Sickle cell anemia, also known as sickle cell disease (SCD), affects the oxygen-carrying red blood cells (RBCs). Healthy RBCs transport oxygen to every body part and organ system. RBCs are typically round and flexible, so they may readily pass through blood vessels. In sickle cell anemia, RBCs are rigid and sticky, giving them the appearance of a C-shaped farm tool called a “sickle,” which causes them to clump together, reducing circulation and oxygenation.
Healthy RBCs typically live 120 days, but in sickle cell anemia, they die in 10-20 days, which reduces the oxygen in the blood and causes anemia.
In this article:
- Causes
- Complications
- Nursing Process
- Nursing Assessment
- Nursing Interventions
- Nursing Care Plans
- Ineffective Tissue Perfusion
- Risk for Decreased Cardiac Output
- Risk for Infection
- Risk for Venous Thromboembolism
Causes
Sickle cell anemia is a genetic condition inherited at birth. Both parents must carry the sickle cell gene and pass it on to their children for them to be born with sickle cell anemia. A child will have a sickle cell trait if only one parent has the sickle cell gene. People with the sickle cell trait can produce both standard and sickle cell hemoglobin because they have one normal hemoglobin gene and one gene with an altered version (sickle cell). They may have some sickle cells in their blood but often do not exhibit symptoms. However, they may pass the gene along to their offspring.
People with ancestry from Africa, the Mediterranean, and the Middle East are most frequently affected by sickle cell anemia.
The morbidity and mortality rates for sickle cell anemia are high. Sickle cell anemia is a chronic condition, and stem cell transplantation is the only cure.
Complications
The following are complications of sickle cell anemia:
- Vaso-occlusive crisis – blood stasis and clumping in the circulation causes painful swelling in the extremities, back, and joints.
- Splenic sequestration – sickle cells that accumulate in the spleen cause spleen enlargement, which can be life-threatening.
- Acute chest syndrome (ACS) – infection in the lungs from sickle cells leads to chest pain, fever, and dyspnea that requires emergency treatment.
- Avascular necrosis (AVN) – 50% of patients will experience AVN, which is a loss of blood supply that causes bone death, often to the femur and humerus.
Other complications include stroke, pulmonary hypertension, leg ulcers, priapism, and DVT.
Nursing Process
Screening for sickle cell anemia is essential at birth, allowing for an early diagnosis and course of treatment. The nurse can create a care plan with an interdisciplinary approach to provide effective therapy and reduce complications.
Many of these patients die prematurely despite improvements in treatment. The morbidity is very high, and patients often struggle with chronic severe pain and may experience a reduced quality of life.
The nursing care plan will include the administration of blood transfusions, oxygen therapy, analgesia, and immunizations. During patient education, the nurse should stress the importance of medication adherence, staying hydrated, getting adequate sleep, not smoking, infection prevention, and following up with their hematologist.
Nursing Assessment
The first step of nursing care is the nursing assessment, during which the nurse will gather physical, psychosocial, emotional, and diagnostic data. In this section, we will cover subjective and objective data related to sickle cell anemia.
Review of Health History
1. Determine the patient’s general symptoms.
Check for the following symptoms of anemia:
- Jaundice
- Extreme fatigue
- Difficulty breathing
- Dizziness
- Irregular heartbeat
SCD is characterized by vaso-occlusive crises, which cause symptoms like:
- Abdominal pain
- Back pain
- Severe deep pain in the extremities
2. Note the client’s age.
SCD typically appears in childhood. Infants are mainly protected for the first six months of life by elevated Hb F levels. SCD symptoms become apparent soon after that.
3. Investigate pain crises.
Pain related to a vaso-occlusive crisis can be challenging to manage in SCD. To develop an accurate plan of care, ask the patient to describe the following:
- Their last pain crisis
- Their last hospital admission
- Analgesics taken and their effect
- SCD treatments (such as disease-modifying drugs) and their effects
4. Investigate triggers of vaso-occlusive crisis.
Infections, dehydration, cold weather, physical exertion, and stress are several causes of a sickle cell crisis.
5. Determine any substance abuse or psychiatric disorders.
Patients with SCD have a high rate of chronic pain, causing a negative impact on mental health. Patients may cope by using alcohol and drugs, which only further exacerbate SCD. Assess for a history of the following:
- Substance abuse
- Substance misuse
- Psychiatric conditions
- Use of psychotropic medications
- Alcohol dependence
6. Obtain a medical history of SCD treatments.
Review the history of blood transfusions and exchange transfusions. Identify the following risks:
- Iron overload
- Alloimmunization from chronic blood transfusions
- Previous transfusion reactions
7. Note the medical history of the patient.
Ask the patient about the presence or history of SCD-related complications, such as:
- Aplastic crisis
- Splenic sequestration
- Priapism
- Acute chest syndrome
- Stroke
- Cholelithiasis
- Avascular necrosis
- Pulmonary hypertension
- Deep vein thrombosis
Physical Assessment
1. Assess pain characteristics.
Pain crises occur abruptly. The crisis could last several hours to many days. The following parts of the body are commonly affected by a pain crisis:
- Abdomen
- Chest
- Back
- Extremities
- Bones
- Joints
- Hands and feet in infants (dactylitis)
2. Assess for symptoms of anemia.
Anemia in SCD is described as chronic and hemolytic. Patients commonly tolerate a Hb level of 6-7 g/dL, but their exercise and exertion tolerance is minimal. Children may demonstrate reduced stamina, which is observed when playing on the playground or in physical education class.
3. Assess the patient’s growth and development.
SCD has been associated with the following effects on growth and development in children and adolescents:
- Growth retardation
- Delayed sexual maturation
- Poor weight gain
- Delayed height growth
4. Monitor for ocular changes.
SCD can cause vascular changes in the eye, with deteriorating effects later in the disease. Jaundice is common in the sclera. Corkscrew-shaped blood vessels may be seen with ophthalmoscopic examination.
5. Assess the patient’s cardiovascular status.
A systolic murmur appears on auscultation throughout the heart. Septic shock or splenic sequestration crisis may manifest as hypotension and tachycardia. Patients with severe anemia may show indications of high-output heart failure.
6. Examine the respiratory status.
Tachypnea indicates pneumonia, heart failure, or acute chest syndrome. Dyspnea can suggest acute chest syndrome, pulmonary hypertension, or heart failure.
7. Look for signs of infection.
Fever in children indicates infection; however, it is less significant in adults until it is a high-grade fever. Meningitis is 200 times more common in children with SCD, so assess for neck stiffness and positive Brudzinski or Kernig signs.
8. Palpate the abdomen.
Palpate the abdomen for enlargement and tenderness that may indicate hepatosplenomegaly or splenic sequestration.
9. Monitor for other signs of complications from SCD.
- Infection:
- Fever of more than 101.3 F (38.5 C)
- Leukocytosis
- Malaise
- Acute chest syndrome:
- Chest pain
- Cough
- Fever
- Dyspnea
- Tachypnea
- Splenic sequestration:
- Pain on the left side of the abdomen
- Spleen enlargement on palpation
- Fatigue
- Pallor
- Tachycardia
- Priapism:
- Prolonged unwanted erection
- Impotence
Diagnostic Procedures
1. Perform a prenatal diagnosis.
SCD can be tested for in utero. A chorionic villus sample can be conducted between 8 and 12 weeks of gestation to collect DNA.
2. Perform newborn screening.
Screening for SCD at birth is mandatory in the United States and allows for earlier detection and treatment.
3. Assist the patient with PFTs.
Children with SCD typically have abnormal pulmonary function test (PFT) results. PFTs should be conducted regularly in children with recurrent acute chest episodes or decreased oxygen saturation.
4. Measure the blood flow velocity.
Transcranial Doppler ultrasound measures blood flow velocity and is a good predictor of stroke risk. Children with SCD have a 1% risk of stroke annually, which increases to >10% with higher cerebral blood flow velocities. Annual screening from the age of 2 to 16 years is recommended.
5. Consider lumbar puncture.
If there is a change in mental status, meningeal symptoms, or fever, a lumbar puncture should be performed to rule out meningitis.
6. Recognize early symptoms of meningitis.
Meningitis in children with SCD requires early detection and aggressive tests such as:
- CBC count
- Urinalysis
- Chest X-rays
- Blood cultures
7. Draw blood for tests.
The patient with SCD will typically present with the following baseline lab abnormalities:
- Hemoglobin level 5-9 g/dL
- Hematocrit 17-29%
- Total leukocyte count 12,000-20,000 cells/mm3 with prominent neutrophils
- Increased platelet count
- Low erythrocyte sedimentation rate
- Elevated reticulocyte count
- Presence of sickled cells on peripheral blood smear
8. Assist the patient with imaging scans.
The following imaging tests may be ordered to diagnose complications associated with SCD:
- Chest radiography: for patients with respiratory symptoms.
- Plain radiography of the extremities: evaluate bone deformities, osteonecrosis, and dactylitis.
- Magnetic Resonance Imaging (MRI): shows avascular necrosis of the femoral and humeral heads, differentiating between osteomyelitis and bony infarction in patients with bone pain.
- Computed Tomography (CT): reflects subtle regions of osteonecrosis and is suggested for patients who cannot have an MRI.
- Nuclear Medicine Scans: detect early osteonecrosis and osteomyelitis.
- Transcranial Doppler Ultrasonography (TCD): reveals stroke risk for children with SCD.
- Abdominal Ultrasonography: for complaints of abdominal pain; rules out ectopic pregnancy, cholelithiasis, or cholecystitis and measures spleen and liver size.
- Echocardiography: to evaluate pulmonary hypertension and LV diastolic function.
Nursing Interventions
Nursing interventions and care are essential for the patients recovery. In the following section, you will learn more about possible nursing interventions for a patient with sickle cell anemia.
Prevent and Manage Vaso-Occlusive Crisis (VOC)
1. Prevent a pain crisis.
Educate the patient on preventing and treating pain at home. This includes hydration, rest, and analgesia.
2. Take the patient’s pain seriously.
Patients with SCD live with chronic severe pain. They may not present with obvious physical or verbal pain cues. Controlling sickle cell pain requires prompt treatment with opioids.
3. Administer oxygen as needed.
Only administer oxygen when Spo2 levels are low. High levels of oxygen can suppress the formation of new red blood cells.
4. Infuse fluids.
Dehydration is a common trigger for VCO. Infuse normal saline or 5% dextrose to replace lost fluids.
5. Administer medications as ordered.
These are common medications given to patients with SCD to prevent VOC and reduce complications:
- Hydroxyurea
- L-glutamine oral powder
- Crizanlizumab
- Voxelotor
6. Transfuse blood as ordered.
Blood transfusion is only necessary for sudden and severe anemia, such as with splenic sequestration, acute chest syndrome, pregnancy, and stroke prevention in children. Excessive blood transfusions increase the risk of transfusion reactions, iron overload, and alloimmunization.
7. Prepare for erythrocytapheresis (red blood cell exchange).
Erythrocytapheresis involves replacing sickle cell-affected blood with red blood cells from a donor free from sickle cells. Red blood cell exchange avoids iron overload.
8. Administer analgesia.
Severe pain requires opioid analgesics. Morphine is often the drug of choice in acute settings. Patient-controlled analgesia (PCA) pumps allow the patient to control their pain continuously. Weaker opioids may control moderate pain. NSAIDs may be used in conjunction with opioids.
9. Recommend taking folic acid as prescribed.
Consume folic acid supplements daily and eat a nutritious diet. The bone marrow needs folic acid and other vitamins to produce new red blood cells.
10. Encourage the patient to increase fluid intake.
Advise the patient to increase their oral fluid intake as dehydration triggers VOC. Emphasize increasing water consumption if they exercise or spend time in hot or humid areas.
11. Advise the patient to be cautious of extreme temperatures.
Extreme heat or cold can increase the patient’s chance of developing a sickle cell crisis.
12. Educate on chronic pain control.
Patients will need to take long-acting morphine as well as NSAIDs to manage pain at home. Breakthrough pain medications like hydrocodone or hydromorphone are also necessary. Tricyclic antidepressants may reduce the need for opioids as well as treat depression.
13. Insist on medication adherence.
Adhering to medications like hydroxyurea is paramount, as this medication decreases the frequency and severity of pain crises.
14. Educate on nonpharmacologic pain methods.
Narcotics carry numerous side effects, so the addition of nonpharmacologic pain control is essential. Instruct on trying heat and cold therapy, acupuncture, yoga, and cognitive-behavioral therapy.
15. Promote regular exercise or physical activity.
Low-to-moderate exercise is recommended for patients with SCD. Remind the patient to pace themselves, take breaks, and refrain from strenuous activities.
16. Promote smoking cessation.
Smoking increases the risk of having a pain crisis. Patients who smoke also have an increased chance of having acute chest syndrome.
17. Administer penicillin prophylaxis as ordered.
Penicillin is used in SCD as a prophylactic treatment. Children with SCD are advised to take penicillin from 2 months until 5 years of age. This helps to prevent potentially fatal infections like pneumonia.
18. Encourage the parents to complete their child’s vaccinations.
Vaccinations are critical for disease prevention in SCD, as infections can be severe and fatal. Ensure that the patient has all recommended childhood vaccinations to prevent the following infections and viruses:
- Pneumonia
- Meningitis
- Hepatitis B
- Influenza
19. Ensure referrals to necessary specialists.
All patients with SCD should receive care from a hematologist. Additional specialists may be necessary for the following reasons:
- Pain management specialists for chronic pain management
- Infectious disease specialists for infection control
- Orthopedists for avascular necrosis or osteomyelitis when joint replacement is necessary
- Retina specialists to manage retinal disease
- Nephrologists for kidney disease
- Cardiologists for pulmonary hypertension or heart failure
20. Refer the patient to a mental health professional and support groups.
Living with a chronic and life-limiting disease commonly results in depression, anxiety, and stress. Refer the patient to a mental health professional, such as a psychologist, counselor, or social worker, to assist with coping. Suggest support groups, as talking with others who are going through similar issues can be beneficial.
21. Encourage the patient and their families to ask questions about SCD.
Understanding SCD allows the patient and their families to make informed treatment options. If the patient is a child with sickle cell anemia, educate the parents or caregivers as much as possible about the disease. This will help them know how to provide the appropriate care for the child. Provide necessary learning materials for better comprehension of symptoms and treatment.
22. Teach patients and their families when to seek urgent medical attention.
SCD causes many life-threatening conditions. Advise the patient and their families to go to the nearest hospital once the following symptoms appear:
- Severe pain
- Fever
- Dyspnea
- Changes in mental status
- Abdominal pain
- Priapism lasting longer than two hours
23. Discuss stem cell transplantation.
Hematopoietic stem cell transplantation can cure SCD and is mostly performed in younger patients with severe complications like stroke, acute chest syndrome, recurrent pain crises, osteonecrosis, and more. Stem cell transplant is limited to patients with siblings who are a match without SCD, which is uncommon. Unrelated donors are possible but come with higher risks.
Treat Complications of SCD
1. Treat iron overload.
Frequent blood transfusions will eventually cause iron overload. Administer iron chelation agents like deferoxamine, deferasirox, and deferiprone.
2. Manage acute chest syndrome (ACS).
ACS is a severe respiratory complication of SCD that causes a pneumonia-like illness with a high rate of morbidity and mortality. Administer antibiotics and blood or exchange transfusions for hypoxia.
3. Instruct on treating priapism.
Priapism is frequent and sustained erections that are painful and unwanted. Instruct patients to drink extra fluids, take oral pain medication, and attempt to urinate when it occurs. If erections last longer than two hours, seek emergency assistance.
4. Treat leg ulcers.
Leg ulcers require proper wound care, including zinc oxide occlusive dressings and elevation. Debridement and antibiotics may be employed.
5. Instruct on modifications with avascular necrosis (AVN).
Patients with ANV of the femoral or humeral heads should not bear weight on these areas. This may require modifications to their careers and lifestyles. Refer to occupational and physical therapists as needed.
Nursing Care Plans
Once the nurse identifies nursing diagnoses for sickle cell anemia, nursing care plans help prioritize assessments and interventions for both short and long-term goals of care. In the following section, you will find nursing care plan examples for sickle cell anemia.
Chronic Pain
Chronic pain associated with sickle cell anemia can be caused by the vaso-occlusive nature of sickle cells.
Nursing Diagnosis: Chronic Pain
Related to:
- Vaso-occlusive crisis
- Splenic sequestration
- Hyperhemolytic crisis
- Aplastic crisis
- Disease process
- Decreased blood supply in the affected area
- Tissue death
- Dehydration
- 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 be able to 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.
Assessment:
1. Ask about the patient’s level and duration of pain.
Patients with sickle cell anemia commonly experience pain. Their pain is often undertreated as they do not present with obvious signs of pain (crying, moaning) causing healthcare providers to dismiss their expression of pain.
Vaso-occlusive crisis four stages:
- First phase – A low-intensity, unbearable pain may accompany tingling or numbness.
- Second phase – Afflicted tissue begins to deteriorate, producing excruciating pain.
- Third phase – Body’s inflammatory reaction to tissue death, with persistent, excruciating pain that frequently lasts several days and may be accompanied by fever.
- Fourth phase – The crisis’s resolution typically takes place over 1-2 days.
2. Determine the pain location.
Pain location will determine where the clumping of cells causes decreased blood flow. It will also help implement specific interventions to alleviate the pain in the affected part.
3. Assess the patient’s fluid intake.
Hydration is often a factor in a pain crisis.
4. Review pain medications taken by the patient.
Identify the pain medications that are effective or not for the patient.
Interventions:
1. Keep the patient hydrated.
Give oral and intravenous (IV) fluid administration to maintain appropriate hydration and blood flow to control pain. Electrolyte replacement may also be required.
2. Administer pain medications.
During an acute crisis, patients often require PCA administration as their pain is severe.
3. Review medications at discharge.
Ensure patients understand their medication regimen. Educate them to manage breakthrough pain before it becomes severe.
4. Administer other medications.
Hydroxyurea, l-glutamine, and crizanlizumab are common medications given to reduce the frequency of pain crises.
5. Encourage nonpharmacologic interventions.
Heating pads can reduce pain in the back or extremities. Encourage relaxation, distraction, and movement if helpful.
6. Refer to a pain management specialist.
Collaborate with pain management specialists for a treatment plan to assist patients with sickle cell anemia in controlling pain crises.
Ineffective Tissue Perfusion
Ineffective tissue perfusion associated with sickle cell anemia can be caused by the vaso-occlusive nature of sickle cells leading to blood stasis and clumping of red blood cells causing poor circulation, decreased blood supply, severe pain, and tissue death.
Nursing Diagnosis: Ineffective Tissue Perfusion
Related to:
- Vaso-occlusive crisis
- Splenic sequestration
- Acute chest syndrome
- Hyperhemolytic crisis (rapid RBC destruction over a short period)
- Aplastic crisis (no RBC production and increased RBC destruction)
- Decreased blood supply in the affected area
- Tissue death
As evidenced by:
- Decreased red blood cells (anemia)
- Fever
- Painful swelling of affected areas
- Severe pain
- Abdominal pain
- Jaundice
- Tingling or numbness
- Fatigue
Expected outcomes:
- Patient will manifest hemoglobin levels at their baseline.
- Patient will be able to verbalize decreased pain or elimination of pain.
- Patient will not experience complications from sickle cell anemia.
Assessment:
1. Review red blood cell laboratory results.
Hemoglobin levels in sickle cell disease patients are typically lower, ranging from 6 to 11 g/dL.
2. Review imaging results.
Patients suspected of complications such as ACS may require a chest X-ray to confirm the diagnosis. Splenic sequestration can be confirmed through a CT scan if questionable.
3. Assess for signs of anemia.
Extreme exhaustion (fatigue), dyspnea, lightheadedness, or an irregular heartbeat are all symptoms of anemia. Severe anemia symptoms can be caused by an aplastic crisis or a splenic sequestration crisis.
Interventions:
1. Oxygenate as needed.
During a vaso-occlusive crisis, sickle cells clump together and cannot carry oxygenated blood to the rest of the body. Administer oxygen as needed when oxygen saturation levels are low. Unnecessary oxygen administration can suppress the formation of new RBCs.
2. Transfuse PRBCs as prescribed.
Transfusions of red blood cells enhance the quantity and quality of healthier, more flexible red blood cells to reduce complications.
3. Administer IV fluids.
Hydration is another priority treatment for a vaso-occlusive crisis. IV fluids are often administered continuously. A 5% dextrose solution is recommended.
4. Administer hydroxyurea.
Hydroxyurea is the most frequent medication prescribed to prevent sickle-shaped red blood cell formation and lower the occurrence of vaso-occlusive crises.
5. Instruct on sickle cell crisis and its prevention.
Ensure the patient and family understand how to prevent vaso-occlusive crises:
- Drink plenty of water
- Do not smoke
- Avoid extremes in temperature
- Avoid stress
- Do not miss doses of medications
- Get adequate sleep
6. Prepare for possible splenectomy.
The blood-filled spleen may enlarge during a severe sequestration crisis. The patient may experience an abrupt decrease in hemoglobin, hypovolemic shock, and death within hours. For patients who develop recurrent splenic sequestration, a splenectomy may be required.
7. Discuss bone marrow transplants with the provider.
Currently, stem cell and bone marrow transplants (BMT) are the only cure for sickle cell disease. Most BMT procedures are carried out on patients who have experienced complications like strokes, acute chest syndrome, and recurrent pain crises. Bone marrow transplants carry significant risks and are not performed frequently.
Risk for Decreased Cardiac Output
Sickle cell anemia causes decreased cardiac function due to the abnormal sickling of red blood cells. The crescent-like shape obstructs the vessels and decreases oxygenated blood to the heart, resulting in cardiomyopathies like pulmonary hypertension and heart failure.
Nursing Diagnosis: Risk for Decreased Cardiac Output
Related to:
- Vaso-occlusive crisis
- Inadequate filling of blood in the heart
- Decreased oxygenated blood to the heart
- Poor cardiac function
- Low red blood cell count
- Low hemoglobin levels
As evidenced by:
A risk diagnosis is not evidenced by signs and symptoms as the problem has not yet occurred. Nursing interventions are aimed at prevention.
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.
Assessment:
1. Review echocardiogram.
Elevated tricuspid regurgitation velocity on echocardiogram suggests pulmonary hypertension. LV diastolic dysfunction is observed with heart failure. An echocardiogram can be performed every few years or when symptoms occur to monitor disease progression.
2. Monitor the RBCs.
Patients with sickle cell anemia live with chronically low hemoglobin levels, but a lack of healthy hemoglobin and RBCs prevents oxygen from circulating to the heart.
3. Perform a physical assessment.
Note for any signs of poor cardiac output, such as:
- Dyspnea on exertion
- Activity intolerance
- Low oxygen saturation
- Edema
- Jugular vein distension
Interventions:
1. Administer oxygen.
Reduced cardiac output may cause symptoms of dyspnea and low SpO2. Apply oxygen as needed for hypoxia.
2. Prepare for blood transfusions or red blood cell exchange.
A blood transfusion may be necessary to boost cardiac output and help the body’s circulation of oxygenated blood in cases of sickle cell crisis. Red blood cell exchange replaces the abnormal RBCs with normal blood cells and can reduce complications like acute chest syndrome.
3. Prepare for heart catheterization.
A heart catheterization is required to diagnose pulmonary hypertension. A pulmonary artery pressure of ≥ 25 mmHg, determined by a right heart catheterization, defines pulmonary hypertension.
4. Administer medications as prescribed.
The patient with pulmonary hypertension or heart failure may benefit from:
- Aspirin
- Diuretics
- Beta-blockers
- ACE inhibitors
Risk for Infection
Risk for infection associated with sickle cell anemia can be caused by diminished red blood cell formation, accelerated RBC destruction, or folic acid deficiency. This can cause a compromised immune system susceptible to viral infection.
Nursing Diagnosis: Risk for Infection
Related to:
- Vaso-occlusive crisis
- Splenic sequestration
- Hyperhemolytic crisis
- Aplastic crisis
- Disease process
- Decreased blood supply in the affected area
- Tissue death
- Compromised immune system
- Treatment such as hydroxyurea and bone marrow transplant
As evidenced by:
A risk diagnosis is not evidenced by signs and symptoms as the problem has not yet occurred. Nursing interventions are aimed at prevention.
Expected outcomes:
- Patient will be able to 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.
Assessment:
1. Assess risk factors.
Sickle cell crisis, complications, and treatments can compromise the immune system of patients with sickle cell anemia.
2. Assess the environment of the patient.
Patients with sickle cell anemia can acquire infections that arise from exposure to environmental or airborne microorganisms.
3. Assess the patient’s hygiene.
Personal hygiene, such as frequent handwashing, can minimize the transmission of microorganisms causing infection.
Interventions:
1. Administer prophylactic antibiotics as prescribed.
In children with sickle cell disease between 2 months and five years, antibiotic prophylaxis with penicillin helps prevent life-threatening infections.
2. Ask the patient to verbalize the signs and symptoms of infection.
Teach parents of patients with sickle cell anemia about signs and symptoms of impending infection (such as increasing temperature) and when to seek medical assistance.
3. Ask parents and patients to demonstrate infection control measures.
Demonstrate and observe effective handwashing and use of alcohol-based hand sanitizers.
4. Ensure vaccinations are given.
Ensure patients receive pneumococcal, meningococcal, and annual influenza vaccines due to functional asplenia’s increased susceptibility to infection.
Risk for Venous Thromboembolism
The crescent-shaped structure of red blood cells in sickle cell anemia causes vascular occlusion and increases the risk of blood clot formation.
Nursing Diagnosis: Risk for Venous Thromboembolism
Related to:
- Sickling red blood cells (RBC)
- Increased clumping of RBCs
- Increased blood viscosity
- Dehydration
As evidenced by:
A risk diagnosis is not evidenced by signs and symptoms as the problem has not yet occurred. Nursing interventions are aimed at prevention.
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.
Assessment:
1. Review Doppler ultrasound.
Children with sickle cell anemia are at a higher risk for stroke. Children ages 2 to 16 should receive a transcranial Doppler ultrasound annually to measure blood flow in the brain. A Doppler ultrasound is also used to assess for the presence of blood clots in the extremities.
2. Assess risk factors for thromboembolism.
Frequent hospitalizations, recent surgery, pregnancy, splenectomy, dehydration, catheters (such as a Port-A-Cath), and smoking are significant risk factors for thrombosis in patients with sickle cell anemia.
3. Assess the patient’s and their family’s ability to recognize a stroke or blood clot.
The following are signs of stroke :
- Facial drooping
- Arm weakness
- Speech difficulty
- Unsteady gait
- Eyesight changes
- Changes in mentation
Recognizing signs of a blood clot may prove challenging as a vaso-occlusive crisis commonly causes pain, and lower extremity edema may occur with other complications of sickle cell anemia. Additional signs that may signal a blood clot include:
- Swelling in one leg that is unequal to the other
- Warmth in the extremity
Interventions:
1. Prepare for monthly blood transfusions.
Regular blood transfusions can lower the risk of blood vessel occlusion and stroke by reducing the number of sickled cells and diluting the blood in circulation.
2. Administer anticoagulants.
Heparin and direct oral anticoagulants like rivaroxaban or apixaban are administered to treat venous thromboembolism. Patients with central venous catheters may require anticoagulant flushes.
3. Maintain hydration.
Dehydration is a common cause of a vaso-occlusive crisis and may also contribute to the formation of blood clots from hyperviscosity. Infuse IV fluids as necessary and instruct the patient to consume enough liquids.
4. Educate on continuing anticoagulation.
After acute venous thromboembolism, it is recommended that patients continue anticoagulant treatment for three months, as shorter durations are associated with a higher risk of recurrence.
References
- American Stroke Association. (2021, September 20). Sickle cell disease and pediatric stroke risk. www.stroke.org. Retrieved August 2023, from https://www.stroke.org/en/about-stroke/stroke-in-children/sickle-cell-disease
- Centers for Disease Control and Prevention. (2023, July 6). What is sickle cell disease? Retrieved July 2023, from https://www.cdc.gov/ncbddd/sicklecell/facts.html
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