• Teach patient about chronicity of disorder and need for monthly vitamin B12injections even when patient has no symptoms. Instruct patient how to self-administer injections, when appropriate.
• Stress importance of ongoing medical follow-up and screen-ing, because gastric atrophy associated with pernicious ane-mia increases the risk of gastric carcinoma.
For more information, see Chapter 33 in Smeltzer, S. C., Bare, B.
G., Hinkle, J. L., & Cheever, K. H. (2010). Brunner and Suddarth’s textbook of medical-surgical nursing (12th ed.). Philadelphia:
Lippincott Williams & Wilkins.
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• Anemia, with hemoglobin values in the range of 7 to 10 g/dL.
• Jaundice is characteristic, usually obvious in the sclera.
• Bone marrow expands in childhood, sometimes causing enlargement of bones of the face and skull.
• Tachycardia, cardiac murmurs, and often cardiomegaly are associated with chronic anemia.
• Dysrhythmias and heart failure may occur in adults.
• Virtually any organ may be affected by thrombosis, but the primary sites involve those areas with slower circulation, such as the spleen, lungs, and central nervous system.
• There is severe pain in various parts of the body. All tissues and organs are vulnerable and susceptible to hypoxic dam-age or ischemic necrosis.
• Sickle cell crisis: sickle crisis, aplastic crisis, or sequestration crisis.
• Acute chest syndrome: fever, cough, tachycardia, and new infiltrates seen on the chest x-ray
• Pulmonary hypertension is a common sequela of sickle cell disease, and often the cause of death.
Assessment and Diagnostic Findings
The patient with sickle cell trait usually has a normal hemo-globin level, a normal hematocrit, and a normal blood smear.
In contrast, the patient with sickle cell anemia has a low hematocrit level and sickled cells on the smear. The diagno-sis is confirmed by hemoglobin electrophorediagno-sis.
Medical Management
Treatment of sickle cell anemia is the focus of continued research. However, aside from the equally important aggressive management of symptoms and complications, there are cur-rently few primary treatment modalities for sickle cell diseases.
• PBSCT: May cure sickle cell anemia but is available to only a small subset of affected patients because of either the lack of a compatible donor or because severe organ damage that may be already present in the patient is a contraindication for PBSCT
• Pharmacologic therapy: Hydroxyurea, a chemotherapy agent, has been shown to be effective in increasing fetal
50 Anemia, Sickle Cell
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hemoglobin (ie, hemoglobin F) levels in patients with sickle cell anemia; arginine may be useful in managing pulmonary hypertension and acute chest syndrome
• Transfusion therapy: Has been shown to be highly effective in several situations (eg, in an acute exacerbation of ane-mia, in the prevention of severe complications from anes-thesia and surgery, and in improving the response to infec-tion and in severe cases of acute chest syndrome).
• Pulmonary function is monitored and pulmonary hypertension is treated early if found. Infections and acute chest syndrome, which predispose to crisis, are treated promptly. Incentive spirometry is performed to prevent pulmonary complications;
bronchoscopy is done to identify source of pulmonary disease.
• Fluid restriction may be beneficial. Corticosteroids may be useful.
• Folic acid is administered daily for increased marrow require-ment.
• Supportive care involves pain management (aspirin or NSAIDs, morphine, and patient-controlled analgesia), oral or IV hydration, physical and occupational therapy, physiother-apy, cognitive and behavioral intervention, and support groups.
N U R S I N G P R O C E S S
T
HEP
ATIENT WITHS
ICKLEC
ELLC
RISIS See “Nursing Management” under “Anemia” for additional information.Assessment
• Question patients in crisis about factors that could have precipitated the crisis and measures used to prevent crisis.
•Assess all body systems, with particular emphasis on pain (0-to-10 scale, quality, and frequency), swelling, fever (all joint areas and abdomen).
•Carefully assess respiratory system, including breath sounds, oxygen saturation levels.
•Assess for signs of cardiac failure (edema, increased point of maximal impulse, and cardiomegaly [as seen on chest x-ray]).
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•Elicit symptoms of cerebral hypoxia by careful neurologic examination.
•Assess for signs of dehydration and history of fluid intake;
examine mucous membranes, skin turgor, urine output, serum creatinine, and BUN values.
•Assess for signs of any infectious process (examine chest and long bones and femoral head, because pneumonia and osteomyelitis are common).
•Monitor hemoglobin, hematocrit, and reticulocyte count and compare with baseline levels.
•Assess current and past history of medical management, particularly chronic transfusion therapy, hydroxyurea use, and prior treatment for infection.
Diagnosis Nursing Diagnoses
•Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels
•Risk for infection
•Risk for powerlessness related to illness-induced helplessness
•Deficient knowledge regarding prevention of crisis Collaborative Problems/Potential Complications
•Hypoxia, ischemia, infection, and poor wound healing leading to skin breakdown and ulcers
•Dehydration
•Cerebrovascular accident (CVA, brain attack, stroke)
•Anemia
•Acute and chronic renal failure
•Heart failure, pulmonary hypertension, and acute chest syndrome
•Impotence
•Poor compliance
•Substance abuse related to poorly managed chronic pain Planning and Goals
The major goals for the patient are relief of pain, decreased incidence of crisis, enhanced sense of self-esteem and power, and absence of complications.
Nursing Interventions Managing Pain
•Use patient’s subjective description of pain and pain rat-ing on a pain scale to guide the use of analgesic agents.
•Support and elevate any joint that is acutely swollen until swelling diminishes.
•Teach patient relaxation techniques, breathing exercises, and distraction to ease pain.
•When acute painful episode has diminished, implement aggressive measures to preserve function (eg, physical ther-apy, whirlpool baths, and transcutaneous nerve stimulation).
Preventing and Managing Infection
•Monitor patient for signs and symptoms of infection.
•Initiate prescribed antibiotics promptly.
•Assess patient for signs of dehydration.
•Teach patient to take prescribed oral antibiotics at home, if indicated, emphasizing the need to complete the entire course of antibiotic therapy.
Promoting Coping Skills
•Enhance pain management to promote a therapeutic rela-tionship based on mutual trust.
•Focus on patient’s strengths rather than deficits to enhance effective coping skills.
•Provide opportunities for patient to make decisions about daily care to increase feelings of control.
Increasing Knowledge
•Teach patient about situations that can precipitate a sickle cell crisis and steps to take to prevent or diminish such crises (eg, keep warm, maintain adequate hydration, avoid stressful situations).
•If hydroxyurea is prescribed for a woman of childbearing age, inform her that the drug can cause congenital harm to unborn children and advise about pregnancy
prevention.
Monitoring and Managing Potential Complications Management measures for many of the potential complica-tions are delineated in the previous seccomplica-tions; additional measures should be taken to address the following issues.
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LEG ULCERS
•Protect the leg from trauma and contamination.
•Use scrupulous aseptic technique to prevent nosocomial infections.
•Refer to a wound–ostomy–continence nurse, which may facilitate healing and assist with prevention.
PRIAPISM LEADING TO IMPOTENCE
•Teach patient to empty the bladder at the onset of the attack, exercise, and take a warm bath.
•Inform patient to seek medical attention if an episode persists more than 3 hours.
CHRONIC PAIN AND SUBSTANCE ABUSE
•Emphasize the importance of complying with prescribed treatment plan.
•Promote trust with patient through adequate management of acute pain during episodes of crisis.
•Suggest to patient that receiving care from a single provider over time is much more beneficial than receiving care from rotating physicians and staff in an emergency department.
•When a crisis arises, emergency department staff should contact patient’s primary health care provider for optimal management.
•Promote continuity of care and establish written contracts with patient.
Promoting Home- and Community-Based Care
•Involve the patient and his or her family in teaching about the disease, treatment, assessment, and monitoring needed to detect complications. Also teach about vascular access device management and chelation therapy.
•Advise health care providers, patients, and families to communicate regularly.
•Provide guidelines regarding when to seek urgent care.
•Provide follow-up care for patients with vascular access devices, if necessary.
Evaluation
Expected Patient Outcomes
•Reports control of pain
•Is free of infection
•Expresses improved sense of control
•Increases knowledge about disease process
•Experiences absence of complications
For more information, see Chapter 33 in Smeltzer, S. C., Bare, B. G., Hinkle, J. L., & Cheever, K. H. (2010). Brunner and Sud-darth’s textbook of medical-surgical nursing (12th ed.).
Philadelphia: Lippincott Williams & Wilkins.