Jr, Ratanawongsa, Lanzkron, & Beach (2011) was chosen for their examination of religious coping and hospital admissions among African Americans with SCD. Their article, Religious Coping and Hospital Admissions Among Adults with Sickle Cell Disease, discusses religion as a positive coping mechanism and how it is associated with positive outcomes. Their study relates to the topic of this paper as it is aimed directly at African Americans with SCD and how religion influences their quality of life.

The article titled, Quality of Life of Adult Patients with Sickle Cell Disease, written by Mann-Jiles and Morris (2009) discusses their study of quality of life in adult patients with SCD in comparison with the general population. This article was chosen for the information provided about specific aspects affecting patients’ quality of life. The psychological and psychosocial information provided relates to the topic of this paper.

The article, Psychosocial and Behavioral Outcomes in Children with Sickle Cell Disease and Their Healthy Siblings, written by Gold, Mahrer, Treadwell, Weissman, and Vichinsky (2008), examines the psychosocial functioning among children with SCD and their healthy siblings including caregiver evaluation of the children’s behavior. This article was chosen for the study of behavioral outcomes among children with SCD. The article relates to the topic of this paper for the discussion of positive psychosocial outcomes and behavioral problems.

The articles by Knight-Madden, Lewis, Tyson, Reid, and MooSang (2011), The Possible Impact of Teachers and School Nurses on the Lives of Children Living with Sickle Cell Disease, and two articles by Haywood, Lanzkron, Ratanawongsa, Bediako, Lattimer, Powe, and Beach (2010), A Video-Intervention to Improve Clinician Attitudes Toward Patients with Sickle Cell Disease: The Results of a Randomized Experiment, and (2010) The Association of Provider Communication with Trust Among Adults with Sickle Cell Disease, were used as support of information found in the additional research articles. These articles were chosen for the relevance of the interventions and positive psychosocial outcomes.

Discussion

African Americans with SCD face stigma, discrimination, lack of specialty care, family and work issues, social isolation, disruptions in social activities and relationships, and significant risk for early mortality, all of which contribute to quality of life (Mann-Jiles & Morris, 2009). Research conducted by Mann-Jiles and Morris (2009) found evidence that psychological and social factors contribute substantially to complaints of pain. These authors identified factors related to pain issues such as race, gender, age, education, socioeconomic status, coping mechanisms, and social support. The disease process itself leads to leg ulcers, delayed growth and sexual maturation creating low self-esteem and poor body image. SCD negatively affects family functioning causing increased stress and depression among all family members. Frequent hospitalizations restrict the parents and siblings’ opportunities to form or sustain relationships causing them to become isolated. One study reviewed by Mann-Jiles and Morris (2010) found that black clients had significantly increased pain scores when compared to white clients, while white clients were prescribed opioids more often than black clients due to the providers concerns of addiction, tolerance, and side effects. This indicates an important need for increased education of SCD among healthcare providers and the importance of nurse advocacy for greater quality of care without discrimination among this population.

The school life of a child with SCD can be a significant factor in their psychosocial functioning with both positive and negative outcomes. Due to the significant symptoms of SCD, children are treated differently among their peers along with increased absenteeism and cognitive deficits (Knight-Madden et al., 2010). Teachers and school nurses with the knowledge of SCD can intervene to improve the child’s quality of life. Complications of SCD can be detected early if the teacher and school nurse are taught what symptoms to watch for and how to detect them. Teachers can provide education to the class about SCD in an attempt reduce teasing and isolation of the affected child from the other students. It is important for the teacher to discourage comparisons between children living with SCD and their unaffected siblings who may have better academic performances (Knight-Madden et al., 2010). Increased disease-related absences will benefit from schoolwork being sent home or to the hospital to encourage the child to continue to learn. Knight-Madden et al. (2010) concludes that school-nurse case management for children aged 5-19 years old with SCD has been shown to improve educational and psychosocial outcomes, and nurses have sought to be at the forefront in trying to reduce health disparities such as those in children who suffer from SCD.

Interviews conducted by Graff et al. (2010) explored parent-sibling communication about SCD along with factors contributing to their experience of living with SCD and/or caring for a child with SCD. There is a significant demand of time and energy from the parents of a child with SCD, which can negatively affect siblings. Graff et al. (2010) found that parents felt uneducated or undereducated about SCD and have difficulty explaining it but were eager to learn more about the disease and communicate more effectively with their family and community. Open and problem-solving communication can lead to better psychological adjustment for siblings (Graff et al., 2010). Members of the community witnessing a crisis are fearful and avoid the child leaving the parent without support or guidance. Parents who are able to communicate clearly the effects and cause of the crisis can educate the community and receive support and acceptance of the child. For parents who find it difficult to communicate with their children or others, the nurse can intervene by providing educational materials, identifying resources, and introducing social networks within the community for guidance.

For clients with SCD and parents of children with SCD, navigating the health care system creates increased stress for the entire family. This author found that negative outcomes related to emergency room visits among clients with SCD was a recurring topic in the literature reviewed. Severe pain that varies in intensity and duration is what brings the client to the ER on numerous occasions. The pain of SCD is misunderstood and undertreated by the health care team. Graff et al. (2010) found that parents preferred a specialized clinic and avoided taking their sick child to the emergency room due to lack of skill and education of the health care workers about SCD. Increased sibling distress was associated with the number of ER visits as well as behavior problems in the child with SCD (Graff et al., 2010). There are long waits in the emergency waiting room despite the child experiencing a sickle cell crisis. Efficient and timely treatment of the client with SCD can help to decrease the amount of stress on the entire family. A nurse-initiated protocol (NIP) for a patient experiencing a sickle cell crisis should be implemented in all emergency rooms and hospitals as well as specialized training for IV starts and blood draws in patients with sickle cell disease.

The research study conducted by Gold et al. (2008) reported no significant behavioral issues between children with SCD and their healthy siblings with some exception. Their report found positive psychosocial functioning without clinically significant behavioral problems with the exception of somatic complaints and internalizing behavior such as withdrawal, anxiety, depression, and thought problems. In addition, Gold et al. (2008) found an increase in externalizing behavior problems such as aggression, delinquency, attention problems, and social problems that led to increased ER visits. Internalizing behaviors may go undetected by medical professionals and lead to increased external behaviors. By reviewing the complete medical history of the child including the number of ER visits, the nurse can determine the extent of increasing external behaviors and intervene by providing resources for additional medical/psychosocial treatment.

Within the African American community, the church is a significant source of support. The members of the church share the burdens of the families struggling with SCD and provide emotional as well as physical support. For single parents who have multiple roles within the family, the church is a source of strength and guidance. Bediako et al. (2010) revealed that religious coping such as Bible study, prayer, and church attendance led to positive health outcomes and fewer hospital admissions. Given that SCD predominately affects African Americans, attention by healthcare professionals to this culture’s dependence on the church can greatly influence health outcomes. Holistic nursing for this population that incorporates increased pastoral care and spiritual support as primary nursing interventions will influence psychological well-being.

The treatment and support of the client with SCD at any age and the family requires education of the entire community. From school nurses and physicians to members of the church, everyone in contact with the client can be given the tools necessary to positively affect the wellbeing of the client providing positive psychosocial outcomes and a greater quality of life. Greater attention is needed for the possibility of an increase in the number of specialized clinics in densely populated areas of clients with SCD to provide care without stigmatization and a decrease in exacerbations by timely treatment and expert skill provided by specially trained nurses. For urgent care, the ER nurse should be armed with the necessary skill and compassion to identify a sickle cell crisis and act quickly to reduce the amount of strain and frustration on the family. Nurse advocacy for education and treatment for the child with SCD greatly increases positive psychosocial outcomes and may influence the adult life of the child by reducing stigmatization and providing opportunities for continued growth and positive functioning within society.

References

Bediako, S. M., Lattimer, L., Haywood Jr, C., Ratanawongsa, N., Lanzkron, S., & Beach, M. C. (2011). Religious Coping and Hospital Admissions Among Adults with Sickle Cell Disease. Journal of Behavioral Medicine, 34, 120-127.

Gold, J. I., Mahrer, N. E., Treadwell, M., Weissman, L., & Vichinsky, E. (2008). Psychosocial and behavioral outcomes in children with sickle cell disease and their healthy siblings. Journal of Behavioral Medicine(31), 506-516.

Graff, C. J., Hankins, J. S., Hardy, B. T., Hall, H. R., Roberts, R. J., & Neely-Barnes, S. L. (2010). Exploring Parent-Sibling Communication in Families of Children with Sickle Cell Disease. Issues in Comprehensive Pediatric Nursing, 33, 101-123.

Haywood Jr, C., Lanzkron, S., Hughes, M. T., Brown, R., Massa, M., Ratanawongsa, N., & Beach, M. C. (2010). A Video-Intervention to Improve Clinician Attitudes Toward Patients with Sickle Cell Disease: The Results of a Randonmized Experiment. Journal of General Internal Medicine, 26(5), 518-24.

Haywood Jr, C., Lanzkron, S., Ratanawongsa, N., Bediako, S., Lattimer, L., Powe, N. R., & Beach, M. C. (2010). The Association of Provider Communication with Trust Among Adults with Sickle Cell Disease. Journal of General Internal Medicine, 25(6), 543-8.

Knight-Madden, J. M., Lewis, N., Tyson, E., Reid, M. E., & MooSang, M. (2011). The Possible Impact of Teachers and School Nurses on the Lives of Children Living With Sickle Cell Disease. Journal of School Health, 81(5), 219-22.

Mann-Jiles, V., & Morris, D. L. (2009). Quality of life of adult patients with sickle cell disease. Journal of the American Academy of Nurse Practitioners(21), 340-349.

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