Sickle Cell Anemia: Class 3

Question 0

Pt is admitted with sickle cell crisis. the FIRST nursing action would be??

Answer 0

1. Administer Pain medication

- You would administer oxygen first which would solve the hypoxia. If oxygen is an option choose O2.

Question 1

What is contraindicated with a young woman who has sickle cell anemia with severe abdominal pain??

Answer 1

1. Forcing Oral fluid therapy

Don’t give oral fluids to someone who has severe abdominal pain

Question 2

Look up transfering sickle cell trait and disease to kids in the book!!

Answer 2

25% chance

Look this up in the book

Question 3

What should a nurse do first for a client with a platelet count of 20,000??

Answer 3

1. 20,000 is critical

2. Check for spontaneous bleeding

Question 4

Idiopathic thrombocytopenia Purpura. What would the INITIAL treatment include?

Answer 4

1. Glucocorticoid therapy

Question 5

What is responsible for the neurologic manifestations associated with anemia?

Answer 5

1. Tissue Hypoxia

Question 6

Compensatory vital sign changes associated with anemia, infection and bleeding result in what??

Answer 6

1. Increased Heart Rate

Question 7

A nursing diagnosis that commonly addresses tissue hypoxia associate with hematologic disorders??

Answer 7

1. Fatigue

Question 8

What do you expect a Pt to have when someone has MODERATE anemia??

Answer 8

1. Dyspnea and Fatigue

Question 9

What is the manifestation of MOST CONCERN with someone with severe anemia?

Answer 9

1. Dyspnea at Rest

Oxygen is not getting where it needs to be
Pt is at risk for ischemia

Question 10

Sickle Cell Disease

Answer 10

1. Both parents must have the gene for the disease to show

Question 11

Sickle Cell Trait

Answer 11

1. If one parent has trait there is a 50% chance each child will have the trait

Question 12

Both Parents with Sickle Cell Trait?

Answer 12

25% chance that each child will have the disease

Question 13

Sickle Cell Disease

-Mortality

Answer 13

1. Incurable disease due to renal and pulmonary failure

Question 14

Sickle Cell in CHildren

-Complications

Answer 14

1. Stroke
2. pulmonary - Acute chest syndrome/pneumonia
3. Gall stone formation
4. Anemia
5. Pain & Infection
6. Jaundice

Question 15

Homozygous for sickle cell

Answer 15

<1% of African americans are homozygous for the disorder which means they have sickle cell disease

Question 16

Sickle Cell Anemia

Answer 16

1. Most Severe
2. Homozygous for hemoglobin S (have disease)
3. Inherited HbS from both parents

Question 17

Sickle Cell Thalassemia

Answer 17

1. Combination of sickle cell trait and thalassemia trait

Question 18

Sickle Cell HbC disease

Answer 18

1. Inherits HbS from one parent and another abnormal type of Hb from another

Question 19

Sickle Cell Trait

Answer 19

1. Inherit HbS from one parent and normal HbA from another
2. People with sickle cell trait are usually asymptomatic unless triggered by a hypoxic event

> 40% of HbS is indicative of Sickle Cell Disease

Question 20

Sickle Cell Episodes

-Triggers

Answer 20

1. Hypoxia or deoxygenation of RBC
2. Dehydration **
3. High Altitudes **

Pg 178 bx 316

Question 21

Sickle Cell Episodes

-Symptoms

Answer 21

1. Pain and swelling
2. Pallor of mucous membranes
3. Fatigue **

Question 22

Sickle Cell Crisis

Answer 22

1. Severe, painful, acute exacerbation with vasoocclusive crisis

Question 23

Sickle Cell Crisis

-Nursing Priority

Answer 23

1. Manage Pain

2. Rehydrate the Pt

Question 24

Sickle Cell Crisis | -Manifestations

Answer 24

1. Severe capillary hypoxia - Change in membrane permeability - Thrombi - Tissue ischemia, Infarction, Necrosis - Shock - Stroke - Abdominal Pain

Question 25

Thrombotic Crisis

Answer 25

1. Occurs when sickling occurs in micro circulation | 2. Extremely painful and can last an average of 4 to 6 days

Question 26

Sickle Cell Disease | -Manifestations

Answer 26

1. Asymptomatic until sickling episode 2. Pallor - may be difficult to assess on dark skin individuals - Look at mucous membranes, conjunctiva, nail beds, palms 3. PAIN - aching in joints, hands, feet gnawing, throbbing

Question 27

Sickle Cell in Infants | -Fetal HgB

Answer 27

1. Affected children are asymptomatic until 4 to 6 months of age 2. Sickling is inhibited by high levels of fetal Hgb 3. Infants have fetal Hgb till 4 to 6 months of age

Question 28

Sickle Cell Disease | -Complications

Answer 28

1. Gradual Involvement of all body systems 2. Often fatal by middle age from RENAL and PULMONARY failure 3. Prone to infection - PNEUMONIA ** most common infection

Question 29

Sickle Cell Disease | -Chronic Disease

Answer 29

1. Retinopathy 2. Cardiomegaly 3. Avascular necrosis 4. Skin ulcers 5. Delayed puberty 6. Anemia and leukocytosis

Question 30

Sickle Cell Disease | -Diagnostics

Answer 30

1. Electrophoresis of Hemoglobin - Uses electrical current to separate HbA and HbS 2. Sickling Test - Checks if RBC will sickle when RBC's are deoxygenated

Question 31

Sickle Cell Disorder | -What you see in a Serum Blood analysis?

Answer 31

1. HgbS (abnormal type) 2. Low Hemoglobin 3. High reticulocyte count

Question 32

Sickle Cell | -Priority Interventions

Answer 32

1. Oxygenate 2. Rehydrate 3. Medicate - Give IV meds around the clock during crisis - -morphine or dilaudid

Question 33

DEMEROL for Sickle Cell Pain Treatment

Answer 33

1. Do not use due to the breakdown of nor-meperidine which can cause seizures

Question 34

Sickle Cells & Hydration

Answer 34

1. Sickled cells can revert back to normal hgb with hydration 2. Pt need to keep well hydrated to prevent crisis

Question 35

Sickle Cell Disorder | -Nursing Management

Answer 35

1. No Treatment 2. Alleviate symptoms of disease complications 3. Minimize end-organ damage

Question 36

Acute Chest Syndrome | -

Answer 36

1. Vasoocclusive crisis in the pulmonary vasculature | - Seen as New infiltrate on chest X-ray associated w/ FEVER, COUGH, SOB

Question 37

Acute Chest Syndrome | -Nursing Management

Answer 37

1. Oxygen therapy 2. Fluid therapy 3. Antibiotics ***

Question 38

Sickle Cell | -Blood Transfusions

Answer 38

1. Improved blood & Tissue oxygenation 2. Reduction in sickling 3. Temporary suppression of production of RBC's containing HbS

Question 39

Blood Transfusions | -Complications

Answer 39

1. Overload of Iron in the body w/ frequent transfusions

Question 40

Hemosiderosis

Answer 40

1. Overload of iron in the body | 2. May require iron-chelating therapy

Question 41

Blood Transfusions | -Stroke

Answer 41

1. Chronic transfusion proven to be effective treatment of stroke complications related to sickle cell disease

Question 42

Sickle Cell | -Nursing MGMT -- Medications

Answer 42

1. Folic Acid daily supplements 2. Hydroxyurea - For pt's with 4-5 crises per year 3. Stem cell transplant - Can cure some pt's with SCD (it may reoccur)

Question 43

Hydroxyurea

Answer 43

1. Chemotherapy agent w/ potent effects on bone marrow 2. Increases the production of fetal hgb in the RBC's - prevents sickled cells from becoming rigid and occluding vessels

Question 44

Fetal Hbg

Answer 44

1. Main oxygen transport protein in the fetus during last 7 months of development 2. Binds to oxygen with greater affinity then the adult form - this gives developing fetus better access to oxygen from mothers blood stream

Question 45

Hydroxyurea | -Side effects

Answer 45

1. Suppression of blood counts - Specifically WBC's 2. Can cause neutropenia or thrombocytopenia 3. Pt can be at risk for INFECTION and BLEEDING

Question 46

Sickle Cell Disorder | -Patient Teaching

Answer 46

1. Avoid high altitude - pt may need to move out of high altitude area 2. Maintain fluid intake 3. Treat infections 4. Control pain

Question 47

Sickle Cell Disorder | -Nursing Dx

Answer 47

1. Acute Pain 2. Risk for impaired tissue perfusion 3. Risk for infection 4. Impaired physical mobility 5. Delayed growth and development

Question 48

SCD | -Goals for treatment

Answer 48

1. Client will experience reduced complications as the result of sickling 2. Client will meet growth and development needs 3. Client will optimize physical mobility as tolerated

Question 49

SCD | -Acute Pain

Answer 49

1. Administer prescribed analgesics (morphine) around the clock during crises 2. PCA education for parent or child 3. Reposition pt's for comfort

Question 50

SCD | -Impaired Tissue Perfusion

Answer 50

1. Hydration - This can stop or reverse sickling 2. Supplemental Oxygen 3. Administer blood transfusions 4. Monitor for transfusion reactions 5. Encourage rest - WITH ALL ANEMIAS

Question 51

SCD | -Risk For Infection (considerations)

Answer 51

1. Teach proper administration of antibiotics for prophylaxis or treatment 2. Assess resources to obtain antibiotics 3. Encourage use of vaccines