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Sickle Cell Anemia

Nurse 211
STUDY
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Sickle Cell Disorder is a hereditary _______________
Hemoglobinopathy
Sickle cell is characterized by
replacement of normal hemoglobin wtih abnormal hemoglobin S in RBCs
Is sickle cell classified as chronic or acute
Chronic Hemolytics anemia
What is sickling?
when the percentage of hemoglobin (Hgb) replaced by HgbS increases
What happens during episodes of Sickling?
Rbcs become abnormally crescent shaped, causing occlusions of small blood vessels, ischemia, and damage to affected organs.
What can occur with repeated episodes of sickling and unsickling
Weakens the cell membranes of RBCs
What occurs with weakened rbcs?
they are hemolyzed and removed
Because of RBCs are misshaped, what happens to the circulation?
Abnormally shaped red blood cells are unable to circulate freely through the blood vessels and become stuck in small vessels, particularly in capillaries forming occlusions with the end result of ischemia because the tissues cannot receive oxygen and nutrients they need.
repeated or prolonged ischemia resulting form sickle cell induced occlusions cause__________
damage to the tissues and organs throughout the body, eventually resulting in scarring and impaired function
What is the infection rate for sickle cell anemia?
High r/t impaired immunity
What can occur with children with sickle cell anemia?
developmental delay, mental retardation, and other neurological outcomes.
What triggers sickling?
hypoxia
dehydration
infection
venous stasis
pregnancy
alcohol
high altitudes
fever
emotional or physical stress
acidosis
strenuous exercise
anesthesia
Define Sickle Cell Crisis?
the term used to describe periods when the percentage of hgb S increases resulting in the appearance of symptoms, often marked by acute pain resulting from ischemia.
Precipitating factors for sickle cell crisis?
increased blood viscosity
low fluid intake/dehydration
fever
hypoxia
low oxygen tension
infection
trauma
How can sickled cells resume a normal shape?
re-hydration and re-oxygenation; however, membranes are more fragile and cell life is shortened to 10-20 days
Etiology of sickle cell anemia?
genetic autosomal recessive with two allele mutations
Sickle Cell Trait
only one allele mutation
Client is a carrier (can only pass the trait)
Manifestations are mild even under severe triggers
Risk Factors?
1 in 100 Latinos
Mediterranean
African American's have sickle cell trait; they remain asymptomatic unless stressed by severe hypoxia
Triggers?
Hypoxia
Low environmental temperature
low body temperature
excessive exercise
anesthesia
dehydration
infection
acidosis
Manifestations of sickle cell? arise from?
RBC sickling
general manifestations?
Pallor
Fatigue
Jaundice
Irritability
Extensive sickling can precipitate what?
a crisis as result of occluded circulation, impaired erythropoiesis, or sequestration or large amounts of blood in the liver or spleen.
Vasoocculusive or thrombotic crisis occurs when?
when sickling develops in the microcirculation
obstruction of blood flow by sickled cells that cannot pass through the vessels triggers _________
vasospasm that halts all blood flow
Lack of blood flow leads to:
tissue ischemia
infarction
Pain
Large joints are affected
abdominal pain may signal infarction or abdominal organs and structures. infarction also may affect bone marrow or lead to aseptic necrosis of affected bones resulting in pain from avascular necrosis of the bone marrow
stroke may result from cerebral vessel occlusion
skin ulcers may develop as the result of occluded vessels supplying the dermis
Cardiovascular changes
SOB
fatigue
weakness
Compare: pulses, temp, cap refill all extremities.
HR may be rapid
BP maybe low,
Decreased pulse pressure.
Skin Changes
pallor
cyanosis (decr oxygenation)
Assessment: check lips, tongue, nail beds, conjunctivae, palms, soles of feet q 8 hr (gray blue tinge)
Jaundice r/t bilirubin from damaged RBC
OPEN ULCERS on lower legs, ankles, necrotic, infected
Abdominal Changes
hepatomegaly
splenomegaly
(blood trapping)
Gallstones
CNS Changes
low grade fever
seizures
manifestations of stroke.
assess gait, hand grasps, coordination.
Collaboration
Neonatal screening
early intervention
prophylactic antibiotics
Diagnostic Tests
Newborns: testing cord blood using hemoglobin electrophoresis
Sickledex may be used for quick screening in children older than 6 months
Serum analysis of blood reveals teh degree of anemia, with hemoglobin of 6-10g/dl
Lab Assessment
TRAIT: 40% Hbs
DISEASE: 80-100% HbS
HCT: low (20%-30%)
RETICULOCYTE: high = anemia long duration
BILIRUBIN: high
WBC: high d/t chronic inflammation, hypoxia, ischemia
Pain Control
parenteral analgesics are generally administered around the clock

pain medications should not be ordered prn

Complementary: (not alone, must use pain meds) warm temp, distraction, relaxation, positioning, aroma, therapeutic touch, warmsoaks and compresses
Hydration
Oral and IV fluid replacement promotes pain relief, since dehydration is often a cause of crisis

Fluids reduce the viscosity of the blood, so adequate hydration is essential
Oxygenation
administered to provide comfort and decrease the incidence of pulmonary complications
Prevention and treatment of infection
children who are functionally asplenic or have had a splenectomy have a resultant decreased capability to fight infection
Transfusion of RBCs
improved blood and tissue oxygenation
reduction in sickling
temporary suppression of the production of RBCs containing hgbS
Complications of transfusion
iron is stored in tissues and organs because the body has no way to excrete it
Assessment
HX of past crisis
precipitating events
medical treatmetn
home management
weigh/height (failure to thrive is common) so compare with past
use pain scale and identify pain perception in each body part
assess pain management protocol

multisystem assessment:
fever
neurologic changes
respiratory symptoms are emergency condition that necessitate prompt treatment

monitor for shock, hypotension, changes in LOC, dizziness, lightheadedness, increased capillary refill
Nursing Dx
risk forimpaired tissue perfusion (cerebral)
Acute Pain
Caregiver role Strain
Interrupted family process
delayed growth and development
Impaired physical mobility
Plan
client will experience reducing complications
caregiver will provide support and assistance
client will meet growth and development needs
client will optimize physical mobility as tolerated
Treatment
Pain Control
Hydration
Oxygenation
Preventino and treatment of infections
Transfusion
Hydroxurea
Stem cell Transplant
Hydroxyurea
Decreased abnormal cell production results in decreased pain
Complications
Hand-Foot Syndrome(Swollen hands and feet may be the first signs of sickle cell anemia)
Splenic Crisis ( spleen traps to much rbcs)
Infections (decreased immune function)
Acute Chest Syndrome: like pneumonia, sickle cells get trapped in lungs resulting in decreased O2 levels
Pulmonary HTN
Delayed Growth and puberty
Stroke
eye problems
priapism ( persistent and usually painful long-lasting erection)
Gallstones
Ulcers on the legs
Multiple organ failure
Fever
Tachycardia
LOC
A patient with sickle cell anemia has a____ hematocrit?
LOW
A newly admitted client has sickle cell crisis. The nurse is planning care based on assessment of the client. The client is complaining of severe pain in his feet and hands. The pulse oximetry is 89. Which of the following interventions would be implemented first? Assume that there are orders for each intervention.
a. Adjust the room temperature
b. Give a bolus of IV fluids
c. Start O2
d. Administer meperidine (Demerol) 75mg IV push
C
A 43-year-old African American male is admitted with sickle cell anemia. The nurse plans to assess circulation in the lower extremities every 2 hours. Which of the following outcome criteria would the nurse use?
a. Body temperature of 99°F or less
b. Toes moved in active range of motion
c. Sensation reported when soles of feet are touched d. Capillary refill of < 3 seconds
D
A 25-year-old male is admitted in sickle cell crisis. Which of the following interventions would be of highest priority for this client?
a. Taking hourly blood pressures with mechanical cuff b. Encouraging fluid intake of at least 200mL per hour c. Position in high Fowler's with knee gatch raised
d. Administering Tylenol as ordered
B
A 14 year old girl has been hospitalized with Sickle Cell Anemia in vasoocclusive crisis. Which of these Nursing diagnoses should receive priority in the Nursing plan of care? --
A. Impaired social interaction --
B. Alteration in body image --
C. Pain --
D. Alteration in tissue perfusion
D
Which of the following foods would the nurse encourage the client in sickle cell crisis to eat? --
A. Peaches --
B. Cottage cheese --
C. Popsicles --
D. Lima beans
C
The male client with sickle cell anemia comes to the emergency room with a temperature of 101.4 F and tells the nurse that he is having a sickle cell crisis. Which diagnostic test should the nurse anticipate the emergency room doctor ordering for the client? --
A. Spinal tap. --
B. Hemoglobin electrophoresis. --
C. Sickle-turbidity test (Sickledex). --
D. Blood cultures.
D
A Client in crisis should:
1. Management of Pain
2. Administration of oxygen
3. Promoting Hydration to decrease blood viscocity
4. Monitor for
The nurse is teaching a group of parents whose children have sickle cell anemia. When a parent asks the cause of the symptoms, the nurse responds with which of the following?
"Sickled cells clump in the smaller blood vessels and obstruct blood flow."

Rationale:
All the symptoms of sickle cell are a result of the clumping of the sickled cells in the microvasculature, causing obstruction of blood flow. The other statements are inaccurate.
A child with suspected sickle cell disease (SCD) is in the clinic for laboratory studies. The parents ask the nurse what results will tell the physician that their child has SCD. The nurse responds that which of the following is increased in this disease?
Reticulocyte count

Rationale:
The reticulocyte count will be increased because the life span of sickled red blood cells is shortened. Hemoglobin, hematocrit, and platelet levels will be decreased.
A pregnant woman tells the nurse that there is a history of sickle cell disease in her family and she is afraid that the baby will have the disease. The nurse provides the client with which of the following information?
Both the mother and father must carry the gene for the baby to be affected.

Rationale:
Sickle cell is inherited as an autosomal recessive disorder. Both parents must carry the gene for the baby to be affected. The other statements are inaccurate.
The nurse is caring for a child who is in the hospital experiencing sickle cell crisis. The parents are asking the nurse which treatment will help cure the child. The nurse responds with which of the following?
Treatment is aimed at pain control, oxygen therapy, and hydration, but does not provide a cure.

Rationale:
Treatment for sickle cell crisis is pain control, oxygenation, and fluid resuscitation. There is no cure for sickle cell disease. The nurse teaches families how to prevent sickle cell crisis.
The nurse is admitting a 7-year-old client who is experiencing sickle cell crisis and plans care based on which of the following nursing diagnoses?
Delayed Growth and Development

Rationale:
The child with sickle cell disease is often developmentally delayed due to the effects of physical disability, pain, and inpatient hospital stays. The nurse would plan activities that help maintain developmental levels the child has reached. The child in sickle cell crisis does not experience ineffective airway clearance, bleeding, or constipation as a result of sickle cell disease. The child may have an illness that could cause one of these symptoms, but they are not common to children with sickle cell disease.
The 24-year old African American female client tells the nurse she has a brother with sickle cell disease. She is engage to be married and is concerned about giving this disease to her future children. which information is most important to provide too the client?
A. tell the client that she won't pass this on if she has never had symptoms
B. Encourage the client to discuss this concern with her fiance
C. Recommend that she and her fiance see a genetic counselor
D. Discuss the possibility of adopting children after she gets married.
C
The nurse is caring for a client in a sickle cell crisis. which is the pain regiment of choice to relieve the pain?
1. Frequent aspirin
2. Motrin
3. Demerol
4. Morphine
4. Morphine
the student nurse asks the nurse, "what is sickle cell anemia?" which statement by the nurse would be the best answer to the student's question?
1. There is some written material at the desk
2. it is a congenital disease of the blood
3. the client has decreased synovial fluid
4. the blood becomes thick when the client is deprived of oxygen
4. sickle cell anemia is a disorder of the client rbcs characterized by abnormally shaped red cells that sickle or clump together, leading to oxygen deprivation and resulting in crisis and severe pain.
the client's nephew has just been diagnosed with sickle cell anemia. the client asks the nurse, "how did my nephew get this disease? which statement would be the best response by the nurse?
1. sickle cell is an inherited autosomal recessive disorder
2. he was born with it and both his parents were carriers of the disease
3. at this time, the cause is unknown
4. your sister was exposed to a virus
2. explained in layman's terms
the client diagnosed with sickle cell anemia comes to the ED c/o joint pain throughout the body. Oral temp 102.4, SpO2 91%. Which action should the nurse implement first:
1. Request ABGs
2. Administer oxygen
3. Start IV
4.Administer analgesic
2
Client is experiencing vaso-occlusive sickle cell crisis secondary to infection. which medical tx should the nurse anticipate
1. administer demerol
2. admit the client to a private room
3. Infuse D5W
4. Insert a 22-french foley
3
Which s/s will the nurse expect to assess in teh client diagnosed with a vaso-occlusive sickle cell crisis
1. lordosis
2.epistaxis
3. hematuria
4. petechia
3. Vaso-occlusive crisis, the most frequent crisis, is characterized by organ infarction, which will result in bloody urine secondary to kidney infarction.
Periods marked by acute pain resulting from ischemia
Sickle cell Crisis
An individual with one normal gene and one hemoglobin S gene has
Sickle Cell Trait
Iron stored in tissues and organs
Hemosiderosis
Characterized by replacement of normal hemoglobin S in RBCS
Sickle Cell Disorder
When the immune system reacts against antigens on the donated tissues
alloimmunization
Persistent, painful erection of teh penis
Priapism
Can occur when sickling develops in the microcirculation
vaso-occlusion
marked by pooling of large amounts of blood in the liver and spleen.
Sequestration crisis
Caused by lack of blood flow
Tissue ischemia
a symptom complex that includes fever, chest pain, increased WBC count and pulmonary infiltrates
Acute Chest Syndrome
When the percentage of hemoglobin replaced by hemoglobin S increases it is called
sickling
a chronic hemolytic anemia that is the most common type of sickle cell disorder
sickle cell anemia