Nursing Study Guide for Sickle Cell Anemia in Pediatric Patients
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Most common hemoglobinopathies
- RBCs do not carry the normal Hgb but instead carry a less effective type |
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Severe chronic blood disorder that occurs 1/2,000 per year in U.S
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Glutamic acid is replaced w/ valine in Hgb molecule → elongated RBC that is rigid & sickle w/ a shortened life spa
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Significant anemia may occur when RBCs sickle
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When cells sickle, blood becomes more viscous b/c cells clump together & prevent normal blood flow to the tissues of that area since their shape cannot pass through the smaller capillaries & venules → vaso-occlusive process leads to local tissue hypoxia → ischemia → infarction & pain crisis
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Hemolysis occurs following sickling
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Inherited autosomal recessive pattern
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African (1 in 400, 8% carry trait), Mediterranean, Middle Eastern, & Indian descent
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Passed on when both parents have gene or trait
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May be triggered by stress or traumatic event
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25% risk of Hgb SS, 25% Hgb AA, 50% Hgb AS
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Infection, fever, acidosis, dehydration, physical exertion, excessive cold exposure, hypoxia
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Hgb AS = carrier & usually have only minimal health problems
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Cardiomegaly, fxn murmur
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Pulmonary HTN, Restrictive lung disease
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Retinopathy
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Cholelithiasis & gallstones
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Jaundice, hepatomegaly
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Functional Asplenia
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Chronic leg ulcers
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MODs common in adulthood
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Stroke
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Sepsis
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Delayed G&D & puberty
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Organs
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Infants asymptomatic until 3-4 months d/t Hgb F protection (later half of 1st year of life)
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Pain crisis, recurrent pain episodes (vaso-occlusive)
- ↑ tachycardia & tachypnea → more sickling - Most common in joints (hot, swollen) |
Acute Chest Syndrome (ACS)
- Cell clumping in lungs - ↓ gas exchange → hypoxia, wheezing, cough, chest pain, fever → more sickling |
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Aplastic crisis (profound anemia)
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Dactylitis (hand-foot syndrome), aseptic infarction
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Pale mucous membranes
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Easily fatigued w/ poor appetite
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↓ BP d/t severe anemia or ↑ BP d/t SC nephropathy
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Acute abdominal pain (most common) d/t sludging & splenomegaly
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Sequestration crisis / Splenic sequestration
- Pooling of blood in liver & spleen w/ ↓ blood volume & shock |
Bacterial meningitis or sepsis
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Bone infarction
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Immunizations & ABX to ↓ risk of infection
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Tx underlying cause (infection)
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H-O-P to it! → Hydration, O2, Pain relief
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O2 during episodes of crisis to prevent further sickling
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↑ fluids to promote hemodilution, 150 mL/kg/day or double maintenance w/ hypotonic, D5W or D5 w/ 0.25% NS
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Adequate pain management helps ↓ stress; always believe pain level
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NO PRN pain meds, use fix dose, can use w/ non-pharmacologic techniques
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Assess pain w/ the right pain tool & look for complications of pain
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Assess for S/S of ineffective tissue perfusion
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Avoid sudden temp change (cooling mattress for fever)
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Cluster care
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Quiet environment & privacy
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Sickle-Turbidity Test (Sickledex) finger stick
Possibility or SCA or SC trait
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Hgb Electrophoresis
Dx, only accurate test for SCA
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Hgb
~6-9 mg/dL (normal 11-15 in infant)
Significantly lower w/ splenic sequestration, ACS, or aplastic crisis |
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Reticulocyte Count
↑ greatly
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Peripheral Blood Smear
Presence of sickle-shaped cells & target cells
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Platelet Count, Erythrocyte Sedimentation Rate,
↑
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LFTs
↑ bilirubin
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X-Ray Studies or Scans
Determine extent of organ or tissue damage d/t vaso-occlusion
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K+
↑ d/t hemolysis of RBCs after transfusion
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Pulmonary Infiltrate
W/ ACS
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Stem cell transplant
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Splenectomy
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Prophylactic ABX
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Immunizations
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Analgesics
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O2
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IV fluids
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PRBCs
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Hydroxyurea
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Family support as they often feel guilty or responsible
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Promote wireless communication w/ NP for collab & coaching
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Disease process, complications, genetics, testing for carrier status
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Regular health maintenance visits, immunizations, PCN, coping, adequate fluids
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Avoid temp fluctuations, overexertion, & stress
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Need 24-hr access to facility that specializes in SCA
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Report & Seek Immediate Medical Attention:
- Suspected pain crisis - Febrile illness - Pale, listlessness, ↑ fatigue - Unusual headache, loss of feeling, sudden weakness (stroke) - Sudden vision changes - Cough, SOB, chest pain (ACS) - Limp or swollen joints - Painful erection that won’t go down (priapism) - Symmetric swelling of hands & feet in (dactylitis) |
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