50%(2)1 out of 2 people found this document helpful
This preview shows page 3 - 7 out of 13 pages.
Newborn screen (in all 50 States)i.Hemoglobin electropheresisD.Autosomal recessive diseasei.Multiple family members have it E.Deoxygenation of RBC will cause the sickle cells to become a sickle cell:
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 bilirubin5. SICKLE CELL ANEMIA MANIFESTATIONS:A.Aplastic or hyperhemolytic crises i.Sudden severe anemia shockii.Parvo virus or spontaneousB.Vasoocclusive (Pain)Crisis i.Ischemia & Pain1.Painful swelling of extremities, joints, backii.Dactylitis– first sign in infants and babies 1.Pain in the fingers and toesiii.Priapism: pain in the penisiv.Acute chest syndromev.Stroke vi.Functional asplenia (non-functioning spleen)1.Higher risk for infection!!C.Sequestration Crisis i.Splenic sequestrationii.Vasocclusive event: pooling of bloodiii.Can cause shockD.Chronic Effectsi.Susceptibility to ii.Overwhelming infectionsiii.“silent stroke” – microocclusions eventsiv.Blindness – microocclusionsv.Hepatomegalyvi.Renal failure – microocclusionsvii.Cardiomegaly – microocclusionsE.Acute chest syndromei.Severe chest, back or abdominal painii.Feveriii.Coughiv.Dyspneav.Tachypneavi.Decreased 02 saturationsF.Strokei.Severe headacheii.Vomitingiii.Seizuresiv.Decreased/abnormal LOC
v.Slurred speechvi.Unsteady gaitvii.Numbness in extremitiesviii.Vision changes6. TRIGGERS FOR VASOOCCLUSIVE CRISISi.Illnessii.Temperature changes (cold = vasoconstriction)iii.Stressiv.Dehydration (not drinking enough)v.Being at high altitudes (less oxygen in air)7. SICKLE CELL ANEMIA NURSING CARE*A.Rest & Hydration**B.Enuresis supportC.Electrolyte Replacement: metabolic AcidosisD.Psychologic supportE.Pain managementi.Heat compressesii.Massageiii.Medication (tylenol, ibuprofen, codeine)1.IV medication is last resortF.Vaccines i.Able to: unless have impaired immune systemii.Influenza and Pneumoccal importantG. Antibiotics:i.Hydroxyurea: increase fetal hgb to help produce healthy hgbii.Folic acid stimulate production HgbH.Monitoring for complicationsI.Transfusion therapyJ.Notes:i.People with sickle cell trait can have symptoms but not usuallyii.Athletes who have sickle cell anemia need to be super hydrate, cannot getover heated, may not have great enduranceiii.Normal Hgb may be 7 – 8
iv.Life expectancy: 50 – 60 y/o8. BETA THALASSEMIAA.Autosomal recessive disorderB.Cooley’s anemia = β-Thalassemia major = life-threatening without transfusionsC.Deficiency in the synthesis of the β-chain of Hgb. Unstable Hgb, disintegratesD.Results in severe anemia requires chronic transfusionsE.Complications: iron overloadF.Who is affected?i.Mediterranean descent (Italian heritage, etc.)G. S/sx i.Anemiaii.FTTiii.Jaundiceiv.Enlarged spleen, liver, heartv.Misshapen bonesH. Treatment:i.Blood Transfusions1.To replenish blood supply; suppress overproduction by bone marrow2.Can cause buildup of iron over time3.Complications: iron overload CHF, DM type 1, Hep C, HIVI.Desferol IV or Deferasirox oral i.Oral = once a day; better complianceJ.Splenectomy?
You've reached the end of your free preview.
Want to read all 13 pages?
Hemoglobin, Sickle cell anemia, bone marrow transplant