Pediatric Sickle Cell Disease
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show | Sickle cell disease
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What are the effects of sickle cells in the capillaries ? | show 🗑
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What kind of triggers precipitate sickle cell crisis? | show 🗑
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show | acute chest syndrome, pulmonary HTN, pulmonary infiltrate, infection, aplastic crisis, temporary cessation of bone marrow blood cell production.
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show | Splenectomy in early childhood due to splenic sequestration from blood trapped in the spleen.
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What is the leading cause of death in children with sickle cell disease? | show 🗑
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What are some precipitating factors to sickly cell crisis? | show 🗑
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What is the life span of a sickle cell ? | show 🗑
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When do sickled cells resume normal shape? | show 🗑
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In response to shortened life span of sickle cells, the bone marrow does what ? | show 🗑
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show | sever hemolytic anemia
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Children with sickle cell are usually asx until what age? | show 🗑
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show | acute painful episodes from vaso occulusion
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What causes the pain from vaso occlusion ? | show 🗑
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show | back, abdomen, chest, joints. Chest tightness and SOB are also sx of acute chest syndrome.
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show | vaso occlusive crisis
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show | dehydration, exposure to cold, acidosis or localized hypoxemia
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show | stasis of blood with clumping in the microcirculation, ischemia and infarction.
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What are the sx of vaso occlusive crisis ? | show 🗑
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What happens if VOC is not tx ? | show 🗑
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What causes splenic sequestration ? | show 🗑
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What are sx of splenic sequestration ? | show 🗑
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What is an aplastic crisis in sickle cell disease ? | show 🗑
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What are sx of aplastic crisis ? | show 🗑
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show | most common cause of hospitalization, most common in 2-4 years, abnormal cells infiltrate into lower resp tract
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What are signs and sx of Acute chest syndrome ? | show 🗑
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What are complications of acute chest syndrome ? | show 🗑
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show | cord blood with hgb electropheresis, sickle turbidity test or sickledex
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show | pain control, hydration, infections mangement, bed rest to reduce energy expenditure.
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__________ is usually ordered for a child in pain with sickle cell disease. | show 🗑
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___________ is also important to reduce pain since that is also the cause of crisis. | show 🗑
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show | fluids , viscosity
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__________ is also administered to provide comfort and reduce pulmonary complications. | show 🗑
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_________ 125mg twice daily is recommended for children from 2m-3y. Double the dosage at 3-5y | show 🗑
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Vaccines should be administered as scheduled | show 🗑
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How is blood transfusion for a child with sickle cell therapeutic ? | show 🗑
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what are some complications of frequent transfusions ? | show 🗑
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show | Exchange
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show | hydroxyurea
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show | hydroxyurea
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show | hydroxyurea
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show | recurrence.
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show | past crisis, precipitating events, medical tx, home management, pain, ht and wt (since FTT is common) , as about chronic or acute pain.
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Where does pain most commonly occur ? | show 🗑
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show | fever, neuro changes, decreased alertness, behavior changes, resp sx, assess pain and inflammation and infection, most importantly SHOCK
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What does nursing management for sickle cell anemia include ? | show 🗑
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Since child is dehydrated in sickle cell anemia it is important to _________ . | show 🗑
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show | around the clock and not PRN .
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show | repositioning, taking pressure off of joints.
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show | high protein and high calorie diet . folic acid and vitamin C
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show | check for increasing anemia, shock (ms change, pallor VS changes) neuro, enlarged spleen, adverse rx of blood transfusion, assess G&D milestones.
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What is one important thing that you should teach the family about. | show 🗑
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children should do activites that promote learning and development but avoid activities that are _______ and _________ sports. | show 🗑
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children with sickle cell disease should not engage in activities, such as ______ and __________, that may increase oxygen demand, resulting in sickling. | show 🗑
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