Transcript Sickle Cell Anemia (SCA)

 Alterations in Hematologic
Status—
Sickle Cell Anemia
 Thalassemia Major
Aplastic Anemia
Hemophilia
Idiopathic Thrombocytopenic Purpura
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NURS 317
Major functions of Bone Marrow
Production of cells
 Oxygenation
 Nutrient distribution to cells
 Immune protection
 Waste collection from cells heat
production
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Common Diagnostic
Assessments
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Family History
Child’s health history
Physical exam
Medication use
Dietary pattern
Lab studies: CBC w/diff, PT, PTT, Hb, Hct,
iron studies, bone marrow aspiration/bx
Prepare child & family for lab tests
Sickle Cell Anemia (SCA)
A global disease
Definition
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http://www.nhlbi.nih.gov/health/dci/Di
seases/Sca/SCA_WhatIs.html
Sickle cell anemia results from the hereditary
presence of abnormal Hgb S in place of Hgb A.
When Hgb S becomes deoxygenated (loses
oxygen), it tends to form polymers that
deform the red blood cells (RBCs) into a sickle
shape. Sickled RBCs cannot pass freely
through capillaries and cause blockage of
small vessels. This can compromise the blood
supply to various organs.
Incidence
Hereditary hemoglobinopathy;
autosomal recessive; 1 African
Americans
 Hgb S trait is found in 8 to 10% of the
American black population.
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Sickle Cell Anemia– homozygous form
(HbSS)—most prevalent in US
In Western African native blacks, up
to 40% of the population carry Hgb
S trait.
Basic Defect
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A defect in the structure of Hemoglobin A—
substitution of 1 amino acid (valine) for
another (glutamine) @ the 6th position of the
beta polypeptide chain.
Hgb partially/completely replaced by Hgb S
When triggered, Hgb S sickles & obstructs
cap. blood flow, s blood viscosity 
tissue ischemia
Results  hemolysis (RBC destruction) &
anemia;
Sickling reversible until repeated cycles
leads to irreversible sickling
Diagnostic Tests
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Rarely seen before 4-6 mos.
Sickledex: sickle turbidity test; finger or
heel stick; results in 3 min
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Sickledex does not distinguish between
sickle cell trait and sickle cell disease.
Hemoglobin electrophoresis is required to
distinguish between these 2 disorders.
Hgb electrophoresis to “fingerprint” protein
Hx, PE, CBC
CXR if symptoms
Diagnostic Tests
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(cont’d)
Peripheral Blood Smear
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The routine peripheral blood smear
does not contain sickled red blood
cells unless hypoxemia (inadequate
levels of oxygen in the blood) or a
precipitating factor (such as a red
blood cell poison or drug) is
present.
Diagnostic Tests
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(cont’d)
Electrophoresis
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Both sickle cell disease (homozygous for
hemoglobin S) and sickle cell trait
(heterozygous for Hgb S) can be detected
by this test.
This test separates different types of
hemoglobin based on electrical charge.
Hgb S can be differentiated from Hgb A
and other types of Hgb.
Diagnostic Tests
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Normal Results
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(cont’d)
In hemoglobin electrophoresis, no Hgb S
should be present. Normal hemoglobins
in an adult are mostly Hgb A with small
amounts of Hgb A2 and Hgb F.
What do abnormal results mean?
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sickle cell trait
sickle cell anemia
Pathophysiology
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Spleen—enlarged, if untreated,
becomes fibrous & ineffective 
susceptibility to infection.
 Liver—failure & necrosis 2ndary to
impaired circulation. Hepatomegaly
and tenderness are common sx.
 Kidney— perfusion 2ndary to
congestion of glomerular capillaries &
tubular arterioles hematuria, inability
to concentrate urine, enuresis, &
possible nephrotic syndrome.
Pathophysiology
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(cont’d)
Bones—
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Hyperplasia of bone marrow osteoporosis
weakened bones lordosis and kyphosis
or other skeletal deformities
Chronic hypoxia possible osteomyelitis
from salmonella
“Hand-foot Syndrome”—occurs in children 6
mos-2yrs. Caused by infarction of shorttubular bones.
Sx: swelling & pain of soft tissue of hands &
feet. Usually resolves in 2-3 wks.
Pathophysiology
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CNS—primarily R/T ischemia
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(cont’d)
MAJOR INFARCT—Stroke or CVA
paralysis
MINOR INFARCT—HA, aphasia,
weakness, convulsions, visual changes
2ndary to retinopathy & retinal
detachment.
Heart—Decompensation & failure
2ndary to chronic anemia  CHF,
tachycardia
Pathophysiology
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(cont’d)
Blood—
Rate of destruction of RBC’s > rate
of RBC production
 RBC’s lifespan is  to 10-15 days
(compared to 120 days for nml RBC)
  Hgb, Hct & RBC count
  Reticulocyte count, bilirubin & uric
acid
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Pathphysiology
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(cont’d)
Other sx:
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Generalized weakness
Anorexia
Joint, back, and abdominal pain
Fever
Vomiting
Possible leg ulcers
Failure to thrive,  rate of growth &
development
Sickle Cell Crisis
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Triggers
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Acute illness
Dehydration
Strenous exercise
Hypoxic conditions
Trauma
Respiratory illness
Results: acute pain, cell destruction,
infarcts, organ damage
Types of Crises
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Vaso-occlusive: PAIN, swollen joints,
vomiting, fever, anorexia, dactylitis
 Acute sequestration: liver & spleen,
HR dyspnea, weakness, pallor,
shock
 Aplastic crisis: weakness, pallor,
dyspnea, tachycardia, shock
 Hyperhemolytic— most rare form.
Caused by excess RBC destruction.
Other complications
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Acute chest syndrome: chest pain,
cough, fever, abdominal pain.
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Hydroxyurea can help prevent this.
Megaloblastic anemia
 Hyperhemolytic crisis
 Cerebrovascular injury (stroke)
 Overwhelming infections
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TREATMENT
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Primary Goals:
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Prevent Crisis (avoid sickling
phenomenon)—through adequate
oxygenation & hydration in every day
life activities. Hydroxyurea may be used
prophylactically in adolescents and older
adults. Folic Acid 1mg daily is also
recommended to restore folate stores
needed for erythropoiesis.
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Treat crisis as an emergency
when it occurs—determine etiology
quickly and treat appropriately
Nursing Management
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Adequate hydration (also any electrolyte
replacement if needed)
Pain management
Promote rest
Treat any infections
Blood transfusions if needed
Monitor O2 – supplement if needed
Oral hygiene
Maintain nutrition
Family support and education
NURSING CARE
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Pain Control—strive to prevent pain.
Opioids as morphine, oxycodone,
hydromorphone & methadone are
preferred. Give IV or PO for severe pain
round-the-clock. PCA pumps are great!
Avoid Demerol (Meperidine) - ↑seizure
risk
 Tylenol & Ibuprofen may be used after dx is made
 Use non-pharmacologic methods also
 Heat (e.g. K-pad)to affected joints may be
soothing by  O2 with vasodilation.
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NURSING CARE
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Hydration–
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Accurate I & O essential
 IV/PO fluids up to 150ml/kg/day will promote
hemodilution
Note color of urine—sometimes brown
Prevent tissue deoxygenation
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Avoid
Avoid
Avoid
Avoid
Avoid
strenuous activity
environments with low O2 concentration
extremes in temp- esp. cold environment
emotional stress
known sources of infection
Child/Family Education
How to manage & minimize crisis:
avoid dehydration, cold, stress,
hypoxia, acute illness, etc.
 Signs of infection
 Importance of immunizations
 Antibiotic administration if ordered
 Education & support re: long term
effects
 Genetic counseling
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 Thalassemia Major
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Autosomal recessive
Defective Hgb; RBCs fragile w/short
life span  anemia, hemolysis
 Main Tx: blood transfusions
 Hemochromatosis – iron overload –
occurs from hemolysis of
transfused and defective RBCs
 Chelation tx done to eliminate
excess Fe
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Nursing Considerations
Numerous blood transfusions
 Assess s/s of hemosiderosis
 Assess s/s infection
 Folic acid supplementation
 Iron chelation tx as ordered
 Family support
 Possible bone marrow transplant
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Aplastic Anemia
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Definition: Condition where all formed
elements of the blood are depressed.
Pancytopenia or Triad:
Anemia (↓ RBC's)
 Leukopenia (↓ WBC's)
 Thrombocytopenia (↓ platelets)
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Etiology of A.A.
Primary (congenital)
 Secondary (acquired) -- often
secondary to drugs e.g. chemotherapy
or other meds that “shut down” bone
marrow function
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Diagnosis -- Bone marrow aspiration
or biopsy
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Therapeutic Management -- Goal:
Restore bone marrow function
Immunosuppressive therapy
 Bone marrow transplantation ** choice
85% survival rate
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Nursing Considerations -- Similar to
child with leukemia
Prepare family for diagnostic &
therapeutic procedures
 Preventing complications from severe
pancytopenia
 Emotionally supporting them in terms
of a potentially fatal outcome
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Hemophilia
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Deficiency in specific clotting factor
X-linked recessive trait; 2 most
common
Hemophilia A (classic)
 Hemophilia B (Christmas Disease)
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Uncontrolled bleeding from wounds
into joints, muscles, & other body
tissues
 Spontaneous & severe uncontrolled
bleeding in severe form
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Common Labs
Hx & PE, symptoms
 PTT prolonged
 Factor VIII, IX levels 
 Normal: PT, thrombin, bleeding
time, fibrinogen, platelet count
 May do liver Bx to r/o other causes
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Signs & symptoms
Excessive bleeding from
circumcision
 Bleeding w/teething
 Hemarthosis – cardinal sign
 Bruises in various stages
 Hematuria
 Nose bleeds
 Oral bleeding
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Nursing Management
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Control localized bleeding:
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Direct pressure for extended period of time like
10 minutes
Ice
Elevation of affected limb
Avoid injections when possible
No rectal temps
Monitor for bleeding (joints, urine, stool, etc)
Provide pain relief
Monitor neuro status if possible head bleeds
Nursing Management
Administration of Factor
replacement as needed
 Factor VIII concentrate
(Antihemophilia factor {AHF} ),
or DDAVP , a synthetic form of
vasopressin that is the treatment of
choice in mild hemophilia & von
Willebrand disease. Should increase
Factor VIII activity 3-4 fold.
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Teaching Considerations
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Teach family: factor administration/storage
 May have Mediport & parents know how to
access & administer via central line in
emergency
DDAVP administration—nasal spray or injection
Avoid ASA type products
Avoid contact sports
Medic alert tag
Avoid constipation
Good nutrition
Good oral hygiene
Genetic counseling
Protective environment for infants/toddlers
Idiopathic Thrombocytopenia
Purpura (ITP)
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Excessive destruction of platelets Purpura
(from petechiae under skin)
Normal bone marrow
Most common bleeding disorder in
children
80% between 2-5 yrs old and usually last
~ 6m
Usually after viral infection
Symptoms when platelet ct < 20,000
Therapeutic Management
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IF medical tx is indicated, then may
use one of the following regimens
Prednisone
 IVIG—expensive and takes long to
administer and monitor pt
 Anti-D antibody—controversial but may
prolong survival of platelets. Can be
given in 1 dose over 5-10 minutes.
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Nursing Considerations
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Stress activity restriction
Prevention of head injuries; instruct
parents to get med eval if head or abd
injury
Assess for bleeding/bruising
Oral care support
No rectal temps – No ASA products
Splenectomy if has for 1 yr and tx not
successful
Family support & teach on how to control
bleeds
 There
you have it!