Newborn screen in all 50 States i Hemoglobin electropheresis D Autosomal

Newborn screen in all 50 states i hemoglobin

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Newborn screen (in all 50 States) i. Hemoglobin electropheresis D. Autosomal recessive disease i. Multiple family members have it E. Deoxygenation of RBC will cause the sickle cells to become a sickle cell:
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i. Rigid shape and easier for a clot to form F. Lifespan of a sickle cell is about 20 days i. More turnover: more build-up of bilirubin 5. SICKLE CELL ANEMIA MANIFESTATIONS: A. Aplastic or hyperhemolytic crises i. Sudden severe anemia  shock ii. Parvo virus or spontaneous B. Vasoocclusive (Pain)Crisis i. Ischemia & Pain 1. Painful swelling of extremities, joints, back ii. Dactylitis – first sign in infants and babies 1. Pain in the fingers and toes iii. Priapism: pain in the penis iv. Acute chest syndrome v. Stroke vi. Functional asplenia (non-functioning spleen) 1. Higher risk for infection!! C. Sequestration Crisis i. Splenic sequestration ii. Vasocclusive event: pooling of blood iii. Can cause shock D. Chronic Effects i. Susceptibility to ii. Overwhelming infections iii. “silent stroke” – microocclusions events iv. Blindness – microocclusions v. Hepatomegaly vi. Renal failure – microocclusions vii. Cardiomegaly – microocclusions E. Acute chest syndrome i. Severe chest, back or abdominal pain ii. Fever iii. Cough iv. Dyspnea v. Tachypnea vi. Decreased 02 saturations F. Stroke i. Severe headache ii. Vomiting iii. Seizures iv. Decreased/abnormal LOC
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v. Slurred speech vi. Unsteady gait vii. Numbness in extremities viii. Vision changes 6. TRIGGERS FOR VASOOCCLUSIVE CRISIS i. Illness ii. Temperature changes (cold = vasoconstriction) iii. Stress iv. Dehydration (not drinking enough) v. Being at high altitudes (less oxygen in air) 7. SICKLE CELL ANEMIA NURSING CARE* A. Rest & Hydration** B. Enuresis support C. Electrolyte Replacement: metabolic Acidosis D. Psychologic support E. Pain management i. Heat compresses ii. Massage iii. Medication (tylenol, ibuprofen, codeine) 1. IV medication is last resort F. Vaccines i. Able to: unless have impaired immune system ii. Influenza and Pneumoccal important G. Antibiotics: i. Hydroxyurea: increase fetal hgb to help produce healthy hgb ii. Folic acid stimulate production Hgb H. Monitoring for complications I. Transfusion therapy J. Notes: i. People with sickle cell trait can have symptoms but not usually ii. Athletes who have sickle cell anemia need to be super hydrate, cannot get over heated, may not have great endurance iii. Normal Hgb may be 7 – 8
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iv. Life expectancy: 50 – 60 y/o 8. BETA THALASSEMIA A. Autosomal recessive disorder B. Cooley’s anemia = β-Thalassemia major = life-threatening without transfusions C. Deficiency in the synthesis of the β-chain of Hgb. Unstable Hgb, disintegrates D. Results in severe anemia requires chronic transfusions E. Complications: iron overload F. Who is affected? i. Mediterranean descent (Italian heritage, etc.) G. S/sx i. Anemia ii. FTT iii. Jaundice iv. Enlarged spleen, liver, heart v. Misshapen bones H. Treatment: i. Blood Transfusions 1. To replenish blood supply; suppress overproduction by bone marrow 2. Can cause buildup of iron over time 3. Complications: iron overload  CHF, DM type 1, Hep C, HIV I. Desferol IV or Deferasirox oral i. Oral = once a day; better compliance J. Splenectomy?
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  • Spring '14
  • Roberts,CristineAnn
  • Hemoglobin, Sickle cell anemia, bone marrow transplant

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