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
Deficient Knowledge
Nursing Diagnosis
May be related to
- Lack of exposure/recall
- Information misinterpretation
- Unfamiliarity with resources
Possibly evidenced by
- Questions, request for information, statement of misconceptions
- Inaccurate follow-through of instructions; development of preventable complications
- Verbal/nonverbal cues of anxiety
Desired Outcomes
- Verbalize understanding of disease process, including symptoms of crisis, potential complications.
- Verbalize understanding of therapeutic needs.
- Initiate necessary behaviors/lifestyle changes to prevent complications.
- Participate in continued medical follow-up; genetic counseling/family planning services.
| Nursing Interventions | Rationale |
|---|---|
| Review disease process and treatment needs. | Provides knowledge base from which patient can make informed choices. Note: The median age at death for females is 48 years old and 42 years old for males. Deaths often occur due to organ failure. |
| Review precipitating factors: | |
| Causes peripheral vasoconstriction, which may result in sludging of the circulation, increased sickling, and may precipitate a vaso-occlusive crisis. |
| Increases metabolic demand for oxygen and increases insensible fluid losses (evaporation and perspiration) leading to dehydration, which may increase blood viscosity and tendency to sickle. |
| Decreased oxygen tension present at higher altitudes causes hypoxia and potentiates sickling of cells. |
| Encourage consumption of at least 4–6 qt of fluid daily, during a steady state of the disease, increasing to 6–8 qt during a painful crisis or while engaging in activities that might precipitate dehydration. | Prevents dehydration and consequent hyperviscosity that can potentiate sickling. |
| Encourage ROM exercise and regular physical activity with a balance between rest and activity. | Prevents bone demineralization and may reduce risk of fractures. Aids in maintaining level of resistance and decreases oxygen needs. |
| Review patient’s current diet, reinforcing the importance of diet including liver, green leafy vegetables, citrus fruits, and wheat germ. Provide necessary instruction regarding supplementary vitamins such as folic acid. | Sound nutrition is essential because of increased demands placed on bone marrow. Folic acid supplements are frequently ordered to prevent aplastic crisis. |
| Discourage smoking and alcohol consumption and identify appropriate community support groups. | Nicotine induces peripheral vasoconstriction and decreases oxygen tension, which may contribute to cellular hypoxia and sickling. Alcohol increases the possibility of dehydration therefore precipitates sickling. Maintaining these changes in behavior and lifestyle may require prolonged support. |
| Discuss principles of skin and extremity care and protection from injury. Encourage prompt treatment of cuts, insect bites, sores. | Because of impaired tissue perfusion, especially in the periphery, distal extremities are especially susceptible to altered skin integrity/infection. |
| Include instructions on care of leg ulcers that might develop. | Fosters independence and maintenance of self-care at home. |
| Instruct patient to avoid persons with infections such as upper respiratory infections. | Altered immune response places patient at risk for infections, especially bacterial pneumonia. |
| Recommend patient avoid cold remedies and decongestants containing ephedrine and large amounts of caffeine. | Those remedies containing vasoconstrictors may decrease peripheral tissue perfusion and cause sludging of sickled cells. |
| Stress the importance of reading labels on over-the counter (OTC) drugs and consulting healthcare provider before consuming any drugs/herbal supplements. | Promotes patient’s sense of control, may avert a crisis. |
| Discuss conditions for which medical attention should be sought: | |
| Symptoms suggestive of sickling in the renal medulla. |
| Dehydration may trigger a vaso-occlusive crisis. |
| May signify a vaso-occlusive crisis due to sickling in the bones or spleen (ischemia or infarction) or onset of osteomyelitis. |
| May reflect angina, impending MI, or pneumonia. |
| Cholelithiasis, primarily with bilirubin stones, is present in more than 50% of adults. |
| Suggestive of infections that may precipitate a vaso-occlusive crisis if dehydration develops. Note: Severe infections are the most frequent cause of aplastic crisis. |
| Assist patient to strengthen coping abilities: deal appropriately with anxiety, get adequate information, use relaxation techniques. | |
| May prevent inappropriate treatment in emergency situations. |
| Screening may identify other family members with sickle cell trait. Hereditary nature of the disease with the possibility of transmitting the mutation may have a bearing on the decision to have children. |
| Provides opportunity to correct misconceptions/present information necessary to make informed decisions. Pregnancy can precipitate a vaso-occlusive crisis because the placenta’s tortuous blood supply and low oxygen tension potentiate sickling, which in turn can lead to fetal hypoxia. |
| Encourage patient to have routine follow-ups: | |
| Monitors changes in blood components; identifies need for changes in treatment regimen. When using hydroxyurea, frequent monitoring of CBC is required because of narrow margin between efficacy (acceptable degree of bone marrow suppression) and toxicity (neutropenia, anemia, thrombocytopenia). |
| Sound oral hygiene limits opportunity for bacterial invasion or sepsis. |
| Detects development of sickle retinopathy with either proliferative or nonproliferative ocular changes. |
| Determine need for career guidance. | A sedentary career may be necessary because of the decreased oxygen-carrying capacity and diminished exercise tolerance. |
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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