Sickle Cell Anemia Nursing Care Plans Diagnosis and Interventions
Sickle Cell Anemia NCLEX Review and Nursing Care Plans
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:
- Stroke. Issues in circulation will result to blockages, therefore predisposing the patient to develop thrombolytic strokes
- Acute chest syndrome. This is characterized by chest pain, fever and difficulty breathing requiring emergency medical treatment
- 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
- 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.
- 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.
- Leg ulcers. Poor wound healing and rampant skin breakdown can be observed for patients suffering from sickle cell anemia.
- 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.
- 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.
- 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
- Blood tests – blood samples for assessment of the defective hemoglobin.
- Stroke risk assessment – must be assessed because of the vaso-occlusive nature of the disease
- Ultrasound – using a special ultrasound machine for the early detection of sickle cell anemia
- 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:
- Antimetabolites– used for the control of excess proliferation of RBC’s
- L-glutamine oral powder – used for alleviating pain crises
- P-selectin inhibitors – aides in preventing the clumping of the blood cells (RBC’s, WBC’s, platelets), thereby reducing vaso-oclussivity of the disease
- Analgesics (Opioids or NSAIDs) – used to address the acute and chronic pain associated with the condition
- 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.
- Antibiotics – due to disorders in blood cell productions associated with the disease, patients are more prone to developing severe infections, especially pneumonia.
- Vaccinations. These are given as preventive measures to disease, thereby inhibiting life-threatening infections
- Blood transfusion. This is utilized to address the decreased hemoglobin levels and limit the effects of the disease process
- 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.
Sickle Cell Anemia Nursing Care Plans
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.
|Sickle Cell Anemia Nursing Interventions||Rationales|
|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 ordered||To 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.
|Sickle Cell Anemia Nursing Interventions||Rationales|
|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.|
Nursing Care Plan 3
Nursing Diagnosis: Ineffective Tissue Perfusion related to infarct, fibrosis, and iron deposits due to myocardial damage secondary to sickle cell anemia, as evidenced by abnormal vital signs, resulting in palpitations, delayed capillary refill time and absence of peripheral pulses upon palpation, disorientation to time, place and situation resulting to restlessness and confusion and will show visible skin discoloration, edema, and unhealing wound.
Desired Outcome: The patient will show improvement in tissue perfusion resulting in stable vital signs, capillary refill time that is less than 2 seconds, palpable peripheral pulses, calm pace and properly oriented, normal pinkish color of nail beds and will demonstrate desirable fluid intake and urine output.
|Sickle Cell Anemia Nursing Interventions||Rationale|
|Assess the patient’s vital signs and strictly monitor the pulses, noting the rate, rhythm, and volume. Determine if the patient has hypotension by checking the BP, tachycardia, or weak, and thready pulses. Note also any changes in the respirations.||Accumulation of sickle red blood cells in peripheral vessels can cause a channel to become completely or partially blocked, reducing perfusion to the surrounding tissues. Acute splenic sequestration happens causing the spleen to enlarge and may cause shock.|
|Determine skin temperature by touch, note changes in skin color, assess for excessive sweating, and always check the capillary refill time by pressing on the nail bed and counting the number of seconds until the patient’s normal color returns.||Deviation from normal values can result in decreased oxygen in the tissues and potential blockage of capillaries.|
|Document any changes in the level of consciousness whether the patient is oriented to time, place, and situation. Ask the patient about the severity and characteristic of headache and onset of dizziness if there’s any. Orient the patient at all times.||Changes in mental state may be caused by deviated blood or oxygen perfusion in the CNS due to an obstruction.|
|Monitor and chart the patient’s fluid intake and urine output, to determine if it is adequate or not. If the patient is conscious and coherent, ask the patient to remember how much is the fluid intake and how many times the patient voided.||Other than hypovolemia or a reduction in blood volume, dehydration induces sickling and capillary blockage. Reduced renal perfusion could be a sign of vascular occlusion.|
|Always maintain a comfortable but warm room temperature and warm body temperature without overheating the patient. Allow adequate room sunlight by opening the window curtains.||This helps to maintain circulation and perfusion by preventing vasoconstriction. Excessive body temperature can promote diaphoresis, which can lead to inadvertent fluid loss and put the patient at risk of dehydration.|
|Assess the patient for presence of edema. Measure the depth of the edema and gently press the skin to record time for the skin to go back to its original form.||Blood artery occlusion and circulatory stasis can cause edema in the limbs, increasing the risk of tissue ischemia and necrosis. Note: Checking men’s genitals for edema as well as priapism is highly advisable.|
|Check for pain and assess for its characteristics such as location, and severity. Inform the patient to tell the staff if he/she has any bone or chest pain.||Increasing pain especially on the chest region or bones may indicate elevation in the number of sickle cells.|
|Hydrate the patient by infusing hypo-osmolar solutions such as 0.45% saline through an intravenous line using a calibrated infusion pump, as prescribed.||To lower the HbS concentration inside the red blood cells, hydration with hypo-osmolar solution is recommended. This can reduce the tendency of sickling, as well as help in decreasing blood viscosity while keeping effective perfusion.|
|Monitor the lower extremities for any formation of ulcerations or changes in skin texture. If edema forms, elevate the legs when patient is on bed or sitting on a chair.||Decrease in peripheral circulation may result from the sickling of cells in patients with sickle cell anemia. Dermal changes may occur. Ulcerations may exhibit delayed healing.|
Nursing Care Plan 4
Nursing Diagnosis: Acute Pain related to the blockage of sickled cells into small blood vessels which transport blood and oxygen as evidenced by localized bone and joint pains as evidenced by a decrease in range of motion and immobility, recurrent abdominal pain, low back pain, and transient headache.
Desired Outcome: The patient will demonstrate knowledge in alleviating pain with effective pain relief measures and exercises that minimize the use of pain relief medications, will frequently perform routine range of motion exercises and be able to move freely, will have adequate rest, and relaxation and will verbalize relief from pain.
|Sickle Cell Anemia Nursing Interventions||Rationale|
|Perform a comprehensive pain assessment upon receiving the patient. Assess for the characteristics of pain such as location, onset, frequency, duration, quality, and severity of pain.||Obstruction of sickled cells may cause a lack of oxygen in the cells that may lead to infarction of tissues and result in pain. Usually, the patient may experience pain in the extremities, back, and ribs.|
|Observe the patient for nonverbal signs of pain including facial grimace, inadequate posture, gait disturbance, guarding behavior, and hesitations to move. Also note physiological signs including increased blood pressure level, rapid heart rate or respiratory rate.||Because each patient’s pain is different, nonverbal indicators may help with pain evaluation and therapeutic success. Because of the diverse perceptions of pain, different descriptions may be encountered with each patient.|
|Discuss with the patient and significant other what pain relief measures were performed in the past and note the effective ones. Advise the patient that it will be best to repeat the effective exercises done before.||This aids in determining individualized therapy requirements. The significant other should help the patient in performing the significant pain relief measures.|
|Create a plan and educate the patient and the significant other on alternative pain alleviation techniques such as meditation, yoga, breathing techniques, music therapy, biofeedback, and therapeutic massage.||The use of cognitive-behavioral pain treatment may lessen the need for pain relief medicines. Thus, it will lessen undesirable side effects. This will also improve the patient’s sensation of control.|
|Demonstrate to significant other how to perform a gentle massage on the affected area or area with pain.||A gentle massage on the affected area helps in reducing muscle tension.|
|Encourage and teach the patient how to perform a range of motion exercises such as rotation of extremities, adduction, abduction, and flexion.||ROM exercises will help in preventing joint stiffness and the possible formation of bone contractures.|
|Always monitor the patient for proper hydration by encouraging adequate fluid intake and eating a balanced diet.||Dehydration will cause more pain and increase vaso-occlusion.|
|Educate the patient about the benefits of applying warm or moist compresses to the affected joints and other painful locations. Instruct that ice or cold compresses should be avoided.||8. Warmth enhances circulation to hypoxic zones by causing vasodilation. The cold causes vasoconstriction, which worsens the pain situation.|
Nursing Care Plan 5
Nursing Diagnosis: Impaired Skin Integrity related to vaso-occlusion and venous stasis secondary to sickle cell anemia, as evidenced by the loss of sensation and decreased physical movement as evidenced by presence of a non-healing stage 2 pressure ulcer.
Desired Outcome: The patient’s pressure ulcer will have optimal healing and will be able to prevent further dermal ischemic injury and the patient will demonstrate knowledge in performing measures on how to reduce skin breakdown such as proper turning, use of protectors, and early detection.
|Sickle Cell Anemia Nursing Interventions||Rationale|
|Educate the client about turning and repositioning. Explain the importance of turning and repositioning in bed or chair every 2 hours.||Reduces the risk of tissue ischemia by preventing prolonged tissue pressure in areas where circulation is already weakened.|
|Carefully and regularly inspect skin and assess pressure points for redness, warmth and swelling. Perform massage to affected areas if necessary.||Skin deterioration can be accelerated by poor circulation.|
|Always check and protect bony prominences with the use of soft cushions and protectors for the heel and elbow.||Soft cushions and protectors on bony prominences reduce tissue pressure and prevent skin deterioration.|
|Educate the patient on how to pat dry and clean skin surfaces with a soft cotton towel and always keep the linens such as bed sheets or under sheets dry and wrinkle-free.||The proliferation of harmful organisms thrives in moist environments and wrinkles on bed may cause skin breakdown.|
|Note the size, shape and other characteristics of ischemic areas, cuts, and bruises. Monitor closely for pressure ulcer formation.||Pathogenic organisms may be able to enter through these points. This increases the danger of infection and delays recovery in the case of a weakened immune system.|
|Demonstrate to the client and explain the importance of always cleaning the open wound ulcers as indicated with hydrogen peroxide or povidone iodine solutions. Document the characteristics of any drainage if present.||The effectiveness of therapies and the condition of tissue perfusion determine whether healing is improved or delayed. Because they have weaker resistance to infection and less nutrients for healing, these individuals are at a higher risk for severe outcomes.|
More Sickle Cell Anemia Nursing Diagnosis
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
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