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.
- 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
Ineffective Tissue Perfusion
May be related to
- Vaso-occlusive nature of sickling, inflammatory response
- Arteriovenous (AV) shunts in both pulmonary and peripheral circulation
- Myocardial damage from small infarcts, iron deposits, and fibrosis
Possibly evidenced by
- Changes in vital signs: diminished peripheral pulses/capillary refill, general pallor
- Decreased mentation, restlessness
- Angina, palpitations
- Tingling in extremities, intermittent claudication, bone pain
- Transient visual disturbances
- Ulcerations of lower extremities, delayed healing
- Demonstrate improved tissue perfusion as evidenced by stabilized vital signs, strong/palpable peripheral pulses, adequate urine output, absence of pain; usual mentation; normal capillary refill; skin warm/dry; nail beds and lips of natural pale, pink color; absence of paresthesias.
|Carefully monitor vital signs: Assess pulse points for rate, rhythm, and volume. Take note of hypotension, rapid, weak, and thready pulses, and increased or shallow respirations.||Accumulation and sickling in peripheral vessels may lead to complete or partial blockage of a vessel with diminished perfusion to surrounding tissues. Sudden massive splenic sequestration of dead cells can lead to shock.|
|Assess skin for pallor, cyanosis, coolness, diaphoresis, and delayed capillary refill.||Changes reflect diminished circulation and/or hypoxia potentiating capillary occlusion.|
|Monitor and note changes in level of consciousness, reports of headache, dizziness, development of sensory and motor deficits (hemiparesis or paralysis), and seizure activity.||Changes observed may reflect diminished perfusion to the central nervous system (CNS) due to ischemia or infarction. Stagnant cells must be mobilized immediately to prevent further ischemia/infarction.|
|Maintain adequate fluid intake and monitor urine output.||Dehydration causes increase in sickling and occlusion of capillaries other than hypovolemia or decrease in blood volume. Decrease renal perfusion may indicate vascular occlusion.|
|Assess the lower extremities for skin texture, ulcerations, and/or edema.||Sickling of blood can cause reduced peripheral circulation and often leads to dermal changes and delayed healing.|
|Note reports of change in character of pain, or development of bone pain, angina, tingling of extremities, eye pain and disturbances in vision.||Changes may reflect increased sickling of cells and diminished circulation where organs are further involved. Watch out for these signs and symptoms as they may indicate myocardial infarction (MI), pulmonary infarction, or occlusion of vasculature of the eye.|
|Maintain room temperature and body warmth without overheating.||This prevents vasoconstriction therefore aids in maintaining circulation and perfusion. Excessive increase in body temperature may cause diaphoresis which may add to insensible fluid losses and may put the patient in risk for dehydration.|
|Assess client for edema.||Occlusion of blood vessels and circulatory stasis may lead to edema of extremities, potentiating risk of tissue ischemia and necrosis. Note: Assess for edema in the genitals of men including priapism.|
|Monitor laboratory studies:|
||Decreased tissue perfusion may lead to gradual infarction of organ tissues, such as the brain, liver, spleen, kidney, skeletal muscle, and so forth, with consequent release of intracellular enzymes.|
||Electrolyte losses (especially sodium) are increased during crisis because of fever, diarrhea, vomiting, diaphoresis.|
||Hydration lowers the HbS concentration within the red blood cells, which decreases the sickling tendency and blood viscosity, therefore helps in maintaining perfusion. Infusion pump may prevent circulatory overload. Lactated Ringer’s solution or D5W may cause RBC hemolysis and potentiate thrombus formation.|
||Hydroxyurea (Droxia) is a cytotoxic agent and dramatically decreases number of sickle-cell episodes and reduces the severity of complications such as fever and severe chest pain by increasing the level of fetal hemoglobin. Levels greater than 20% may prolong life.Antisickling agents (sodium cyanate) are aimed at prolonging erythrocyte survival and prevents sickling by affecting cell membrane changes.The use of anticoagulants, plasma expanders, nitrates, vasodilators, and alkylating agents has proved essentially unsuccessful in the management of the vaso-occlusive crisis.|
|Assist with surgical diathermy or photocoagulation.||Diathermy is the direct coagulation of bleeding sites in the eye resulting in vascular stasis. This may prevent progression of proliferative changes if initiated early.|
|Assist with needle aspiration of blood from corpora cavernosa.||Priapism can be caused by sickling of blood cells within the penis. It is characterized by sustained and painful erection and edema. Removal of occluded sickled cells can improve circulation, decreasing psychological trauma and risk of necrosis and infection.|
|Surgical intervention.||Direct incision and ligation of the dorsal arteries of the penis and sapheno cavernous shunting may be necessary in severe cases of priapism to prevent tissue necrosis.|
Recommended nursing diagnosis and nursing care plan books and resources.
- 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.
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