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Nursing Guide to Sickle Cell Anemia: Nursing Diagnosis, Interventions, & Care Plans

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Sickle cell anemia is a genetic hemoglobinopathy that causes red blood cells to adopt an abnormal, rigid, sickle shape. These cells tend to clump and block blood flow, leading to severe complications such as pain crises, anemia, and organ damage.  

This chronic condition is most prevalent in individuals of Sub-Saharan African, Spanish-speaking regions in the Western Hemisphere, Saudi Arabian, Indian, and Mediterranean ancestry. Sickle cell anemia requires ongoing multidisciplinary care, and nurses play a pivotal role in the identification, acute management, and education of patients and their families. 

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Etiology and epidemiology 

Sickle cell anemia results from a single point mutation in the beta-globin gene located on chromosome 11. This mutation causes the substitution of thymine for adenine causing coding for valine in place of glutamic acid at the sixth position of the beta-globin chain, leading to the production of hemoglobin S (HbS).  

When exposed to low oxygen conditions, HbS undergoes decreased solubility, and increased viscosity, and polymer formation distorting red blood cells into a crescent or "sickle" shape. Individuals who inherit two identical copies of the sickle cell gene (one from each parent) acquire sickle cell anemia, whereas those who inherit two different versions of the sickle cell gene typically exhibit no or minimal symptoms and are classified as having sickle cell trait. It’s important to note that heterozygous inheritance leads to sickle cell trait, whereas homozygous inheritance leads to sickle cell anemia, also known as HbSS disease.  

This autosomal recessive disorder disproportionately affects individuals of Sub-Saharan African descent, with much less frequency in Mediterranean, Middle Eastern, and Indian populations. Worldwide, sickle cell anemia impacts millions of people, particularly in sub-Saharan Africa, where it contributes significantly to childhood morbidity and mortality.  

In the U.S., sickle cell anemia affects at least 100,000 individuals. The incidence is highest among Black or African American individuals, with a prevalence of 1 in 365 births, and it occurs in about 1 in every 16,300 Hispanic-American births.  

Thanks to widespread implementation of newborn screening and early preventive care for complications associated with sickle cell disease, such as penicillin prophylaxis for infants and children under 5 years of age, and immunizations, outcomes for children diagnosed with sickle cell anemia have improved markedly in high-resource settings. 

ICD-10 code 

The American ICD-10-CM code for sickle cell disorders is D57 and various subcategories exist for specific complications or without crisis: 

  • D57.1 — Sickle-cell disease without crisis 
  • D57.0 — Hb-SS disease with crisis 
  • D57.00 — Hb-SS disease with crisis, unspecified 
  • D57.01 — Hb-SS disease with acute chest syndrome 
  • D57.02 — Hb-SS disease with splenic sequestration 
  • D57.03 — Hb-SS disease with cerebral vascular involvement 
  • D57.04 — Hb-SS disease with dactylitis 
  • D57.09 — Hb-SS disease with crisis with other specified complication 

Assessment 

Assessing sickle cell anemia involves a thorough evaluation of both clinical presentation and diagnostic testing to ensure accurate diagnosis and timely management. 

Clinical presentation 

Patients with sickle cell anemia typically present with: 

  • Chronic hemolytic anemia 
  • Intermittent vaso-occlusive crisis with pain episodes (pain crises)  
  • Fatigue and pallor 
  • Jaundice and scleral icterus 
  • Delayed growth and puberty 
  • Frequent infections 
  • Splenic sequestration, most seen in the first five years of life in children 
  • Hand-foot syndrome in infants or toddlers 
  • Acute chest syndrome 
  • Stroke, typically ischemic in children and hemorrhagic in adults 
  • Coronary artery ectasia with systolic murmur 
  • Cholelithiasis, which can lead to cholecystitis or common bile duct blockage 
  • Priapism in teenage and adult males 
  • Avascular necrosis with hip pathology 
  • Pulmonary hypertension 

Diagnostic tests 

  • Hemoglobin electrophoresis: This is considered the gold standard test that identifies HbS by separating different types of hemoglobin based on their electric charge. It confirms the presence of hemoglobin S and genetic testing differentiates between sickle cell trait and sickle cell disease. 
  • Complete blood count (CBC): This reveals normocytic, normochromic anemia, typically with low hemoglobin levels and an elevated reticulocyte count, reflecting increased red blood cell turnover due to chronic hemolysis.  
  • Peripheral blood smear: This examines the morphology and evaluates microscopic changes in blood cells. Performing this test shows the presence of sickled erythrocytes, target cells, and Howell-Jolly bodies, which suggest functional asplenia. 
  • Solubility sickling test: This is the most widely used test to diagnose sickle cell disease based on the polymerization of hemoglobin S (HgS) in a deoxygenated state. Easy and affordable to perform, but limitations cause false-negative results when used with newborns or in patients with coinheritance of a-thalassemia trait.  
  • Transcranial Doppler ultrasound: This is recommended in children aged two to 16 years to evaluate cerebral blood flow velocities. Elevated velocities indicate increased risk of stroke and may necessitate chronic transfusion therapy to reduce this risk. 

Management 

Management of sickle cell anemia focuses on preventing complications, alleviating symptoms, and improving quality of life through a combination of medical, pharmacologic, and supportive interventions. Treatment plans are tailored to the severity of the disease and individual patient needs. 

Medical interventions 

  • Hydroxyurea: A disease-modifying agent that increases total and fetal hemoglobin (HbF) levels, which reduces red cell sickling and significantly decreases the number of pain crises, need for transfusions, and risk of acute chest syndrome (ACS). It is approved for both adults and children age six months and older, and is generally well tolerated. 
  • L-glutamine: An amino acid supplement approved for use in patients aged 5 years and older. It reduces oxidative stress in red blood cells, thereby decreasing the frequency of acute complications such as pain crises. It is often used in conjunction with other therapies like hydroxyurea. 
  • Crizanlizumab: A monoclonal antibody targeting P-selectin, a molecule involved in cell adhesion and vaso-occlusion. It reduces the frequency of pain episodes by preventing sickled cells from sticking to blood vessel walls. Administered via monthly IV infusion, it is approved for patients aged 16 years and older. 
  • Blood transfusions: Used to manage severe anemia, acute chest syndrome, and stroke prevention. Chronic transfusion therapy is especially important in children with high stroke risk as indicated by abnormal transcranial doppler findings. Monitoring for iron overload and iron chelation therapy is often necessary with long-term transfusion use. 
  • Bone marrow transplantation: The only curative option currently available. It replaces the patient's defective bone marrow with healthy stem cells from a matched donor. Though potentially curative, it carries risks such as graft-versus-host disease and is limited by the availability of appropriate donors. It is most effective in children and young adults with minimal organ damage. 
  • Gene Therapy: Exagamglogene autotemcel and Lovotibeglogene autotemcel are two recently FDA approved gene therapies aimed at adding new DNA or modifying existing DNA to improve the cell’s function or restore activity of faulty or missing genes and can provide an option for those who don’t have a well-matched donor to undergo a bone marrow transplant.  

Acute crisis management 

Those with sickle cell anemia are prone to a variety of acute crises that can become life-threatening without prompt and appropriate care. Recognizing the type of crisis is the first step in determining the correct clinical response. 

Types of sickle cell crises 

Sickle cell crises vary in presentation and severity. Here are the most common and clinically significant types: 

  • Vaso-occlusive crisis (VOC) 
  • Acute chest syndrome (ACS) 
  • Splenic sequestration crisis 
  • Acute pain crisis 
  • Aplastic crisis 
  • Stroke (ischemic or hemorrhagic) 
  • Avascular necrosis (AVN) 
  • Severe anemia 
  • Serious infection 
  • Priapism 

Managing sickle cell crises 

Once the specific type of crisis is identified, treatment must be tailored accordingly. Core principles of managing acute complications in sickle cell disease include: 

  • Pain control with opioids and NSAIDs 
  • Vigorous intravenous hydration 
  • Oxygen therapy if hypoxic 
  • Antibiotics for suspected infection 
  • Hospitalization for severe complications (e.g., acute chest syndrome, stroke) 
  • Emergent blood transfusions 
  • Recognition and treatment of priapism lasting longer than 4 hours 
  • Surgical intervention for cholecystitis or common bile duct blockage 

Nursing care plan 

Nursing care plans for individuals with sickle cell anemia are essential for addressing both acute symptoms and long-term health needs. These plans focus on managing pain, preventing complications, promoting adequate hydration and oxygenation, and supporting psychosocial well-being 

Nursing considerations 

Nurses must provide individualized, culturally sensitive, and family-centered care, recognizing the unique physiological and psychosocial needs of both pediatric and adult populations with sickle cell anemia. This includes fostering trust through effective communication and ensuring that care plans respect the patient’s cultural values and beliefs.  

Nurses should vigilantly monitor for early signs of complications such as infection, stroke, or acute chest syndrome, and act promptly to prevent escalation. They’re responsible for accurate administration of prescribed treatments such as pain management, hydration therapy, and oxygen support.  

Additionally, nurses serve as advocates, not only for adequate pain control but also for equitable access to comprehensive care and psychosocial resources, particularly for patients from underserved communities disproportionately affected by this disease. 

Assessment 

  • Obtain medical history of SCD-related complications: Aplastic crisis, splenic sequestration, priapism in males, acute chest syndrome, stroke, cholelithiasis, avascular necrosis, pulmonary hypertension, or deep vein thrombosis. 
  • Pain characteristics: Intensity, quality, duration, location, and relieving and aggravating factors, if any.  
  • Signs of anemia: Jaundice, extreme fatigue, dyspnea, dizziness, and irregular heartbeat.  
  • Respiratory status: Tachypnea or dyspnea. 
  • Cardiovascular status: Systolic murmur, hypotension, tachycardia, or high-output heart failure. 
  • Hydration status 
  • Palpate abdomen: Enlarged liver or spleen, tenderness. 
  • Signs of infection: Fever in children, neck stiffness, and positive Brudzinski or Kernig signs. 
  • Growth and development in children: Delayed growth, delayed sexual maturation, inadequate weight gain, and delayed height growth. Psychosocial and developmental assessment in pediatric patients. 

Nursing diagnosis/risk for 

Acute pain related to:  

  • Vaso-occlusive crisis 
  • Tissue hypoxia 
  • Bone marrow expansion 
  • Joint inflammation 

Risk for infection related to: 

  • Functional asplenia 
  • Chronic anemia 
  • Frequent hospitalizations 
  • Invasive procedures 

Activity intolerance related to: 

  • Chronic anemia 
  • Reduced oxygen-carrying capacity 
  • Fatigue  
  • Pain 
  • Impaired tissue perfusion 
  • Sickling of blood cells 
  • Vaso-occlusion 
  • Chronic anemia 
  • Hypoxia  

Interventions 

  • Administer pain medications as ordered and assess efficacy. 
  • Encourage fluid intake and administer IV fluids. 
  • Maintain oxygen therapy as needed. 
  • Monitor temperature and signs of infection. 
  • Transfuse blood as ordered. 
  • Provide emotional support and psychosocial counseling. 
  • Educate caregivers on crisis prevention. 

Expected outcomes 

  • Patient reports effective pain relief. 
  • Vital signs remain stable and within normal limits. 
  • No signs of infection or complications observed. 
  • Patient and family verbalize understanding of disease management. 

Individual/caregiver education 

Education is essential in the ongoing management of sickle cell anemia. Nurses should teach patients and caregivers about: 

  • Importance of routine follow-up visits 
  • Adherence to prescribed medications 
  • Recognizing early signs of a pain crisis 
  • Avoiding triggers (extreme temperatures, dehydration, stress, smoking) 
  • Importance of vaccination and infection prevention 
  • When to seek emergency care (e.g., chest pain, severe headache, weakness) 

FAQs

Resources 

References 

Amanda Gibson, BSN, RN

Amanda Gibson, BSN, RN

Amanda Gibson, BSN, RN, has been a nurse for 15 years, serving in critical care, perioperative care, emergency medicine, and nursing education. Driven by a passion for advancing healthcare for American Indians and Alaska Natives (AI/AN), Amanda is currently pursuing a master’s degree in nursing leadership and business administration for healthcare. A proud member of the Cherokee Nation, she works with leaders to customize solutions for Native American learning and staffing needs, increase efficiency, and improve the lives of clinicians caring for AI/AN patients.