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.
CARE SETTING
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.
RELATED CONCERNS
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.
ACTIVITY/REST
May report: Lethargy, fatigue, weakness, general malaise
Loss of productivity; decreased exercise tolerance; greater need for sleep and rest
May exhibit: 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: Palpitations or anginal chest pain (concomitant coronary artery disease [CAD]/myocardial ischemia, acute chest syndrome)
Intermittent claudication
May exhibit: Apical pulse: Point of maximal impulse (PMI) may be displaced to the left (cardiomegaly)
Tachycardia, dysrhythmias (hypoxia), systolic murmurs
BP: Widened pulse pressure
Generalized symptoms of shock, e.g., hypotension, rapid thready pulse, and shallow respirations (sequestration crisis)
Peripheral pulses throbbing on palpation
Bruits (reflects compensatory mechanisms of anemia; 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, mucous membranes, and conjunctiva. (Note: Pallor may appear as yellowish brown color in brown-skinned patients and as ashen gray in black-skinned patients). Jaundice: Scleral icterus, generalized icteric coloring (excessive RBC hemolysis)
Dry skin/mucous membranes
Diaphoresis (either sequestration or vaso-occlusive crisis; acute pain or shock)
ELIMINATION
May report: Frequent voiding, voiding in large amounts, nocturia
May exhibit: Right upper quadrant (RUQ) abdominal tenderness, enlargement/distension (hepatomegaly); 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, pale, straw-colored urine; 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, fear of rejection from others
Negative feelings about self, ability to deal with life/situation.
Concern regarding being a burden to significant others (SOs); financial concerns, possible loss of insurance/benefits, lost time at work/school; fear of genetic transmission of disease
May exhibit: Anxiety, restlessness, irritability, apprehension, withdrawal, narrowed focus, self-focusing, unresponsiveness to questions, regression, depression, decreased self-concept
Dependent relationship with whoever can offer security and protection
FOOD/FLUID
May report: Thirst
Anorexia, nausea/vomiting
May exhibit: Height/weight usually in the lower percentiles
Poor skin turgor with visible tenting (crisis, infection, and dehydration)
Dry skin/mucous membranes
Jugular venous distension (JVD) and general peripheral edema (concomitant HF)
HYGIENE
May report: Difficulty maintaining ADLs (pain or severe anemia)
May exhibit: Unkempt appearance, poor personal hygiene
NEUROSENSORY
May report: Headaches or dizziness
Visual disturbances (e.g., hemianopsia, retinopathy, nystagmus)
Tingling in the extremities
Disturbances in pain and position sense
May exhibit: Mental status usually unaffected except in cases of severe sickling (cerebral infarction/intracranial hemorrhage)
Weakness of the mouth, tongue, and facial muscles; aphasia (in cerebral infarction of dominant hemisphere)
Abnormal reflexes, decreased muscle strength/tone; abnormal involuntary movements; hemiplegia or sudden hemiparesis, quadriplegia
Ataxia, seizures
Meningeal irritation (intracranial hemorrhage), e.g., decreasing level of consciousness
(LOC), nuchal rigidity, focal neurological deficits, vomiting, severe headache
PAIN/DISCOMFORT
May report: Pain as severe, throbbing, gnawing of varied location (localized, migratory, or generalized)
Recurrent, sharp, transient headaches
Back pain (changes in vertebral column from recurrent infarctions); joint/bone pain accompanied by warmth, tenderness, erythema, and occasional effusions (vasoocclusive crisis); 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)
May exhibit: Sensitivity to palpation over affected areas
Guarding/holding joints in position of comfort; decreased ROM (result of joint pain and swelling)
Maladaptive pain behaviors, e.g., guilt for being ill, denial of any aspect of disease, indulgence in precipitating factors (overwork, strenuous exercise)
RESPIRATION
May report: Dyspnea on exertion or at rest
History of repeated pulmonary infections/infarctions, pulmonary fibrosis, pulmonary hypertension or cor pulmonale
May exhibit: Acute respiratory distress, e.g., dyspnea, chest pain, and cyanosis (especially in crisis)
Bronchial/bronchovesicular sounds in lung periphery; diminished breath sounds (pulmonary fibrosis)
Crackles, rhonchi, wheezes, diminished breath sounds (HF)
Increased anteroposterior (AP) diameter of the chest (barrel chest)
SAFETY
May report: History of repeated/frequent transfusions
May exhibit: Low-grade fever
Impaired vision (sickle retinopathy), decreased visual acuity (temporary/permanent blindness)
Leg ulcers (common in adult patients, especially found on the internal and external malleoli and the medial aspect of the tibia)
Lymphadenopathy
SEXUALITY
May report: Loss of libido; amenorrhea; priapism, impotence
May exhibit: Delayed sexual maturity
Pale cervix and vaginal walls (anemia)
TEACHING/LEARNING
May report: History of HF (chronic anemic state); pulmonary hypertension or cor pulmonale (multiple pulmonary infections/infarctions); chronic leg ulcers, delayed healing
Discharge plan
DRG projected mean length of inpatient stay: 4.3 days
May need assistance with shopping, transportation, self-care, homemaker/maintenance tasks
Refer to section at end of plan for postdischarge considerations.
DIAGNOSTIC STUDIES
CBC: Reticulocytosis (count may vary from 30%–50%); leukocytosis (especially in vaso-occlusive crisis), with counts over 20,000 indicate infection, decreased Hb (5–10 g/dL) and total RBCs, elevated platelets, and a normal to elevated MCV.
Stained RBC examination: Demonstrates partially or completely sickled, crescent-shaped cells; anisocytosis; poikilocytosis; polychromasia; target cells; Howell-Jolly bodies; basophilic stippling; occasional nucleated RBCs (normoblasts).
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.
Hemoglobin electrophoresis: Identifies any abnormal hemoglobin types and differentiates between sickle cell trait and sickle cell anemia. Results may be inaccurate if patient has received a blood transfusion within 3–4 mo before testing.
ESR: Elevated.
Erythrocyte fragility: Decreased (osmotic fragility or RBC fragility); RBC survival time decreased (accelerated breakdown).
ABGs: May reflect decreased PO2 (defects in gas exchange at the alveolar capillary level); acidosis (hypoxemia and acidic states in vaso-occlusive crisis).
Serum bilirubin (total and indirect): Elevated (increased RBC hemolysis).
Acid phosphatase (ACP): Elevated (release of erythrocytic ACP into the serum).
Alkaline phosphatase: Elevated during vaso-occlusive crisis (bone and liver damage).
LDH: Elevated (RBC hemolysis)
Serum potassium and uric acid: Elevated during vaso-occlusive crisis (RBC hemolysis).
Serum iron: May be elevated or normal (increased iron absorption due to excessive RBC destruction).
Total iron-binding capacity (TIBC): Normal or decreased.
Urine/fecal urobilinogen: Increased (more sensitive indicators of RBC destruction than serum levels).
Intravenous pyelogram (IVP): May be done to evaluate kidney damage.
Bone radiographs: May demonstrate skeletal changes, e.g., osteoporosis, osteosclerosis, osteomyelitis, or avascular necrosis.
X-rays: May indicate bone thinning, osteoporosis.
NURSING PRIORITIES
1. Promote adequate cellular oxygenation/perfusion.
2. Alleviate pain.
3. Prevent complications.
4. Provide information about disease process/prognosis, and treatment needs.
DISCHARGE GOALS
1. Oxygenation/perfusion adequate to meet cellular needs.
2. Pain relieved/controlled.
3. Complications prevented/minimized.
4. Disease process, future expectations, potential complications, and therapeutic regimen understood.
5. Plan in place to meet needs after discharge.
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.
CARE SETTING
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.
RELATED CONCERNS
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.
ACTIVITY/REST
May report: Lethargy, fatigue, weakness, general malaise
Loss of productivity; decreased exercise tolerance; greater need for sleep and rest
May exhibit: 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: Palpitations or anginal chest pain (concomitant coronary artery disease [CAD]/myocardial ischemia, acute chest syndrome)
Intermittent claudication
May exhibit: Apical pulse: Point of maximal impulse (PMI) may be displaced to the left (cardiomegaly)
Tachycardia, dysrhythmias (hypoxia), systolic murmurs
BP: Widened pulse pressure
Generalized symptoms of shock, e.g., hypotension, rapid thready pulse, and shallow respirations (sequestration crisis)
Peripheral pulses throbbing on palpation
Bruits (reflects compensatory mechanisms of anemia; 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, mucous membranes, and conjunctiva. (Note: Pallor may appear as yellowish brown color in brown-skinned patients and as ashen gray in black-skinned patients). Jaundice: Scleral icterus, generalized icteric coloring (excessive RBC hemolysis)
Dry skin/mucous membranes
Diaphoresis (either sequestration or vaso-occlusive crisis; acute pain or shock)
ELIMINATION
May report: Frequent voiding, voiding in large amounts, nocturia
May exhibit: Right upper quadrant (RUQ) abdominal tenderness, enlargement/distension (hepatomegaly); 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, pale, straw-colored urine; 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, fear of rejection from others
Negative feelings about self, ability to deal with life/situation.
Concern regarding being a burden to significant others (SOs); financial concerns, possible loss of insurance/benefits, lost time at work/school; fear of genetic transmission of disease
May exhibit: Anxiety, restlessness, irritability, apprehension, withdrawal, narrowed focus, self-focusing, unresponsiveness to questions, regression, depression, decreased self-concept
Dependent relationship with whoever can offer security and protection
FOOD/FLUID
May report: Thirst
Anorexia, nausea/vomiting
May exhibit: Height/weight usually in the lower percentiles
Poor skin turgor with visible tenting (crisis, infection, and dehydration)
Dry skin/mucous membranes
Jugular venous distension (JVD) and general peripheral edema (concomitant HF)
HYGIENE
May report: Difficulty maintaining ADLs (pain or severe anemia)
May exhibit: Unkempt appearance, poor personal hygiene
NEUROSENSORY
May report: Headaches or dizziness
Visual disturbances (e.g., hemianopsia, retinopathy, nystagmus)
Tingling in the extremities
Disturbances in pain and position sense
May exhibit: Mental status usually unaffected except in cases of severe sickling (cerebral infarction/intracranial hemorrhage)
Weakness of the mouth, tongue, and facial muscles; aphasia (in cerebral infarction of dominant hemisphere)
Abnormal reflexes, decreased muscle strength/tone; abnormal involuntary movements; hemiplegia or sudden hemiparesis, quadriplegia
Ataxia, seizures
Meningeal irritation (intracranial hemorrhage), e.g., decreasing level of consciousness
(LOC), nuchal rigidity, focal neurological deficits, vomiting, severe headache
PAIN/DISCOMFORT
May report: Pain as severe, throbbing, gnawing of varied location (localized, migratory, or generalized)
Recurrent, sharp, transient headaches
Back pain (changes in vertebral column from recurrent infarctions); joint/bone pain accompanied by warmth, tenderness, erythema, and occasional effusions (vasoocclusive crisis); 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)
May exhibit: Sensitivity to palpation over affected areas
Guarding/holding joints in position of comfort; decreased ROM (result of joint pain and swelling)
Maladaptive pain behaviors, e.g., guilt for being ill, denial of any aspect of disease, indulgence in precipitating factors (overwork, strenuous exercise)
RESPIRATION
May report: Dyspnea on exertion or at rest
History of repeated pulmonary infections/infarctions, pulmonary fibrosis, pulmonary hypertension or cor pulmonale
May exhibit: Acute respiratory distress, e.g., dyspnea, chest pain, and cyanosis (especially in crisis)
Bronchial/bronchovesicular sounds in lung periphery; diminished breath sounds (pulmonary fibrosis)
Crackles, rhonchi, wheezes, diminished breath sounds (HF)
Increased anteroposterior (AP) diameter of the chest (barrel chest)
SAFETY
May report: History of repeated/frequent transfusions
May exhibit: Low-grade fever
Impaired vision (sickle retinopathy), decreased visual acuity (temporary/permanent blindness)
Leg ulcers (common in adult patients, especially found on the internal and external malleoli and the medial aspect of the tibia)
Lymphadenopathy
SEXUALITY
May report: Loss of libido; amenorrhea; priapism, impotence
May exhibit: Delayed sexual maturity
Pale cervix and vaginal walls (anemia)
TEACHING/LEARNING
May report: History of HF (chronic anemic state); pulmonary hypertension or cor pulmonale (multiple pulmonary infections/infarctions); chronic leg ulcers, delayed healing
Discharge plan
DRG projected mean length of inpatient stay: 4.3 days
May need assistance with shopping, transportation, self-care, homemaker/maintenance tasks
Refer to section at end of plan for postdischarge considerations.
DIAGNOSTIC STUDIES
CBC: Reticulocytosis (count may vary from 30%–50%); leukocytosis (especially in vaso-occlusive crisis), with counts over 20,000 indicate infection, decreased Hb (5–10 g/dL) and total RBCs, elevated platelets, and a normal to elevated MCV.
Stained RBC examination: Demonstrates partially or completely sickled, crescent-shaped cells; anisocytosis; poikilocytosis; polychromasia; target cells; Howell-Jolly bodies; basophilic stippling; occasional nucleated RBCs (normoblasts).
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.
Hemoglobin electrophoresis: Identifies any abnormal hemoglobin types and differentiates between sickle cell trait and sickle cell anemia. Results may be inaccurate if patient has received a blood transfusion within 3–4 mo before testing.
ESR: Elevated.
Erythrocyte fragility: Decreased (osmotic fragility or RBC fragility); RBC survival time decreased (accelerated breakdown).
ABGs: May reflect decreased PO2 (defects in gas exchange at the alveolar capillary level); acidosis (hypoxemia and acidic states in vaso-occlusive crisis).
Serum bilirubin (total and indirect): Elevated (increased RBC hemolysis).
Acid phosphatase (ACP): Elevated (release of erythrocytic ACP into the serum).
Alkaline phosphatase: Elevated during vaso-occlusive crisis (bone and liver damage).
LDH: Elevated (RBC hemolysis)
Serum potassium and uric acid: Elevated during vaso-occlusive crisis (RBC hemolysis).
Serum iron: May be elevated or normal (increased iron absorption due to excessive RBC destruction).
Total iron-binding capacity (TIBC): Normal or decreased.
Urine/fecal urobilinogen: Increased (more sensitive indicators of RBC destruction than serum levels).
Intravenous pyelogram (IVP): May be done to evaluate kidney damage.
Bone radiographs: May demonstrate skeletal changes, e.g., osteoporosis, osteosclerosis, osteomyelitis, or avascular necrosis.
X-rays: May indicate bone thinning, osteoporosis.
NURSING PRIORITIES
1. Promote adequate cellular oxygenation/perfusion.
2. Alleviate pain.
3. Prevent complications.
4. Provide information about disease process/prognosis, and treatment needs.
DISCHARGE GOALS
1. Oxygenation/perfusion adequate to meet cellular needs.
2. Pain relieved/controlled.
3. Complications prevented/minimized.
4. Disease process, future expectations, potential complications, and therapeutic regimen understood.
5. Plan in place to meet needs after discharge.
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