Sickle Cell Anemia

Sickle Cell Anemia Nursing Diagnosis Interventions and Care Plans

Sickle Cell Anemia NCLEX Review Care Plans

Nursing Study Guide on Sickle Cell Anemia

Sickle cell anemia is a congenital blood disorder characterized by irregularly shaped red blood cells, commonly crescent and/or “sickles” in shape.

These asymmetrical cells get stuck on small blood vessels which can slow down and even block blood flow and oxygen supply throughout the body.

There is no cure for sickle cell anemia yet; however, a variety of treatments are available to control pain and to mitigate complications of the disease.

Signs and Symptoms of Sickle Cell Anemia

Anemia – The normal life span of red blood cells is up to 120 days before being replaced. In the case of sickle cell anemia, the cells die within 10 to 20 days.

This shortened life span, of the oxygen-carrying erythrocytes, results in decreased cell count and thus depriving the body of oxygen and causing fatigue.

Pain – Patients with sickle cell anemia experience acute pain called pain crisis.

This symptom is brought about by the abnormal erythrocytes blocking the blood flow of the tiny vessels on the chest, abdomen and joints, thereby causing pain.

This episodic pain varies in severity and may last from a few hours to a few weeks, sometimes prompting hospital admissions for the most painful.

Swelling of Hands and feet – The irregularly-shaped cells impede the natural processes of circulation by clumping on the blood vessels of the body.

This blockage of blood flow thereby causes swelling of the lower extremities, particularly of the hands and feet.

Infections – One of the spleen’s functions is to filter the blood of old and damaged erythrocytes.

Due to the rapid deterioration of the abnormal red blood cells in Sickle cell anemia, the spleen is overwhelmed, thereby causing damage to the organ.

This in turn will inhibit another function of the spleen, which is the production the infection-fighting leukocytes.

Delayed Growth or Puberty – Problems in circulation cause oxygen and other nutrients to be impeded for use in the body.

This in turn causes delays in growth for young children and late onset of puberty in adolescents.

Vision problems – The retina is primarily responsible for the processing of images seen by the eye.

But due to the clumping of the abnormal cells in the blood vessels of the retina, its functions are impeded and may cause vision problems. 

Causes and Risk Factors of Sickle Cell Anemia

In sickle cell anemia, the gene responsible for the binding of hemoglobin to red blood cells is affected. This mutation causes for the red blood cells to act abnormally to the altered hemoglobin by becoming inelastic and malformed.

This in turn limits the erythrocytes capacity to carry oxygen effectively all throughout the body.

Considering the genetic repercussions, it takes both the mother and father to have the defective gene for Sickle cell anemia to develop.

If only one parent has the malfunctioning gene, their offspring may both carry the normal and defective sickle cell hemoglobin.

Meaning, the patient will be carrying the abnormal gene but may not develop Sickle cell anemia.

However, he/she will still pass on the sickle cell trait to his/her children.

Patients with sub-Saharan African family backgrounds are more susceptible in developing the sickle cell trait.

Other ethnicities at risk for sickle cell disease are descendants coming from South America, Cuba, Central America, Saudi Arabia, India, Turkey, Greece, and Italy.

Complications of Sickle Cell Anemia

The following are the complications of sickle cell anemia but are not limited to:

  1. Stroke. Issues in circulation will result to blockages, therefore predisposing the patient to develop thrombolytic strokes
  2. Acute chest syndrome. This is characterized by chest pain, fever and difficulty breathing requiring emergency medical treatment
  3. Pulmonary hypertension. This type of anemia can cause build-up of unnecessary lung pressure due to problems with circulation as a result of erythrocyte clumping
  4. Organ damage. Due to the chronic inability of the red blood cells to provide essential oxygen for normal organ function, patients with sickle cell anemia may develop organ failure, which can be fatal.
  5. Blindness. One of the potential complications of having abnormal red blood cells circulating in the body is damage to smaller blood vessels, particularly the eye. This in turn will cause eye damage and eventually blindness.
  6. Leg ulcers. Poor wound healing and rampant skin breakdown can be observed for patients suffering from sickle cell anemia.
  7. Gallstones. The build of bilirubin caused by the metabolism of the abnormal erythrocytes will result to gall stones that will block the flow of bile.
  8. Priapism. This is a condition wherein men with Sickle cell anemia will present with painful and long-lasting erections due to the blockages of the tiny blood vessels of the penis.
  9. Pregnancy complications. Sickle cell anemia increases the risk of high blood pressure and the presence of clots that will impede with the normal development of the fetus

Diagnosis of Sickle Cell Anemia

  1. Blood tests – blood samples for assessment of the defective hemoglobin.
  2. Stroke risk assessment – must be assessed because of the vaso-occlusive nature of the disease
  3. Ultrasound – using a special ultrasound machine for the early detection of sickle cell anemia
  4. Newborn screening – includes testing the baby for sickle cell anemia; this can be done early on, in-vitro, wherein amniotic fluid is obtained in the womb. Once detected, referral to a genetic counselor is warranted for added management of the disease.

Treatment for Sickle Cell Anemia

            The treatment of sickle cell anemia involves control of the signs and symptoms of the condition.

Medications: a variety of medications are utilized and they are:

  1. Antimetabolites– used for the control of excess proliferation of RBC’s
  2. L-glutamine oral powder – used for alleviating pain crises
  3. P-selectin inhibitors – aides in preventing the clumping of the blood cells (RBC’s, WBC’s, platelets), thereby reducing vaso-oclussivity of the disease
  4. Analgesics (Opioids or NSAIDs) – used to address the acute and chronic pain associated with the condition
  5. Hemoglobin oxygen-affinity modulators – inhibits RBC conversion to sickle RBC’s and improves RBC deformability and in turn, reduces thickening of blood, often associated with the illness.
  6. Antibiotics – due to disorders in blood cell productions associated with the disease, patients are more prone to developing severe infections, especially pneumonia.
  7. Vaccinations. These are given as preventive measures to disease, thereby inhibiting life-threatening infections
  8. Blood transfusion. This is utilized to address the decreased hemoglobin levels and limit the effects of the disease process
  9. Stem cell transplant/Bone marrow transplant. Transplantation involves the elimination of latent blood cells in the marrow and replacing it with the donated healthy cells. 

Nursing Care Plans for Sickle Cell Anemia

Nursing Care Plan 1

Nursing Diagnosis: Impaired Gas Exchange related to decreased oxygen-carrying capacity of the blood and abnormal RBC structure life span secondary to sickle cell anemia, as evidenced by shortness of breath, oxygen saturation of 82%, mild confusion(GCS 14), use of accessory muscles, cyanosis of the lips, heart rate of 122 bpm, restlessness, and reduced activity tolerance

Desired Outcome: The patient will demonstrate adequate oxygenation as evidenced by normal heart rate, easy of breathing, GCS 15, absence of restlessness, and oxygen saturation within the target range set by the physician.

InterventionsRationales
Assess the patient’s vital signs, especially the respiratory rate and depth, as well as the use of accessory muscles. Assess the heart rate.To create a baseline set of observations for the patient, and to monitor any changes in the vital signs as the patient receives medical treatment.
Monitor the color of skin and mucous membrane.Peripheral cyanosis (bluish discoloration of the skin, ear lobes, or nail beds) may be evident with hypoxemia. Central cyanosis involving the mucosa may indicate further reduction of oxygen levels.
Assess the level of consciousness every hour using Glasgow coma scale (GCS).Decline in level of consciousness indicate worsening of hypoxia.
Provide humidified oxygen as prescribed.To increase oxygen levels while aiming to reduce the risk of drying out the lungs.
Reposition the patient by elevating the head of the bed and encouraging him/her to sit on an upright position. Encourage pursed lip breathing and deep breathing exercises.To improve the delivery of oxygen in the airways and to reduce shortness of breath and risk for airway collapse.
Refer the patient to a chest physiotherapist.Patients with sickle cell anemia are prone to respiratory infections such as pneumonia., To enable the patient to receive more information and specialized care in the removal of lung secretions and enabling of improved gas exchange.
Administer packed red blood cells as orderedTo maintain adequate oxygen supply by delivering healthy red blood cells via transfusion.

Nursing Care Plan 2

Nursing Diagnosis: Risk for Deficient Fluid Volume Deficit

Desired Outcome: The patient will be able to maintain fluid balance in terms of input and output.

InterventionsRationales
Assess vital signs, particularly blood pressure level.Sickle cell anemia may cause malaise and anorexia, which can lead to decreased oral fluid intake. This may lower blood pressure levels and put the patient at risk for hypotensive episodes that lead to shock.
Commence a fluid balance chart, monitoring the input and output of the patient. Include episodes of vomiting, gastric suctioning, and other gastric losses in the I/O charting.To monitor patient’s fluid volume accurately.
Start intravenous therapy as prescribed. Electrolytes may need to be replaced intravenously.     Encourage oral fluid intake of at least 2000 mL per day if not contraindicated.To replenish the fluids and electrolytes lost from vomiting or other gastric losses, and to promote better blood circulation around the body.
Educate the patient (or guardian) on how to fill out a fluid balance chart at bedside.To help the patient or the guardian take ownership of the patient’s care, encouraging them to drink more fluids as needed, or report any changes to the nursing team. 
Administer blood transfusion as prescribed.To increase the hemoglobin level and manage sickle cell anemia.

Other Nursing Diagnoses:

  • Ineffective Tissue Perfusion
  • Acute Pain
  • Fatigue

Nursing References

Ackley, B. J., Ladwig, G. B., Makic, M. B., Martinez-Kratz, M. R., & Zanotti, M. (2020). Nursing diagnoses handbook: An evidence-based guide to planning care. St. Louis, MO: Elsevier.  Buy on Amazon

Gulanick, M., & Myers, J. L. (2017). Nursing care plans: Diagnoses, interventions, & outcomes. St. Louis, MO: Elsevier. Buy on Amazon

Ignatavicius, D. D., Workman, M. L., Rebar, C. R., & Heimgartner, N. M. (2018). Medical-surgical nursing: Concepts for interprofessional collaborative care. St. Louis, MO: Elsevier.  Buy on Amazon

Silvestri, L. A. (2020). Saunders comprehensive review for the NCLEX-RN examination. St. Louis, MO: Elsevier.  Buy on Amazon

Disclaimer:

Please follow your facilities guidelines and policies and procedures.

The medical information on this site is provided as an information resource only and is not to be used or relied on for any diagnostic or treatment purposes.

This information is not intended to be nursing education and should not be used as a substitute for professional diagnosis and treatment.

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