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
May be related to
- Intravascular sickling with localized stasis, occlusion, and infarction/necrosis
- Activation of pain fibers due to deprivation of oxygen and nutrients, accumulation of noxious metabolites
Possibly evidenced by
- Localized, migratory, or more generalized pain, described as throbbing, gnawing, or severe and incapacitating; affecting peripheral extremities, bones, joints, back, abdomen, or head (headaches recurrent/transient)
- Decreased ROM, guarding of the affected areas
- Facial grimacing, narrowed/self-focus
- Patient shall verbalize relief or control of pain.
- Demonstrate relaxed body posture, have freedom of movement, be able to sleep/rest appropriately.
|Assess for pain. Note location, duration, and intensity (scale of 0-10).||Sickling of cells potentiates cellular hypoxia and may lead to infarction of tissues with resultant pain. Pain usually occurs in the back, ribs, and limbs and may last for days.|
|Observe nonverbal pain cues: gait disturbances, positioning of the body, guarding behavior, facial grimacing, reluctance to move, and physiological manifestations of acute pain (increased BP, tachycardia, increased RR).||Nonverbal cues may aid in evaluation of pain and effectiveness of therapy since pain is unique to each patient. One may encounter varying description because of individualized perceptions.|
|Discuss what pain relief measures were effective in the past. Involve the patient and SO in care.||Involve the patient or SO in care to allow for identification of effective remedies that have been found to relieve pain. This helps in establishing individualized treatment needs.|
|Teach and discuss alternative pain relief measures: relaxation techniques, biofeedback, yoga, meditation, progressive relaxation techniques, distraction techniques, guided imagery and breathing techniques.||Cognitive-behavioral pain management may reduce reliance on pharmacological means of pain control. This also enhances the patient’s sense of control.|
|Provide support and carefully position affected extremities.||To reduce edema, discomfort, and risk of injury, especially if osteomyelitis is present.|
|Massage gently affected areas.||Helps reduce muscle tension.|
|Encourage ROM exercises.||Prevents joint stiffness and possible contracture formation.|
|Plan activities during peak analgesic effect.||Maximizes movement of joints, enhancing mobility.|
|Maintain adequate fluid intake.||Dehydration increases sickling/vaso-occlusion and corresponding pain.|
|Apply warm, moist compresses to affected joints and other painful areas. Avoid use of ice or cold compresses.||Warmth causes vasodilation and increases circulation to hypoxic areas. Cold causes vasoconstriction and compounds the crisis.|
|Administer medications as indicated:|
||Reduces pain and promotes rest and comfort. Note: Narcotics are the mainstay of pain control during crisis.|
||Acetaminophen (Tylenol) can be used for control of headache, pain, and fever. Aspirin should be avoided because it alters blood pH and can make cells sickle more easily.|
||Meperidine (Demerol) should not be used because its metabolite, normeperidine, can cause CNS excitation.|
|Administer and monitor RBC transfusions.||Frequency of painful sickle-cell crises may be reduced by routine partial exchange transfusions to maintain the population of normal RBCs.|
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