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. The 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 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 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 an 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, decrease incidences of sickle cell crisis, enhanced sense of self-esteem and power, and absence of complications.
Here are six (6) nursing care plans (NCP) and nursing diagnosis (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
Risk for Deficient Fluid Volume
Nursing Diagnosis
- Risk for Deficient Fluid Volume
Risk factors may include
- Increased fluid needs, e.g., hypermetabolic state/fever, inflammatory processes
- Renal parenchymal damage/infarctions limiting the kidney’s ability to concentrate urine (hyposthenuria)
Possibly evidenced by
- Not applicable. Existence of signs and symptoms establishes an actual nursing diagnosis.
Desired Outcomes
- Maintain adequate fluid balance as evidenced by individually appropriate urine output with a near-normal specific gravity, stable vital signs, moist mucous membranes, good skin turgor, and prompt capillary refill.
| Nursing Interventions | Rationale |
|---|---|
| Maintain accurate I&O and weigh daily. | Patient may reduce fluid intake during periods of crisis because of malaise and anorexia. Dehydration from vomiting, diarrhea, and fever may reduce urine output and initiate a vaso-occlusive crisis. |
| Note urine characteristics and specific gravity. | Kidney can lose its ability to concentrate urine, resulting in excessive losses of dilute urine and fixation of the specific gravity. |
| Monitor vital signs, comparing with patient’s previous readings. Measure BP in lying, sitting, and standing positions if possible. | Reduction of circulating blood volume can occur from increased fluid loss, resulting in hypotension and tachycardia. |
| Assess patient for fever, changes in level of consciousness, poor skin turgor, dryness of skin and mucous membranes, pain. | Symptoms may indicate dehydration or hemoconcentration with consequent vaso-occlusive state. |
| Closely monitor vital signs during blood transfusions and note presence of dyspnea, crackles, rhonchi, wheezes, jugular vein distention, diminished breath sounds, cough, frothy sputum and cyanosis. | The patient’s heart may already be weakened and prone to failure because of the chronic demands placed on it by the anemic state. The heart may be unable to tolerate the added fluid volume from transfusions or rapid IV fluid administration administered to treat crisis or shock. |
| Administer IV fluids as indicated. | IV fluids replaces losses and fills deficit. It may reverse renal concentration of RBCs and presence of failure. Fluids must be given immediately to decrease hemoconcentration and prevent further infarction. |
| Monitor laboratory studies: Hb/Hct, serum and urine electrolytes. | Elevation in Hb/Hct may indicate hemoconcentration. The loss kidneys’ ability to concentrate urine may result in serum depletions of Na+, K–, and Cl–, therefore necessitating replacement. |
Recommended Resources
Recommended nursing diagnosis and nursing care plan books and resources.
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- Nursing Care Plans: Nursing Diagnosis and Intervention (10th Edition)
An awesome book to help you create and customize effective nursing care plans. We highly recommend this book for its completeness and ease of use. - Nurse’s Pocket Guide: Diagnoses, Prioritized Interventions and Rationales
A quick-reference tool to easily select the appropriate nursing diagnosis to plan your patient’s care effectively. - NANDA International Nursing Diagnoses: Definitions & Classification, 2021-2023 (12th Edition)
The official and definitive guide to nursing diagnoses as reviewed and approved by the NANDA-I. This book focuses on the nursing diagnostic labels, their defining characteristics, and risk factors – this does not include nursing interventions and rationales. - Nursing Diagnosis Handbook, 12th Edition Revised Reprint with 2021-2023 NANDA-I® Updates
Another great nursing care plan resource that is updated to include the recent NANDA-I updates. - Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5(TM))
Useful for creating nursing care plans related to mental health and psychiatric nursing. - Ulrich & Canale’s Nursing Care Planning Guides, 8th Edition
Claims to have the most in-depth care plans of any nursing care planning book. Includes 31 detailed nursing diagnosis care plans and 63 disease/disorder care plans. - Maternal Newborn Nursing Care Plans (3rd Edition)
If you’re looking for specific care plans related to maternal and newborn nursing care, this book is for you. - Nursing Diagnosis Manual: Planning, Individualizing, and Documenting Client Care (7th Edition)
An easy-to-use nursing care plan book that is updated with the latest diagnosis from NANDA-I 2021-2023. - All-in-One Nursing Care Planning Resource: Medical-Surgical, Pediatric, Maternity, and Psychiatric-Mental Health (5th Edition)
Definitely an all-in-one resources for nursing care planning. It has over 100 care plans for different nursing topics.
See also
Other recommended site resources for this nursing care plan:
- Nursing Care Plans (NCP): Ultimate Guide and Database
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 | 4 Care Plans
- Aortic Aneurysm | 4 Care Plans
- Deep Vein Thrombosis | 5 Care Plans
- 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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