painful edematous hands and feet. Often fist painful VOC episode in 6 months- 2 years.
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what should family do to prevent hypoxia
keep child from exercising strenuously, keep child away from high altitudes, avoid letting the child become infected and seek care at first sign of infection, use prophylactic penicillin if prescribed, keep child well hydrated, do not withhold fluids at night
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what is done for a child who is hospitalized with a vasoocclusive crisis
administer iv fluids and electrolytes, monitor i/o, administer blood products, administer analgesics, administer PCA pump, use warm compresses, and administer prescribed antibiotics to treat infection
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hemophilia
lifelong hereditary blood disorder, can be a result of spontaneously mutated genes, (x-linked recessive)
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what can be done to identify carriers
genetic testing, chorionic villi sampling and amniocentesis
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when can hemophilia be evident
after circumcision or when the child becomes mobile, bleeding can be mild, moderate, or severe
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Hemarthrosis
bleeding in the joint , can become swollen and then become imobile
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what can you notice in a nursing assessment with a patient who has hemophilia
hemarthrosis, spontaneous bleeding into muscles and tissues, loss of motions in joints, pain, prolonged bleeding into muscles and tissues
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what are some lab values you may see with a patient who has hemophilia
prolonged PTT, low factor VII and factor IX
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what are some nursing interventions that may be needed with hemophilia
no rectal temp, physical therapy can be initiated when bleeding is controlled, DDAVP is administered, injury prevention is key, use soft toothbrush, adolescents should use electric razors, avoid asprin, wear medical alert bracalet
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lead poisoning
condition involving chronic ingestion or inhalation of materials containing lead, with mental and physical dysfunction
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what should you assess when dealing with a child with lead poisoning
inquire sources of lead, signs of anemia, abdominal pain, vomiting, constipation, anorexia, headache, fever, lethargy, and central nervous system issues
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diagnostic test for lead poisoning
blood lead level, erthrocyte protoporphyrin level
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interventions for lead poisoning
prevent further exposure to lead, use only cold water, chelation therapy
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chelation therapy
process of removing lead from blood and or organs, may be painful so child is given local anesthetic prior to therapy
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neoplasm
tumor that arises from new abnormal cell growth
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benign tumor
does not reoccur
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malignant tumor
can reoccur and metastasize to other locations
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categories that predispose children to cancer
genetic, enviornmental , microbal
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s&s of cancer in children
unusual mass or swelling, unexplained paleness and loss of energy, easy bruising, presistant or localized pain, prolonged unexplained fever or illness, frequent headaches often with vomiting, sudden eye or vision changes, unexplained rapid weight loss
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why can cancer be hard to diagnose
because parents dont want to think about their children having cancer, children may mask pain and symptoms
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what is therapeutic management of cancer
surgery, radiation therapy, palliative
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what is hematopoietic stem cell transplant
replacement of stem cells in the body
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what can HSCT treat
aplastic anemia, malignant disorders, non malignant disorders, immunodeficiency disorders
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HSCT complications
graft v. host disease
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what is graft v. host disease
immune response related to difference in donor/recipient human lymphocyte antigens , the donors WBCs think the childs body is foreign and attack it
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s&s of graft v. host disease
liver (elevated enzyme levels), skin(macular skin reactions), Gi tract(mild to copious diarrhea)
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treatment of graft v. host disease
immunosuppressive drugs and steroids , (prednisone, cyclisporin, tacrolimus)
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HSCT complications
pancytopenia, mucositis
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Pancytopenia
reductions in red and white blood cells
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Mucositis
inability to eat
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if a pt has Pancytopenia what should you do
transfusions, bleeding precautions, and isolation, use electric razor, soft toothbrush, do not use tampons, do not use enemas
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if a pt has mucositis what should you do
this pt will be on iv fluids and on TPN, will also be on stool softners
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what is chemotherapy
use of antineoplastic agents to kill cancer cells
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s&s of chemo (1)
affects the hematologic system, GI tract , and skin, anorexia, nausea and vomiting (1)
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how should chemo be administered
must be given through a free flowing IV line and stopped immediately if any signs of infiltration occur
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side effects of chemo (2)
bone marrow suppression, bruising and bleeding, alopecia, malaise/fatigue, anorexia, stomatitis
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stomatitis
oral ulcers that are red, painful, and eroded in the mouth and pharynx
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interventions for stomatitis
soft sponge toothbrush, frequently rinsing mouth with chlorahexidine mouthwash or sodium bicarb and salt mouth rinses , avoid lemons due to acidity
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radiation s&s
skin reactions, bruising ,fatigue, bone marrow suppression, nausea and vomiting, anorexia
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pediatric leukemia
cancer of blood forming tissues , malignant diseases of the bone marrow and lymphatic system in which bone marrow elements are replaced by abnormal immature blast cells
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acute lymphocytic leukemia
most common form of childhood cancer, chromosome problems, prenatal exposure to radiation, snd chemo
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s&s of ALL
pallor, tiredness, weakness, lethargy, petechia, bleeding, bruising, infections, fever, bone joint pain, enlarged lymph nodes, headache, vomiting, anorexia, weight loss
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labs for ALL
bone marrow aspiration that reveals 80% to 90% immature blast cells
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what is the most definitive way to diagnose leukemia
bone marrow aspiration
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nursing interventions for leukemia
prevent infection, reduction of exposure to enviornmental molds and organisms from plants and fresh fruits, provide child with age appropriate explanations for diagnostic tests, examine child for infection, administer blood products, monitor for chemo, proper nutrition, promote self esteem, family and patient education
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hodgkins disease
cancer in the lymph nodes
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what is hodgkins disease charcterized by
reed sternberg
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Hodgkin’s disease s&s
enlarged firm, nontender, moveable nodes often in supraclavicular or cervical area
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Hodgkin’s disease s&s if metastasized
fever, weight loss, night sweats, cough, anorexia, nausea
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diagnosis of hodgkin’s disease
lymph node biopsy , reed sternberg cells
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diagnosis of hodgkin’s disease if metastisized
CBC, liver function, CT/MRI, chest X-ray, bone marrow biopsy
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Hodgkin’s disease treatment
radiation , involved field, extended field, total nodal irradiation, and chemo
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involved field
specific nodes
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extended field
involved nodes plus those immediately adjacent
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osteosarcoma
most common bone cancer in children due to growth, peaks at ages 10-25 and more than half occurs in femur
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s&s of osteosarcoma
pain, edema, limping, inability to bear weight, palpable mass, and fracture
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diagnosis of osteosarcoma
biopsy, X-ray, MRI, to rule out metastasis there needs to be a whole body PET scan, CT scan
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osteosarcoma treatment
surgery (amputation) , chemo,
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phantom limb pain
tingling, itching, and pain felt in amputated limb