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Increased blood viscosity due to dehydration oHypoxia(from high altitude, poorly pressurized airplanes, hypoventilation, vasoconstriction due to hypothermia)Acute exacerbations that vary in severity and frequencyTypesoVaso-occlusive (VOC) thromboticPriority to promote restoSplenic SequestrationLife threatening – death can occur within hoursBlood pools in spleenoAplastic CrisesDiminished production and increased destruction of RBCTriggered by viral infection or depletion of folic acidSigns include profound anemia, pallorAcute Chest Syndrome(similar to pneumonia)WANT TO AVOID THIS with children with Sickle CellVOC or infection results in sickling in the lungsChest pain, fever, cough, tachypnea, wheezing, hypoxiaRepeated episodes may lead to pulmonary hypertensionoTX: Aggressive treatment of infectionPossibly prophylactic with oral Penicillin from age 2 months to 5 yearsMonitor reticulocyte count regularly to evaluate bone marrow functionBlood transfusion, if given early in crisis, may reduce ischemiaExchange transfusion may be appropriate in some situationsFrequent transfusion leads to hemosiderosis (iron in tissues)Treat with iron chelation (same treatment for lead poisoning)oParenterally – Deferoxamine (Desferal)oOral—deferasirox or deferiproneoUsed alone or in combination
o+ Vitamin C to promote iron excretionMonitor child’s growth – watch for failure to thriveAssess pain and hydration status, Minimize CrisesoManaging Pain in Sickle Cell DiseasePrompt Treatment of pain, Position of comfort, ROM exercises, Warm compress, Rest and gentle massageHemophiliaA group of hereditary bleeding disorders that result from deficiencies of specific clotting factorsS/S:oBleeding tendencies range from mild to severeoSymptoms may not occur until 6 months of ageoHemarthrosis = Bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobilityoEcchymosis (bruising), Epistaxis (nose bleed)TX lead forward and pinch nose because risk of chokingoBleeding after proceduresMinor trauma, tooth extraction, minor surgeriesLarge subcutaneous and intramuscular hemorrhages may occurBleeding into neck, chest, mouth may compromise airwayTreatment:oDDAVP (Desmopressin) – SteroidGiven IV, Used for mild hemophiliaCauses 2 to 4 times increase in factor VIII activityoReplace missing clotting factorsTransfusionsoAt home with (factor 8 replacement)promptintervention to decrease complications… Following major or minor hemorrhagesGoal is to PREVENT BLEEDING!!!During a bleedoElevate and immobilize the joint, ICE, analgesics, ROM after bleeding stops to prevent contractures, physical therapy, and avoid obesity to minimize joint stressvon Willebrand Disease (vWD)A hereditary bleeding disorder involving deficiency of von Willebrand factor (a plasma protein and the carrier for factor 8 (VIII))