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patel sickle cell

Pediatric Sickle Cell Disease

QuestionAnswer
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