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Pediatric Sickle Cell Disease

Quiz yourself by thinking what should be in each of the black spaces below before clicking on it to display the answer.
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Question
Answer
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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