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155 Cards in this Set

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a disorder in which the body launches an immunologic response against itself.
autoimmune disorder
a type of medication therapy in which there is a binding of a metallic ion with a structure so that the ion is inactivated (such as iron)
chellation
the production of erythrocytes (RBCs)
erythropoiesis
production of blood cells, normally occurs in the bone marrow but may occur in extramedullary sites
hematopoiesis
breakdown of RBC's
hemolysis
focal or general increase in tissue iron stores without associated tissue damage
hemosiderosis
process of vasoconstriction and coagulation to stop bleeding
hemostasis
reduction in all types of blood cells
pancytopenia
immature RBC
reticulocyte
In fetal life, various tissues produce RBC's but after birth, their production is controlled exclusively by by the _____ _____, particularly in the _____ bones.
bone marrow
long
The function of RBC's is to transport oxygen to the tissues. Essential to this ability to carry oxygen is an appropriate amount of _______, whose production depends on sufficient amounts of circulating ____.
hemoglobin
iron
Iron is absorbed from dietary intake by the intestines and is stored by the _____.
liver
What stimulates the production of RBCs?
What organ(s) is stimulated and what what hormone is then produced?
a decrease in circulating oxygen
The kidneys are stimulated to produce erythropoietin. This hormone stimulates the production of RBC precursors and casuses them to mature rapidly.
Disorders of the ____ can affect an individual's ability to produce erythropoietin and thus can affect RBC production by the bone marrow.
kidney
The life span of erythrocytes in neonates is shorter/longer than in older infants and children because of increased/decreased destruction during rapid growth.
shorter
increased
Where are some places hematopoises takes place?
Hematopoises primarily in the marrow of the ribs, sternum, vertebrae, pelvis, skull, clavicles, and scapulas.
___________ can occur as a result of hypoxia, such as that experienced at high altitudes or when oxygen is not sufficiently directed to the tissues, as in cyanotic heart disease. This is a ________ mechanism,
polycythemia
compensatory
____ or leukocytes are formed in the bone marrow and in _______ tissue. They assist in the body's ability to distinguish "self" from "nonself."
WBC's
lymphatic
______ are the cells that promote hemostasis- the prevention of blood loss. They are formed in bone marrow from megakaryocytes.
platelets
Platelets can circulate in the blood for about ___ days before they die; however, disease, fever, and infection can shorten a platelet's lifetime. Platelet disorders occur when the bone marrow cannot meet the production demands of the body.
10
______ are made in the thymus and are responsible for cell mediated immunity.
T- Cells
______ are responsible for humoral immunity and produce antibodies.
B Cells
____ _____ _____ are lymphocyte-like cells that can kill certain types of tumor cells and viruses directly.
natural killer cells
_____ end in phil, while ______ don't!
Granulocytes , agranulocytes
________ fight bacteria.
neutrophils
_______ fight parasites and respond to allergens.
eosinophils
_____ activate the inflammatory process and contain histamine.
They also produce heparin and speed fat removal.
basophils
________ have a U- or kidney shaped nucleus and phagocytize large cells, including necrotic tissue. They have an important role in chronic infection.
monocytes
________ are found in the bone marrow, spleen, thymus, lymph glands, tissues, and circulation. There are B and T types.
lymphocytes
What is the most common cause of anemia during infancy, childhood, and adolescence?
iron-deficiency anemia
Several factors can contribute to iron-deficiency anemia. What are the main 3?
decreased iron intake
increased iron or blood loss
periods of increased growth
True or False: iron-deficiency anemia occurs most often in children 9 to 24 months old as maternal iron stores are depleted.
true
True or False: premature infants are less likely to have iron-deficiency anemia than term infants.
false, preterm infants are more likely
Early transition from breast milk or infant formula to ___ ____ can precipitate and chronic diarrhea with occult intestinal bleeding in children younger than 2 years old due to the exposure to a protein found in this substance/food.
cow's milk
________ are also at risk for iron-deficiency anemia because they go through an increased growth stage and often have poor dietary habits. _______ further complicates blood loss for young women.
adolescents
menstruation
Clinical manifestations of iron-deficiency anemia vary with the degree of anemia but may include which symptoms?
pallor
pale mucous membranes
pale conjunctiva
tachycardia
tachypnea
lethargy
irritablity
Before age 20, blood cells are produced mainly in what 3 bones?
ribs
sternum
vertebrae
Basophils increase when?
in the healing phase of inflammatory response
______ suppress the immune response.
lymphocytes (T & B)
____ or ________ are the cell fragments responsible for clotting and promote _______.
platelets (thrombocytes)
hemeostasis
Platelets have a short or long life span?
short
If a child is younger than ___ iron-fortified formula or breast milk with iron-fortified supplements is recommended.
12 months
How often is iron given? What are some implications?
TID
Give with Vit. C rich fluids
empty stomach (between meals)
avoid administration with milk or formula
wipe off teeth/ use straw
What do stools like when iron supplements an taken?
black, tarry
In iron deficiency anemia, what might you hear if it is severe when you listen to the heart?
murmor
If the child is unresponsive, to oral iron supplements, what can be given and how?
iron dextran (Imferon)

Given deep IM using Z track
True or False: After beginning iron supplement therapy, the reticulocyte count should peak in 4 to 30 days and the HGB levels should increase in 5 to 7 days. This is a good way to measure compliance with therapy.
False. The reticulocyte count should peak in 5 to 7 days and the HGB levels should increase in4 to 30 days.
If the child is older than 12 months, restrict milk intake to _____ oz per day or less.
24
Name some examples of iron rich foods. (9)
egg yolks
liver
meat
prunes/ dried fruit
dark, leafy green vegetables
apricots
beans
cream of wheat
iron fortified cereals
Name some sources of Vitamin C
(3)
strawberries
orange juice
tomatoes
True or False: iron deficiency anemia is most common between 4-6 months.
False, infants still have maternal iron stores at this age
True or False: blood transfusions are common therapies in iron deficiency anemia
false. Response to iron therapy can often be positively predicted and blood transfusions are rarely indicated.
What are some diagnostic evaluations for iron deficiency anemia
nutritional intake
CBC
serum ferritin level
serum iron levels
iron binding capacity
If serum iron levels are _____ iron binding capacity will be ______.
decreased
increased
3 types of sickle cell disease
sickle cell anemia
sickle beta-thalassemia
sickle C disease
In Sickle Cell Disease, a group of hemoglobinopathies, _____ hemoglobin is partially or totally replaced with ____ hemoglobin.
normal
abnormal (HbS)
IN Sickle cell disease, RBCs assume a sickle shape, which prevents them from flowing easily through the smallest/largest blood vessels.
smallest
Describe sickled RBCs
stiff (stiffen into a rod)
rigid
nonpliable
Sickled cells clump together, causing _______ in the small vessels. With reoxygenation, most of the sickled RBCs resume their normal shape. However, with repeated sickling and unsickling, the cells become irreversibly ____ and their life span is reduced from 120 days to ___ days.
occlusions
12
Sickled cells cause microvascular occlusion, leading to tissue ______, i_____, and organ _____.
ischemia, infarct, organ damage
What are some causes of cell sickling?
acidosis
decreased oxygenation
dehydration
What 3 organs are the most seriously affected by the complications of sickle cell disease?
lungs, spleen, brain
What is the spleen's normal function? The spleen of a child with sickle cell disease does not function much after what age?
to filter bacteria in the blood

5
What is the most frequent complication and reason for hospitalization in sickle cell disease?
vaso-occlusive crisis
How does someone get sickle cell disease? What ethnicities most commonly present this disease?
inherited autosomal recessive

African-American
Mediterranean
Indian
Middle Eastern
All of the clinical manifestations of Sickle Cell Disease are a result of the obstructions caused by the sickled RBCs and the increased destruction of sickled and normal RBCs caught in microcirculation obstructions. What are some of the clinical manifestations of sickle cell disease? (9)
chronic hemolytic anemia
pallor
jaundice
fatigue
renal dysfunction
retinopathy
delayed growth & puberty
cholelithiasis (gall stones)
Avascular necrosis of hips & shoulders
Symptoms of sickle cell disease are not usually present until what age? (hint: when infant begins to manufacture hemoglobin)
4 to 6 months
an acute episodic exacerbation of sickle cell disease is known as a what?
sickle cell crisis
What are some precipitating factors to a sickle cell crisis?
infection
dehydration
hypoxia
trauma
stress

D- SHIT!
With sickle cell disease, are reticulocyes high or low?
high because of the chronic loss and destruction as a result of the shorted life span of the RBCs
In sickle cell disease, the _____ does not function properly or has been surgically removed because of complications. Surgical removal of this organ places the patient at high risk of what?
spleen

infection because they are in an immunocompromised state
Because people with sickle cell disease have an improperly functioning spleen (if they have one) they are at greater risk of Strep. pneumoniae and H. influenzae. These are usually destroyed by the spleen. Because of this, these people should get what?
routine vaccines against H. influenzae, pneumococcal polyvalent vaccine, as well as hepatitis B.
Also penicillin V therapy is recommended for all children with suspected or actual diagnosis to prevent pneumonia.
What are the 3 types of sickle cell crises?
vaso-occlusive crisis
acute- sequestration crisis
aplastic crisis
describe a vaso-occlusive crisis
pain in joints & limbs
chest syndrome
dactylitis
cerebrovascular infarction (CVA caused by vaso-occlusion of vessels in brain)
what is dactylitis?
swelling of hands or feet, pain, warmth in affected area
What is chest syndrome?
chest pain, fever, cough, abdominal pain
Describe a acute sequestration crisis
pooling of blood in spleen causing sleep enlargement
hypovolemic shock (can be life threatening)
acutely- ill looking child
pallor
irritability
Describe an aplastic crisis
decreased RBC production causes profound anemia

can be caused by exposure to toxic chemicals, ionizing radiation, some antibiotics, viral infections
symptoms include pallor, lethargy, headache, fainting
_______ can be the first sign of bactermia.
fever
A child with SCD and a temperature of ____ or higher should receive prompt medical attention because of the risk for infectious complications.
38.5 C
Diagnosis of SCD is made at newborn screening in the first __ days of life. Tests can include a ___ and a hemoglobin _______.
1-7
CBC
electrophoresis
Therapeutic management for SCD includes what?
prophylactic penicillin (antibiotic therapy)
hydration- oral & IV
analgesics- NSAIDs, IV opioids
RBC transfusion
Rest
oxygen (keep above 95%)
PHARRO
Nursing considerations for a patient with SCD include strict ____, pain management, hydration, and monitor the child for signs of _____ and monitor the child's condition for complications. Should warmth or cold be used to soothe pain?
I & O
infection
warmth (not cold)
Teach patient & families with SCD about the need for follow up appts. and the prevention of precipitating causes such as (3)
avoid low oxygen environment
avoid infection, get immunized
prevent tissue deoxygenation
What is the opioid of choice for treating pain in a sickle cell crisis?
What should be avoided?
morphine
(opiods and NSAIDS are recommended)

Tell patients to avoid aspirin and don't take antipyretics for fever until directed by a doctor.
Beta-Thalassemia is an _____ caused by diminished _______ of beta ________ _____ of ________.
How does one get this disorder?
an autosomal recessive disorder

an anemia caused by a diminished synthesis of beta polypeptide chains of HGB
Beta-Thalassemia is also known as ...
thalassemia major
cooley's anemia
What ethnicities is Beta-Thalassemia found in?
primarily Mediterranean
also has been reported in Asian and African populations
A buildup of iron in organs such as the spleen, liver, pancreas, skin and heart is known as _________
hemosiderosis
Symptoms of Beta-Thalassemia include...
bronze skin tone
growth and maturation retardation
characteristic facial features
hepatosplenomegaly
anemia (severe)
pallor

BiG CHAP
Explain the pathophysiology of Beta-Thalassemia
a defect in beta chain of HGB synthesis which leads to the RBCs being hemolyzed as they are produced which frees large amounts of iron into the bloodstream. This causes hemosiderosis. The excessive erythropoises leads to thin, fragile bones. Hepatosplenomegaly occurs due to hemosiderosis.
What are some characteristic facial features of Beta-Thalassemia
enlarged head
protruding forehead
prominent maxillary (upper lip)
wide set eyes
flattened nose
green/yellow skin tone (bronze?)
The major complication of long term transfusion therapy for the tx of Beta-Thalassemia is ________. To prevent organ damage from excessive iron overload, ____ therapy is initiated. What medications are we talking about?
hemosiderosis
chelation
deferoxamine (Desferal) IV or SQ
desferasirox (Exjade) PO children over 2
The diagnostics for Beta-Thalassemia include exam of RBCs show they are m_____ and h______, reticulocyte count to see how fast ___ is being made, serum iron levels, total ___ binding capacity, and ________ electrophoresis.
microlytic, hypochromic
RBC
hemoglobin
true or false: hemoglobin electrophoresis determines the type of hemoglobin that is deformed.
true
management of Beta-Thalassemia includes
erythrocyte transfusion
chellation therapy
splenectomy
In erythrocyte transfusions for Beta-Thalassemia, _____ provides a RBC a longer life.
neocytes
If there is a splenectomy, what are the 3 recommendations?
-immunizations
-prophylactic penicillin
-aggressive antibiotic therapy for febrile illnesses
What is the only known cure for Beta-Thalassemia?
bone marrow transplant
Hemophilia is a lifelong blood disorder with no cure. How does one get this disorder?
It is hereditary
x-linked autosomal recessive
Hemophilia involves a deficiency in what?
the factor necessary to coagulate blood
Name the 2 types of hemophilia and their deficiency.
classic hemophilia (hemophilia a)
deficiency in factor VIII (75%)

Christmas disease (hemophilia b)
deficiency in facor IX (15%)
What are the s/s of hemophilia?
bruise easily
epistaxis (bloody nose)
hematuria (maybe)
prolonged bleeding (with pokes/cuts/tooth loss etc)

Recurrent bleeding (hemarthrosis) in same joint
Swelling, pain, bleeding
stiffness in joints/ muscles
Diagnosis for hemophilia includes a history of ______; prolonged ___, ____, and _____ time, _____ level, ____ count, and factor ___ & ___ assays.
bleeding
prothrombin time (PT)
partial thromboplastin time (PTT)
bleeding
fibrinogen
platelet
XIII & IV
What is the therapeutic management for a patient with hemophilia?
factor replacement (recombinant antihemolytic factor)
DDAVP - for milld A (synthetic ADH)
Avoid activities that induce bleeding, avoid aspirin
How do we treat a bleeding part?
RICE
For a patient with hemophilia, apply gentle pressure for 10 to 15 minutes to superficial wounds and assess area for bleeding. Pressure facilitates what? Patients with hemophilia should be caught up on their what?
clot formation

immunizations
Severe side effects of chelation therapy that should be reported to the primary HCP can include...
hearing loss, ringing in the ears, fever, diarrhea, visual disturbances, allergic reactions, respiratory compromis
True or False: Von Willebrand's Disease (VWD) is the most commonly inherited bleeding disorder and includes at least 20 subtypes.
true
True or False: Von Willebrand's Disease is an x- linked autosomal recessive disorder and only affects males.
False.
It is an autosomal dominant disorder and affects both males and females
Children with Von Willebrand's Disease have either __________ or _______ of von Willebrand's ______
underproduction or dysfunction
protein
VWD protein occurs together with factor ____ in circulation, making it a carrier for this factor.
VIII
One of Vonn Willebrand's protein's important functions is to bind and attract ____ to the endothelial tissue injury, thus facilitating the formation of a ____. Deficiency of Von Willebrand's protein may result in a corresponding ______ of factor VIII.
platelets
clot
deficiency
Clinical manifestations and the need for treatment for Von Willebrand's Disease vary depending on the severity of the disorder. What are some clinical manifestations?
-epistaxis
-bleeding from gums
-prolonged bleeding from cuts
-excessive bleeding after surgery or trauma
-menorrhagia in females
In diagnosing Von Willebrand's Disease, a thorough history will ascertain whether or not episodes of bruising are proportional to thedegree of trauma. A family history of _____ disorders is relevant. Lab tests may include a bleeding time and PTT, which will likely be _____. What is the most clinically significant lab test for diagnosing the disorder? What will it show?
bleeding
normal

quantitative immunoelectrophoretic assay, which will reveal a discrepancy in the quantity and function of Von Willebrand's factor in the plasma.
The therapeutic management for Von Willebrand's Disease is aimed at controlling bleeding episodes and replacing the missing or dysfunctional factor in the blood. What is the treatment? What are 2 nursing implications for this disease?
DDAVP- IV or intranasally
(vasoconstricts)

-wear a medic alert bracelet
Avoid NSAIDS and ASA
(thins blood)
With all blood disorders, the patient should wear a ____ _____ ______ and should avoid ____ and _____ (medications).
medic alert bracelet
aspirin, NSAIDS
If DDAVP is administered during a bleeding episode for Von Willebrand's Disease, the parent should monitor the child's ____ intake for 24 h following administration to prevent ______, a complication seen in young children.
fluid
hyponatremia
Thrombocytopenia is when platelet count falls below _____ per mm3
150,000
Immune thrombocytopenic purpura (ITP) is an _____ hemorrhagic disorder characterized by ______, a _____ rash, ______ bone marrow, and the absence of signs of other identifiable causes of _________.
acquired
thrombocytopenia
purpura
normal
thrombocytopenia
Immune thrombocytopenic purpura is classified as either acute, or chronic. How is each defined?
acute- less than 6 months
chronic- greater than 6 months
What season(s) is Immune thrombocytopenic purpura most prevalent?
late winter and spring
While acute Immune thrombocytopenic purpura affects males and females equally, chronic Immune thrombocytopenic purpura affects _____ more often.
females
The etiology of Immune thrombocytopenic purpura is unknown, but in the majority of children, it follows a _____ illness and is considered to be an _______ process.
viral
autoimmune
What are some s/s of Immune thrombocytopenic purpura ? 3
-sudden onset of bruising & petechiae
-bleeding of mucous membranes
-bleeding of gums

(otherwise in good health)
How is Immune thrombocytopenic purpura diagnosed?
low platelet count, < 50,000 /mm3
normal physical exam

may be hx of recent live virus vaccination, illness, fever, bruising, petechiae, purpura,
Immune thrombocytopenic purpura results in destruction of circulating ____ and decreased ____ _____ production of new platelets.
platelets
bone marrow
The greatest risk of a low platelet count is _______ ________, so a _______ assessment is important. This is especially important if platelets fall below _____.
intracranial hemorrhage
neurologic
20,000
The therapeutic management for Immune thrombocytopenic purpura includes monitoring the ______ count, and medications such as _____ and _____ . If the ITP is still not controlled, a _____ may be indicated.
platelet
IVIG
steroids (methylprednisone, prednisone)
splenectomy

Steroids block the autoimmune destruction of platelets.
True or False: Immune thrombocytopenic purpura is usually self limiting
true
If a splenectomy is indicated, avoid until after age ____ and there needs to be adequate measures taken to prevent infection.
5
What should be avoided if receiving IG (immunoglobulin)?
live viruses because the IG can suppress the normal immune response
In treating Immune thrombocytopenic purpura, oral steroids are taken at what dose?
1-2 mg/kg/day
True or False: steroids need to be weened off gradually.
true
_____is an acquired hemorrhagic syndrome that involves overstimulation of clotting and anticlotting process in response to a disease or injury (caused by something else). There is excessive bleeding and excessive clotting at the same time.
disseminated intravascular coagulation (DIC)
What is the leading cause of DIC?
sepsis
What are some other causes of DIC?
disease
injury
shock
trauma
hypoxia
burns
poisonous snake bite
necrotizing entercolitis
viral or bacterial infection
pregnancy induced
Explain the pathophysiology of DIC
A clotting mechanism is triggered
--> increase thrombin, and rapid fibrinogen converts to fibrin
--> destruction of platelets, then obstruction and necrosis of vessel
-->fibrinolytic mechanism activated with less clotting factors available
-->leads to hemolysis of RBC's

basically a hyperstimulation of clotting mechanism and anti-clotting mechanism where clots are destroyed faster than they can be produced. Eventually run out of clotting factors
What are some initial clinical manifestations of DIC?
Excessive bruising
Petechiae
Purpura
Mild GI bleeding
Bleeding/oozing from mild tissue trauma such as IM injections, NG insertion site, catheter site
What are some signs of worsening DIC?
worsening of bleeding
progressive organ failure
intracranial hemorrhage
hemoptysis
hypoxia
(all leads to organ failure)
what is hemoptysis?
the expectoration of blood or blood streaked sputum
What are some confirmatory diagnostic lab findings for DIC?
platelet count?
RBC count?
fibrinogen level?
PT time?
level of fibrin degradation products?
You will see what on the smear?
decreased RBC count
decreased platelet count
decreased fibrinogen level
Prolonged PT
Elevated levels of fibrin degradation products.
RBC fragments on smear
What is an example of a fibrin degradation product?
d dimer
_______ are the substances left behind whet clots dissolve in blood (relating to DIC)
fibrin degradation products
Therapeutic management for DIC includes treating the underlying cause. Treatment and nursing considerations? (hint: mnemonic is 5 letters)
HAVAS
Heparin (sometimes, it’s controversial)
Administer blood- platelets, plasma, RBC (rapid mass transfusion)
Vitamin K
Avoid tissue trauma, monitor labs, support family
Strict I & O
True or False: patients with DIC are usually in an outpatient setting and the nurse must monitor vital signs, look for signs of bleeding, and administer medications as ordered.
False. These patients are in the ICU. Everything else is true.
What is the opioid of choice for treating acute pain in DIC?
morphine
What is aplastic anemia?
a condition in which the bone marrow stops producing the cells it normally makes, resulting in pancytopenia
How is aplastic anemia contracted?
congenital or acquired
congenital- Fanconi syndome
acquired- drugs, chemicals, radiation exposure, viruses
idiopathic- no known cause in 70% cases
What is pancytopenia?
reduction of all blood cell types
What are the clinical manifestations of aplastic anemia?
(7)
A PEEP FIT
anorexia
petechiae (low platelet)
ecchymosis (low platelet)
epistaxis (low platelet
pallor (anemia)
fatigue(low O2)
infection (low WBC)
tachycardia (compensating)
Explain what is required for diagnosis of aplastic anemia
1. granulocytes <500
2.platelets < 20,000
3.reticulocytes <1%
4. bone marrow sample: <25% of normal cellularity
two of these must be present for diagnosis, one must be #4
____ ____ biopsy for aplastic anemia should reveal the presence or absence of precursors of the mature cells found in a peripheral blood sample. In aplastic anemia, these precursors are notably absent from the sample. This type us described as hypocellular and often contains a predominance of lymphocytes and yellowish fatty tissues.
bone marrow
Therapeutic management for aplastic anemia is based on the severity of the symptoms. If there is exposure to a known causative agent, the exposure is discontinued immediately. Describe some treatment options.
Platelet & RBC transfusion
Bone Marrow transplant
Stem Cell transplant
(if matches found)
If matches are not found,
immunosuppresive therapy
cyclosporine, antithymocyte globulin effectively treat the condition
Name some Nursing considerations for aplastic anemia..
infection & bleeding precautions...
no rectal temps, meds, or exams
no injections
extra soft toothbrush
avoid contact sports
infection precautions- private room, screen visitors for illness, visitors/hcp may need to gown up,