Sickle cell anemia affects the oxygen-carrying blood cells, known as red blood cells (RBCs). Round, healthy RBCs transport oxygen to every body part and organ system. RBCs typically have a round shape and are flexible, so they may readily pass through blood vessels. In sickle cell anemia though, 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.
Sickle cell anemia is a genetic condition inherited at birth. Both parents must carry the sickle cell gene and pass it on to their child 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 they 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 with the only cure being stem cell or bone marrow transplants.
The following are common complications of sickle cell anemia:
- Vaso-occlusive crisis – blood stasis and clumping in the circulation causes fever and 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.
The 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 avoid complications such as vaso-occlusive crises.
Many of these patients die prematurely despite improvements in treatment. The morbidity is very high, and nearly all individuals experience a sickle cell crisis at some point in their lives. These patients often struggle with chronic severe pain and may experience a reduced quality of life.
The nursing care plan will include the administration of penicillin prophylaxis for children, blood transfusions, oxygen therapy, pain management, and immunization. During patient education the nurse should stress the importance of staying hydrated, getting adequate sleep, not smoking, infection prevention, and following up with their hematologist.
Nursing Care Plans Related to Sickle Cell Anemia
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
Ineffective Tissue Perfusion 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.
Ineffective Tissue Perfusion Interventions
1. Keep the patient oxygenated.
During a vaso-occlusive crisis, sickle cells clump together and are not able to carry oxygenated blood to the rest of the body. The administration of oxygen will help increase oxygen and tissue perfusion.
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.
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
Chronic Pain 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.
Chronic Pain 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.
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
Risk for 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.
Risk for 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.
References and Sources
- Centers for Disease Control and Prevention. (2022). What is sickle cell disease? https://www.cdc.gov/ncbddd/sicklecell/facts.html
- Doenges, M. E., Moorhouse, M. F., & Murr, A. C. (2019). Nurse’s pocket guide: Diagnoses, interventions, and rationales (15th ed.). F A Davis Company.
- Mangla, A., Ehsan, M., Agarwal, N., Maruvada, S., & Doerr, C. (2022). Sickle Cell Anemia (Nursing). National Library of Medicine – National Center for Biotechnology Information. https://www.ncbi.nlm.nih.gov/books/NBK568706/
- Silvestri, L. A., & Silvestri, A. E. (2022). Saunders comprehensive review for the NCLEX-RN examination (9th ed.). Elsevier Inc.
- US National Heart, Lung, and Blood Institute. (2022). Sickle cell disease – Treatment | NHLBI, NIH. Advancing Heart, Lung, Blood, and Sleep Research & Innovation | NHLBI, NIH. https://www.nhlbi.nih.gov/health/sickle-cell-disease/treatment
