6 Sickle Cell Anemia Crisis Nursing Care Plans

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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.

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Here are six (6) nursing care plans (NCP) and nursing diagnosis (NDx) for patients with sickle cell anemia:

  1. Impaired Gas Exchange
  2. Ineffective Tissue Perfusion
  3. Risk for Deficient Fluid Volume
  4. Acute Pain
  5. Risk for Impaired Skin Integrity
  6. Deficient Knowledge
  7. Other Possible Nursing Care Plans
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Deficient Knowledge

Nursing Diagnosis

May be related to

  • Lack of exposure/recall
  • Information misinterpretation
  • Unfamiliarity with resources

Possibly evidenced by

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  • 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 InterventionsRationale
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: 
  • Cold environmental temperatures, failure to dress warmly when engaging in winter activities, wearing tight and restrictive clothing.
Causes peripheral vasoconstriction, which may result in sludging of the circulation, increased sickling, and may precipitate a vaso-occlusive crisis.
  • Stressful situations, strenuous physical activity and contact-type sports, and extremely warm temperatures.
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.
  • Traveling to place more than 7000 ft above sea level or flying in an unpressurized aircraft.
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: 
  • Urine that appears blood tinged or smoky.
Symptoms suggestive of sickling in the renal medulla.
  • Indigestion, persistent vomiting, diarrhea, high fever, excessive thirst.
Dehydration may trigger a vaso-occlusive crisis.
  • Severe joint or bone pain.
May signify a vaso-occlusive crisis due to sickling in the bones or spleen (ischemia or infarction) or onset of osteomyelitis.
  • Severe chest pain, with or without cough.
May reflect angina, impending MI, or pneumonia.
  • Abdominal pain; gastric distress following meals.
Cholelithiasis, primarily with bilirubin stones, is present in more than 50% of adults.
  • Fever, swelling, redness, increasing fatigue/pallor, dizziness, drowsiness, non healing leg ulcers.
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.
  • Suggest wearing a medical alert bracelet or carrying a wallet card.
May prevent inappropriate treatment in emergency situations.
  • Discuss genetic implications of the condition. Encourage SO/family members to seek testing to determine presence of HbS.
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.
  • Explore concerns regarding childbearing and family planning and refer to community resources and obstetrician knowledgeable about sickle cell disease, as indicated.
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: 
  • Periodic laboratory studies: CBC
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).
  • Biannual dental examination.
Sound oral hygiene limits opportunity for bacterial invasion or sepsis.
  • Annual ophthalmologic examination.
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.
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Recommended Resources

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See also

Other recommended site resources for this nursing care plan:

Other care plans for hematologic and lymphatic system disorders:

Matt Vera is a registered nurse with a bachelor of science in nursing since 2009 and is currently working as a full-time writer and editor for Nurseslabs. During his time as a student, he knows how frustrating it is to cram on difficult nursing topics. Finding help online is nearly impossible. His situation drove his passion for helping student nurses by creating content and lectures that are easy to digest. Knowing how valuable nurses are in delivering quality healthcare but limited in number, he wants to educate and inspire nursing students. As a nurse educator since 2010, his goal in Nurseslabs is to simplify the learning process, break down complicated topics, help motivate learners, and look for unique ways of assisting students in mastering core nursing concepts effectively.
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