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 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: Excess or deficit in oxygenation and/or carbon dioxide elimination at the alveolar-capillary membrane.
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.|
Ineffective Tissue Perfusion
Ineffective Tissue Perfusion: Decreased in the oxygen resulting in the failure to nourish the tissues at the capillary level.
May be related to
- Vaso-occlusive nature of sickling, inflammatory response
- Arteriovenous (AV) shunts in both pulmonary and peripheral circulation
- Myocardial damage from small infarcts, iron deposits, and fibrosis
Possibly evidenced by
- Changes in vital signs: diminished peripheral pulses/capillary refill, general pallor
- Decreased mentation, restlessness
- Angina, palpitations
- Tingling in extremities, intermittent claudication, bone pain
- Transient visual disturbances
- Ulcerations of lower extremities, delayed healing
- Demonstrate improved tissue perfusion as evidenced by stabilized vital signs, strong/palpable peripheral pulses, adequate urine output, absence of pain; usual mentation; normal capillary refill; skin warm/dry; nail beds and lips of natural pale, pink color; absence of paresthesias.
|Carefully monitor vital signs: Assess pulse points for rate, rhythm, and volume. Take note of hypotension, rapid, weak, and thready pulses, and increased or shallow respirations.||Accumulation and sickling in peripheral vessels may lead to complete or partial blockage of a vessel with diminished perfusion to surrounding tissues. Sudden massive splenic sequestration of dead cells can lead to shock.|
|Assess skin for pallor, cyanosis, coolness, diaphoresis, and delayed capillary refill.||Changes reflect diminished circulation and/or hypoxia potentiating capillary occlusion.|
|Monitor and note changes in level of consciousness, reports of headache, dizziness, development of sensory and motor deficits (hemiparesis or paralysis), and seizure activity.||Changes observed may reflect diminished perfusion to the central nervous system (CNS) due to ischemia or infarction. Stagnant cells must be mobilized immediately to prevent further ischemia/infarction.|
|Maintain adequate fluid intake and monitor urine output.||Dehydration causes increase in sickling and occlusion of capillaries other than hypovolemia or decrease in blood volume. Decrease renal perfusion may indicate vascular occlusion.|
|Assess the lower extremities for skin texture, ulcerations, and/or edema.||Sickling of blood can cause reduced peripheral circulation and often leads to dermal changes and delayed healing.|
|Note reports of change in character of pain, or development of bone pain, angina, tingling of extremities, eye pain and disturbances in vision.||Changes may reflect increased sickling of cells and diminished circulation where organs are further involved. Watch out for these signs and symptoms as they may indicate myocardial infarction (MI), pulmonary infarction, or occlusion of vasculature of the eye.|
|Maintain room temperature and body warmth without overheating.||This prevents vasoconstriction therefore aids in maintaining circulation and perfusion. Excessive increase in body temperature may cause diaphoresis which may add to insensible fluid losses and may put the patient in risk for dehydration.|
|Assess client for edema.||Occlusion of blood vessels and circulatory stasis may lead to edema of extremities, potentiating risk of tissue ischemia and necrosis. Note: Assess for edema in the genitals of men including priapism.|
|Monitor laboratory studies:|
|Decreased tissue perfusion may lead to gradual infarction of organ tissues, such as the brain, liver, spleen, kidney, skeletal muscle, and so forth, with consequent release of intracellular enzymes.|
|Electrolyte losses (especially sodium) are increased during crisis because of fever, diarrhea, vomiting, diaphoresis.|
|Hydration lowers the HbS concentration within the red blood cells, which decreases the sickling tendency and blood viscosity, therefore helps in maintaining perfusion. Infusion pump may prevent circulatory overload. Lactated Ringer’s solution or D5W may cause RBC hemolysis and potentiate thrombus formation.|
|Hydroxyurea (Droxia) is a cytotoxic agent and dramatically decreases number of sickle-cell episodes and reduces the severity of complications such as fever and severe chest pain by increasing the level of fetal hemoglobin. Levels greater than 20% may prolong life.Antisickling agents (sodium cyanate) are aimed at prolonging erythrocyte survival and prevents sickling by affecting cell membrane changes.The use of anticoagulants, plasma expanders, nitrates, vasodilators, and alkylating agents has proved essentially unsuccessful in the management of the vaso-occlusive crisis.|
|Assist with surgical diathermy or photocoagulation.||Diathermy is the direct coagulation of bleeding sites in the eye resulting in vascular stasis. This may prevent progression of proliferative changes if initiated early.|
|Assist with needle aspiration of blood from corpora cavernosa.||Priapism can be caused by sickling of blood cells within the penis. It is characterized by sustained and painful erection and edema. Removal of occluded sickled cells can improve circulation, decreasing psychological trauma and risk of necrosis and infection.|
|Surgical intervention.||Direct incision and ligation of the dorsal arteries of the penis and sapheno cavernous shunting may be necessary in severe cases of priapism to prevent tissue necrosis.|
Risk for Deficient Fluid Volume
Risk for Deficient Fluid Volume: At risk for decreased intravascular, interstitial, and intracellular fluid.
Risk factors may include
- Increased fluid needs, e.g., hypermetabolic state/fever, inflammatory processes
- Renal parenchymal damage/infarctions limiting the kidney’s ability to concentrate urine (hyposthenuria)
Possibly evidenced by
- Not applicable. Existence of signs and symptoms establishes an actual nursing diagnosis.
- Maintain adequate fluid balance as evidenced by individually appropriate urine output with a near-normal specific gravity, stable vital signs, moist mucous membranes, good skin turgor, and prompt capillary refill.
|Maintain accurate I&O and weigh daily.||Patient may reduce fluid intake during periods of crisis because of malaise and anorexia. Dehydration from vomiting, diarrhea, and fever may reduce urine output and initiate a vaso-occlusive crisis.|
|Note urine characteristics and specific gravity.||Kidney can lose its ability to concentrate urine, resulting in excessive losses of dilute urine and fixation of the specific gravity.|
|Monitor vital signs, comparing with patient’s previous readings. Measure BP in lying, sitting, and standing positions if possible.||Reduction of circulating blood volume can occur from increased fluid loss, resulting in hypotension and tachycardia.|
|Assess patient for fever, changes in level of consciousness, poor skin turgor, dryness of skin and mucous membranes, pain.||Symptoms may indicate dehydration or hemoconcentration with consequent vaso-occlusive state.|
|Closely monitor vital signs during blood transfusions and note presence of dyspnea, crackles, rhonchi, wheezes, jugular vein distention, diminished breath sounds, cough, frothy sputum and cyanosis.||The patient’s heart may already be weakened and prone to failure because of the chronic demands placed on it by the anemic state. The heart may be unable to tolerate the added fluid volume from transfusions or rapid IV fluid administration administered to treat crisis or shock.|
|Administer IV fluids as indicated.||IV fluids replaces losses and fills deficit. It may reverse renal concentration of RBCs and presence of failure. Fluids must be given immediately to decrease hemoconcentration and prevent further infarction.|
|Monitor laboratory studies: Hb/Hct, serum and urine electrolytes.||Elevation in Hb/Hct may indicate hemoconcentration. The loss kidneys’ ability to concentrate urine may result in serum depletions of Na+, K–, and Cl–, therefore necessitating replacement.|
Acute Pain: Unpleasant sensory and emotional experience arising from actual or potential tissue damage or described in terms of such damage; sudden or slow onset of any intensity from mild to severe with anticipated or predictable end and a duration of <6 months.
May be related to
- Intravascular sickling with localized stasis, occlusion, and infarction/necrosis
- Activation of pain fibers due to deprivation of oxygen and nutrients, accumulation of noxious metabolites
Possibly evidenced by
- Localized, migratory, or more generalized pain, described as throbbing, gnawing, or severe and incapacitating; affecting peripheral extremities, bones, joints, back, abdomen, or head (headaches recurrent/transient)
- Decreased ROM, guarding of the affected areas
- Facial grimacing, narrowed/self-focus
- Patient shall verbalize relief or control of pain.
- Demonstrate relaxed body posture, have freedom of movement, be able to sleep/rest appropriately.
|Assess for pain. Note location, duration, and intensity (scale of 0-10).||Sickling of cells potentiates cellular hypoxia and may lead to infarction of tissues with resultant pain. Pain usually occurs in the back, ribs, and limbs and may last for days.|
|Observe nonverbal pain cues: gait disturbances, positioning of the body, guarding behavior, facial grimacing, reluctance to move, and physiological manifestations of acute pain (increased BP, tachycardia, increased RR).||Nonverbal cues may aid in evaluation of pain and effectiveness of therapy since pain is unique to each patient. One may encounter varying description because of individualized perceptions.|
|Discuss what pain relief measures were effective in the past. Involve the patient and SO in care.||Involve the patient or SO in care to allow for identification of effective remedies that have been found to relieve pain. This helps in establishing individualized treatment needs.|
|Teach and discuss alternative pain relief measures: relaxation techniques, biofeedback, yoga, meditation, progressive relaxation techniques, distraction techniques, guided imagery and breathing techniques.||Cognitive-behavioral pain management may reduce reliance on pharmacological means of pain control. This also enhances the patient’s sense of control.|
|Provide support and carefully position affected extremities.||To reduce edema, discomfort, and risk of injury, especially if osteomyelitis is present.|
|Massage gently affected areas.||Helps reduce muscle tension.|
|Encourage ROM exercises.||Prevents joint stiffness and possible contracture formation.|
|Plan activities during peak analgesic effect.||Maximizes movement of joints, enhancing mobility.|
|Maintain adequate fluid intake.||Dehydration increases sickling/vaso-occlusion and corresponding pain.|
|Apply warm, moist compresses to affected joints and other painful areas. Avoid use of ice or cold compresses.||Warmth causes vasodilation and increases circulation to hypoxic areas. Cold causes vasoconstriction and compounds the crisis.|
|Administer medications as indicated:|
|Reduces pain and promotes rest and comfort. Note: Narcotics are the mainstay of pain control during crisis.|
|Acetaminophen (Tylenol) can be used for control of headache, pain, and fever. Aspirin should be avoided because it alters blood pH and can make cells sickle more easily.|
|Meperidine (Demerol) should not be used because its metabolite, normeperidine, can cause CNS excitation.|
|Administer and monitor RBC transfusions.||Frequency of painful sickle-cell crises may be reduced by routine partial exchange transfusions to maintain the population of normal RBCs.|
Risk for Impaired Skin Integrity
Risk factors may include
- Impaired circulation (venous stasis and vaso-occlusion)
- Altered sensation
- Decreased mobility/bedrest
- Prevent dermal ischemic injury.
- Display improvement in wound/lesion healing if present.
- Participate in behaviors to reduce risk factors/skin breakdown.
|Help and educate the client to reposition frequently, even when sitting in chair.||Prevents prolonged tissue pressure where circulation is already compromised, reducing risk of tissue ischemia.|
|Inspect skin and pressure points regularly for redness, provide gentle massage.||Poor circulation may predispose to rapid skin breakdown.|
|Protect bony prominences with pillows, sheepskin, and heel or elbow protectors.||Decreases pressure on tissues, preventing skin breakdown.|
|Keep skin surfaces dry and clean and linens dry and wrinkle-free.||Moist and contaminated areas provide excellent media for growth of pathogenic organisms.|
|Note and monitor ischemic areas, cuts, bumps, and bruises closely for ulcer formation.||Potential entry sites for pathogenic organisms. In presence of altered immune system, this increases risk of infection and delayed healing.|
|Elevate lower extremities when sitting.||Enhances venous return, reducing venous stasis or edema formation.|
|Provide egg-crate, alternating air pressure, or water mattress.||Reduces tissue pressure and aids in maximizing cellular perfusion to prevent dermal injury.|
|Cleanse open wounds and ulcers with hydrogen peroxide, boric acid, or povidone iodine solutions as indicated. Document the distribution, size, depth, appearance and drainage.||Improvement or delayed healing reflects status of tissue perfusion and effectiveness of interventions. Note: These patients are at increased risk of serious complications because of lowered resistance to infection and decreased nutrients for healing.|
|Assist and prepare patient with hyperbaric oxygenation to ulcer sites.||Maximizes oxygen delivery to tissues, enhancing healing.|
Deficient Knowledge: Absence or deficiency of cognitive information related to specific topic.
May be related to
- Lack of exposure/recall
- Information misinterpretation
- Unfamiliarity with resources
Possibly evidenced by
- Questions, request for information, statement of misconceptions
- Inaccurate follow-through of instructions; development of preventable complications
- Verbal/nonverbal cues of anxiety
- Verbalize understanding of disease process, including symptoms of crisis, potential complications.
- Verbalize understanding of therapeutic needs.
- Initiate necessary behaviors/lifestyle changes to prevent complications.
- Participate in continued medical follow-up; genetic counseling/family planning services.
|Review disease process and treatment needs.||Provides knowledge base from which patient can make informed choices. Note: The median age at death for females is 48 years old and 42 years old for males. Deaths often occur due to organ failure.|
|Review precipitating factors:|
|Causes peripheral vasoconstriction, which may result in sludging of the circulation, increased sickling, and may precipitate a vaso-occlusive crisis.|
|Increases metabolic demand for oxygen and increases insensible fluid losses (evaporation and perspiration) leading to dehydration, which may increase blood viscosity and tendency to sickle.|
|Decreased oxygen tension present at higher altitudes causes hypoxia and potentiates sickling of cells.|
|Encourage consumption of at least 4–6 qt of fluid daily, during a steady state of the disease, increasing to 6–8 qt during a painful crisis or while engaging in activities that might precipitate dehydration.||Prevents dehydration and consequent hyperviscosity that can potentiate sickling.|
|Encourage ROM exercise and regular physical activity with a balance between rest and activity.||Prevents bone demineralization and may reduce risk of fractures. Aids in maintaining level of resistance and decreases oxygen needs.|
|Review patient’s current diet, reinforcing the importance of diet including liver, green leafy vegetables, citrus fruits, and wheat germ. Provide necessary instruction regarding supplementary vitamins such as folic acid.||Sound nutrition is essential because of increased demands placed on bone marrow. Folic acid supplements are frequently ordered to prevent aplastic crisis.|
|Discourage smoking and alcohol consumption and identify appropriate community support groups.||Nicotine induces peripheral vasoconstriction and decreases oxygen tension, which may contribute to cellular hypoxia and sickling. Alcohol increases the possibility of dehydration therefore precipitates sickling. Maintaining these changes in behavior and lifestyle may require prolonged support.|
|Discuss principles of skin and extremity care and protection from injury. Encourage prompt treatment of cuts, insect bites, sores.||Because of impaired tissue perfusion, especially in the periphery, distal extremities are especially susceptible to altered skin integrity/infection.|
|Include instructions on care of leg ulcers that might develop.||Fosters independence and maintenance of self-care at home.|
|Instruct patient to avoid persons with infections such as upper respiratory infections.||Altered immune response places patient at risk for infections, especially bacterial pneumonia.|
|Recommend patient avoid cold remedies and decongestants containing ephedrine and large amounts of caffeine.||Those remedies containing vasoconstrictors may decrease peripheral tissue perfusion and cause sludging of sickled cells.|
|Stress the importance of reading labels on over-the counter (OTC) drugs and consulting healthcare provider before consuming any drugs/herbal supplements.||Promotes patient’s sense of control, may avert a crisis.|
|Discuss conditions for which medical attention should be sought:|
|Symptoms suggestive of sickling in the renal medulla.|
|Dehydration may trigger a vaso-occlusive crisis.|
|May signify a vaso-occlusive crisis due to sickling in the bones or spleen (ischemia or infarction) or onset of osteomyelitis.|
|May reflect angina, impending MI, or pneumonia.|
|Cholelithiasis, primarily with bilirubin stones, is present in more than 50% of adults.|
|Suggestive of infections that may precipitate a vaso-occlusive crisis if dehydration develops. Note: Severe infections are the most frequent cause of aplastic crisis.|
|Assist patient to strengthen coping abilities: deal appropriately with anxiety, get adequate information, use relaxation techniques.|
|May prevent inappropriate treatment in emergency situations.|
|Screening may identify other family members with sickle cell trait. Hereditary nature of the disease with the possibility of transmitting the mutation may have a bearing on the decision to have children.|
|Provides opportunity to correct misconceptions/present information necessary to make informed decisions. Pregnancy can precipitate a vaso-occlusive crisis because the placenta’s tortuous blood supply and low oxygen tension potentiate sickling, which in turn can lead to fetal hypoxia.|
|Encourage patient to have routine follow-ups:|
|Monitors changes in blood components; identifies need for changes in treatment regimen. When using hydroxyurea, frequent monitoring of CBC is required because of narrow margin between efficacy (acceptable degree of bone marrow suppression) and toxicity (neutropenia, anemia, thrombocytopenia).|
|Sound oral hygiene limits opportunity for bacterial invasion or sepsis.|
|Detects development of sickle retinopathy with either proliferative or nonproliferative ocular changes.|
|Determine need for career guidance.||A sedentary career may be necessary because of the decreased oxygen-carrying capacity and diminished exercise tolerance.|
Other Possible Nursing Care Plans
Nursing diagnoses you can use to develop your own care plan for sickle cell anemia:
- Acute/Chronic Pain—intravascular sickling with localized stasis, occlusion, and infarction/necrosis; activation of pain fibers due to deprivation of oxygen and nutrients, accumulation of noxious metabolites.
- Risk for Deficient Fluid Volume—increased fluid needs, e.g., hypermetabolic state/fever, inflammatory processes; renal parenchymal damage/infarctions limiting the kidney’s ability to concentrate urine (hyposthenuria).
- Risk for Infection—chronic disease process, tissue destruction, e.g., infarction, fibrosis, loss of spleen (autosplenectomy); inadequate primary defenses (broken skin, stasis of body fluids, decreased ciliary action).
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