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patel sickle cell
Pediatric Sickle Cell Disease
| Question | Answer |
|---|---|
| hereditary hemoglobinopathy characterized by the partial or complete replacement of normal hemoglobin with abnormal hemoglobin S in red blood cells. | Sickle cell disease |
| What are the effects of sickle cells in the capillaries ? | The sickled cells are rigid and obstruct capillary blood. Microscope obstruction lead to engorgement and tissue ischemia. This local tissue hypoxia causes further sickling and ultimately larg infarctions. |
| What kind of triggers precipitate sickle cell crisis? | Any condition that increases the body's need for oxygen of alters the transport of oxygen like infection, trauma and dehydration |
| What are complications of sickle cell disease? | acute chest syndrome, pulmonary HTN, pulmonary infiltrate, infection, aplastic crisis, temporary cessation of bone marrow blood cell production. |
| Why do children with sickle cell disease have impaired immunity ? | Splenectomy in early childhood due to splenic sequestration from blood trapped in the spleen. |
| What is the leading cause of death in children with sickle cell disease? | Infections |
| What are some precipitating factors to sickly cell crisis? | fever, dehydrations, altitude, extremes in temp, vomiting emotional distress, fatigue, alcohol consumption, pregnancy, elevated hgb levels, elevated reticulocyte counts, excessive exercise or physical activity, acidosis |
| What is the life span of a sickle cell ? | 10-20 days as opposed to 120 |
| When do sickled cells resume normal shape? | when rehydrated and reoxygenated |
| In response to shortened life span of sickle cells, the bone marrow does what ? | enlarges to produce more RBC |
| continuous formation and destruction of the red blood cells contributes to what ? | sever hemolytic anemia |
| Children with sickle cell are usually asx until what age? | 4-6months |
| The most common reason for hospitalization of the child with sickle cell anemia is ________. | acute painful episodes from vaso occulusion |
| What causes the pain from vaso occlusion ? | microinfarctions and ishchemia , avascular necrosis of the bone marrow. |
| Where does the pain from necrosis manifest during a sickle cell crisis? | back, abdomen, chest, joints. Chest tightness and SOB are also sx of acute chest syndrome. |
| what is the most common type of crisis that can last for days or weeks. | vaso occlusive crisis |
| What are the precipitants of vaso occlusive crisis ? | dehydration, exposure to cold, acidosis or localized hypoxemia |
| What causes vaso occlusive crisis ? | stasis of blood with clumping in the microcirculation, ischemia and infarction. |
| What are the sx of vaso occlusive crisis ? | painful sx, fever, tissue engorgement, swelling of joints, priaprism, severe abdominal pain . |
| What happens if VOC is not tx ? | cerebral occlusion, infarction, thrombosis |
| What causes splenic sequestration ? | pooling of blood in the spleen, cardiovascular collapse can occur. |
| What are sx of splenic sequestration ? | profound anemia, hypovolemia, shock (life threatening event) |
| What is an aplastic crisis in sickle cell disease ? | increased production and destruction of RBC's triggered by viral infection or depletion of folic acid. |
| What are sx of aplastic crisis ? | profound anemia, pallor, fatigue |
| What is acute chest syndrome ? | most common cause of hospitalization, most common in 2-4 years, abnormal cells infiltrate into lower resp tract |
| What are signs and sx of Acute chest syndrome ? | fever, cough, chest and back pain, dyspnea, hypoxemia |
| What are complications of acute chest syndrome ? | pulmonary infection, infarct, fat embolism, leading to pulmonary failure and death. |
| diagnostic tests in newborns is ________ and for quick screening in children over 6 months is __________. | cord blood with hgb electropheresis, sickle turbidity test or sickledex |
| Clinical therapy focuses on | pain control, hydration, infections mangement, bed rest to reduce energy expenditure. |
| __________ is usually ordered for a child in pain with sickle cell disease. | morphine PCA |
| ___________ is also important to reduce pain since that is also the cause of crisis. | fluid replacement (dehydration might cause sickle cell crisis) |
| _________ also decrease the _________ of the blood . | fluids , viscosity |
| __________ is also administered to provide comfort and reduce pulmonary complications. | oxygen |
| _________ 125mg twice daily is recommended for children from 2m-3y. Double the dosage at 3-5y | penicillin VK (bicillin or amoxicillin injections if child is allergic) |
| Vaccines should be administered as scheduled | Hib, flu. meningitis |
| How is blood transfusion for a child with sickle cell therapeutic ? | improved blood and tissue oxygenation, a reduction in sickling and a temporary suppression of the production of RBC's containing HbS |
| what are some complications of frequent transfusions ? | iron overload (iron chelating drugs (deferoxamine with vitamine c), development of alloimmunization to RBC and plt's antigens, |
| ________ transfusion is preferred in order to reduce the potential of fluid vlume excess. | Exchange |
| A cytotoxic medication that decreases production of abnormal blood cells and leads to a lesser amount of pain being experienced. | hydroxyurea |
| increases fetal hgb production and red cell mean corpuscular volume. | hydroxyurea |
| bone marrow suppression, HA, dizziness, N&V are side effects of ________ | hydroxyurea |
| Hematopoietic stem cell transplantation may be considered but there is a 10% chance of ________ . | recurrence. |
| what kind of physiologic assessment do you need ? | past crisis, precipitating events, medical tx, home management, pain, ht and wt (since FTT is common) , as about chronic or acute pain. |
| Where does pain most commonly occur ? | abdomen, extremities, head |
| What emergency sx need immediate care ? | fever, neuro changes, decreased alertness, behavior changes, resp sx, assess pain and inflammation and infection, most importantly SHOCK |
| What does nursing management for sickle cell anemia include ? | increasing tissue perfusion, promoting hydration, controlling pain, preventing infection, ensuring nutrition, preventing complications, emotional support. |
| Since child is dehydrated in sickle cell anemia it is important to _________ . | hydrate child adequately with IV fluids. monitor I and O |
| Pain medication should be _________ . | around the clock and not PRN . |
| _________ is also a good technique to relieve some pain. | repositioning, taking pressure off of joints. |
| a child with sickle cell anemia should eat a _______ and ________ diet. emphasis should be put on ______ and _______ supplements . | high protein and high calorie diet . folic acid and vitamin C |
| how can you prevent complications of crisis ? | check for increasing anemia, shock (ms change, pallor VS changes) neuro, enlarged spleen, adverse rx of blood transfusion, assess G&D milestones. |
| What is one important thing that you should teach the family about. | how to avoid triggers like avoiding hot weather, hydration, stressfull situations, |
| children should do activites that promote learning and development but avoid activities that are _______ and _________ sports. | strenuous/overexerting and contact |
| children with sickle cell disease should not engage in activities, such as ______ and __________, that may increase oxygen demand, resulting in sickling. | running and heavy exercise |
Created by:
puja
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