NUR 2214 Study Guide Summer 2020 Exam 3 Latest 2023 /2024 Sickle Cell Anemia • Definition/Pathophysiology
- The clinical features of SCA are primarily the result of
- Pain is most common symptom
- Cardiovascular changes – risk for high cardiac output, SOB, general
- Respiratory changes – occur over time – usually pts develop pulmonary
▪ (1) obstruction caused by the sickled RBCs with other cells ▪ (2) vascular inflammation ▪ (3) Increased RBC destruction,****** abnormal adhesion, entanglement, and enmeshing of rigid sickle-shaped cells accompanied by the inflammatory process intermittently blocks the microcirculation causing vaso-occlusion.• The resultant absence of blood flow to adjacent tissues causes local hypoxia, leading to tissue ischemia and infarction (cellular death).• Pathologic changes
fatigue, increase jugular venous distension, increase HR, low to normal BP
hypertension, and all are at risk for recurrent pneumonia ▪ Acute chest syndrome is a common life-threatening condition that is usually associated with respiratory infection and can also be caused by fat embolism and pulmonary debris from sickled
cells. Symptoms are similar to pneumonia with:
• Cough, shortness of breath, abnormal breath sounds, and an infiltrate on chest x-ray, Chest pain, fever, cough, tachypnea, wheezing, and hypoxia • Fever may or may not be present.• Without intervention, this complication can lead to respiratory failure and failure of all other organ systems.
- Abdominal changes - damage to the spleen and liver, pain from
reduced PERFUSION is diffuse and steady, also involving the back and legs.▪ The liver or spleen may feel firm and enlarged with a nodular or “lumpy” texture in later stages of the disease. 1 / 4
- Musculoskeletal changes occur because arms and legs are often sites
- Central nervous system (CNS) –
- The clumped masses of sickled RBCs block blood flow and PERFUSION
of blood vessel occlusion.▪ Joints may be damaged from hypoxic episodes and have necrotic degeneration.
▪ During crises, patients may have a low-grade fever.▪ Long-term effects of reduced PERFUSION to the CNS may result in infarcts with repeated episodes of hypoxia, causing the patient to have seizures or symptoms of a stroke • Vaso occlusive crisis
leading to further tissue hypoxemia (reduced oxygen supply) and more sickle-shaped cells, which then leads to more blood vessel obstruction, inadequate perfusion, and ischemia in the affected tissues
▪ Manifestations:
• Fever, swelling of hands, feet, and joints, abdominal pain • Acute Exacerbation • Caused by hypoxia, exercise, high altitude, fever (temp extremes) • Pallor • Fatigue • Severe Pain (Due to micro-occlusions) • Client will have low RBCs.• WBC (leukocytes and neutrophils) will not be abnormal from the sickle cell crisis, neither will the platelets, which are used for clotting.
- Sequestration crisis
- Aplastic crisis
- Hyperhemolytic crisis
▪ A pooling of a large amount of blood usually in the spleen and infrequently in the liver that causes a decreased blood volume and ultimately shock.
▪ Diminished RBC production usually triggered by viral infection that may result in profound anemia.
▪ An accelerated rate of RBC destruction characterized by anemia, jaundice, and reticulocytosis • Precipitating factors for a crisis. 2 / 4
o DEHYDRATION: Encouraging fluids and hydration of the client
will help to prevent sickling of the cells. Because clients in sickle cell crisis are often dehydrated, the fluid of choice is a hypotonic solution such as 0.45% normal saline.
- High Altitude
- Extreme stress
- Pregnancy
- Vigorous activities
- Rest to minimize energy expenditure and to improve oxygen utilization
- Rehydration through oral and IV therapy (No caffeine)
- Electrolyte replacement because hypoxia results in metabolic acidosis,
- Analgesia for the severe pain from vaso-occlusion
- Blood replacement to treat anemia and to reduce the viscosity of the
- Antibiotics to treat any existing infection
- Administer oxygen.(Remember ABC’s)
- Remove any constrictive clothing.
- Encourage the patient to keep extremities extended to promote
- Do not raise the knee position of the bed. Avoid flexing knees and hips
- Elevate the head of the bed no more than 30 degrees.
- Keep room temperature at or above 72° F (22.2° C).
- Avoid taking blood pressure with external cuff.
- Blood transfusions as ordered, be cautious to prevent iron overload
• Nursing care during a crisis
which also promotes sickling
sickled blood
venous return.
o Check circulation in extremities every hour:
▪ Pulse oximetry of fingers and toes ▪ Capillary refill ▪ Peripheral pulses ▪ Toe temperature • Prevention of crisis
- Interventions focus on preventing infection, controlling infection, and
- Drink at least 3 to 4 liters of liquids every day.
- Avoid alcoholic beverages and smoking cigarettes or using tobacco in
- Contact your primary health care provider at the first sign of illness
starting drug therapy early when infection is present.
any form.
or infection. 3 / 4
- Be sure to get a “flu shot” every year.
- Ask your primary health care provider about taking the pneumonia
- Avoid temperature extremes of hot or cold.
- Be sure to wear socks and gloves when going outside on cold days.
- Avoid planes with unpressurized passenger cabins.
- Avoid travel to high altitudes (e.g., cities such as Denver and Santa
- Ensure that any health care professional who takes care of you knows
- Consider genetic counseling.
- Avoid strenuous physical activities.
- Engage in mild, low-impact exercise at least 3 times a week when you
- Take all antibiotics as prescribed.
- Repot fever of 101.3 or higher to you PCP
- Report to PCP if S&S of a cold occur
- Wearing sunscreen even when cloudy
- Vaccination (HPV)
- Avoid sun from 10-2
- No tanning beds
- No tobacco uses
- Asbestos
- Avoid carcinogens
- Use PPE in workplace
- Modify behaviors that can cause cancer
- Change in bowels or bowel habits
- A sore that doesn’t heal***
- Unusual bleeding or discharge***
- Thickening or lump in breast or elsewhere
- / 4
vaccine.
Fe).
that you have sickle cell disease, especially the anesthesia provider and radiologist.
are not in crisis.• Pt Teaching***
Cancer • Primary prevention
▪ Limit intake of alcohol no more than 1 ounce per day ▪ Include more fruits and veggies and whole grains in diet (Fiber) ▪ Limit number of sexual partners and safe sex practices • Seven warning signs of cancer (CAUTION UP)