Sickle cell disease (SCD), or sickle cell anemia (SCA) is a group of hereditary blood disorders characterized by an abnormality in the oxygen-carrying hemoglobin molecule in red blood cells. The most common forms of SCDs are: homozygous hemoglobin SS disease (sickle cell anemia), hemoglobin SC disease, and sickle [beta]-thalassemia.
Sickle cell anemia is a severe hemolytic anemia that results from the inheritance of the sickle hemoglobin gene. This gene causes the hemoglobin molecule to be defective. The sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension. The oxygen level in venous blood can be low enough to cause this change; consequently, the erythrocyte containing HbS loses its round, pliable, biconcave disk shape and becomes deformed, rigid, and sickle shaped. These long, rigid erythrocytes can adhere to the endothelium of small vessels; when they adhere to each other, blood flow to a region or an organ may be reduced. If ischemia or infarction results, the patient may have pain, swelling, and fever. The sickling process takes time; if the erythrocyte is again exposed to adequate amounts of oxygen before the membrane becomes too rigid, it can revert to a normal shape. For this reason, the “sickling crises” are intermittent. Cold can aggravate the sickling process, because vasoconstriction slows the blood flow. Oxygen delivery can also be impaired by an increased blood viscosity, with or without occlusion due to adhesion of sickled cells; in this situation, the effects are seen in larger vessels, such as arterioles.
Sickle cell anemia is most common in tropical Africans in in people of African descent; about 1 in 10 African-American carries the abnormal gene. If two parents who are both carriers of the sickle cell trait have an offspring, each child has a 25% chance of developing sickle cell anemia. However, sickle cell anemia also appears in other ethnic populations, including people of Mediterranean or East Indian Ancestry. Overall, 1 in every 400 to 600 black children has sickle cell anemia. The defective HbS-producing gene may have persisted because, in areas where malaria is endemic, the heterozygous sickle cell trait provides resistance to malaria and is actually beneficial.
Nursing Care Plans
Nursing care planning and goals for patients with sickle cell anemia include: providing relief for pain, decrease incidences of sickle cell crisis, enhanced sense of self-esteem and power, and absence of complications.
- Impaired Gas Exchange
- Ineffective Tissue Perfusion
- Risk for Deficient Fluid Volume
- Acute Pain
- Risk for Impaired Skin Integrity
- Deficient Knowledge
- Other Possible Nursing Care Plans
Impaired Gas Exchange
- Impaired Gas Exchange
May be related to
- Decreased oxygen-carrying capacity of the blood, reduced RBC life span/premature destruction, abnormal RBC structure; sensitivity to low oxygen tension (strenuous exercise, increase in altitude)
- Increased blood viscosity (occlusions created by sickled cells packing together within the capillaries) and pulmonary congestion (impairment of surface phagocytosis)
- Predisposition to bacterial pneumonia, pulmonary infarcts
Possibly evidenced by
- Use of accessory muscles
- Cyanosis (hypoxia)
- Demonstrate improved ventilation/oxygenation as evidenced by respiratory rate within normal limits, absence of cyanosis and use of accessory muscles; clear breath sounds.
- Participate in ADLs without weakness and fatigue.
- Display improved/normal pulmonary function tests.
|Monitor respiratory rate, depth, use of accessory muscles, and areas of cyanosis.||These are indicators of adequacy of respiratory function or degree of compromise and the effectiveness or need for therapy.|
|Auscultate and note the presence or absence of breath sounds and adventitious sounds.||Development of atelectasis and stasis of secretions can impair gas exchange.|
|Monitor vital signs and note changes in cardiac rhythm.||Compensatory changes in vital signs and development of dysrhythmias reflect effects of hypoxia on cardiovascular system.|
|Check thoroughly reports of chest pain and increasing fatigue. Monitor for signs of fever, couch, adventitious breath sounds.||May reflect development of acute chest syndrome which increases the workload of the heart and oxygen demand. The crisis is a common complication in sickle-cell patients and can be associated with one or more symptoms including fever, cough,excruciating pain, sputum production, shortness of breath, or low oxygen levels.|
|Regularly assess level of consciousness.||Brain tissue is very sensitive to decreases in oxygen. Doing the assessment may be an early indicator of developing hypoxia.|
|Assist client in turning, coughing, and deep-breathing exercises.||Promote expansion of chest optimally, mobilization of secretions, and aeration of all lung fields. This also reduces the risk of stasis of secretions and pneumonia.|
|Evaluate patient’s tolerance to activity, limit activities within patient’s tolerance. Assist with ADLs and mobility as needed.||Reduces the metabolic requirements of the body would reduce the oxygen requirements.|
|Schedule rest periods and encourage patient to alternate rests and activity.||To protect the patient from excessive fatigue and reduce oxygen demands.|
|Teach and demonstrate the use of relaxation techniques: guided imagery, deep breathing exercises, and visualization.||Relaxation decreases muscle tension and anxiety and hence the metabolic demand for oxygen.|
|Encourage adequate fluid intake (2 to 3 L/day) within cardiac tolerance.||Adequate intake is necessary to provide for mobilization of secretions and prevent hyperviscosity of blood occlusion.|
|Screen health status of patient’s visitors and staff. Use PPEs when necessary.||Protects from potential sources of respiratory infection.|
|Administer supplemental humidified oxygen as indicated.||Supplemental oxygen maximizes the transport of oxygen to the tissues. Oxygen should only be given in the presence of confirmed hypoxemia because oxygen can suppress erythropoietin levels, further reducing the production of RBCs.|
|Monitor laboratory studies: CBC, ABGs, pulse oximetry, cultures, chest x-rays, pulmonary function tests.|
||Patients with SSD are prone to pneumonia, which can be potentially fatal because of its hypoxic effect of increasing sickling. Mobilizes secretions and increases aeration of lung fields.|
||Increases number of oxygen-carrying cells, dilutes the percentage of HbS to prevent sickling, improves circulation, and decreases number of sickled cells. Plain red blood cells (PRCs) are usually used because they are less likely to create circulatory overload. Partial transfusions are sometimes used prophylactically in high-risk situations (e.g., preparation for general anesthesia, third trimester of pregnancy.)|
|Administer medications as indicated:|
||Maintains normal temperature to reduce metabolic oxygen demands without affective serum pH, which may occur with aspirin.|
|A broad-spectrum antibiotic is started immediately pending culture results of suspected infections. This may change when the specific pathogen is identified.|
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