sickle cell crisis, an acute episodic condition that occurs in children with sickle cell anemia. The crisis may be vasoocclusive, resulting from the aggregation of misshapen erythrocytes, or anemic, resulting from bone marrow aplasia, increased hemolysis, folate deficiency, or splenic sequestration of erythrocytes. See also hemoglobin S, sickle cell anemia. ▪ OBSERVATIONS: Painful vasoocclusive crisis is the most common of the sickle cell crises. It is usually preceded by an upper respiratory or GI infection without an exacerbation of anemia. The clumps of sickled erythrocytes obstruct blood vessels, resulting in occlusion, ischemia, and infarction of adjacent tissue. Characteristics of this kind of crisis are leukocytosis; acute abdominal pain from visceral hypoxia; painful swelling of the soft tissue of the hands and feet (hand-foot syndrome); and migratory, recurrent, or constant joint pain, often so severe that movement of the joint is limited. Persistent headache, dizziness, convulsions, visual or auditory disturbances, facial nerve palsies, coughing, shortness of breath, and tachypnea may occur if the central nervous system or lungs are affected. Other problems associated with vasoocclusion include priapism, hematuria, and retinopathy. Anemic crisis is characterized by a dramatic, rapid drop in hemoglobin levels resulting from various causes. Aplastic crisis resulting in severe anemia occurs because red blood cell production is diminished by acute viral, bacterial, or fungal infection. Megaloblastic anemia (another form of anemic crisis) results from folic acid deficiency during periods of accelerated erythropoiesis. Severe anemia between crises is not common unless a generalized state of malnutrition exists. Hyperhemolytic crisis, characterized by anemia, jaundice, and reticulocytosis, results from glucose-6-phosphate dehydrogenase deficiency or occurs as a reaction to multiple transfusions. Acute sequestration crisis, which occurs in young children 6 months to 5 years of age, results when large quantities of blood suddenly accumulate in the spleen, causing massive splenic enlargement, severe anemia, shock, and, ultimately, death. Susceptibility to infection is a common problem of young children with sickle cell anemia and may be greatly increased during periods of crisis. Systemic infection and septicemia from pneumococcus or Haemophilus influenzae are not uncommon and may be rapidly fatal. In older children, local infection, especially osteomyelitis, rather than generalized septicemia is frequently a complicating factor. ▪ INTERVENTIONS: Therapy consists of immediate transfusion of packed red blood cells in the acute anemic crisis and alleviation of severe abdominal and joint pain with analgesics or narcotics as needed in vasoocclusive crisis. Short-term oxygen therapy, hydration by oral or IV means, electrolyte replacement to counteract metabolic acidosis resulting from hypoxia, and antibiotics to treat any existing infection may be necessary. Pneumococcal and meningococcal vaccine is recommended for children between 2 and 5 years of age because they are highly susceptible to infection. Partial exchange transfusions are often mandatory in life-threatening crises, such as when sickling occurs in the vessels of the brain or lungs, and may be used as a preventive technique; multiple transfusions, however, increase the risk of hepatitis, hemosiderosis, and transfusion reactions. Oral anticoagulants have been used to relieve the pain of vasoocclusion, but these increase the risk of bleeding. Priapism, a painful condition frequently seen in vasoocclusive crisis, may be treated by aspirating the corpora cavernosa. In children with recurrent splenic sequestration, splenectomy may be a lifesaving procedure. The process is not routinely recommended because surgery increases the risk of acidosis and hypoxia from anesthesia, and, in time, the spleen usually atrophies through progressive fibrotic changes. Infarction of tissue in any organ is a potential hazard in sickle cell crisis, and special management and treatment are warranted by the specific site of damage. Typical complications include uremia (requiring renal transplantation or hemodialysis), chronic functional pulmonary impairment, aseptic necrosis of the hip, and microvascular occlusion that may lead to venous thrombosis. ▪ PATIENT CARE CONSIDERATIONS: The primary concern of the nurse during a crisis is to initiate procedures that reduce sickling. Foremost is prevention of tissue deoxygenation and resulting hypoxia by maintaining bed rest to minimize energy expenditure and oxygen use, although some exercise is necessary to promote circulation. Hydration and electrolyte balance are essential. A complete record of fluid intake and output is maintained, and adequate therapy is calculated accordingly. Serum sodium level is monitored closely to prevent hyponatremia. Oxygen is given in severe anoxia, although prolonged administration depresses bone marrow activity and thus aggravates anemia. Management of pain in vasoocclusion is often difficult and may require experimentation with various drugs and schedules before the patient receives adequate relief. The application of warmth is often soothing; cold is contraindicated because it enhances vasoconstriction and sickling. The nurse constantly monitors the child’s condition for splenomegaly, infection, evidence of shock or cerebrovascular accident, hypervolemia, transfusion reaction, or increasing anemia. An important aspect of nursing care is the continued emotional support of parents whose child has a chronic illness that is potentially fatal.
