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Unformatted text preview: SICKLE CELL CRISIS Sickle cell disease (sickle hemoglobinopathies) is a group of genetic diseases, the most common forms being homozygous hemoglobin SS disease (sickle cell anemia), hemoglobin SC disease, and sickle [beta]-thalassemia. Sickle cell disease primarily affects black populations and people of Mediterranean, South/Central American, Arabian, and East Indian descent. It renders the individual vulnerable to repeated painful crises that can progressively destroy vital organs. These crises are: Vaso-occlusive/thrombocytic crisis: Related to infection, dehydration, fever, hypoxia, and characterized by multiple infarcts of bones, joints, and other target organs, with tissue pain and necrosis caused by plugs of sickled cells in the microcirculation. Hypoplastic/aplastic crisis: May be secondary to severe (usually viral) infection or folic acid deficiency, resulting in cessation of production of RBCs and bone marrow. Hyperhemolytic crisis: Reticulocytes are increased in peripheral blood; and bone marrow is hyperplastic. Characterized by anemia and jaundice (effects of hemolysis). Sequestration crisis ( more commonly occurs in infants ) : Massive, sudden erythrostasis with pooling of blood in the viscera (splenomegaly), resulting in hypovolemic shock/possible death. This crisis occurs in patients with intact splenic function. CARE SETTING Sickle cell disease is generally managed at the community level, with many of the interventions included here being appropriate for this focus; however, this plan of care addresses sickle cell crisis, which usually requires hospitalization during the acute phase. RELATED CONCERNS Cerebrovascular accident (CVA)/stroke Cholecystitis with cholelithiasis Chronic obstructive pulmonary disease (COPD) and asthma Cirrhosis of the liver Heart failure: chronic Pneumonia: microbial Psychosocial aspects of care Seizure disorders Sepsis/septicemia Patient Assessment Database Depends on severity of condition, presence of complications. ACTIVITY/REST May report: Lethargy, fatigue, weakness, general malaise Loss of productivity; decreased exercise tolerance; greater need for sleep and rest May exhibit: Listlessness; severe weakness and increasing pallor (aplastic crisis) Gait disturbances (pain, kyphosis, lordosis); inability to walk (pain) Poor body posture (slumping of shoulders indicative of fatigue) Decreased range of motion (ROM) (swollen, inflamed joints); joint, bone deformities Generalized retarded growth; tower-shaped skull with frontal bossing; disproportionately long arms and legs; short trunk; narrowed shoulders/hips; and long, tapered fingers CIRCULATION May report: Palpitations or anginal chest pain (concomitant coronary artery disease [CAD]/myocardial ischemia, acute chest syndrome) Intermittent claudication May exhibit: Apical pulse: Point of maximal impulse (PMI) may be displaced to the left (cardiomegaly) Tachycardia, dysrhythmias (hypoxia), systolic murmurs BP: Widened pulse pressure Generalized symptoms of shock, e.g., hypotension, rapid thready pulse, and shallow Generalized symptoms of shock, e....
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