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 symptoms later after decrease in levels of fetal hemoglobin (HbF) and can be identified by routine screening procedures (SCDG, 1993; NSTF, 2000 and Pass et.al, 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 et.al, 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 et.al, 2000), evaluation of blood pressure (Pegelow et.al, 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 et.al, 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 et.al, 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 et.al, 1997; Vichinsky, 2001; Rosse et.al, 1990; Tahhan et.al, 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 et.al, 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 et.al, 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 et.al, 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 et.al, 2004). Poorly coordinated and resourced haemoglobinopathy service has been identified as major problems for SCD care (Atkin et.al, 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).

Conclusion
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 and resourced haemoglobinopathy service has been identified as major problems for SCD care.

References

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