Increased blood viscosity due to dehydration o Hypoxia from high altitude

Increased blood viscosity due to dehydration o

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Increased blood viscosity due to dehydration o Hypoxia (from high altitude, poorly pressurized airplanes, hypoventilation, vasoconstriction due to hypothermia) Acute exacerbations that vary in severity and frequency Types o Vaso-occlusive (VOC) thrombotic Priority to promote rest o Splenic Sequestration Life threatening – death can occur within hours Blood pools in spleen o Aplastic Crises Diminished production and increased destruction of RBC Triggered by viral infection or depletion of folic acid Signs include profound anemia, pallor Acute Chest Syndrome (similar to pneumonia) WANT TO AVOID THIS with children with Sickle Cell VOC or infection results in sickling in the lungs Chest pain, fever, cough, tachypnea, wheezing, hypoxia Repeated episodes may lead to pulmonary hypertension o TX: Aggressive treatment of infection Possibly prophylactic with oral Penicillin from age 2 months to 5 years Monitor reticulocyte count regularly to evaluate bone marrow function Blood transfusion, if given early in crisis, may reduce ischemia Exchange transfusion may be appropriate in some situations Frequent transfusion leads to hemosiderosis (iron in tissues) Treat with iron chelation (same treatment for lead poisoning) o Parenterally – Deferoxamine (Desferal) o Oral—deferasirox or deferiprone o Used alone or in combination
o + Vitamin C to promote iron excretion Monitor child’s growth – watch for failure to thrive Assess pain and hydration status, Minimize Crises o Managing Pain in Sickle Cell Disease Prompt Treatment of pain, Position of comfort, ROM exercises, Warm compress, Rest and gentle massage Hemophilia A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors S/S: o Bleeding tendencies range from mild to severe o Symptoms may not occur until 6 months of age o Hemarthrosis = Bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobility o Ecchymosis (bruising), Epistaxis (nose bleed) TX lead forward and pinch nose because risk of choking o Bleeding after procedures Minor trauma, tooth extraction, minor surgeries Large subcutaneous and intramuscular hemorrhages may occur Bleeding into neck, chest, mouth may compromise airway Treatment: o DDAVP (Desmopressin) – Steroid Given IV, Used for mild hemophilia Causes 2 to 4 times increase in factor VIII activity o Replace missing clotting factors Transfusions o At home with (factor 8 replacement) prompt intervention to decrease complications… Following major or minor hemorrhages Goal is to PREVENT BLEEDING!!! During a bleed o Elevate and immobilize the joint, ICE, analgesics, ROM after bleeding stops to prevent contractures, physical therapy, and avoid obesity to minimize joint stress von Willebrand Disease (vWD) A hereditary bleeding disorder involving deficiency of von Willebrand factor (a plasma protein and the carrier for factor 8 (VIII))

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