The RBCs that contain more Hb S than Hb A are prone to sickling when they are exposed to decreased oxygen tension in the blood. The cells become more elongated, thus the term “sickle.” Once sickled, RBCs are more rigid, fragile, and rapidly destroyed. The RBCs therefore have a short survival time (30 to 40 days, as compared with a normal 120-day survival rate), a decreased oxygen-carrying capacity, and low Hb content. They cannot flow easily through tiny capillary beds and may become clumped and cause obstructions. The obstructions can lead to ischemia and necrosis, which produce the major clinical manifestations of pain. In people with sickle cell disease, approximately 50% do not survive beyond age 20 years, and most people do not live past 50 years of age. Complications include chronic obstructive pulmonary disease, congestive heart failure, and infarction of organs such as the spleen, retina, kidneys, and even the brain.
Two factors have been identified as producing sickling, although the exact cause is unknown. The first is hypoxemia, which is caused by low oxygen tension in the blood from high altitudes, strenuous exercise, or low oxygen concentration during anesthesia. The second is a change in the
condition of the blood, such as decreased plasma volume, decreased blood pH, or increased plasma osmolality as a result of dehydration.
Most infants do not develop symptoms during the first 6 months because fetal Hb has a protective effect. The parents or the child may describe a history of lung infections or cardiomegaly (hypertrophy of the heart). Children with the disease may have a history of chronic fatigue, dyspnea, joint pain and swelling, and chest pain.
The extent of the symptoms depends on the amount of Hb S that is present. The general signs are similar to the other types of hemolytic anemia (anemia as a result of destruction of RBCs): malaise, fatigue, pallor, jaundice, and irritability. Children begin to fall below the growth curve in height and weight at around 7 years, and puberty is usually delayed. They are often small for their age and may have narrow shoulders and hips, long extremities, and a curved spine. You may note jaundice and pale skin. Often, the children have heart rates that are faster than normal and heart murmurs; you may find a large liver and spleen. Eventually, all body systems, including the heart, lungs, central nervous system, kidneys, liver, bones and joints, skin, and eyes, are affected. The most severe problem is sickle cell crisis.
Children with sickle cell disease have a chronic, potentially fatal genetic disorder. Frequent hospitalizations and delayed growth and development put them at risk for low self-esteem and body image problems. In addition, because of the genetic nature of the disease, parents may experience guilt feelings. Families need extensive genetic and psychological counseling to avoid problems. Assess the child and the family for coping skills and knowledge deficits about the cause and prevention of sickle cell crisis.
Nursing care plan primary nursing diagnosis: Altered growth and development related to physical disabilities secondary to poor tissue perfusion.
Nursing care plan intervention and treatment plan
Although sickle cell disease cannot be cured, there are many treatment alternatives to prevent exacerbations, limit complications, and manage sickle cell crises. Medical management centers on the treatment of anemia and the prevention of crisis. Families are counseled to avoid the causes of crisis (i.e., dehydration, infection, hypoxia, high altitudes, vigorous exercise, and stress). To prevent aplastic crisis, prophylactic daily doses of penicillin are given to infants beginning when they are about 4 months old. Sequestration and aplastic crises are treated with transfusions of packed RBCs. A vasoocclusive crisis is treated with analgesia and increased hydration. Pain levels should be assessed frequently and corrected quickly. Avoid using aspirin, which may increase acidosis. Children may not express the need for pain medication because of fear of the route of administration. Patient-controlled analgesia, therefore, may be used, with morphine sulfate as the drug of choice. Iron supplements may be used if folic acid levels are lower than normal.
Counsel children and families on the importance of maintaining hydration even when the child is ill or during hot weather. Encourage oral fluid intake in addition to intravenous fluids when children are in the hospital. Increase fluid intake to 1.5 times the normal maintenance volume if the child’s cardiac function is adequate.
In cases of acute crisis, pain is the overriding problem. In addition to prescription medicines, employ other pain-reducing interventions, such as diversion, imagery, and general comfort measures. Keep the pain level within tolerable limits for the individual. Encourage families to maintain a normal life for the child with sickle cell disease. Arrange for genetic counseling so that families can make informed decisions. When appropriate, and depending on the age, include siblings in the care.
Nursing care plan discharge and home health care guidelines
Teach the patient and family the causes, signs, and symptoms of crisis and ways to avoid future crisis. Emphasize good nutrition and the avoidance of caffeine and smoking. Patients and families need to be taught the signs and symptoms of complications, such as cardiopulmonary dysfunction, increased intracranial pressure, and renal impairment. The patient and family need to be taught the importance of taking daily antibiotics and the side effects, dosage, and route of medication. If the patient is on pain medications, care should be taken to ensure that the medication is not abused but is taken when the patient is in need. Patients and families need to understand the need for genetic counseling, the potential long-term effects of sickle cell disease, and the possible complications. Older children and parents need to deal with the delayed sexual maturity that occurs. Long-term follow-up care is essential for patients with sickle cell disease.
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