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Hyperhemolytic crisis accelerated rate of rbc

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Hyperhemolytic Crisis: Accelerated rate of RBC destruction characterized by anemia, jaundiceand reticulocytosis.Vaso-Occlusive CrisisResults from sickled cells obstructing blood vessels = PAIN in area of involvementSigns & SymptomsDistal ischemia and painHand foot syndrome = occlusion in the small distal bones of the hands and feet = painand swelling*Immediately report symmetric swelling of the hands and feet in the infant or toddler. Termeddactylitis, aseptic infarction occurs in the metacarpals and the metatarsalsAbdomen = severe pain resembling acute surgical conditionCerebrum = Stroke, visual disturbancesChest = Resembles pneumonia, chest pain, SOB, coughArthralgia = pain in the jointPriapismKidney = hematuriaHHOP TO IT!!! (Heat, Hydration, Oxygenation & Pain Management)Prevent Sickle Cell DiseasePrevent Sickle CrisisMaintain Adequate Hydration / Avoid dehydrationMaintain OxygenationProphylactic antibioticsTreat medical emergenciesVaccinesControl Pain*Do not use meperidine for pain management during sickle cell crisis because multiple dosinghas been associated with an increased risk of seizuresHYDRATION FORMULAPediatric Hematologic & Neoplastic Alterations-10 of 171st10 KgKg x 100 mL (Max 1000 mL)Next 10 – up to 20 Kg1000 + Kg x 50 mL (max 1500 mL)Over 20 Kg1500 + Kg x 20 mL
Acute Chest Syndrome -SCA complicationPresence of pulmonary infiltrateAcute Chest Syndrome is a medical emergency!One of the most common reasons forhospitalization, and a common cause of death for adolescent patients with sickle celldisease.Increased risk for pneumonia due to decreased oxygen to lung tissueClinical Manifestations:oChest pain, back pain, abdominal pain, fever 101.3 or higher, congested cough,dyspnea/wheezing, tachypnea, retractions and declining O2 sats.TX: deep breathing, antibiotics, O2, pain meds, blood transfusion, chest tubeCVA (Stroke)-SCA complicationSudden and severe complication, may occur in the absence of related illnessMajor blood vessels of the brain are blocked leading to cerebral infarction, may causevariable degrees of neurologic impairment.Current treatment involves chronic transfusion therapy, performed monthly to prevent arepeat strokeClinical Manifestations:oSevere headache, vomiting, jerking of the face, legs, arms, seizures, strangebehavior, inability to move arm or leg, unsteady gait, slurred speech, weakness inhands and feet and vision changes.Therapeutic ManagementAims of therapy include prevention of sickling and treatment of medical emergencies due tocrises.Crisis management is generally supportive and symptomaticMINIMIZE TRIGGERS!Fever, hypoxia, emotional or physical stress.Rest to minimize oxygen use - Short term O2 therapyOral and IV hydrationElectrolyte replacement to combat MA that results from hypoxiaPain managementoTreat mild moderate pain with acetaminophen or ibuprofen.

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Term
Summer
Professor
Keyser

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