Sickle Cell Anemia Flashcards
1
Q
Pathophysiology of Sickle Cell Anemia
A
- Significant anemia may occur when the RBCs sickle
- Sickling is triggered by any stress or traumatic event such as (1) infection, (2) fever, (3) dehydration, (4) physical exertion, (5) excessive cold exposure, or (6) hypoxia, (7) psychological stress and emotions
- As the cell sickle, the blood becomes more vicious because the sickled cells clumped together and prevent normal blood flow to the tissues of that area. The sickle-shaped RBC cannot pass through the smaller capillaries and venues of the circulatory system.
- This vaso-occlusive process leads to local tissue hypoxia followed by ischemia and may result in infarction.
2
Q
Risk Factors for Sickle Cell Anemia
A
- Functional asplenia places the child at significant risk for serious infection with Steptococcus Pneumoniae or other encapsulated organisms.
3
Q
Signs and Symptoms
A
- Infants are usually asymptomatic until 3-4 months of age because Hgb F protects against sickling
- Pain
> Which causes tachycardia and tachypnea - Frequent infection
- Dehydration
4
Q
Complications of Sickle Cell Anemia
A
- Recurrent vaso-occlusive pain crisis
- Stroke
- Sepsis
- Acute Chest Syndrome
- Splenic sequestration
- Reduced visual acuity related to decreased retinal blood flow
- Chronic Leg Ulcers
- Cholestasis and Gallstones
- Delayed Growth and Development
- Delayed Puberty
- Priapism
5
Q
Therapeutic Management for Sickle Cell Anemia
A
- Focuses on preventing vaso-occlusive episodes and infection
- Prophylactic antibiotics in the young child and appropriate immunization in all children with SCA can reduce the risk of serious infection
- Treatment of vaso-occlusive focuses on pain control
> Oxygen administration is necessary during episodes of crisis to prevent additional cell sickling
> Adequate hydration with IV fluids is critical
> Close monitoring of Hgb, Hct, and reticulocytes determines the point at which transfusion of PRBCs is necessary
> Electrolyte analysis: when RBCs are administered, there is a potential for hemolysis of the cell, increasing potassium levels
> Antibiotic therapy is necessary when infection is present
6
Q
Nursing Assessment: Health History
A
- Note:
> Growth and Development
> Frequency and extent of vaso-occlusive crises
> Past hospitalizations and treatment for pain crises
> History of immunizations (pneumococcal, flu, and meningococcal vaccinations) - Determine history of blood transfusions
- Document current medication regimen
- Note history of recurrent infections
- Determine history of present illness and precipitating factors
> Hypoxia
> Infection
> Dehydration - Note onset, character and quality of pain, as well as relieving factors
7
Q
Nursing Assessment: Physical Examination (Inspection and Observation)
A
- Inspect conjunctiva, palms, and soles for pallor and skin for pallor, lesions or ulcers
- Note jaundice of the skin or scleral icterus
- Document color and moisture of oral mucosa
- Measure Temperature to evaluate for infection
- Note blood pressure (may be decreased with severe anemia or increased with sickle cell nephropathy)
- Determine baseline mental status
- Perform neurologic assessment frequently
8
Q
Nursing Assessment: Physical Examination (Auscultation)
A
- Auscultate heart sounds for murmurs
- Heart rate is elevated with pain, hyperthermia, or dehydration
- Listen to breath sounds (note rate and depth of respirations and adequacy of aeration)
- Adventitious breath sounds may be present if respiratory infection has triggers the sickle cell crisis
9
Q
Nursing Assessment: Physical Examination (Palpation)
A
- Palpate the joints for warmth, tenderness, and range of motion
- Palpate the abdomen for areas of tenderness
- Note hepatomegaly or splenomegaly
10
Q
Lab and Diagnostics
A
- Newborn screening is required by law
- Hgb electrophoresis is performed promptly to confirm the diagnosis
> Will demonstrate the presence of Hgb S and Hgb F only in the young infant
> In the older infant or child, the result will be Hgb SS
Labs:
1. Hemoglobin: baseline is 7-10 mg/dL; will be significantly lower with splenic sequestration, ACS or aplastic crisis
2. Reticulocyte Count: Greatly elevated
3. Peripheral Blood Smear: Presence of sickle-shaped cells and target cells
4. Platelet count: Increased
5. Erythrocyte Sedimentation Rate: Elevated
6. Abnormal liver function test with elevated bilirubin
*X-rays or other scans may be performed to determine the extent of organ and tissue damage resulting from vaso-occlusion
11
Q
Nursing Management: Educating the Family and Child
A
- Begin child and family education immediately after SCA is confirmed
- Teach about the genetics of the disease and encourage family members to be tested for carrier status
- Educate about the disease process
- Emphasize the importance of regularly scheduled health maintenance visits and immunizations
- Teach how to administer prophylactic penicillin for infection prevention and hydroxyurea and or L-glutamine for prevention of acute pain episodes
- Encourage families to seek medical evaluation urgently for any febrile illness
- Educate families about how to prevent and recognize vaso-occlusive events
12
Q
Prevention or Early Recognition of Vaso-Occlusive Events
A
- Seek immediate attention for ANY febrile illness
- Obtain vaccinations and penicillin prophylaxis
- Encourage adequate fluid intake daily
- Avoid temperatures that are too hot or too cold
- Avoid overexertion or stress
- Have 24-hour access to physician, nurse practitioner, or facility familiar with sickle cell care
- Contact medical provider promptly if you suspect a pain crisis is developing
- Seek medical attention immediately if any of the following develop:
> Child is pale and listless
> Abdominal pain
> Limp or swollen joints
> Cough, shortness of breath,, chest pain
> Increasing fatigue
> Unusual headache, loss of feeling, sudden weakness
> Sudden vision change
> Painful erection that won’t go down (priapism)
13
Q
Nursing Management: Managing Pain During a Vaso-Occlusive Episode
A
- Adequate pain management helps to decrease the child’s stress level; elevated stress may contribute to further sickling and additional pain*
- Initiate pain assessment with standardized pain scale
- Provide frequent evaluations of pain
- Always believe the child’s report of pain
- Moderate to severe pain usually requires opioid medication
- To bring pain under control, initially administer analgesics routinely rather than on an PRN basis
- Monitor patient-controlled analgesia in the child or adolescent
- Ensure the child is adequately hydrated with hypotonic fluid
- Nonsteriodal anti-inflammatory medications and acetaminophen are often used for less severe pain
- Use distraction with non-pharmacologic pain management techniques
> Relaxation or hypnosis
> Music
> Massage
> Play
> Guided imagery
> Therapeutic touch
> Behavior modification to augment the pain medication regimen
14
Q
Nursing Management: Managing Vaso-Occlusive Episodes
A
- Treat underlying condition
- Increase fluid requirements (promote hemodilution, provide 150 mL/kg of fluids per day or as much as double maintenance; IV or orally)
- Maintain appropriate electrolyte and pH balance
- Frequently evaluate respiratory and circulatory status
- Encourage incentive spirometer
- Administer oxygen is pulse ox is reading 92%
- Monitor LOC and immediately report changes
15
Q
Nursing Management: Preventing Infection
A
- By 2 months of age, begin administration of oral penicillin V potassium as prophylaxis against pneumococcal infection
- Continue prophylaxis until age five
- Administer childhood immunizations according to current schedule
- To prevent sepsis or meningitis as a result of infection:
> the child should receive not only the 7-valent pneumococcal vaccine series in infancy but also the 23-valent pneumococcal conjugate vaccine series in infancy
> Meningococcal vaccination is also warranted - Provide influenza vaccination before the onset of flu season
