Sickle cell anemia is a severe hemolytic anemia resulting from the inheritance of the sickle hemoglobin (HbS) gene, which causes a defective hemoglobin molecule. Learn the ways on how to intervene as a nurse and manage patients with sickle cell anemia.
Pathophysiology of Sickle Cell Anemia
The defective hemoglobin molecule assumes a sickle shape when exposed to low oxygen tension. These long, rigid RBCs become lodged in small vessels and can obstruct blood ﬂow to body tissue. 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 (eg, when it travels through the pulmonary circulation) before the membrane becomes too rigid, it can revert to a normal shape. For this reason, the “sickling crises” are intermittent.
The HbS gene is inherited, with some people having the sickle cell trait (a carrier, inheriting one abnormal gene) and some having sickle cell disease (inheriting two abnormal genes).
Sickle cell disease is found predominantly in people of African descent and less often in people who have descended from the Mediterranean countries, the Middle East, or aboriginal tribes of India.
Symptoms of sickle cell anemia vary and are only somewhat based on the amount of HbS. Symptoms and complications result from chronic hemolysis or thrombosis.
- 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 damage 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 xray
- 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 hemoglobin 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 diagnosis is conﬁrmed by hemoglobin electrophoresis.
Medical Management of Sickle Cell Anemia
- 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 currently 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 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 anemia, in the prevention of severe complications from anesthesia and surgery, and in improving the response to infection 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 requirement.
- Supportive care involves pain management (aspirin or NSAIDs, morphine, and patientcontrolled analgesia), oral or IV hydration, physical and occupational therapy, physiotherapy, cognitive and behavioral intervention, and support groups.
Nursing Process for patients with Sickle cell Anemia
- 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 (0to10 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 xray]).
- 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.
Nursing Diagnoses for Sick Cell Anemia
- Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels
- Risk for infection
- Risk for powerlessness related to illnessinduced helplessness
- Deﬁcient knowledge regarding prevention of crisis
Collaborative Problems/Potential Complications
- Hypoxia, ischemia, infection, and poor wound healing leading to skin breakdown and ulcers
- Cerebrovascular accident (CVA, brain attack, stroke)
- Acute and chronic renal failure
- Heart failure, pulmonary hypertension, and acute chest syndrome
- 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 selfesteem and power, and absence of complications.
- Use patient’s subjective description of pain and pain rating 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 therapy, 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 relationship 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.
- 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 complications are delineated in the previous sections; additional measures should be taken to address the following issues.
- 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 followup care for patients with vascular access devices, if necessary.
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