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295 Cards in this Set
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what is palliative care
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focus on relieving symptoms in order to maximize quality of life and optimize function
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palliative care is commonly used to describe care when
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at the end of life
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when is end of life care used
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when an illness/condition is no longer responsive to curative treatment
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what kind of treatment is used with end of life care
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aggressive
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end of life care is what
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active
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what are some Issues related to transition to EOL care
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Timing - dependent on illness, circumstances, desires of the family
Symbolic vs. direct communication Conflicting perspectives – child, family, care team Responsibility for care – familiar vs. new caregivers |
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what are the critical elements of EOL care
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Shared decision making
Symptom management Supporting social relationships Assisting with living Assisting with dying Post-death bereavement support |
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what is the goal of palliative care
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relieving suffering
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what are some Common EOL symptoms in children
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pain
dyspnea agitation mood disorders (depression, anxiety) neurological distress (seizures, tremors) |
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what is the symptom management for EOL care
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No holds-barred approach: anything and everything is acceptable if it’s in the service of relieving suffering
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where are the cellular components of the blood produced from
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Produced from pluripotent hematopoeitic stem cells in the bone marrow
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what are the cellular components of the blood
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Erythrocytes (red blood cells)
Leukocytes (white blood cells) Platelets |
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what are some problems seen with hematologic function
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production
maturation destruction |
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how do you assess hematologic function
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CBC - complete blood count
Hemoglobin electrophoresis Bone marrow aspiration/biopsy |
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what is done from the treatment of a hematological problem
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correction of underlying problem
management of clinical manifestations |
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transfusion therapy is typically directed toward what
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at a single cell line
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how are PRBC given
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1 unit harvested from whole blood ~ 250 cc
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what is the amount of PRBC given dependent on
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weight/goals of treatment
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how are platelets usually harvested
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typical adult transfusion harvested from 8 units whole blood – “random donor” or “pooled”
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multiple units of platlets are harvested from single donor via what
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plasmapheresis
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what is the function of erythrocytes
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transport oxygen to the tissues
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erythrocyte production is regulated by what
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tissue oxygenation
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what is hemoglobin
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the oxygen-carrying protein molecule
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what is hematocrit
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% of RBC’s in whole blood
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what is reticulocyte count
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% of progenitor cells in circulating blood
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what can be a cause of anemia
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Deficiency
Abnormal morphology |
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what are the types of deficiency that can cause anemia
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decrease in production
blood loss increase in destruction (hemolysis) |
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what are the types of abnormal morphologies
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sickle cell diseases
thalassemia |
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what are the consequences of anemia
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Decreased oxygen-carrying capacity
Hemodilution Growth retardation (chronic) |
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what does decreased oxygen-carrying capacity lead to
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tissue hypoxia
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what does tissue hypoxia cause
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weakness
fatigue pallor |
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what does Hemodilution lead to
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increased cardiac load
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what does an increase in cardiac load lead to
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tachycardia
murmur |
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when is iron deficiency anemia most common
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Common nutritional deficiency between 6 & 24 months
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what can cause iron deficiency anemia in infants
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Depletion of maternal iron stores
Under-nourishment Excessive milk consumption |
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what makes adolescents at risk for iron deficiency anemia
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Decreased intake
Rapid growth Menorrhagia |
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what screening is done for iron deficiency anemia
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Decrease in Hgb/Hct
Decrease in serum iron concentration Increase in total iron-binding capacity growth developmental delays |
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what is the dietary management for iron deficiency anemia
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limited milk intake
iron-fortified infant foods (cereal, formula) foods naturally high in iron |
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what is the iron supplementation for iron deficiency anemia
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oral iron supplements: 3-6 mg/kg/day
IM iron dextran: reserved for chronic severe anemia |
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what is the education for iron deficiency anemia
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Limiting milk intake
Introduction of solid foods Administration of oral supplements |
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what is a sickle cell disease
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Inherited disorder of hemoglobin production
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what are the normal forms of hemoglobin
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Hgb A – normal
HgbF – fetal hemoglobin |
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what is the Abnormal hemoglobin seen in SCD
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HgbS – tendency to sickle (assume abnormal shape)
HgbC – less likely to sickle than hgbS |
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what kind of inheritance of SCD
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Autosomal recessive
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what is HbSS
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most severe (homozygous) form
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what is HbSC
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heterozygous form, milder pathology
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what is HbSA
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sickle cell trait; 8-12% incidence in Americans of African descent
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when are clinical Manifestations of HbSS apparent
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Usually not apparent until fetal hemoglobin abates (4 - 6 months)
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what are precipitating factors that lead to a vasocclusive crises
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dehydration
infection/fever increased altitude |
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what are symptoms caused by
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Ischemia in affected tissues
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what are the symptoms cause by ischemia in affected tissues
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PAIN
swelling specific to tissues affected |
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what are the Common manifestations of acute vasocclusive episodes
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extremities – distal swelling, warmth, loss of function
priapism – penile engorgement pulmonary infiltrate – respiratory compromise stroke – may or may not be symptomatic |
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what are the types of life threatening sickle cell crisis
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splenic sequestration
acute chest syndrome (ACS) |
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what causes splenic sequestration
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large volume of sickled cells trapped in spleen
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what does a splenic sequestration cause
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splenomegaly, hypovolemia which leads to shock
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what happens in acute chest syndrome
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rapid onset, severe respiratory compromise
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what is the chronic organ damage that happens with sickle cell disease
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lungs → pulmonary hypertension
kidneys → progressive renal disease spleen → functionally asplenic by ~ 3 years cirrhosis brain – multiple CVA’s musculoskeletal – avascular necrosis |
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what is the life expectancy of a person with sickle cell disease
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50-60 yrs
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what is the supportive care for acute manifestations of sickle cell disease
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hydration
pain management antibiotics to treat/prevent infection +/- transfusion, oxygenation |
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what is done for infection prophylaxis in SCD
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early vaccination (pneumococcus, meningococcus)
oral penicillin |
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what are the therapeutic modalities for SCD
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Hydroxyurea, Tranfusion therapy, and Hematopoietic stem cell transplantation (HSCT)
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what is hydroxyurea
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chemotherapeutic agent
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what does hydroxyurea do
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stimulates production of hgb F, suppresses sickling
very low rate of side effects/long term complications at HbSS doses significantly reduces risk of stroke |
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what does transfusion therapy do
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maintain adequate levels of normal hemoglobin, reduce likelihood of sickling
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what does a Hematopoietic stem cell transplantation (HSCT) do
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donor cells migrate to marrow and produce normal hematopoiesis
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what is a main social issue with treating SCD
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perceived risk of addiction for pain
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what are the physical developmental delays with SCD
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reduced linear growth, delayed pubertal development
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what are the cognitive developmental delays with SCD
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recurrent strokes → cognitive deficits, academic disabilities
psychosocial |
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what is ß-Thalassemia
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Autosomal recessive inheritence - deficiency in the synthesis of ß-polypeptide chain of Hgb molecule leads to instability & destruction of RBC’s which leads to excessive erythropoeisis
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what are the three forms of thalassemia
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Thalassemia minor (trait)
Thalassemia intermedia Thalassemia major (Cooley Anemia) |
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how is the diagnosis of thalassemia confirmed
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Hgb electrophoresis
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what is the goal of thalassemia management
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maintain normal Hgb levels
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what is done for the management of thalassemia
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Transfusions to maintain Hgb > 10 g/dl
Chelation for iron overload (Desferal) HSCT |
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what do leukocytes do
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Protect the body from invasion of foreign organisms
Distribute antibodies & other immune factors |
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what are the two types of leukocytes
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granulocytes (neutrophils, eosinophils, basophils)
agranulocytes (lymphocytes, monocytes) |
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leukocytes relative % are reported on what
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the differential
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what is the Major function of granulocytes
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phagocytosis of foreign cells and substances, particularly bacteria
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what do neutrophils do
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neutrophils most important for fighting bacterial infection
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what do monocytes do
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responsible for phagocytosis of bacteria and cellular debris
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what do lymphocytes do
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they are not phagocytic but protect against specific antigens
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what do T-lymphocytes to
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synthesize cytotoxic agents and stimulate macrophage production (cell-mediated immunity)
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what do B-lymphocytes do
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produce immunoglobulins and release antibodies into the bloodstream (humoral immunity)
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Acquired neutropenic abnormalities result most commonly from what
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infection, cancer, or cancer treatment
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what is the consequence of altered neutropenic cells
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immune compromise
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what is a result of immune compromise
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Decreased ability to fight bacterial infection
Susceptibility to morbidity from common (e.g., varicella) or opportunistic (e.g., p. carinii) infections |
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what is the management of neutropenia
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Transfusion of WBC’s seldom indicated
Prevention of infection Hand washing Monitor contacts with infectious individuals Rapid initiation of antibiotics with fever or other signs of infection |
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what are Primary (congenital) immunodeficiencies
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Severe combined immunodeficiency disease (SCID)
Wiskott-Aldrich syndrome |
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what are Secondary (acquired) immunodeficiencies
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Human immunodeficiency virus (HIV)
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what are the types of Congenital Immunodeficiency:SCID
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X-linked recessive, autosomal recessive, & sporadic forms
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what happens in SCID
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Mutations of cellular receptors to interleukin
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what happens when there are Mutations of cellular receptors to interleukin
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decreased T-cell production & defective B-cell function
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what is the life expectancy of SCID without treatment
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2 years
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what is the treatment of choice for SCID
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allo-BMT; experimental gene transfer studies on-going
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what is the Most common route of infection in young children
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perinatal
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how is HIV diagnosed in infants
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less than 18 months complicated by persistence of maternal antibodies
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what can diagnose infants with HIV by 1 month
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PCR (polymerase chain reaction)
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Classification of severity for HIV is based on what
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both on clinical presentation and evidence of CD4 suppression
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what are the clinical manifestations of HIV
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FTT
development delays organomegaly persistent/recurrent infections marrow suppression |
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what is done for the management of HIV
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Antiretroviral drugs to slow the growth of the virus
Preventing & treating opportunistic infectionsSupportive care/symptom management Life-long adherence required |
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what are some predictors of non adherence
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biological parent as primary caregiver
anxiety/depressive symptoms Increase in age Increase in duration of treatment |
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what are platelets
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Fragments of megakaryocytes produced in the marrow and stored in the spleen
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what do platelets do
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Adhere to the inner surface of a damaged vessel to form a homeostatic plug
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what initiates the clotting cycle
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Degradation
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what causes thrombocytopenia
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Can be caused by deficient production (marrow suppression) or by excessive destruction
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when is transfusion indicated for thrombocytopenia
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Transfusion indicated only for acute life-threatening bleeding episodes or prophylaxis when prolonged marrow suppression anticipated
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what is Idiopathic Thrombocytopenic Purpura (ITP) characterized by
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normal production and increased destruction of platelets
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what is the cause of ITP
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Exact etiology unknown - probably precipitated by an autoimmune response
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what does ITP commonly follow
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acute viral infection
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what is the highest incidence age of ITP
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2 - 8 years
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what is the management of ITP
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Treatment minimized as disease is usually self-limiting & treatments often don’t change the course of recovery
Restricted activity until platelet count > 50,000 Splenectomy for persistence > 1 year |
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what is pantocytopenia
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aplastic anemia
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what happens in pantocytopenia
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All cell lines are simultaneous depressed; marrow is virtually empty of cellular components
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Fanconi’s anemia is present when
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at birth
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what is pantocytopenia
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aplastic anemia
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what can cause acquired aplastic anemia
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infection (parvovirus, heptatitis, unspecified viral)
chemicals (household, chemotherapeutic) radiation idiopathic |
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what happens in pantocytopenia
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All cell lines are simultaneous depressed; marrow is virtually empty of cellular components
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what is the management of aplastic anemia directed towards
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Directed at restoring function to the marrow
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Fanconi’s anemia is present when
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at birth
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what the the management of aplastic anemia
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Immunosuppressive therapy to remove presumed immunologic etiology
Limit transfusions - may sensitize to WBC & HLA antigens & limit therapeutic options HSCT |
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what can cause acquired aplastic anemia
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infection (parvovirus, heptatitis, unspecified viral)
chemicals (household, chemotherapeutic) radiation idiopathic |
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what is the immunosuppressive therapy used for aplastic anemia
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Anti-thymocyte globulin (ATG) - suppresses T cell-dependent autoimmune responses
Cyclosporine, high dose steroids |
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what is the management of aplastic anemia directed towards
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Directed at restoring function to the marrow
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what are clotting factors
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Complex system of clotting, anti-clotting, and clot breakdown mechanisms are designed to ensure clot formation only in the presence of blood vessel injury
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what the the management of aplastic anemia
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Immunosuppressive therapy to remove presumed immunologic etiology
Limit transfusions - may sensitize to WBC & HLA antigens & limit therapeutic options HSCT |
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what are some alterations in clotting
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Deficiencies or abnormalities in specific factors (chronic bleeding disorders)
Inappropriate systemic activation & acceleration of the normal clotting mechanism (DIC) |
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what is the immunosuppressive therapy used for aplastic anemia
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Anti-thymocyte globulin (ATG) - suppresses T cell-dependent autoimmune responses
Cyclosporine, high dose steroids |
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what are clotting factors
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Complex system of clotting, anti-clotting, and clot breakdown mechanisms are designed to ensure clot formation only in the presence of blood vessel injury
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what are some alterations in clotting
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Deficiencies or abnormalities in specific factors (chronic bleeding disorders)
Inappropriate systemic activation & acceleration of the normal clotting mechanism (DIC) |
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what is the most common type of hemophilia
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80% X-linked recessive & therefore occur only in males
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what are the types of X-linked hemophilias
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Hemophilia A (Factor VIII deficiency)
Hemophilia B (Factor IX deficiency) |
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what is von Willebrand disease
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hemophilia that is autosomal dominant and affects both males & females
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hemophilia has a high rate of what
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spontaneous mutation
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what are the manifestations of hemophilia
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Clots cannot form or remain unstable
Bleeding can occur anywhere but is common in joint spaces (hemarthrosis) and at the site of tissue injury (including surgery) Spontaneous bleeding can occur without known trauma Epistaxis, mucosal membrane bleeds, hematuria |
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what can follow chronic hemarthrosis
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Limited motion, bone changes, contractures
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Life-threatening bleeds include what
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neck, mouth, chest (airway obstruction), retroperitoneal, and head
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Serious bleeding is uncommon when with hemophilia
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in the first year of life
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Mild/moderate cases of hemophilia may not be diagnosed until when
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later in childhood with trauma or surgery
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when is the management for hemophilia
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Factor replacement therapy
Joint care during and following bleed prevent bleeding maximize self care at home |
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when are factor replacement therapies used for hemophilia
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Acute or life-threatening bleeds
Prophylaxis in severe cases DDAVP in mild cases |
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what is the type of joint care used in hemophilia
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RICE
Supervised physical therapy to strengthen muscles and maintain function |
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what are some known exposure risks to childhood cancers
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diagnostic irradiation
medications (DES, chemotherapy) specific viruses (EBV) |
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what are the treatment modalities for cancer in children
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Chemotherapy
Radiation Surgery Hematopoeitic stem cell (bone marrow) transplantation |
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Antineoplastic agents are used in which type of treatment
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chemotherapy
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Antineoplastic agents are used to treat what
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malignancies
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what does chemotherapy do
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Takes advantage of rapid rates of cellular division
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what types of intensity is used in chemotherapy
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Maximum dose intensity to avoid development of resistance
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what are common regimens for chemotherapy
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combination regimens
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what are the advantages of combination regimens
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maximize cell-cycle specific cell kill
synergy minimize specific organ toxicity |
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what are the Most common dose-limiting toxicities
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myelosuppression
nausea/vomiting stomatitis/mucositis end-organ toxicities (heart, liver, lungs) |
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what are the Principles of Nursing Management for Patient on Chemotherapy
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Pre-treatment assessment
Dose calculation & verification Safe handling & administration Monitoring for acute and delayed onset toxicities Patient & family education |
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what is the Pre-treatment assessment for chemotherapy
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baseline laboratory findings
allergies/prior reactions interactions with other medications tolerance of previous courses self-care skills |
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how are doses of chemotherapy calculated
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body surface area
body weight age |
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what is done for safe handling and administration of chemotherapy
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exposure risks
disposal of wastes intravenous administration of vesicants and irritants documentation |
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what is done for Monitoring for and preventing toxicities for chemotherapy
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adverse reactions
side effects hydration status organ function |
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what is done for Patient and family education for chemotherapy
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“information overload”
expected side effects with time frames supportive medications precautions home care when to call the treatment team |
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what does ionization cause in DNA
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Ionization causes single-strand or double-strand breaks in DNA
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what happens to the cell when ionization is done
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inability of cells to divide
inability to repair DNA damage cell death |
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side effects of radiation are dependent on what
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location and dose
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what is essential for children receiving radiation
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Reproducibility of the treatment field
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immobilization for radiation may require what for radiation
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Immobilization may require conscious sedation or anesthesia
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what are the side effects
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myelosuppression
skin breakdown fatigue/post-radiation somnolence syndrome late complications, including diminished organ function, cognitive limitations, second cancers |
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the primary goal of surgery may be what
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diagnostic or therapeutic
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what is the rationale of Hematopoeitic Stem Cell Transplantation
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to overcome myelosuppression as dose-limiting toxicity and/or to restore normal marrow function
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Ablation of the marrow is achieved with what
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chemotherapy +/- radiation
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how are stem cells infused
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peripherally (regardless of harvest mechanism) and migrate to marrow beds to resume hematopoeisis
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what are some life threatening complications of HSCT
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pancytopenia
end-organ toxicities graft vs. host disease |
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what happens in pantocytopenia
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infection and bleeding
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what are the Principles of HSCT Nursing
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Intensive supportive care during period of pancytopenia
Prevention/ameliorating of other end-organ toxicities Management of acute & chronic GVHD Intensive outpatient management continues long after discharge from hospital Chronic immune deficiency and sequelae to therapy profoundly impact children’s daily lives |
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what causes myelosuppression
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Caused by chemotherapy, radiation, infiltration of marrow with cancer cells
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what is Nadir
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period of time when blood counts are at their lowest
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what is the timing and duration of nadir dependent on
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timing and duration dependent on agent(s), dose, intensity
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what is done for the prevention of infection
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avoid potential contacts
frequent handwashing good oral hygiene colony stimulating factors (G-CSF) to reduce duration of nadir prophylaxis against pneumocystis carinii (PCP) |
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what is done for the management of infection
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initiate broad-spectrum antibiotics for fever during period of neutropenia
monitor for subtle signs/symptoms |
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where are the most common documented infections
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lung
soft tissues mucosa blood |
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what is the most distressing side effect of cancer treatment
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nausea/vomiting
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when is nausea and vomiting most common
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Common with many chemotherapy agents
Can also follow radiation, especially GI or CNS |
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what are the types of nausea and vomiting
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acute – during or immediately following chemo
delayed – starting or persisting beyond 24 hrs anticipatory – conditioned response when n/v poorly controlled with prior courses |
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what is the goal with nausea/vomiting
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prevention
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what is the Emetogenic potential
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likelihood that specific agents will cause vomiting (low, moderate, high)
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what is done for agents with moderate or high emetogenic potentials
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administer anti-emetics before, during, and beyond chemotherapy regimen
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what are some additional toxicity's of cancer treatment
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constipation
diarrhea mucositis/stomatitis fatigue hair loss weight loss/nutritional deficits |
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what are 5 essential strategies for caring for children and their families
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education
supportive presence active monitoring/vigilance technical competence advocacy |
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what is leukemia
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Cancer of the blood-forming tissues
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what happens in leukemia
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unrestricted proliferation of immature WBC’s that infiltrate and replace normal body tissues
proliferating cells depress production of normal blood cells in the marrow |
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major presenting symptoms of leukemia are secondary to what
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marrow suppression (infection, anemia, bleeding)
tissue infiltration (pain, organomegaly) |
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how are leukemia's classified
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Classification according to leukocyte cell line of origin
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what are the types of leukemia's
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Lymphocytic (ALL, CLL)
Myelocytic (AML, CML) |
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how is leukemia diagnosed
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by bone marrow aspirate/biopsy
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what do bone marrow biopsys look at
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Cytochemical, chromosomal, immunologic markers
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what is the most common childhood malignancy
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ALL
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when is the peak for ALL
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early childhood (2 - 5 years)
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classification of ALL is based on what
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Classification according to lymphocyte cell line of origin
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what is the most common classification of ALL
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pre-cursor (progenitor, early) B cell
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what are the rare types of ALL
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T-cell and B-cell ALL more rare
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what is done for the treatment of ALL
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Combination chemotherapy, 3 phases:
Intrathecal chemotherapy - administered into CSF via lumbar puncture HSCT |
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what are the 3 phases of chemotherapy for ALL
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induction (to achieve remission)
intensification/consolidation (to eradicate residual leukemic cells, particularly in CNS) maintenance (preserve remission & reduce risk of relapse) |
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HSCT is reserved for who in ALL
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refractory/relapsed disease
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Intensified CNS treatment in ALL poses risk of what
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cognitive sequelae – decreased academic ability, ADHD, other learning disabilities
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what is the peak age of AML
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Age peaks < 2 years and in adolescence
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what may distinguish AML from ALL
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higher white count, spontaneous bleeding, soft tissue infiltrates (chloromas) may raise index of suspicion
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how is AML classified
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Classified according to specific myeloid cell line affected (M1 - M7) but treatment is typically the same
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what is induction therapy like in AML treatment
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intensive to the point of ablation
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what is the intensity like like AML treatment
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Intensification is shorter and very intense
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children with what go right to BMT
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with HLA-compatible sibling
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what are the most common solid tumors in children
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CNS tumors
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what are the most common types of CNS tumors
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60% develop in the posterior fossa (cerebellum, brain stem), 40% are supratentorial (cerebrum
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treatability of CNS tumors are a function of what
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Treatability is a function of both grade (rate of growth) and location (surgical resectability)
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what are common diagnosis of CNS tumors
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Medulloblastoma/PNET (cerebellum)
Astrocytomas/gliomas (supra- or infra-tentorial) Ependymoma (4th ventricle) Brainstem gliomas (Pons) Spinal cord tumors |
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what is the goal of surgery for a CNS tumor
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Goal is most extensive resection possible while preserving neurological function
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when is surgery curative for CNS tumors
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Curative only in low-grade tumors with clear demarcation
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what is a problem with radiation in CNS tumors
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Irradiating developing brain-cognitive impairment
growth retardation sensory impairment |
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how is chemotherapy commonly used in children with CNS tumors
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Usually part of multi-modal therapy
Can be used to delay radiation in young children Intensive chemotherapy followed by auto-BMT in aggressive tumors |
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when do the majority of bone tumors occur
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in the 2nd decade of life
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bone tumors are almost always what
|
primary tumors of bone (not metastases)
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what are the common presentations of bone tumors
|
Most common presentation is pain, with or without soft tissue mass
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what can limit treatment options for a bone tumor
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Pathological fracture
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what is common with bone tumors
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metastatic disease
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where does Osteogenic sarcoma most commonly occur
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in the long bones
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osteogenic sarcoma may be associated with what
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with pubertal growth spurt (earlier onset in girls, prolonged incidence in boys)
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osteogenic sarcoma commonly metastasizes where
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to the lungs
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where does ewings sarcoma most commonly occur
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– occurs most commonly in extremities or pelvis and involves axial skeleton
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where does ewings sarcoma typically metastasize to
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to lungs, marrow, other bones
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what is the goal of surgery for bone tumors
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obtaining clean, wide margin of normal tissue while providing optimum function
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when is amputation indicated for bone tumors
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“expendable” bones
skeletal immaturity preservation of maximum function |
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what are limb sparing surgeries
|
affected bone(s) removed and replaced with internal prosthesis
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what is pre-operative chemotherapy used for in bone tumors
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to reduce tumor bulk and immediately treat micro-metastatic disease
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when does post-operative chemotherapy occur for bone tumors
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chemo resumes ASAP following wound healing
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what are lymphomas
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Neoplastic disorders arising from lymphoid and hematopoeitic systems
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what are the classifications of lymphomas
|
Hodgkin’s Disease (HD)
Non-Hodgkin Lymphomas (NHL) |
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when is hodkin's lymphoma rare and when does it increase
|
Rare before age 5, increased incidence after age 15 and into young adulthood
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Hodkin's lymphoma primarily involves what
|
lymph nodes organ involvement (spleen, liver, marrow, lungs) is not uncommon
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staging of hodgekin's lymphoma is based on what
|
based on extent of disease (I-IV) and presence of clinical symptoms
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what are the symptoms of hodgkin's lymphoma
|
fever, night sweats, weight loss
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what are the treatments for hodgkin's lymphoma
|
radiation
chemotherapy choice of modalities based on extent of disease, balancing curative goals with concerns about long-term toxicities |
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what are some life threatening emergencies associated with NHL
|
obstruction
metabolic (tumor lysis syndrome) |
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what can precipitate a life threatening emergency in NHL
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“bulky disease” (mediastinal, abdominal, marrow) and rapid cell reproduction
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what is the treatment for NHL
|
Multi-agent chemotherapy indicated even for limited-stage disease
Radiation therapy limited to chemotherapy-resistant disease |
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what is the Most common soft tissue sarcoma in childhood
|
Rhabdomyosarcoma
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|
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where does Rhabdomyosarcoma typically arise from
|
immatural mesenchymal cells in skeletal muscle lineage, but can arise in any soft tissue
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what is the peak incidence for Rhabdomyosarcoma
|
Peak incidence < 6 years (esp. genitourinary, orbital) and adolescents (extremities, paratesticular)
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the presentation of Rhabdomyosarcoma is based on what
|
anatomical location of primary tumor, metastases, age
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|
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what is the treatment for Rhabdomyosarcoma
|
Multi-agent chemotherapy
Complexity and duration of treatment dependent on stage Radiation Timing and dose dependent on site Surgery Primary modality only if wide resection is possible “Second look” to determine response to treatment |
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what is Neuroblastoma
|
Peripheral nervous system tumor arising along the sympathetic chain
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|
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what are the most common places for neuroblastoma tumors
|
Most commonly abdominal (adrenal), cervical, thoracic, and pelvic chains
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2/3 of neuroblastoma patients present with metastasis where
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to marrow, bone, liver, lymph nodes
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neuroblastoma symptoms may mimic what
|
leukemia symptoms
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|
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neuroblastoma may do what in infants
|
May spontaneously regress in infants
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neuroblastoma is rare in who
|
Rare in children > 10 years
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|
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what is the treatment for neuroblastoma
|
Multi-modal therapy dictated by stage
Intensive chemotherapy/radiation regimen for high-risk disease +/- hematopoeitic stem cell transplant “Second look” surgery to debulk remaining tumor and assess response to therapy Infants with 4S disease may be monitored with supportive care only, treated only for life-threatening complication |
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what is a wilms tumor
|
Primary malignant renal tumor
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what is the peak age for a wilms tumor
|
age 2 - 3 years
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a small percentage of wilms tumors are associated with what
|
Small percentage associated with congenital anomalies, familial predisposition
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what is the most common presentation of wilms tumors
|
Most commonly presents as asymptomatic abdominal mass, less commonly with hematuria, malaise, hypertension
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what is the treatment of wilms tumors
|
Surgery - goal is to completely resect tumor without causing capsule rupture (nephrectomy)
Chemotherapy - intensity and duration dependent on stage Radiation - reserved for stage III & IV patients Bilateral disease requires delayed surgical intervention and intensive chemotherapy to preserve renal function |
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what is Retinoblastoma
|
Rare intraocular tumor
|
|
|
90% of patients with Retinoblastoma are diagnosed when
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by 5 years
|
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what is the treatment of Retinoblastoma
|
Goal is cure (> 90%) while maximizing useful vision
Surgery (enucleation), cryotherapy, photocoagulation (laser) Radiation - may be used to preserve vision in multifocal disease Chemotherapy - advanced disease, neo-adjuvantly to reduce tumor burden and/or delay radiation |
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|
diabetes is a chronic disorder of what
|
carbohydrate metabolism
|
|
|
diabetes has a partial or complete deficiency in what
|
insulin
|
|
|
what is type 1 diabetes
|
autoimmune disorder that destroys pancreatic islet cells; 75% diagnosed in childhood
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|
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what is types 2 diabetes
|
insulin resistance with or without deficiency; increasing incidence of childhood/ adolescent onset
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|
|
what is the cause of type 1 diabetes
|
defect on chromosome 6
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|
|
onset of type 1 diabetes usually follows what
|
precipitating event, most often viral infection
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|
|
types 1 diabetes is associated with what
|
Association with other auto-immune disorders
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|
|
types 2 diabetes may be associated with what
|
insulin-resistance syndrome and obesity
|
|
|
what happens in the cells when insulin is missing
|
glucose can't enter the cells
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|
|
what does hyperglycemia cause
|
osmotic gradient that draws fluid out of the intracellular space into the blood stream with increased renal excretion (polyuria)
other essential elements pulled along and excreted (particularly potassium) |
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|
without glucose for energy, what is used for fuel
|
protein and fats are metabolized for fuel which leads to ketosis
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|
|
progressive deterioration from diabetes leads to what
|
dehydration, ketoacidosis, hypokalemia, coma and death
|
|
|
what are long term complications of diabetes
|
With poor diabetic control, microvascular compromise results from protein/glycosol deposits in endothelium of small vessels
nephropathy retinopathy neuropathy |
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|
what reflects post-pubertal duration
|
Degree of vascular compromise
|
|
|
what are the cardinal symptoms of diabetes
|
weight loss
polyphagia polyuria polydipsia |
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|
what are other symptoms of diabetes
|
abdominal discomfort, nausea
fatigue slowed healing eneuresis |
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|
replacement of insulin is what for diabetics
|
life long requirement
|
|
|
what are the 3 types of preparations of insulin
|
rapid
intermediate long-acting |
|
|
how are the classifications of insulin used
|
combined to maintain BG 80-120 mg/dL
|
|
|
what is the goal for BG monitoring
|
independent self-monitoring and adjustment to maintain optimal control
|
|
|
what is Hemoglobin A1c (glycosylated Hgb)
|
reflects average BG levels over previous 2 - 3 months
|
|
|
what is the ideal value for hemoglobin A1c
|
less than 7
|
|
|
when is urine stick testing done
|
if there is poor control of BG to check for ketones
|
|
|
what is the nutritional management for diabetics
|
supervision by registered dietician
caloric intake should balance energy expenditures and meet needs for growth timing of food intake coordinated with insulin regimen frequent meals, snacks intake before and during exercise well-balanced diet limit concentrated sweets & fats counting carbohydrates maintain dietary fiber |
|
|
what may precipitate ketoacidosis if diabetes is not in good control
|
vigorous exercise
|
|
|
when do you delay vigorous exercise
|
delay until BG < 240 and no ketonuria
|
|
|
what is used for insulin injection
|
non-exercised extremities for insulin injection
|
|
|
episodes of hypoglycemia represent what
|
incompatibility between insulin regimen and dietary intake
|
|
|
what happens in hypoglycemia
|
impaired brain function & increased adrenergic stimulation
behavior changes pallor, sweating palpitations weakness, drowsiness, change in LOC, coma |
|
|
what happens in an insulin reaction
|
severe CNS compromise
|
|
|
what is done to treat hypoglycemia
|
Administer simple carbohydrate
concentrated sweet milk or juice Repeat and/or provide more complex carbohydrate source Glucagon/IV glucose with loss of consciousness Monitor BG and adjust insulin dosing |
|
|
what is diabetic ketoacidosis
|
complete state of insulin deficiency
|
|
|
what is done to treat hypoglycemia
|
Administer simple carbohydrate
concentrated sweet milk or juice Repeat and/or provide more complex carbohydrate source Glucagon/IV glucose with loss of consciousness Monitor BG and adjust insulin dosing |
|
|
what are the symptoms of DKA
|
dehydration
electrolyte imbalance - hypokalemia cardiac arrhythmias metabolic acidosis with compensatory Kussmaul respirations cyanosis risk of cerebral edema |
|
|
what is diabetic ketoacidosis
|
complete state of insulin deficiency
|
|
|
what is done for DKA
|
Immediate intervention/intensive care
|
|
|
what are the symptoms of DKA
|
dehydration
electrolyte imbalance - hypokalemia cardiac arrhythmias metabolic acidosis with compensatory Kussmaul respirations cyanosis risk of cerebral edema |
|
|
what is done for DKA
|
Immediate intervention/intensive care
|
