Topic: Sickle Cell Anemia        
Researched and written by: Maritta H. Lee
                                                I attest that the following report is a product of my own original work.

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OVERVIEW/DEFINITION OF SICKLE CELL ANEMIA      Sickle Cell Anemia is a blood disorder in which red blood cells are affected, making them abnormal.  As cited in K. D. Scott & A. A. Scott (1999), Sickle Cell Anemia is rated the most common genetic disease in the United States (Behram & Vaughn, 1983), and is most common among the African American population (Carter,1983; Nash, 1994; Powars, Overturf, and Weiss, 1981).  Adults carrying the Sickle Cell trait can pass the trait on to their children at birth.  The newborn child can inherit the Sickle Cell trait or just Sickle Cell Anemia.  As cited in K. D. Scott & A. A. Scott (1999), Sickle Cell Anemia is more of a chronic type of anemia, where the Sickle Cell trait is more of a mutation of the hemoglobin (Francis & Johnson, 1991).  However, a high mortality rate exists in the first five years of a child’s life (Chapar, Blair, & Stowe, 1991). STATISTICS AND CHARACTERISTICS OF SICKLE CELL ANEMIA      Sickle Cell Anemia can manifest itself in both males and females.  Although Sickle Cell Anemia is most dominant in the African American population, no information was found isolating Sickle Cell Anemia to one race.  As cited in V. E. Maikler, M. E. Broome, P. Bailey, & G. Lea (2001), one out of every 375 African American children are affected by Sickle Cell disease, and over 50,000 African Americans suffer from this disease (Agency For Health Care Policy and Research).  As with any other disorder/disease, there are many complications to having Sickle Cell Anemia. <>     As cited in K. D. Scott & A. A. Scott (1999), Sickle Cell Anemia can be traced to changes in the way the red blood cells are made up (Wang et al., 1993).  These abnormal red blood cells can take different forms, such as being sickled, rigid, or elongated.  When the red blood cells take on these forms, they become trapped in the blood vessels and block the flow of  oxygen (Sergeant, 1992).  This process is called hypoxia, and can cause a variety of difficulties to take place.  These difficulties can be fatigue, damage to multiple organ systems, such as the liver and spleen, heart, lungs, kidneys, and causing chronic pain (Carter, 1983; Whitten, 1992).  For example, when the spleen is affected, jaundice becomes more visible.  Sickle Cell can also contribute to infectious diseases, such as urinary tract infections. <>     As cited in K. D. Scott & A. A. Scott (1999), there is an infectious disease that is detrimental to a child’s life.  This disease is called Sepsis, and can lead to death in the first three to five years of a child’s life (Berg, 1994).  A child’s central nervous system is also affected by the abnormal red blood cells, which can cause the child to have a stroke.  Strokes can affect motor, intellectual, and sensory impairments from which a child can either recover or die.  Vision can also be affected, as well as suffering from seizures.  However, the most common symptom of Sickle Cell Anemia is the chronic continuous pain, and physical limitations of the child due to having frail bone structure.  These symptoms can affect adolescent children as well.  Adolescent children will have impairments in sustained attention and academic achievement.  Children with Sickle Cell Anemia will also have complications with cognitive and psychological problems, and delays in developmental milestones, such as learning to walk.  Their lives are greatly impacted by the Sickle Cell disease.  They spend most of their time isolated from their peers, as well as their school, due to the effects that Sickle Cell has on the body.  This has an impact on their social skills, which are poor, and on their self- image.  Most children who suffer from Sickle Cell Anemia come from low socioeconomic families, and do not have insurance. <> <> EDUCATION/CLASSROOM ISSUES L. Nettles’ (1994) research focused on the scholastic performance of children with the Sickle Cell disease.  As cited in A. L. Nettles (1994), education is very important to have when you are a child that has Sickle Cell disease.  However, children with Sickle Cell Anemia, have difficulties with their academics.  Their absenteeism was thought to be an indicator as to why they were having difficulties with their academic achievement (Vaughn & Cooke, 1979; Whitten & Nishiura, 1985; Williams, Earles, & Pack, 1983).  Research has found that absenteeism does play a part in their academic struggle.  Their constant absence at school affects not only their academic achievement, but also their peer relations.  Another factor is the child’s socioeconomic status, especially, if their status is low.  Children of a higher socioeconomic status score higher on intelligence tests than children of lower socioeconomic status.  These children have learning deficits in reading and spelling skills, making them feel less competent and accepted by their peers in school.  They attend regular classes unless their disease has affected them in such a way that they need assistance with their schoolwork.  They will be placed in a special education curriculum.  Girls seem to perform much higher than boys, in their academic achievement (Farber et al., 1985).     In another study the focus is on the social interactions between children with cancer, or children with Sickle Cell Anemia, and their peers at school.  Teachers were to observe children with cancer, brain tumors, and Sickle Cell disease, and rate them on their social skills with their classmates.  The focus was on the children that were between 8 and 15 years of age.  The age of 8 is when a child’s chronic illness, race, gender, and closest to their date of birth (Noll R., LeRoy S. S., Bukowski W. M., Davies  W. H. et al., 1991).  Their match was a child who did not have a chronic illness.  The teachers were asked to assess the children and fill out the Revised Class Play (RCP) assessment.  This tool is used for measuring a child’s social reputation, and focuses on the roles that the child takes while interacting with their peers.  First, is the socialbility leadership role where everyone likes the child.  Second, is the aggressive-disruptive role where the child displays bullying behavior by picking on his peers, or is bossy.  Third, is the sensitive-isolated role where the child is always sad, and is not included in any activities with his/her peers (Masten, A. S., Morison, P., Pellegrini, D. S., 1985; Morison, P., Masten,  A. S., 1991; Rubin, K. D., Cohen, J. S., 1986).  Three additional roles were added to the (RCP) assessment for measurement by the teachers.  These roles focus on the chronic illnesses of the children.  The teachers rated the children on how often they get sick, how often they miss school, and how exhausted they become.  Primary classroom teachers conducted the Revised Class Play assessment for elementary school children, English teachers conducted the (RCP) assessment for middle school children.           The results of the teachers ratings found that the children with cancer were rated higher in their socialbility-leadership roles, and lower in their aggressive-disruptive roles.  There was no difference in the sensitive isolated role between the children with cancer and the children without cancer.  The children with brain tumors, who were survivors, were rated high by the teachers in the sensitive-disruptive roles of the children with brain tumors, and the children without brain tumors.  There were no differences in the roles for the children with Sickle Cell disease.  Althought, they were rated low on the socialbility-leadership role.  The children with brain tumors, and cancer were not rated by their teachers as being exhausted, even though these children were receiving chemotherapy.  The children with Sickle Cell disease were rated as always being exhausted.  As cited in B. S. Shapiro, D. F. Dinges, E. C. Orne, N. Bauer, et al., (1994), the pain related to Sickle Cell disease is reported to affect girls more so than boys. TREATMENT ISSUES RELATING TO THE CLASSROOM      There is not a lot of information about treatment issues of Sickle Cell disease in the Classroom, the focus is geared more toward how Sickle Cell disease is treated, and how the effects of the treatment affect the child.  What we do know is that chronic pain is the most common effect on the body of a child with Sickle Cell disease.  As cited in L. P. Barakat, K. Smith-Whitley, & K. Ohene-Frempong (2002), this chronic pain can cause poor attendance in school if not treated.  A child may have to visit the emergency room or be hospitalized, at least twice a year (Hurtig & White, 1986).          As cited in K. D. Scott & A. A. Scott (1999), there isn’t a lot of information readily available for families of children with Sickle Cell Anemia to educate themselves on the disease.  A lot of times the families of children with Sickle Cell Anemia do not have the right insurance to cover treatment of their child’s disease, because the disease received minimal funding.  This keeps children from receiving the right kind of health care.  Sickle Cell Anemia can affect the children while in the classroom.  They become distracted more easily, their ability to keep attention is poor, as well as having poor concentration (Brown, Buchanan, et al., 1993; Fowler et al., 1988; Swift et al., 1989).  There are days at a time when children with Sickle Cell disease are absent, because of being medically treated for their interacting with peers their own age, their social skills start to become affected.  The severity of the disease will determine how long the child will be absent from school.  These absences from school lead to the child having problems with their self-esteem, and the child being reluctant to learn new things.  This puts the child at psychosocial risk, and also places the child at risk for poor school performance and academic achievement.       As cited in L. P. Barakat et al., (2002), treatment must be implemented when the child with Sickle Cell disease starts to exhibit signs of discomfort.  When this treatment is implemented, the child will have to be treated in the home.  This is due to the medication he or she will have to take.  The child will start taking analgesic medication that will help fight infectious disease in the child’s body.  The effects of the treatment of Sickle Cell disease are unpredictable and can be fatal (Lemanek, Buckloh, Woods, & Butler, 1995).  As children with Sickle Cell disease grow older they have a tendency to not sticking to their treatment regimens.  There are many factors as to why this happens.  These factors are the children being under stress because of coming from poor backgrounds, and being the ethnic minority in the United States, which affects African Americans directly (Baskin, et al., 1998).  Being able to measure adherence for treatment is more complicated by the type of disease and it’s treatment.  There are some recommendations concerning the adherence to treatment of children with Sickle Cell disease that vary.  This depends on how severe the disease is and prior displays of symptoms that occur.  Managing pain and fever receive standard recommendations for treatment, which are usually limited.  Treatment that prevents a relapse of the disease is preventative treatment, which can be implemented in the home by using medication.  Treatment of pain requires the use of prescription medication to help with the pain, in some cases.  Normally prescription medication is used to fight infection.  Lack of trust of health care professionals, and the inconsistency of health care professionals is due to families seeking treatment in emergency rooms (Viera, 1986).          As cited in V. E. Maikler, et al., (2001), the focus is on the levels of pain that a child with Sickle Cell Anemia experiences.  Some forms of treatment for Sickle Cell Anemia are transfusions, transplantation, and gene therapy (Bunn, 1999; Reed & Vichinsky, 1998; Steinberg, 1996).  There is also treatment for acute pain episodes such as hydration (Shapiro, 1993; Steinberg, 1999).  Children who have the Sickle Cell disease are learning to deal with their illness.  During treatment the child is given a diary to keep track of his or her pain, the severity of the pain, and how often it occurs.  This is typically done in the home while in treatment, but can also be done while hospitalized.  These diaries are more of a record keeper or log of the data the children will record.  They are self-report techniques that will help the child keep track of how often the chronic pain occurs and  how severe (painful) it gets (Shapiro et al., 1990).  These pain intensity reports rate pain as being mild, moderate, and severe.  These diaries will help determine future possibilities for adolescent children with Sickle Cell disease to be able to self medicate themselves, and for younger children with Sickle Cell disease to have a parent administer the medication.  Hospitalization would not be needed for children who were suffering from chronic pain due to Sickle Cell disease.   There are certain risk factors that affect the adaptation of children with Sickle Cell disease and their parents.  These risk factors are stress-processing (coping), social-ecological (family functioning), and intrapersonal factors (Wallander et al., 1988).  If the parent and child aren’t actively using coping strategies, the greater the use of health care services when a child is in pain.  On the other hand, less health care services are used when the parent and child are actively using coping strategies.  For further information on  Sickle Cell disease please go to the following web sites:     <>