Sickle cell anemia is a genetic blood disorder that affects millions of people worldwide. It is characterized by the abnormal shape of red blood cells, which can lead to numerous complications. Nursing care plans are critical in managing sickle cell anemia crisis and providing quality care for patients. In this article, we will discuss the nursing diagnosis for sickle cell anemia crisis nursing care plans and how to effectively care for patients with this condition.
What is Sickle Cell Anemia?
Sickle cell disease (SCD), or sickle cell anemia (SCA) is a group of hereditary blood disorders characterized by an abnormality in the oxygen-carrying hemoglobin molecule in red blood cells. The most common forms of SCDs are homozygous hemoglobin SS disease (sickle cell anemia), hemoglobin SC disease, and sickle [beta]-thalassemia.
Sickle cell anemia is a severe hemolytic anemia that results from the inheritance of the sickle hemoglobin gene. This gene causes the hemoglobin molecule to be defective. Sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension. The oxygen level in venous blood can be low enough to cause this change; consequently, the erythrocyte containing HbS loses its round, pliable, biconcave disk shape and becomes deformed, rigid, and sickle-shaped. These long, rigid erythrocytes can adhere to the endothelium of small vessels; when they adhere to each other, blood flow to a region or an organ may be reduced. 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 before the membrane becomes too rigid, it can revert to a normal shape. For this reason, the “sickling crises” are intermittent. Cold can aggravate the sickling process because vasoconstriction slows the blood flow. Oxygen delivery can also be impaired by an increased blood viscosity, with or without occlusion due to the adhesion of sickled cells; in this situation, the effects are seen in larger vessels, such as arterioles.
Sickle cell anemia is most common in tropical Africans in people of African descent; about 1 in 10 African-American carries the abnormal gene. If two parents who are both carriers of the sickle cell trait have offspring, each child has a 25% chance of developing sickle cell anemia. However, sickle cell anemia also appears in other ethnic populations, including people of Mediterranean or East Indian Ancestry. Overall, 1 in every 400 to 600 black children has sickle cell anemia. The defective HbS-producing gene may have persisted because, in areas where malaria is endemic, the heterozygous sickle cell trait provides resistance to malaria and is actually beneficial.
Nursing Care Plans
Nursing care planning and goals for patients with sickle cell anemia include: providing relief for pain, decreasing incidences of sickle cell crisis, preventing and treating complications, promoting adherence to treatment plans, and helping the patient and family members understand the condition and how to manage it.
Here are six (6) nursing care plans (NCP) and nursing diagnoses (NDx) for patients with sickle cell anemia:
- Impaired Gas Exchange
- Ineffective Tissue Perfusion
- Risk for Deficient Fluid Volume
- Acute Pain
- Risk for Impaired Skin Integrity
- Deficient Knowledge
- Other Possible Nursing Care Plans
Impaired Gas Exchange
Impaired gas exchange can occur in patients with sickle cell disease due to the combined effects of decreased oxygen-carrying capacity of the blood, reduced red blood cell lifespan, and increased blood viscosity. The characteristic sickle-shaped red blood cells in sickle cell anemia have a shorter lifespan and are less able to deliver oxygen to the body’s tissues. In addition, patients with sickle cell anemia are more susceptible to bacterial pneumonia, which can further compromise gas exchange.
- Impaired Gas Exchange
May be related to
- The decreased oxygen-carrying capacity of the blood, reduced RBC life span/premature destruction, abnormal RBC structure; sensitivity to low oxygen tension (strenuous exercise, increase in altitude)
- Increased blood viscosity (occlusions created by sickled cells packing together within the capillaries) and pulmonary congestion (impairment of surface phagocytosis)
- Predisposition to bacterial pneumonia, pulmonary infarcts
Possibly evidenced by
- Use of accessory muscles
- Cyanosis (hypoxia)
- The client will demonstrate improved ventilation/oxygenation as evidenced by respiratory rate within normal limits, absence of cyanosis and use of accessory muscles; clear breath sounds.
- The client will participate in ADLs without weakness and fatigue.
- The client will display improved/normal pulmonary function tests.
Nursing Assessment and Rationales
1. Monitor respiratory rate, depth, use of accessory muscles, and areas of cyanosis.
These are indicators of the adequacy of respiratory function or degree of compromise and the effectiveness or need for therapy.
2. Auscultate and note the presence or absence of breath sounds and adventitious sounds.
Development of atelectasis and stasis of secretions can impair gas exchange.
3. Monitor vital signs and note changes in cardiac rhythm.
Compensatory changes in vital signs and the development of dysrhythmias reflect the effects of hypoxia on the cardiovascular system.
4. Check thoroughly reports of chest pain and increasing fatigue. Monitor for signs of fever, cough, and adventitious breath sounds.
This may reflect the development of acute chest syndrome which increases the workload of the heart and oxygen demand. The crisis is a common complication in sickle-cell patients and can be associated with one or more symptoms including fever, cough, excruciating pain, sputum production, shortness of breath, or low oxygen levels.
5. Regularly assess the level of consciousness.
Brain tissue is very sensitive to decreases in oxygen. Doing the assessment may be an early indicator of developing hypoxia.
6. Monitor laboratory studies: CBC, ABGs, pulse oximetry, cultures, chest x-rays, and pulmonary function tests.
These tests can provide valuable information about the patient’s overall health status and help identify any underlying respiratory or infectious complications.
Nursing Interventions and Rationales
1. Assist the client in turning, coughing, and deep-breathing exercises.
Promote expansion of the chest optimally, mobilization of secretions, and aeration of all lung fields. This also reduces the risk of stasis of secretions and pneumonia.
2. Evaluate the patient’s tolerance to activity, and limit activities within the patient’s tolerance. Assist with ADLs and mobility as needed.
Reducing the metabolic requirements of the body would reduce the oxygen requirements.
3. Schedule rest periods and encourage the patient to alternate rests and activities.
To protect the patient from excessive fatigue and reduce oxygen demands.
4. Teach and demonstrate the use of relaxation techniques: guided imagery, deep breathing exercises, and visualization.
Relaxation decreases muscle tension and anxiety and hence the metabolic demand for oxygen.
5. Encourage adequate fluid intake (2 to 3 L/day) within cardiac tolerance.
Adequate intake is necessary to provide for the mobilization of secretions and prevent hyperviscosity of blood occlusion.
6. Screen the health status of patients’ visitors and staff. Use PPEs when necessary.
Protects from potential sources of respiratory infection.
7. Administer supplemental humidified oxygen as indicated.
Supplemental oxygen maximizes the transport of oxygen to the tissues. Oxygen should only be given in the presence of confirmed hypoxemia because oxygen can suppress erythropoietin levels, further reducing the production of RBCs.
8. Perform and assist with chest physiotherapy, intermittent positive-pressure breathing (IPPB), and incentive spirometry.
Patients with SSD are prone to pneumonia, which can be potentially fatal because of its hypoxic effect of increasing sickling. Mobilizes secretions and increases the aeration of lung fields.
9. Administer packed RBCs or exchange transfusion as ordered.
Increasing the number of oxygen-carrying cells dilutes the percentage of HbS to prevent sickling, improves circulation, and decreases the number of sickled cells. Plain red blood cells (PRCs) are usually used because they are less likely to create a circulatory overload. Partial transfusions are sometimes used prophylactically in high-risk situations (e.g., preparation for general anesthesia, third trimester of pregnancy.)
10. Administer medications as indicated:
- 10.1. Antipyretics: acetaminophen (Tylenol)
Maintains normal temperature to reduce metabolic oxygen demands without affective serum pH, which may occur with aspirin.
- 10.2. Antibiotics
A broad-spectrum antibiotic is started immediately pending culture results of suspected infections. This may change when the specific pathogen is identified.
Recommended nursing diagnosis and nursing care plan books and resources.
Ackley and Ladwig’s Nursing Diagnosis Handbook: An Evidence-Based Guide to Planning Care
We love this book because of its evidence-based approach to nursing interventions. This care plan handbook uses an easy, three-step system to guide you through client assessment, nursing diagnosis, and care planning. Includes step-by-step instructions showing how to implement care and evaluate outcomes, and help you build skills in diagnostic reasoning and critical thinking.
Nursing Care Plans – Nursing Diagnosis & Intervention (10th Edition)
Includes over two hundred care plans that reflect the most recent evidence-based guidelines. New to this edition are ICNP diagnoses, care plans on LGBTQ health issues, and on electrolytes and acid-base balance.
NANDA International Nursing Diagnoses: Definitions & Classification, 2021-2023
The definitive guide to nursing diagnoses is reviewed and approved by NANDA International. In this new version of a pioneering text, all introductory chapters have been rewritten to provide nurses with the essential information they need to comprehend assessment, its relationship to diagnosis and clinical reasoning, and the purpose and application of taxonomic organization at the bedside. A total of 46 new nursing diagnoses and 67 amended nursing diagnostics are presented.
Nurse’s Pocket Guide: Diagnoses, Prioritized Interventions, and Rationales
Quick-reference tool includes all you need to identify the correct diagnoses for efficient patient care planning. The sixteenth edition includes the most recent nursing diagnoses and interventions from NANDA-I 2021-2023 and an alphabetized listing of nursing diagnoses covering more than 400 disorders.
Nursing Diagnosis Manual: Planning, Individualizing, and Documenting Client Care
Identify interventions to plan, individualize, and document care for more than 800 diseases and disorders. Only in the Nursing Diagnosis Manual will you find for each diagnosis subjectively and objectively – sample clinical applications, prioritized action/interventions with rationales – a documentation section, and much more!
All-in-One Nursing Care Planning Resource – E-Book: Medical-Surgical, Pediatric, Maternity, and Psychiatric-Mental Health
Includes over 100 care plans for medical-surgical, maternity/OB, pediatrics, and psychiatric and mental health. Interprofessional “patient problems” focus familiarizes you with how to speak to patients.
Other recommended site resources for this nursing care plan:
- Nursing Care Plans (NCP): Ultimate Guide and Database MUST READ!
Over 150+ nursing care plans for different diseases and conditions. Includes our easy-to-follow guide on how to create nursing care plans from scratch.
- Nursing Diagnosis Guide and List: All You Need to Know to Master Diagnosing
Our comprehensive guide on how to create and write diagnostic labels. Includes detailed nursing care plan guides for common nursing diagnostic labels.
Other care plans for hematologic and lymphatic system disorders:
- Anaphylactic Shock | 4 Care Plans
- Anemia | 6 Care Plans UPDATED!
- Aortic Aneurysm | 4 Care Plans
- Deep Vein Thrombosis | 5 Care Plans UPDATED
- Disseminated Intravascular Coagulation | 4 Care Plans
- Hemophilia | 5 Care Plans
- Leukemia | 5 Care Plans
- Lymphoma | 3 Care Plans
- Sepsis and Septicemia | 6 Care Plans
- Sickle Cell Anemia Crisis | 6 Care Plans
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