Sickle Cell Anemia: Class 3 Flashcards

0
Q

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

A
  1. Administer Pain medication

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

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1
Q

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

A
  1. Forcing Oral fluid therapy

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

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2
Q

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

A

25% chance

Look this up in the book

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3
Q

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

A
  1. 20,000 is critical

2. Check for spontaneous bleeding

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4
Q

Idiopathic thrombocytopenia Purpura. What would the INITIAL treatment include?

A
  1. Glucocorticoid therapy
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5
Q

What is responsible for the neurologic manifestations associated with anemia?

A
  1. Tissue Hypoxia
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6
Q

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

A
  1. Increased Heart Rate
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7
Q

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

A
  1. Fatigue
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8
Q

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

A
  1. Dyspnea and Fatigue
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9
Q

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

A
  1. Dyspnea at Rest

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

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10
Q

Sickle Cell Disease

A
  1. Both parents must have the gene for the disease to show
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11
Q

Sickle Cell Trait

A
  1. If one parent has trait there is a 50% chance each child will have the trait
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12
Q

Both Parents with Sickle Cell Trait?

A

25% chance that each child will have the disease

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13
Q

Sickle Cell Disease

-Mortality

A
  1. Incurable disease due to renal and pulmonary failure
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14
Q

Sickle Cell in CHildren

-Complications

A
  1. Stroke
  2. pulmonary - Acute chest syndrome/pneumonia
  3. Gall stone formation
  4. Anemia
  5. Pain & Infection
  6. Jaundice
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15
Q

Homozygous for sickle cell

A

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

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16
Q

Sickle Cell Anemia

A
  1. Most Severe
  2. Homozygous for hemoglobin S (have disease)
  3. Inherited HbS from both parents
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17
Q

Sickle Cell Thalassemia

A
  1. Combination of sickle cell trait and thalassemia trait
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18
Q

Sickle Cell HbC disease

A
  1. Inherits HbS from one parent and another abnormal type of Hb from another
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19
Q

Sickle Cell Trait

A
  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

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20
Q

Sickle Cell Episodes

-Triggers

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

Pg 178 bx 316

21
Q

Sickle Cell Episodes

-Symptoms

A
  1. Pain and swelling
  2. Pallor of mucous membranes
  3. Fatigue **
22
Q

Sickle Cell Crisis

A
  1. Severe, painful, acute exacerbation with vasoocclusive crisis
23
Q

Sickle Cell Crisis

-Nursing Priority

A
  1. Manage Pain

2. Rehydrate the Pt

24
Q

Sickle Cell Crisis

-Manifestations

A
  1. Severe capillary hypoxia
    - Change in membrane permeability
    - Thrombi
    - Tissue ischemia, Infarction, Necrosis
    - Shock
    - Stroke
    - Abdominal Pain
25
Q

Thrombotic Crisis

A
  1. Occurs when sickling occurs in micro circulation

2. Extremely painful and can last an average of 4 to 6 days

26
Q

Sickle Cell Disease

-Manifestations

A
  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
27
Q

Sickle Cell in Infants

-Fetal HgB

A
  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
28
Q

Sickle Cell Disease

-Complications

A
  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
29
Q

Sickle Cell Disease

-Chronic Disease

A
  1. Retinopathy
  2. Cardiomegaly
  3. Avascular necrosis
  4. Skin ulcers
  5. Delayed puberty
  6. Anemia and leukocytosis
30
Q

Sickle Cell Disease

-Diagnostics

A
  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
31
Q

Sickle Cell Disorder

-What you see in a Serum Blood analysis?

A
  1. HgbS (abnormal type)
  2. Low Hemoglobin
  3. High reticulocyte count
32
Q

Sickle Cell

-Priority Interventions

A
  1. Oxygenate
  2. Rehydrate
  3. Medicate
    - Give IV meds around the clock during crisis
    - -morphine or dilaudid
33
Q

DEMEROL for Sickle Cell Pain Treatment

A
  1. Do not use due to the breakdown of nor-meperidine which can cause seizures
34
Q

Sickle Cells & Hydration

A
  1. Sickled cells can revert back to normal hgb with hydration
  2. Pt need to keep well hydrated to prevent crisis
35
Q

Sickle Cell Disorder

-Nursing Management

A
  1. No Treatment
  2. Alleviate symptoms of disease complications
  3. Minimize end-organ damage
36
Q

Acute Chest Syndrome

-

A
  1. Vasoocclusive crisis in the pulmonary vasculature

- Seen as New infiltrate on chest X-ray associated w/ FEVER, COUGH, SOB

37
Q

Acute Chest Syndrome

-Nursing Management

A
  1. Oxygen therapy
  2. Fluid therapy
  3. Antibiotics ***
38
Q

Sickle Cell

-Blood Transfusions

A
  1. Improved blood & Tissue oxygenation
  2. Reduction in sickling
  3. Temporary suppression of production of RBC’s containing HbS
39
Q

Blood Transfusions

-Complications

A
  1. Overload of Iron in the body w/ frequent transfusions
40
Q

Hemosiderosis

A
  1. Overload of iron in the body

2. May require iron-chelating therapy

41
Q

Blood Transfusions

-Stroke

A
  1. Chronic transfusion proven to be effective treatment of stroke complications related to sickle cell disease
42
Q

Sickle Cell

-Nursing MGMT – Medications

A
  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)
43
Q

Hydroxyurea

A
  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
44
Q

Fetal Hbg

A
  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
45
Q

Hydroxyurea

-Side effects

A
  1. Suppression of blood counts
    - Specifically WBC’s
  2. Can cause neutropenia or thrombocytopenia
  3. Pt can be at risk for INFECTION and BLEEDING
46
Q

Sickle Cell Disorder

-Patient Teaching

A
  1. Avoid high altitude
    - pt may need to move out of high altitude area
  2. Maintain fluid intake
  3. Treat infections
  4. Control pain
47
Q

Sickle Cell Disorder

-Nursing Dx

A
  1. Acute Pain
  2. Risk for impaired tissue perfusion
  3. Risk for infection
  4. Impaired physical mobility
  5. Delayed growth and development
48
Q

SCD

-Goals for treatment

A
  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
49
Q

SCD

-Acute Pain

A
  1. Administer prescribed analgesics (morphine) around the clock during crises
  2. PCA education for parent or child
  3. Reposition pt’s for comfort
50
Q

SCD

-Impaired Tissue Perfusion

A
  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
51
Q

SCD

-Risk For Infection (considerations)

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