Sickle Cell Anemia: Class 3 Flashcards
Pt is admitted with sickle cell crisis. the FIRST nursing action would be??
- Administer Pain medication
- You would administer oxygen first which would solve the hypoxia. If oxygen is an option choose O2.
What is contraindicated with a young woman who has sickle cell anemia with severe abdominal pain??
- Forcing Oral fluid therapy
Don’t give oral fluids to someone who has severe abdominal pain
Look up transfering sickle cell trait and disease to kids in the book!!
25% chance
Look this up in the book
What should a nurse do first for a client with a platelet count of 20,000??
- 20,000 is critical
2. Check for spontaneous bleeding
Idiopathic thrombocytopenia Purpura. What would the INITIAL treatment include?
- Glucocorticoid therapy
What is responsible for the neurologic manifestations associated with anemia?
- Tissue Hypoxia
Compensatory vital sign changes associated with anemia, infection and bleeding result in what??
- Increased Heart Rate
A nursing diagnosis that commonly addresses tissue hypoxia associate with hematologic disorders??
- Fatigue
What do you expect a Pt to have when someone has MODERATE anemia??
- Dyspnea and Fatigue
What is the manifestation of MOST CONCERN with someone with severe anemia?
- Dyspnea at Rest
Oxygen is not getting where it needs to be
Pt is at risk for ischemia
Sickle Cell Disease
- Both parents must have the gene for the disease to show
Sickle Cell Trait
- If one parent has trait there is a 50% chance each child will have the trait
Both Parents with Sickle Cell Trait?
25% chance that each child will have the disease
Sickle Cell Disease
-Mortality
- Incurable disease due to renal and pulmonary failure
Sickle Cell in CHildren
-Complications
- Stroke
- pulmonary - Acute chest syndrome/pneumonia
- Gall stone formation
- Anemia
- Pain & Infection
- Jaundice
Homozygous for sickle cell
<1% of African americans are homozygous for the disorder which means they have sickle cell disease
Sickle Cell Anemia
- Most Severe
- Homozygous for hemoglobin S (have disease)
- Inherited HbS from both parents
Sickle Cell Thalassemia
- Combination of sickle cell trait and thalassemia trait
Sickle Cell HbC disease
- Inherits HbS from one parent and another abnormal type of Hb from another
Sickle Cell Trait
- Inherit HbS from one parent and normal HbA from another
- People with sickle cell trait are usually asymptomatic unless triggered by a hypoxic event
> 40% of HbS is indicative of Sickle Cell Disease
Sickle Cell Episodes
-Triggers
- Hypoxia or deoxygenation of RBC
- Dehydration **
- High Altitudes **
Pg 178 bx 316
Sickle Cell Episodes
-Symptoms
- Pain and swelling
- Pallor of mucous membranes
- Fatigue **
Sickle Cell Crisis
- Severe, painful, acute exacerbation with vasoocclusive crisis
Sickle Cell Crisis
-Nursing Priority
- Manage Pain
2. Rehydrate the Pt
Sickle Cell Crisis
-Manifestations
- Severe capillary hypoxia
- Change in membrane permeability
- Thrombi
- Tissue ischemia, Infarction, Necrosis
- Shock
- Stroke
- Abdominal Pain
Thrombotic Crisis
- Occurs when sickling occurs in micro circulation
2. Extremely painful and can last an average of 4 to 6 days
Sickle Cell Disease
-Manifestations
- Asymptomatic until sickling episode
- Pallor
- may be difficult to assess on dark skin individuals
- Look at mucous membranes, conjunctiva, nail beds, palms - PAIN
- aching in joints, hands, feet gnawing, throbbing
Sickle Cell in Infants
-Fetal HgB
- Affected children are asymptomatic until 4 to 6 months of age
- Sickling is inhibited by high levels of fetal Hgb
- Infants have fetal Hgb till 4 to 6 months of age
Sickle Cell Disease
-Complications
- Gradual Involvement of all body systems
- Often fatal by middle age from RENAL and PULMONARY failure
- Prone to infection
- PNEUMONIA ** most common infection
Sickle Cell Disease
-Chronic Disease
- Retinopathy
- Cardiomegaly
- Avascular necrosis
- Skin ulcers
- Delayed puberty
- Anemia and leukocytosis
Sickle Cell Disease
-Diagnostics
- Electrophoresis of Hemoglobin
- Uses electrical current to separate HbA and HbS - Sickling Test
- Checks if RBC will sickle when RBC’s are deoxygenated
Sickle Cell Disorder
-What you see in a Serum Blood analysis?
- HgbS (abnormal type)
- Low Hemoglobin
- High reticulocyte count
Sickle Cell
-Priority Interventions
- Oxygenate
- Rehydrate
- Medicate
- Give IV meds around the clock during crisis
- -morphine or dilaudid
DEMEROL for Sickle Cell Pain Treatment
- Do not use due to the breakdown of nor-meperidine which can cause seizures
Sickle Cells & Hydration
- Sickled cells can revert back to normal hgb with hydration
- Pt need to keep well hydrated to prevent crisis
Sickle Cell Disorder
-Nursing Management
- No Treatment
- Alleviate symptoms of disease complications
- Minimize end-organ damage
Acute Chest Syndrome
-
- Vasoocclusive crisis in the pulmonary vasculature
- Seen as New infiltrate on chest X-ray associated w/ FEVER, COUGH, SOB
Acute Chest Syndrome
-Nursing Management
- Oxygen therapy
- Fluid therapy
- Antibiotics ***
Sickle Cell
-Blood Transfusions
- Improved blood & Tissue oxygenation
- Reduction in sickling
- Temporary suppression of production of RBC’s containing HbS
Blood Transfusions
-Complications
- Overload of Iron in the body w/ frequent transfusions
Hemosiderosis
- Overload of iron in the body
2. May require iron-chelating therapy
Blood Transfusions
-Stroke
- Chronic transfusion proven to be effective treatment of stroke complications related to sickle cell disease
Sickle Cell
-Nursing MGMT – Medications
- Folic Acid daily supplements
- Hydroxyurea
- For pt’s with 4-5 crises per year - Stem cell transplant
- Can cure some pt’s with SCD (it may reoccur)
Hydroxyurea
- Chemotherapy agent w/ potent effects on bone marrow
- Increases the production of fetal hgb in the RBC’s
- prevents sickled cells from becoming rigid and occluding vessels
Fetal Hbg
- Main oxygen transport protein in the fetus during last 7 months of development
- Binds to oxygen with greater affinity then the adult form
- this gives developing fetus better access to oxygen from mothers blood stream
Hydroxyurea
-Side effects
- Suppression of blood counts
- Specifically WBC’s - Can cause neutropenia or thrombocytopenia
- Pt can be at risk for INFECTION and BLEEDING
Sickle Cell Disorder
-Patient Teaching
- Avoid high altitude
- pt may need to move out of high altitude area - Maintain fluid intake
- Treat infections
- Control pain
Sickle Cell Disorder
-Nursing Dx
- Acute Pain
- Risk for impaired tissue perfusion
- Risk for infection
- Impaired physical mobility
- Delayed growth and development
SCD
-Goals for treatment
- Client will experience reduced complications as the result of sickling
- Client will meet growth and development needs
- Client will optimize physical mobility as tolerated
SCD
-Acute Pain
- Administer prescribed analgesics (morphine) around the clock during crises
- PCA education for parent or child
- Reposition pt’s for comfort
SCD
-Impaired Tissue Perfusion
- Hydration
- This can stop or reverse sickling - Supplemental Oxygen
- Administer blood transfusions
- Monitor for transfusion reactions
- Encourage rest
- WITH ALL ANEMIAS
SCD
-Risk For Infection (considerations)
- Teach proper administration of antibiotics for prophylaxis or treatment
- Assess resources to obtain antibiotics
- Encourage use of vaccines
