Sickle cell anemia, also known as sickle cell disease (SCD), is a genetic blood disorder that significantly impacts the oxygen-carrying capacity of red blood cells (RBCs). In healthy individuals, RBCs are flexible and disc-shaped, allowing them to navigate smoothly through blood vessels and deliver oxygen throughout the body. However, in SCD, RBCs become rigid, sticky, and crescent-shaped, resembling a sickle. This sickling distorts their shape and function, leading to a cascade of complications due to impaired blood flow and oxygen delivery.
Unlike healthy RBCs that survive for about 120 days, sickled RBCs have a significantly shorter lifespan of just 10-20 days. This rapid turnover leads to chronic anemia, reducing the amount of oxygen circulating in the bloodstream and causing significant health challenges. For nurses, understanding the complexities of sickle cell anemia and formulating appropriate nursing diagnoses is crucial for delivering effective and comprehensive patient care. This article aims to provide an in-depth guide to nursing diagnoses relevant to sickle cell anemia, enhancing your ability to manage and improve the quality of life for individuals affected by this condition.
Understanding Sickle Cell Anemia: Causes and Complications
Sickle cell anemia is an inherited condition, meaning it is passed down genetically from parents to their children. For a child to develop sickle cell anemia, they must inherit two copies of the sickle cell gene, one from each parent. If a child inherits only one copy of the gene, they will have sickle cell trait. Individuals with sickle cell trait typically do not exhibit symptoms but can still pass the gene on to their offspring.
Sickle cell anemia disproportionately affects individuals of African, Mediterranean, and Middle Eastern descent, highlighting the importance of culturally sensitive nursing care. The disease is associated with high morbidity and mortality rates, making early diagnosis and comprehensive management essential. While stem cell transplantation offers a potential cure, sickle cell anemia remains a chronic condition requiring lifelong care and management of its numerous complications.
Key Complications of Sickle Cell Anemia
Sickle cell anemia can lead to a range of severe complications, often requiring immediate nursing interventions:
- Vaso-occlusive Crisis (VOC): This is a hallmark of SCD, occurring when sickled RBCs block small blood vessels, leading to excruciating pain, swelling in extremities, back, and joints, and tissue ischemia.
- Splenic Sequestration: Sickled cells can become trapped in the spleen, causing it to enlarge rapidly. This can be life-threatening, leading to a sudden drop in hemoglobin levels and hypovolemic shock.
- Acute Chest Syndrome (ACS): A severe lung complication characterized by chest pain, fever, cough, and dyspnea. ACS is often triggered by infection and can be fatal.
- Avascular Necrosis (AVN): Reduced blood flow to bones, particularly the femur and humerus, can cause bone tissue death. AVN affects a significant proportion of SCD patients, leading to chronic pain and impaired mobility.
- Stroke: Sickled cells can block blood vessels in the brain, leading to stroke, a devastating neurological complication.
- Pulmonary Hypertension: Increased blood pressure in the arteries of the lungs, leading to shortness of breath, fatigue, and heart failure.
- Leg Ulcers: Chronic skin ulcers, often on the lower legs, due to poor circulation.
- Priapism: Prolonged, painful erections due to blood clots in the penis.
- Deep Vein Thrombosis (DVT): Blood clots in deep veins, increasing the risk of pulmonary embolism.
Understanding these complications is crucial for nurses to anticipate potential health issues and formulate effective nursing care plans and nursing diagnoses for sickle cell patients.
The Nursing Process in Sickle Cell Anemia Care
Early screening for sickle cell anemia, ideally at birth, is vital for prompt diagnosis and initiation of treatment. Nurses play a central role in the care of individuals with SCD, utilizing the nursing process to create individualized care plans and collaborate with interdisciplinary teams. The nursing process encompasses assessment, nursing diagnosis, planning, implementation, and evaluation, ensuring holistic and patient-centered care. Despite advancements in treatment, sickle cell anemia remains a challenging condition with high morbidity. Nurses are critical in providing supportive care, managing symptoms, and educating patients and families to improve their quality of life. Nursing interventions often include blood transfusions, oxygen therapy, pain management, and preventative measures against infections. Patient education is paramount, emphasizing medication adherence, hydration, adequate rest, smoking cessation, infection prevention, and regular follow-up with hematologists.
Nursing Assessment: Gathering Subjective and Objective Data
The nursing assessment is the foundation of effective care, involving the collection of comprehensive data – subjective and objective – to understand the patient’s condition fully.
Review of Health History: Subjective Data
Gathering subjective data through a detailed health history is essential to understand the patient’s experience with sickle cell anemia.
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General Symptoms of Anemia: Inquire about common anemia symptoms such as jaundice, extreme fatigue, difficulty breathing, dizziness, and irregular heartbeat.
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Vaso-occlusive Crisis Symptoms: Assess for symptoms indicative of VOC, including abdominal pain, back pain, and severe deep pain in the extremities.
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Age of Onset: Note the patient’s age, as SCD typically manifests in early childhood after the protective effects of fetal hemoglobin (Hb F) diminish around six months of age.
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Pain Crisis History: Thoroughly investigate the patient’s pain crisis history, including:
- Frequency and severity of past pain crises.
- Last pain crisis episode and its characteristics.
- History of hospital admissions for pain management.
- Effectiveness of analgesics used previously.
- Current SCD treatments, including disease-modifying drugs, and their impact.
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Triggers for Vaso-occlusive Crisis: Identify potential triggers for VOC, such as infections, dehydration, cold weather, physical exertion, and stress.
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Substance Abuse and Psychiatric History: Assess for substance abuse, misuse, psychiatric conditions, and alcohol dependence, as chronic pain in SCD can significantly impact mental health, leading to maladaptive coping mechanisms.
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History of SCD Treatments: Review the patient’s history of blood transfusions and exchange transfusions, noting any complications like iron overload, alloimmunization, or previous transfusion reactions.
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Medical History of SCD Complications: Determine if the patient has a history of SCD-related complications, such as aplastic crisis, splenic sequestration, priapism, acute chest syndrome, stroke, cholelithiasis, avascular necrosis, pulmonary hypertension, and deep vein thrombosis.
Physical Assessment: Objective Data
The physical assessment provides crucial objective data to complement the subjective history.
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Pain Characteristics: Evaluate the characteristics of pain during a crisis, noting the abrupt onset, duration (hours to days), and common locations like abdomen, chest, back, extremities, bones, and joints. In infants, dactylitis (swelling of hands and feet) is a characteristic sign.
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Symptoms of Anemia: Assess for objective signs of anemia, such as pallor, fatigue, and reduced stamina, particularly in children during physical activities. Note that patients often tolerate lower hemoglobin levels (6-7 g/dL) but with limited exercise tolerance.
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Growth and Development: Monitor growth and development in children and adolescents for signs of growth retardation, delayed sexual maturation, poor weight gain, and delayed height growth, which can be associated with SCD.
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Ocular Changes: Examine the eyes for jaundice in the sclera and corkscrew-shaped blood vessels upon ophthalmoscopic examination, indicating vascular changes due to SCD.
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Cardiovascular Status: Auscultate for systolic murmurs, a common finding in SCD. Monitor for hypotension and tachycardia, which may indicate septic shock or splenic sequestration crisis. Assess for signs of high-output heart failure in patients with severe anemia.
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Respiratory Status: Observe respiratory rate and depth. Tachypnea may suggest pneumonia, heart failure, or acute chest syndrome. Dyspnea can indicate acute chest syndrome, pulmonary hypertension, or heart failure.
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Signs of Infection: Monitor for fever. In children, fever is a significant sign of infection, whereas in adults, high-grade fever is more concerning. Assess for neck stiffness and positive Brudzinski or Kernig signs, particularly in children, as meningitis is more common in SCD.
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Abdominal Palpation: Palpate the abdomen for enlargement and tenderness, which could indicate hepatosplenomegaly or splenic sequestration.
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Signs of SCD Complications: Systematically assess for objective signs of complications:
- Infection: Fever (>101.3°F/38.5°C), leukocytosis, malaise.
- Acute Chest Syndrome: Chest pain, cough, fever, dyspnea, tachypnea.
- Splenic Sequestration: Left upper quadrant abdominal pain, palpable spleen enlargement, fatigue, pallor, tachycardia.
- Priapism: Prolonged, unwanted erection.
Diagnostic Procedures: Confirming Diagnosis and Monitoring Complications
Diagnostic procedures are essential for confirming the diagnosis of sickle cell anemia and monitoring for complications.
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Prenatal Diagnosis: SCD can be diagnosed in utero through chorionic villus sampling (8-12 weeks gestation) to collect DNA for analysis.
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Newborn Screening: Mandatory newborn screening in many regions ensures early detection and treatment initiation.
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Pulmonary Function Tests (PFTs): Regular PFTs are recommended for children with SCD, especially those with recurrent ACS or decreased oxygen saturation, to assess for pulmonary function abnormalities.
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Transcranial Doppler Ultrasound (TCD): Annual TCD from ages 2 to 16 years is crucial to measure cerebral blood flow velocity and assess stroke risk. Increased velocity indicates higher stroke risk.
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Lumbar Puncture: Perform lumbar puncture if there are changes in mental status, meningeal signs, or fever to rule out meningitis.
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Early Meningitis Workup: In suspected meningitis, initiate rapid diagnostic tests: CBC count, urinalysis, chest X-rays, and blood cultures.
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Blood Tests: Typical baseline lab abnormalities in SCD patients include:
- Hemoglobin level: 5-9 g/dL
- Hematocrit: 17-29%
- Leukocyte count: 12,000-20,000 cells/mm3 (neutrophils prominent)
- Increased platelet count
- Low erythrocyte sedimentation rate
- Elevated reticulocyte count
- Sickled cells on peripheral blood smear
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Imaging Scans: Various imaging modalities are used to diagnose SCD complications:
- Chest Radiography: For respiratory symptoms, to detect ACS or pneumonia.
- Plain Radiography of Extremities: To evaluate bone deformities, osteonecrosis, and dactylitis.
- Magnetic Resonance Imaging (MRI): Superior for detecting avascular necrosis, differentiating osteomyelitis from bony infarction.
- Computed Tomography (CT): Useful for subtle osteonecrosis, alternative for patients unable to undergo MRI.
- Nuclear Medicine Scans: Detect early osteonecrosis and osteomyelitis.
- Transcranial Doppler Ultrasonography (TCD): Assesses stroke risk in children.
- Abdominal Ultrasonography: For abdominal pain, to rule out ectopic pregnancy, cholelithiasis, cholecystitis, and measure spleen and liver size.
- Echocardiography: Evaluates pulmonary hypertension and left ventricular diastolic function.
By systematically gathering subjective and objective data and utilizing appropriate diagnostic procedures, nurses can accurately assess patients with sickle cell anemia and formulate relevant nursing diagnoses.
Alt text: Microscopic view of a blood smear revealing characteristic crescent-shaped sickle cells in sickle cell anemia, alongside normal red blood cells.
Nursing Interventions for Sickle Cell Anemia: Managing Crises and Complications
Nursing interventions are crucial for managing sickle cell anemia, focusing on preventing and treating vaso-occlusive crises and addressing complications.
Preventing and Managing Vaso-Occlusive Crisis (VOC)
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Pain Crisis Prevention Education: Educate patients about home management strategies including hydration, rest, and appropriate use of analgesics.
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Prioritize Pain Management: Acknowledge and address patient pain promptly and effectively. Recognize that patients with chronic pain may not exhibit overt pain cues. Opioid analgesics are often necessary for effective pain control during VOC.
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Oxygen Administration: Administer oxygen only when SpO2 levels are low. Over-oxygenation can suppress red blood cell production.
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Fluid Infusion: Combat dehydration, a common VOC trigger, with intravenous normal saline or 5% dextrose solutions to maintain hydration and blood volume.
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Medication Administration: Administer prescribed medications to prevent VOC and reduce complications:
- Hydroxyurea: Reduces frequency and severity of pain crises.
- L-glutamine oral powder
- Crizanlizumab
- Voxelotor
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Blood Transfusion: Reserve blood transfusions for severe anemia, such as in splenic sequestration, acute chest syndrome, pregnancy, and stroke prevention in children. Be mindful of risks associated with excessive transfusions (reactions, iron overload, alloimmunization).
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Erythrocytapheresis (Red Blood Cell Exchange): Prepare for erythrocytapheresis, which replaces sickled RBCs with healthy donor RBCs, minimizing iron overload.
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Analgesia Management: Utilize opioid analgesics like morphine for severe pain, often via patient-controlled analgesia (PCA) pumps. Employ weaker opioids for moderate pain and consider NSAIDs as adjuncts.
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Folic Acid Supplementation: Encourage daily folic acid supplements and a nutritious diet to support bone marrow function and RBC production.
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Fluid Intake Promotion: Advise increased oral fluid intake, particularly water, to prevent dehydration-induced VOC, especially during exercise or in hot/humid climates.
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Temperature Extremes Caution: Educate patients to avoid extreme temperatures (heat or cold), which can trigger sickle cell crises.
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Chronic Pain Control Education: Instruct on managing chronic pain at home with long-acting morphine and NSAIDs. Prescribe breakthrough pain medications (hydrocodone, hydromorphone) and consider tricyclic antidepressants for pain and depression management.
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Medication Adherence Emphasis: Stress the importance of adhering to prescribed medications, especially hydroxyurea, to reduce crisis frequency and severity.
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Nonpharmacologic Pain Management: Integrate nonpharmacologic pain relief methods: heat/cold therapy, acupuncture, yoga, cognitive-behavioral therapy to minimize narcotic side effects.
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Regular Exercise Promotion: Recommend low-to-moderate exercise, advising patients to pace themselves, take breaks, and avoid strenuous activities.
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Smoking Cessation Advocacy: Emphasize smoking cessation to reduce VOC and acute chest syndrome risk.
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Penicillin Prophylaxis: Administer penicillin prophylaxis (ages 2 months to 5 years) to prevent pneumococcal infections.
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Vaccination Promotion: Ensure patients receive all recommended childhood vaccinations, including pneumococcal, meningococcal, hepatitis B, and influenza vaccines, to prevent severe infections.
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Specialist Referrals: Refer patients to necessary specialists:
- Hematologist: Primary SCD care.
- Pain Management Specialist: Chronic pain management.
- Infectious Disease Specialist: Infection control.
- Orthopedist: Avascular necrosis, osteomyelitis, joint replacement.
- Retina Specialist: Retinal disease management.
- Nephrologist: Kidney disease management.
- Cardiologist: Pulmonary hypertension, heart failure.
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Mental Health Support: Refer to mental health professionals (psychologist, counselor, social worker) and support groups to address depression, anxiety, and stress associated with chronic illness.
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Patient and Family Education: Encourage questions and provide comprehensive education about SCD, treatment options, symptom management, and when to seek urgent medical attention. Provide learning materials for better understanding.
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Urgent Medical Attention Guidance: Instruct patients and families on when to seek immediate medical care: severe pain, fever, dyspnea, mental status changes, abdominal pain, priapism lasting >2 hours.
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Stem Cell Transplantation Discussion: Discuss stem cell transplantation as a potential cure, particularly for younger patients with severe complications. Explain limitations, donor requirements, and risks.
Treating Complications of SCD
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Iron Overload Management: Treat iron overload from frequent transfusions with iron chelation agents (deferoxamine, deferasirox, deferiprone).
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Acute Chest Syndrome (ACS) Management: Treat ACS aggressively with antibiotics and blood or exchange transfusions for hypoxia.
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Priapism Management Education: Instruct patients on managing priapism: increased fluids, oral pain medication, urination attempts. Advise seeking emergency care for erections >2 hours.
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Leg Ulcer Care: Provide proper wound care for leg ulcers: zinc oxide occlusive dressings, elevation, debridement, antibiotics as needed.
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Avascular Necrosis (AVN) Modifications: Advise patients with AVN to avoid weight-bearing on affected joints. Refer to occupational and physical therapists for lifestyle modifications.
Common Nursing Diagnoses for Sickle Cell Anemia
Based on the assessment data and understanding of sickle cell anemia, several key nursing diagnoses are commonly identified. These diagnoses guide the development of individualized care plans to address the specific needs of each patient. Here are some frequently used nursing diagnoses for sickle cell anemia:
1. Chronic Pain
Nursing Diagnosis: Chronic Pain related to vaso-occlusive crisis, splenic sequestration, hyperhemolytic crisis, aplastic crisis, disease process, decreased blood supply, tissue death, dehydration, and infection.
As evidenced by: Decreased red blood cells (anemia), painful swelling of affected areas, pain in the back and chest, abdominal pain, elevated pain scale.
Expected Outcomes:
- Patient will verbalize decreased pain using a pain scale.
- Patient will demonstrate the ability to sleep and rest without interruptions.
- Patient will verbalize their prescribed pain medication regimen.
Nursing Interventions:
- Assess pain level, location, and duration.
- Assess fluid intake and hydration status.
- Review current pain medications and their effectiveness.
- Maintain hydration with oral and IV fluids.
- Administer pain medications as prescribed, including PCA for acute crises.
- Educate on medication regimen and breakthrough pain management.
- Administer medications like hydroxyurea to reduce crisis frequency.
- Encourage nonpharmacologic pain relief methods (heat, relaxation, distraction).
- Refer to pain management specialists.
2. Ineffective Tissue Perfusion
Nursing Diagnosis: Ineffective Tissue Perfusion related to vaso-occlusive crisis, splenic sequestration, acute chest syndrome, hyperhemolytic crisis, aplastic crisis, decreased blood supply, and tissue death.
As evidenced by: Decreased red blood cells (anemia), fever, painful swelling, severe pain, abdominal pain, jaundice, tingling or numbness, fatigue.
Expected Outcomes:
- Patient will manifest hemoglobin levels at their baseline.
- Patient will verbalize decreased pain or elimination of pain.
- Patient will not experience complications from sickle cell anemia.
Nursing Interventions:
- Review red blood cell laboratory results (hemoglobin levels).
- Review imaging results for complications (chest X-ray for ACS).
- Assess for signs of anemia (fatigue, dyspnea, lightheadedness).
- Administer oxygen as needed for low SpO2.
- Transfuse PRBCs as prescribed.
- Administer IV fluids for hydration.
- Administer hydroxyurea to prevent sickling.
- Educate on sickle cell crisis prevention (hydration, avoid triggers).
- Prepare for potential splenectomy for recurrent splenic sequestration.
- Discuss bone marrow transplant as a curative option.
3. Risk for Decreased Cardiac Output
Nursing Diagnosis: Risk for Decreased Cardiac Output related to vaso-occlusive crisis, inadequate cardiac filling, decreased oxygenated blood to the heart, poor cardiac function, low red blood cell count, and low hemoglobin levels.
Expected Outcomes:
- Patient will demonstrate blood pressure and pulse rate within normal limits.
- Patient will manifest normal sinus rhythm in ECG.
- Patient will have no complaints of chest pain or dyspnea.
Nursing Interventions:
- Review echocardiogram results for pulmonary hypertension or heart failure.
- Monitor RBC counts and hemoglobin levels.
- Assess for signs of poor cardiac output (dyspnea, activity intolerance, edema, JVD).
- Administer oxygen for hypoxia.
- Prepare for blood transfusions or red blood cell exchange.
- Prepare for heart catheterization to diagnose pulmonary hypertension.
- Administer medications for pulmonary hypertension or heart failure (aspirin, diuretics, beta-blockers, ACE inhibitors).
4. Risk for Infection
Nursing Diagnosis: Risk for Infection related to vaso-occlusive crisis, splenic sequestration, hyperhemolytic crisis, aplastic crisis, disease process, decreased blood supply, tissue death, compromised immune system, and treatments like hydroxyurea and bone marrow transplant.
Expected Outcomes:
- Patient will maintain an infection-free health status.
- Patient will be able to identify infection prevention measures.
- Patient will be able to verbalize the signs and symptoms of infection.
Nursing Interventions:
- Assess risk factors for infection.
- Assess patient’s environment for infection risks.
- Assess patient’s hygiene practices.
- Administer prophylactic antibiotics (penicillin in children).
- Educate patient and parents on signs and symptoms of infection.
- Educate and demonstrate infection control measures (handwashing).
- Ensure vaccinations are up-to-date (pneumococcal, meningococcal, influenza).
5. Risk for Venous Thromboembolism
Nursing Diagnosis: Risk for Venous Thromboembolism related to sickling red blood cells, increased clumping of RBCs, increased blood viscosity, and dehydration.
Expected Outcomes:
- Patient will not experience stroke or other cardiovascular complications from thrombosis.
- Patient will verbalize personal risk factors for thrombosis.
- Patient will adhere to their anticoagulant therapy.
Nursing Interventions:
- Review Doppler ultrasound results (transcranial Doppler).
- Assess risk factors for thromboembolism (hospitalizations, surgery, dehydration).
- Assess patient and family’s ability to recognize stroke or blood clot signs.
- Prepare for monthly blood transfusions to reduce stroke risk.
- Administer anticoagulants as prescribed for VTE treatment.
- Maintain hydration.
- Educate on continuing anticoagulation therapy.
By utilizing these nursing diagnoses and implementing the corresponding interventions, nurses can provide comprehensive and effective care for patients with sickle cell anemia, addressing their complex needs and improving their overall health outcomes. This guide provides a framework for understanding and applying nursing diagnoses in the context of sickle cell anemia, enhancing the quality of care provided to these individuals.
Alt text: A nurse is shown compassionately educating a patient about sickle cell anemia, using visual aids to explain the condition and its management, fostering patient understanding and empowerment.
References
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