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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.
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.
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.
May report: May exhibit: Lethargy, fatigue, weakness, general malaise Loss of productivity; decreased exercise tolerance; greater need for sleep and rest 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: May exhibit:
Palpitations or anginal chest pain (concomitant coronary artery disease [CAD]/myocardial ischemia, acute chest syndrome) Intermittent claudication Apical pulse: Point of maximal impulse (PMI) may be displaced to the left (cardiomegaly) Tachycardia, dysrhythmias (hypoxia), systolic murmurs
tongue. and facial muscles. regression. seizures May exhibit: . restlessness. (Note: Pallor may appear as yellowish brown color in brown-skinned patients and as ashen gray in black-skinned patients).. nocturia Right upper quadrant (RUQ) abdominal tenderness.g. withdrawal. Jaundice: Scleral icterus. self-focusing. abnormal involuntary movements. infection. decreased muscle strength/tone. and conjunctiva. nausea/vomiting Height/weight usually in the lower percentiles Poor skin turgor with visible tenting (crisis. enlargement/distension (hepatomegaly). pale. ability to deal with life/situation. and shallow respirations (sequestration crisis) Peripheral pulses throbbing on palpation Bruits (reflects compensatory mechanisms of anemia.. mucous membranes. hemianopsia. hemiplegia or sudden hemiparesis. quadriplegia Ataxia. hypotension. possible loss of insurance/benefits. narrowed focus. straw-colored urine. decreased self-concept Dependent relationship with whoever can offer security and protection May exhibit: FOOD/FLUID May report: May exhibit: Thirst Anorexia. retinopathy. fear of genetic transmission of disease Anxiety.g. apprehension. irritability.BP: Widened pulse pressure Generalized symptoms of shock. rapid thready pulse. fear of rejection from others Negative feelings about self. as-cites Left upper quadrant (LUQ) abdominal fullness (spleen may be enlarged or may be atrophic and nonfunctional from repeated splenic infarcts and fibrosis) Dilute. financial concerns. nystagmus) Tingling in the extremities Disturbances in pain and position sense Mental status usually unaffected except in cases of severe sickling (cerebral infarction/intracranial hemorrhage) Weakness of the mouth. generalized icteric coloring (excessive RBC hemolysis) Dry skin/mucous membranes Diaphoresis (either sequestration or vaso-occlusive crisis. voiding in large amounts. hematuria or smoky appearance (multiple renal infarcts) Urine specific gravity decreased (may be fixed with progressive renal disease) EGO INTEGRITY May report: Resentment and frustration with disease. acute pain or shock) ELIMINATION May report: May exhibit: Frequent voiding. Concern regarding being a burden to significant others (SOs). depression. and dehydration) Dry skin/mucous membranes Jugular venous distension (JVD) and general peripheral edema (concomitant HF) HYGIENE May report: May exhibit: Difficulty maintaining ADLs (pain or severe anemia) Unkempt appearance. e. poor personal hygiene NEUROSENSORY May report: Headaches or dizziness Visual disturbances (e. aphasia (in cerebral infarction of dominant hemisphere) Abnormal reflexes. lost time at work/school. may also be auscultated over the spleen because of multiple splenic infarcts) Capillary refill delayed (anemia or hypovolemia) Skin color: Pallor or cyanosis of skin. unresponsiveness to questions.
decreased ROM (result of joint pain and swelling) Maladaptive pain behaviors. vomiting. Howell-Jolly bodies. and a normal to elevated MCV. tenderness. transient headaches Back pain (changes in vertebral column from recurrent infarctions). erythema. joint/bone pain accompanied by warmth. leukocytosis (especially in vaso-occlusive crisis). impotence Delayed sexual maturity Pale cervix and vaginal walls (anemia) TEACHING/LEARNING May report: Discharge plan considerations: History of HF (chronic anemic state). pulmonary fibrosis. e. . severe headache PAIN/DISCOMFORT May report: Pain as severe. dyspnea. with counts over 20. especially found on the internal and external malleoli and the medial aspect of the tibia) Lymphadenopathy SEXUALITY May report: May exhibit: Loss of libido. or generalized) Recurrent. decreased visual acuity (temporary/permanent blindness) Leg ulcers (common in adult patients. sharp. crescent-shaped cells. guilt for being ill.g. chest pain.. poikilocytosis. denial of any aspect of disease. pulmonary hypertension or cor pulmonale Acute respiratory distress. pulmonary hypertension or cor pulmonale (multiple pulmonary infections/infarctions). diminished breath sounds (HF) Increased anteroposterior (AP) diameter of the chest (barrel chest) May exhibit: SAFETY May report: May exhibit: History of repeated/frequent transfusions Low-grade fever Impaired vision (sickle retinopathy). DIAGNOSTIC STUDIES CBC: Reticulocytosis (count may vary from 30%–50%). indulgence in precipitating factors (overwork. deep bone infarctions may have no apparent signs of irritation Gallbladder tenderness and pain (excessive accumulation of bilirubin as a result of increased erythrocyte destruction) Sensitivity to palpation over affected areas Guarding/holding joints in position of comfort. self-care. and cyanosis (especially in crisis) Bronchial/bronchovesicular sounds in lung periphery. gnawing of varied location (localized.. e. polychromasia. rhonchi. migratory. e. decreased Hb (5–10 g/dL) and total RBCs. strenuous exercise) May exhibit: RESPIRATION May report: Dyspnea on exertion or at rest History of repeated pulmonary infections/infarctions. basophilic stippling. decreasing level of consciousness (LOC).g. amenorrhea. target cells. focal neurological deficits. priapism.3 days May need assistance with shopping. and occasional effusions (vasoocclusive crisis). Stained RBC examination: Demonstrates partially or completely sickled.g. transportation. occasional nucleated RBCs (normoblasts). throbbing.Meningeal irritation (intracranial hemorrhage). wheezes.000 indicate infection. nuchal rigidity.. delayed healing DRG projected mean length of inpatient stay: 4. anisocytosis. elevated platelets. chronic leg ulcers. diminished breath sounds (pulmonary fibrosis) Crackles. homemaker/maintenance tasks Refer to section at end of plan for postdischarge considerations.
clear breath sounds. use of accessory muscles Restlessness. 5. NURSING PRIORITIES 1. . Urine/fecal urobilinogen: Increased (more sensitive indicators of RBC destruction than serum levels). Disease process. future expectations. osteomyelitis. acidosis (hypoxemia and acidic states in vaso-occlusive crisis). impaired May be related to Decreased oxygen-carrying capacity of the blood. osteoporosis. pulmonary infarcts Possibly evidenced by Dyspnea. Total iron-binding capacity (TIBC): Normal or decreased. and treatment needs. RBC survival time decreased (accelerated breakdown). Serum iron: May be elevated or normal (increased iron absorption due to excessive RBC destruction). Display improved/normal pulmonary function tests. confusion Tachycardia Cyanosis (hypoxia) DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Respiratory Status: Gas Exchange (NOC) Demonstrate improved ventilation/oxygenation as evidenced by respiratory rate within normal limits. Results may be inaccurate if patient has received a blood transfusion within 3–4 mo before testing. 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. Promote adequate cellular oxygenation/perfusion. reduced RBC life span/premature destruction. osteosclerosis. Oxygenation/perfusion adequate to meet cellular needs. Acid phosphatase (ACP): Elevated (release of erythrocytic ACP into the serum). Prevent complications. X-rays: May indicate bone thinning. ESR: Elevated. 4. 3. Bone radiographs: May demonstrate skeletal changes. 4. NURSING DIAGNOSIS: Gas Exchange. Complications prevented/minimized. potential complications. DISCHARGE GOALS 1. absence of cyanosis and use of accessory muscles. 3. Serum bilirubin (total and indirect): Elevated (increased RBC hemolysis). Participate in ADLs without weakness and fatigue. Intravenous pyelogram (IVP): May be done to evaluate kidney damage.g.. Erythrocyte fragility: Decreased (osmotic fragility or RBC fragility). Alkaline phosphatase: Elevated during vaso-occlusive crisis (bone and liver damage). or avascular necrosis. Alleviate pain. LDH: Elevated (RBC hemolysis) Serum potassium and uric acid: Elevated during vaso-occlusive crisis (RBC hemolysis). Pain relieved/controlled. sensitivity to low oxygen tension (strenuous exercise. and therapeutic regimen understood. e. abnormal RBC structure. Provide information about disease process/prognosis.Sickle-turbidity tube test (Sickledex): Routine screening test that determines the presence of hemoglobin S (HbS) but does not differentiate between sickle cell anemia and trait. Plan in place to meet needs after discharge. osteoporosis. 2. 2. Hemoglobin electrophoresis: Identifies any abnormal hemoglobin types and differentiates between sickle cell trait and sickle cell anemia. ABGs: May reflect decreased PO2 (defects in gas exchange at the alveolar capillary level).
Monitor vital signs. Collaborative Administer supplemental humidified oxygen as indicated. further reducing the production of RBCs. Reflective of developing acute chest syndrome (i. Brain tissue is very sensitive to decreases in oxygen and may be an early indicator of developing hypoxia. Indicators of adequacy of respiratory function or degree of compromise and therapy needs/effectiveness. Reduction of the metabolic requirements of the body reduces the oxygen requirements/degree of hypoxia. chest pain. Promote adequate fluid intake. Auscultate breath sounds. Protects from potential sources of respiratory infection. fever. Note: Oxygen should be given only in the presence of confirmed hypoxemia because oxygen can suppress erythropoietin levels.. use of accessory muscles. Ventilation Assistance (NIC) Assist in turning. and adventitious sounds. Protects from excessive fatigue.g. Schedule rest periods as indicated. coughing. Assess level of consciousness/mentation regularly. Screen health status of visitors/staff. and deep-breathing exercises. noting presence/absence. Sufficient intake is necessary to provide for mobilization of secretions and to prevent hyperviscosity of blood/capillary occlusion. Evaluate activity tolerance. Investigate reports of chest pain and increasing fatigue. Observe for signs of increased fever. particularly in presence of pulmonary insults/pneumonia.. areas of cyanosis. Maximizes oxygen transport to tissues. e. and aeration of all lung fields. and leukocytosis). reduces oxygen demands/degree of hypoxia. Relaxation decreases muscle tension and anxiety and hence the metabolic demand for oxygen. which increases the workload of the heart and oxygen demand. note changes in cardiac rhythm. Encourage patient to alternate periods of rest and activity. Demonstrate and encourage use of relaxation techniques.ACTIONS/INTERVENTIONS Respiratory Monitoring (NIC) RATIONALE Independent Monitor respiratory rate/depth.g. adventitious breath sounds. dyspnea. reduces risk of stasis of secretions/pneumonia. 2–3 L/day within cardiac tolerance. guided imagery and visualization. Promotes optimal chest expansion. limit activities to those within patient tolerance or place patient on bedrest. Assist with ADLs and mobility as needed.. cough. . mobilization of secretions. Compensatory changes in vital signs and development of dysrhythmias reflect effects of hypoxia on cardiovascular system.e. Development of atelectasis and stasis of secretions can impair gas exchange. e.
e.g. third trimester of pregnancy. acetaminophen (Tylenol): Maintains normothermia to reduce metabolic oxygen demands without affecting serum pH. restlessness Angina. bone pain Transient visual disturbances Ulcerations of lower extremities. Antibiotics. Increases number of oxygen-carrying cells. Note: Partial transfusions are sometimes used prophylactically in high-risk situations. which is potentially fatal because of its hypoxemic effect of increasing sickling. A broad-spectrum antibiotic is started immediately pending culture results of suspected infections. preparation for general anesthesia. intermittent positive-pressure breathing (IPPB). Patients are particularly prone to pneumonia. inflammatory response Arteriovenous (AV) shunts in both pulmonary and peripheral circulation Myocardial damage from small infarcts. nailbeds and lips of natural pale. and fibrosis Possibly evidenced by Changes in vital signs. cultures. CBC. intermittent claudication..g. diminished peripheral pulses/capillary refill. palpitations Tingling in extremities. Perform/assist with chest physiotherapy. Mobilizes secretions and increases aeration of lung fields. e. pink color. usual mentation. chronic. severe leg ulcers. . absence of paresthesias.g. then may be changed when the specific pathogen is identified. dilutes the percentage of HbS to prevent sickling. and emoves/decreases number of sickled cells. general pallor Decreased mentation.. Administer medications as indicated: Antipyretics.. absence of pain. improves circulation. chest x-ray. which may occur with aspirin. normal capillary refill. ineffective (specify) May be related to Vaso-occlusive nature of sickling. delayed healing DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Circulation Status (NOC) Demonstrate improved tissue perfusion as evidenced by stabilized vital signs. pulmonary function tests. Administer packed RBCs (PRCs) or exchange transfusions as indicated. strong/palpable peripheral pulses. and incentive spirometer. adequate urine output. NURSING DIAGNOSIS: Tissue Perfusion. PRCs are usually used because they are less likely to create circulatory overload. ABGs/pulse oximetry. skin warm/dry. e. iron deposits.ACTIONS/INTERVENTIONS Respiratory Monitoring (NIC) RATIONALE Collaborative Monitor laboratory studies.
g. CPK. Maintain environmental temperature and body warmth without overheating. with consequent release of intracellular enzymes. (Refer to ND: Gas Exchange. thready pulse. Reduced peripheral circulation often leads to dermal changes and delayed healing. LDH. Dehydration not only causes hypovolemia but increases sickling and occlusion of capillaries. Stagnant cells must be mobilized immediately to reduce further ischemia/infarction.g. seizure activity. and volume.. Prevents vasoconstriction. hemiparesis or paralysis). edema. Note hypotension. kidney.: ABGs. e. Changes may reflect increased sickling of cells/diminished circulation with further involvement of organs. Investigate reports of change in character of pain.g. following. development of sensory/motor deficits (e. liver. Changes reflect diminished circulation/hypoxia potentiating capillary occlusion. Sludging and sickling in peripheral vessels may lead to complete or partial obliteration of a vessel with diminished perfusion to surrounding tissues. (Refer to ND: Fluid Volume. ulcerations (especially of internal and external malleoli). pallor. Assess skin for coolness. e.ACTIONS/INTERVENTIONS Circulatory Care: Arterial [or] Venous Insufficiency (NIC) RATIONALE Independent Monitor vital signs carefully.) Monitor urine output. diarrhea. spleen. Evaluate for developing edema (including genitals in men). risk for deficient. occlusion of vasculature of the eye. and so forth. Vaso-occlusion/circulatory stasis may lead to edema of extremities (and priapism in men). Decreased renal perfusion/failure may occur because of vascular occlusion. skeletal muscle. Serum electrolytes. Assess lower extremities for skin texture. delayed capillary refill. . Sudden massive splenic sequestration of cells can lead to shock. Excessive body heat may cause diaphoresis. Assess pulses for rate. reports of headaches. Maintain adequate fluid intake. tingling of extremities. diaphoresis. angina. Provide replacements as indicated. Collaborative Monitor laboratory studies. weak. aids in maintaining circulation and perfusion. cyanosis. impaired) Changes may reflect diminished perfusion to the central nervous system (CNS) due to ischemia or infarction. Decreased tissue perfusion may lead to gradual infarction of organ tissues. vomiting. eye pain/vision disturbances. myocardial infarction (MI) or pulmonary infarction. AST/ALT.. CBC. BUN. rapid. potentiating risk of tissue ischemia/necrosis. or development of bone pain. Electrolyte losses (especially sodium) are increased during crisis because of fever. Note changes in level of consciousness. adding to insensible fluid losses and risk of dehydration. diaphoresis. such as the brain. dizziness. and increased/shallow respirations. rhythm.
stable vital signs. Hydroxyurea. moist mucous membranes. Sickling within the penis can cause sustained erection (priapism) and edema. Antisickling agents (currently under investigational use) are aimed at prolonging erythrocyte survival and preventing sickling by affecting cell membrane changes. and prompt capillary refill.] DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Hydration (NOC) Maintain adequate fluid balance as evidenced by individually appropriate urine output with a near-normal specific gravity.. NURSING DIAGNOSIS: Fluid Volume.g. good skin turgor. inflammatory processes Renal parenchymal damage/infarctions limiting the kidney’s ability to concentrate urine (hyposthenuria) Possibly evidenced by [Not applicable. e. hypermetabolic state/fever. sodium cyanate) carefully. 0. Observe for possible lethal side effects. Surgical intervention. Assist with/prepare for needle aspiration of blood from corpora cavernosa. which decreases the sickling tendency and also reduces blood viscosity. dramatically decreases the number of sickle cell episodes (by as much as 50%) and reduces the severity of complications such as fever and severe chest pain by increasing the level of fetal hemoglobin (hemoglobin F). a cytotoxic agent. nitrates. Removal of sludged sickled cells can improve circulation. risk for deficient Risk factors may include Increased fluid needs. or experimental antisickling agents (e. presence of signs and symptoms establishes an actual diagnosis. Note: Lactated Ringer’s solution or D5W may cause RBC hemolysis and potentiate thrombus formation.g. Direct coagulation of bleeding sites in the eye (resulting from vascular stasis/edema) may prevent progression of proliferative changes if initiated early.ACTIONS/INTERVENTIONS Circulatory Care: Arterial [or] Venous Insufficiency (NIC) RATIONALE Independent Administer hypo-osmolar solutions (e. Administer hydroxyurea (Droxia). Infusion pump may prevent circulatory overload. Direct incision and ligation of the dorsal arteries of the penis and saphenocavernous shunting may be necessary in severe cases of priapism to prevent tissue necrosis. Hydration lowers the HbS concentration within the RBCs. .45 normal saline) via an infusion pump..g. and alkylating agents has proved essentially unsuccessful in the management of the vaso-occlusive crisis. which helps to maintain perfusion. plasma expanders. decreasing psychological trauma and risk of necrosis/infection. Assist with/prepare for surgical diathermy or photocoagulation. Note: Use of anticoagulants.. Levels greater than 20% may prolong life. vasodilators.
serum and urine electrolytes. sitting. frothy sputum. Reduction of circulating blood volume can occur from increased fluid loss. and standing positions if possible. Fluids must be given immediately(especially in CNS involvement) to decrease hemoconcentration and prevent further infarction. Kidney can lose its ability to concentrate urine. . K-. Heart may be unable to tolerate the added fluid volume from transfusions or rapid IV fluid administered to treat crisis/shock. necessitating replacement. Monitor vital signs closely during blood transfusions and note presence of dyspnea. pain. and so on. resulting in hypotension and tachycardia. Take BP in lying.. diarrhea. resulting in excessive losses of dilute urine and fixation of the specific gravity.ACTIONS/INTERVENTIONS Fluid Monitoring (NIC) RATIONALE Independent Maintain accurate I&O. Weigh daily. crackles. rhonchi. cough. Collaborative Administer IV fluids as indicated. Monitor vital signs. wheezes. Kidneys’ loss of ability to concentrate urine may result in serum depletions of Na+. Note urine characteristics and specific gravity. and Cl-. anorexia. Observe for fever. may reverse renal concentration of RBCs/presence of failure. Dehydration from vomiting. e. Replaces losses/deficits. changes in level of consciousness. fever may reduce urine output and precipitate a vaso-occlusive crisis. Elevations may indicate hemoconcentration. Symptoms reflective of dehydration/hemoconcentration with consequent vaso-occlusive state. diminished breath sounds. poor skin turgor. comparing with patient’s normal/ previous readings. and cyanosis.g. Patient’s heart may already be weakened and prone to failure because of chronic demands placed on it by the anemic state. Patient may reduce fluid intake during periods of crisis because of malaise. dryness of skin and mucous membranes. Monitor laboratory studies. Hb/Hct. JVD.
auditory. have freedom of movement. meditation. Encourage ROM exercises. occlusion. and intensity (scale of 0–10). and risk of injury. enhancing mobility. accumulation of noxious metabolites Possibly evidenced by Localized. increased respiratory rate). Nonverbal cues may aid in evaluation of pain and effectiveness of therapy. bones. Pain is unique to each patient. duration. Dehydration increases sickling/vaso-occlusion and corresponding pain. yoga. Explore discrepancies between verbal and nonverbal cues. Reduces edema. facial expressions.. one may encounter varying descriptions because of individualized perceptions. or severe and incapacitating. and limbs and lasts 5–7 days. therefore. Explore alternative pain relief measures. Helps reduce muscle tension. Sickling of cells potentiates cellular hypoxia and may lead to infarction of tissues with resultant pain. and physiological manifestations of acute pain (e. affecting peripheral extremities. Helpful in establishing individualized treatment needs. Provide support for and carefully position affected extremities. be able to sleep/rest appropriately. back. Plan activities during peak analgesic effect. guided imagery. kinesthetic. and infarction/necrosis Activation of pain fibers due to deprivation of oxygen and nutrients. Prevents joint stiffness and possible contracture formation. Discuss with the patient/SO what pain relief measures were effective in the past. Typically pain occurs in the back. especially if osteomyelitis is present. e. joints. abdomen. Apply local massage gently to affected areas. Involves patient/SO in care and allows for identification of remedies that have already been found to relieve pain. gnawing.. Observe nonverbal pain cues. elevated BP. or head (headaches recurrent/transient) Decreased ROM. Demonstrate relaxed body posture. tactile. progressive relaxation techniques. biofeedback. e. gait disturbances. including location. migratory. described as throbbing. body positioning. visual. narrowed/self-focus DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Pain Level (NOC) Verbalize relief/control of pain.g. or more generalized pain. . ACTIONS/INTERVENTIONS Pain Management (NIC) RATIONALE Independent Assess reports of pain.. ribs. Maintain adequate fluid intake. acute/chronic May be related to Intravascular sickling with localized stasis. distraction (e.g. relaxation techniques.. tachycardia.NURSING DIAGNOSIS: Pain. guarding of the affected areas Facial grimacing. Cognitive-behavioral interventions may reduce reliance on pharmacological therapy and enhance patient’s sense of control. reluctance to move.g. and breathing techniques). Maximizes movement of joints.g. discomfort.
ND: Mobility.g. acetaminophen (Tylenol).. possible joint effusions Osteoporosis with fragmentation/collapse of femoral head or vertebra (compression deformities) Bacterial infections (osteomyelitis) Possibly evidenced by Reports of pain Limited joint ROM. e. Meperidine (Demerol) should not be used because its metabolite. Administer medications as indicated: narcotics.. Acetaminophen (Tylenol) can be used for control of headache. anxiety. Reduces pain and promotes rest and comfort. gait disturbances Generalized weakness. nonnarcotic analgesics. hydromorphone (Dilaudid). seizures. moist compresses to affected joints or other painful areas. Aspirin should be avoided because it alters blood pH and can make cells sickle more easily. NURSING DIAGNOSIS: Mobility. therapeutic restrictions (e. e. continuous infusion around-the-clock morphine (Astramorph. Avoid use of ice or cold compresses. or sedatives. Participate in activities with absence of or improvement in gait disturbances. hydroxyzine (Vistaril). Duramorph). e. Administer/monitor RBC transfusion..g. tremors. increased joint ROM. e. Refer to CPs: Extended Care.g. Frequency of painful crises may be reduced by routine partial exchange transfusions to maintain population of normal RBCs. . inability to walk/perform ADLs. pain.g. Note: Narcotics are the mainstay of pain control during crisis. impaired physical May be related to Multiple/recurrent bone infarctions or infections (weight-bearing bones) Pain/discomfort: kyphosis of upper back/lordosis of lower back. can cause CNS excitation. guarding of joints. and fever..ACTIONS/INTERVENTIONS Pain Management (NIC) RATIONALE Collaborative Apply warm. and absence of inflammatory signs. reluctance to move.g. normeperidine. Warmth causes vasodilation and increases circulation to hypoxic areas. bedrest) DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Mobility Level (NOC) Maintain/increase strength and function of affected body parts. Cold causes vasoconstriction and compounds the crisis. impaired physical..
ACTIONS/INTERVENTIONS Skin Surveillance (NIC) RATIONALE Independent Reposition frequently. Enhances venous return. Keep skin surfaces dry and clean. Collaborative Provide egg-crate. Maximizes oxygen delivery to tissues. alternating air pressure. heel/elbow protectors. presence of signs and symptoms establishes an actual diagnosis. boric acid. this increases risk of infection/delayed healing. as indicated. Decreases pressure on tissues. In presence of altered immune system. bumps closely for ulcer formation. cuts. and drainage. even when sitting in chair. character. Cleanse open wounds/ulcers with hydrogen peroxide. reducing venous stasis/edema formation. Monitor ischemic areas. depth. leg bruises. Inspect skin/pressure points regularly for redness. Moist. contaminated areas provide excellent media for growth of pathogenic organisms. Display improvement in wound/lesion healing if present. Elevate lower extremities when sitting. preventing skin breakdown. size. Protect bony prominences with sheepskin. Note: These patients are at increased risk of serious complications because of lowered resistance to infection and decreased nutrients for healing. Prevents prolonged tissue pressure where circulation is already compromised. altered sensation Decreased mobility/bedrest Possibly evidenced by [Not applicable. pillows. Potential entry sites for pathogenic organisms. Monitor distribution. enhancing healing. reducing risk of tissue trauma/ischemia. Improvement or delayed healing reflects status of tissue perfusion and effectiveness of interventions. or water mattress. Prepare for/assist with hyperbaric oxygenation to ulcer sites.NURSING DIAGNOSIS: Skin integrity. Poor circulation may predispose to rapid skin breakdown. Risk Control (NOC) Participate in behaviors to reduce risk factors/skin breakdown. or povidone iodine (Betadine) solutions as indicated. Reduces tissue pressure and aids in maximizing cellular perfusion to prevent dermal injury. linens dry/wrinkle-free.] DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Tissue Integrity: Skin and Mucous Membranes (NOC) Prevent dermal ischemic injury. . risk for impaired Risk factors may include Impaired circulation (venous stasis and vaso-occlusion). provide gentle massage.
Refer to CPs: Pneumonia: Microbial. including symptoms of crisis. Participate in continued medical follow-up. NURSING DIAGNOSIS: Knowledge. fibrosis. Provides knowledge base from which patient can make informed choices. with death often due to organ failure. treatment. ND: Infection. Note: The median age at death for females is 48 yr. stasis of body fluids. Sepsis/Septicemia. Identify interventions to prevent/reduce risk of infection. prognosis. Knowledge: Treatment Regimen (NOC) Verbalize understanding of therapeutic needs. e. self-care. risk for. and 42 yr for males. potential complications. development of preventable complications Verbal/nonverbal cues of anxiety DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Knowledge: Disease Process (NOC) Verbalize understanding of disease process. and discharge needs May be related to Lack of exposure/recall Information misinterpretation Unfamiliarity with resources Possibly evidenced by Questions.g.. request for information.NURSING DIAGNOSIS: Infection. statement of misconceptions Inaccurate follow-through of instructions. loss of spleen (autosplenectomy) Inadequate primary defenses (broken skin. decreased ciliary action) Possibly evidenced by [Not applicable. presence of signs and symptoms establishes an actual diagnosis] DESIRED OUTCOMES/EVALUATION CRITERIA—PATIENT WILL: Knowledge: Infection Control (NOC) Verbalize understanding of individual causative/risk factors. genetic counseling/family planning services. infarction. risk for Risk factors may include Chronic disease process. tissue destruction. Fractures. . Initiate necessary behaviors/lifestyle changes to prevent complications. ACTIONS/INTERVENTIONS Teaching: Disease Process (NIC) RATIONALE Independent Review disease process and treatment needs. deficient [Learning Need] regarding condition.
Provide necessary instruction regarding supplementary vitamins such as folic acid. Alcohol increases the possibility of dehydration (precipitating sickling). Include instructions on care of leg ulcers that might develop. Stress the importance of reading labels on overthe counter (OTC) drugs and consulting healthcare provider before consuming any drugs/herbal supplements. and wheat germ.: Cold environmental temperatures. Altered immune response places patient at risk for infections. identify appropriate community support groups. especially bacterial pneumonia.ACTIONS/INTERVENTIONS Teaching: Disease Process (NIC) RATIONALE Independent Review precipitating factors. increased sickling. Encourage prompt treatment of cuts. especially in the periphery. and may precipitate a vaso-occlusive crisis. during a steady state of the disease. Causes peripheral vasoconstriction. distal extremities are especially susceptible to altered skin integrity/infection. Review patient’s current diet. Strenuous physical activity/contact-type sports. Because of impaired tissue perfusion. Nicotine induces peripheral vasoconstriction and decreases oxygen tension. Prevents bone demineralization and may reduce risk of fractures. Folic acid supplements are frequently ordered to prevent aplastic crisis. insect bites. failure to dress warmly when engaging in winter activities. Decreased oxygen tension present at higher altitudes causes hypoxia and potentiates sickling of cells.. . increasing to 6–8 qt during a painful crisis or while engaging in activities that might precipitate dehydration. Aids in maintaining level of resistance and decreases oxygen needs. which may increase blood viscosity and tendency to sickle. Prevents dehydration and consequent hyperviscosity that can potentiate sickling/crisis.g. Fosters independence and maintenance of self-care at home. Discuss principles of skin/extremity care and protection from injury. Those remedies containing vasoconstrictors may decrease peripheral tissue perfusion and cause sludging of sickled cells. folate and vitamin B12 are used in greater quantities than usual). and extremely warm temperatures. reinforcing the importance of diet including liver. sores. which may contribute to cellular hypoxia and sickling. wearing tight. e. restrictive clothing. Sound nutrition is essential because of increased demands placed on bone marrow (e.g. Recommend patient avoid cold remedies and decongestants containing ephedrine and large amounts of caffeine. Instruct patient to avoid persons with infections such as upper respiratory infections (URIs). Encourage consumption of at least 4–6 qt of fluid daily. which may result in sludging of the circulation. citrus fruits. green leafy vegetables. Maintaining these changes in behavior/lifestyle may require prolonged support. stressful situations. Encourage ROM exercise and regular physical activity with a balance between rest and activity. Increases metabolic demand for oxygen and increases insensible fluid losses (evaporation and perspiration) leading to dehydration. Travel to places more than 7000 ft above sea level or flying in unpressurized aircraft. Discourage smoking and alcohol consumption.
dizziness. May signify a vaso-occlusive crisis due to sickling in the bones or spleen (ischemia or infarction) or onset of osteomyelitis. CBC. May reflect angina. deal appropriately with anxiety. Dehydration may trigger a vaso-occlusive crisis. Detects development of sickle retinopathy with either proliferative or nonproliferative ocular changes. gastric distress following meals. increasing fatigue/pallor. e. Hereditary nature of the disease with the possibility of transmitting the mutation may have a bearing on the decision to have children. Abdominal pain. . is present in more than 50% of adults. high fever. Severe joint or bone pain. May prevent inappropriate treatment in emergency situation. Promotes patient’s sense of control. with or without cough. identifies need for changes in treatment regimen. primarily with bilirubin stones. e. diarrhea. Encourage SO/family members to seek testing to determine presence of HbS. as indicated.: Periodic laboratory studies. Fever.. nonhealing leg ulcers.g. Suggestive of infections that may precipitate a vasoocclusive crisis if dehydration develops. Assist patient to strengthen coping abilities. Annual ophthalmologic examination. may avert a crisis. When using hydroxyurea. Monitors changes in blood components. Discuss genetic implications of the condition.: Urine that appears blood tinged or smoky. Cholelithiasis. impending MI.g. Explore concerns regarding childbearing/family planning and refer to community resources and obstetrician knowledgeable about sickle cell disease. or pneumonia. Indigestion. Biannual dental examination. Screening may identify other family members with sickle cell trait. Sound oral hygiene limits opportunity for bacterial invasion/sepsis. Suggest wearing a medical alert bracelet or carrying a wallet card. excessive thirst. Provides opportunity to correct misconceptions/present information necessary to make informed decisions. use relaxation techniques. e. persistent vomiting. Note: Severe infections are the most frequent cause of aplastic crisis. swelling. Pregnancy can precipitate a vaso-occlusive crisis because the placenta’s tortuous blood supply and low oxygen tension potentiate sickling. e. Severe chest pain. anemia. drowsiness. redness. Symptoms suggestive of sickling in the renal medulla.. thrombocytopenia). which in turn can lead to fetal hypoxia.g. get adequate information. frequent monitoring of CBC is required because of narrow margin between efficacy (acceptable degree of bone marrow suppression) and toxicity (neutropenia. Encourage patient to have routine follow-ups.g.ACTIONS/INTERVENTIONS Teaching: Disease Process (NIC) RATIONALE Independent Discuss conditions for which medical attention should be sought.
. inflammatory processes. activation of pain fibers due to deprivation of oxygen and nutrients. reduces feelings of isolation and enhances problem solving through sharing of common experiences. March of Dimes. risk for deficient—increased fluid needs. infarction. physical condition/presence of complications. Sedentary career may be necessary because of the decreased oxygen-carrying capacity and diminished exercise tolerance.g.g. hypermetabolic state/fever. tissue destruction. e. occlusion. POTENTIAL CONSIDERATIONS following acute hospitalization (dependent on patient’s age. accumulation of noxious metabolites. and infarction/necrosis. stasis of body fluids. and life responsibilities) Pain.. inadequate primary defenses (broken skin. fibrosis. decreased ciliary action). acute/chronic—intravascular sickling with localized stasis. public health/visiting nurse. e. Encourage participation in community support groups available to sickle cell patients/SO. Helpful in adjustment to long-term situation. loss of spleen (autosplenectomy). personal resources.ACTIONS/INTERVENTIONS Teaching: Disease Process (NIC) RATIONALE Independent Determine need for vocational/career guidance. Fluid Volume.. such as the Sickle Cell Disease Association of America. Infection. risk for—chronic disease process. Note: Failure to resolve concerns/deal with situation may require more intensive therapy/psychological support. renal parenchymal damage/infarctions limiting the kidney’s ability to concentrate urine (hyposthenuria).
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