Hematology/Immunology Flashcards
1
Q
Anemia is the ?
and in conjuction does what?
A
Reduction in RBCs mass and/or hemoglobin concentration
- Decrease in the Hgb available to carry O2 to cells
2
Q
Main causes of anemia
A
Inadequate production of RBCs
- Does not enough made
Increased destruction of RBCs
- Kills them too fast
Excessive lossof RBCs (Trauma)
3
Q
Anemia s/s
A
fatigue, high heart rate and BP, tachypnea, pale and cold, low cap refill, blue extremities (extreme),
blood count – low RBCs
4
Q
RBCs carry
A
O2 and nutrients
5
Q
Low RBCs cause the body to
A
- pumps blood faster
- causes demand for more O2 in the body
6
Q
Retic Count
A
what the body does in an anemic state (High = increased RBCs, low = decrease RBCs)
7
Q
Reticulocyte
A
last stage of development in RBCs
8
Q
Erythrocyte is
A
RBCs
9
Q
Erythrocyte life span = _____ days
A
120
10
Q
homeostatic balance of RBCs
A
RBC production = RBC destruction
11
Q
RBCs transport what to the cells
A
hemoglobin to cells O2
12
Q
Hemoglobin is composed of what 4 globulin chains?
A
Fetal Hgb: 2 α and 2 y globin chains
Later in pregnancy, adult Hgb: 2 α and 2 β globin chains
13
Q
fetal defect in Hgb synthesis present (e.g., Sickle Cell Disease, Beta Thalassemia Major) = Fetal Hgb
A
may be produced into adulthood
14
Q
What disease kills the erythrocytes too fast and they live less than 120 days?
A
sickle cell anemia
15
Q
Why is fetal hemoglobin used in hemolytic diseases like sickle cell anemia?
A
Fetal hemoglobin and O2 have a pos. charge joining together and grabbing each other
- help compensation
16
Q
Basic regulators of RBC production
A
Tissue oxygenation
Kidney production of erythropoietin**
17
Q
Chronic kidney disease can produce anemia, why?
A
kidney is unable to make erythropoietin and help production of RBCs
18
Q
As RBCs age, the membrane
A
ruptures
19
Q
When hemoglobin is broken down, what is released?
A
Iron pigment (Hemosiderin)
Bile pigment (Bilirubin)
20
Q
Hemosiderin most reused in
A
bone marrow for RBC production
21
Q
Bilirubin is excreted by what organ after the rupture of hemoglobin?
A
liver in bile
22
Q
When too many RBCs are broken down, then what can occur in relation to bile and iron?
A
jaundice from too much bile and the body has excess iron in blood leading to organ dysfunction and impact the brain
Elation = getting rid of excess iron = storage = liver
23
Q
Anemia assessment
A
pallor, fatigue, weakness, tachycardia, increased respiratory rate, dizziness, headache (no O2), irritability
24
Q
Severe anemia assessment
A
hyperventilation
25
Children with anemia are prone to
infection
26
Infections worsen
anemia
27
Normal Ranges of Anemia
- newborn
- infant
- child
- adult
Newborn – 14-24
Infant – 10-17
Child – 9.5-15.5
Adult – 12-18
- the younger the child the more hemoglobin needed
28
Iron Deficiency Anemia (IDA)
**Hgb Levels Below Normal** Range
Because Of Body’s **Inadequate Supply, decreased Intake, chronic blood loss Or lack of Absorption Of Iron**
29
Iron Deficiency Anemia (IDA) causes
**Inadequate Supply during fetal development
decreased Intake,
chronic blood loss
Or lack of Absorption Of Iron**
- last trimester in pregnant women = iron goes to the baby
30
Iron Deficiency Anemia (IDA)
children at high risk
- Preterm infants, multiple births (don’t get the iron stores or not getting enough bc sharing with siblings)
- Mother has iron deficiency
- Infants 6 to 24 months old (growth spurts)
- Toddlers (don’t eat enough and picky eaters)
- Female adolescents (puberty periods and growth spurts, not great diets)
31
Iron Deficiency Anemia (IDA) s/s
**Pallor/paleness of mucous membranes
Tiredness/**fatigue**
32
Iron Deficiency Anemia (IDA)
- possible findings
Overweight **“milk baby”**
Dietary intake low in iron
Milk intake > 32 oz./day
**Pica habit** (eating nonfood substances) = trying to get iron from other sources
33
Milk baby
drinking too much cow's milk
chunky overweight
not good source of iron
34
Iron Deficiency Anemia (IDA) Diet
Iron fortified formula or cereal
Dietary addition of iron-rich foods
35
Iron Deficiency Anemia (IDA) Tx
Oral iron supplements
- Ferrous sulfate (Fer-in-Sol)
36
Iron Deficiency Anemia (IDA)
Tx for severe anemia
Blood transfusions
**Packed RBCs** to minimize chance of circulatory overload
Tissue hypoxia – **supplemental O2**
37
Iron-rich foods
green leafy veggies, red meat (steak and liver), fish, legumes
38
Ferrous sulfate (Fer-in-Sol)
side effects
Upset GI, feces black/tarry/dark green, constipation (increase fluids and fiber),
39
How long do you take Ferrous sulfate (Fer-in-Sol) until mature RBCs
3 months (120 days) for mature RBCs to venture out
1 month have H&H see changes
If not chronic bleeding or iron disorder
40
Iron supplement Administration
Give on **empty stomach**
Give with citrus juices **(vitamin C)**
- Increases absorption of iron in bloodstream
Use **dropper** or straw
**Stain the teeth
Stools will be tarry**
Safety
No overdose, stored correctly and **out of reach**
**NO milk = Decrease absorption**
41
During iron-deficient anemia, limit milk to
< 32 oz per day
and dietary sources of iron
42
If iron-deficient anemia is severe and longstanding, what is the prognosis?
Diminished cognitive function
Behavioral changes
Delayed growth and development
(extreme = death)
43
Sickle Cell Anemia
**Autosomal Recessive Genetic Disorder** That Results In The **Formation Of Abnormal Hemoglobin Chains**
- Normal Adult Hemoglobin A (HbA) Is Partly Or Completely Replaced By Abnormal Sickle Hemoglobin (HbS)
44
Under Certain Circumstances, Abnormal RBC “Sickles” cell anemia, results in
**Occlusion Of Small Blood Vessels, Ischemia, And Damage To Affected Organs**
45
If both parents have the sickle cell trait, what are the percentages of the child?
is 25% not have it at all, 50% trait carrier, 25% have disease
90,000-100,000 in the US have it
46
What race and gender is more likely to have sickle cell?
AA at risk, males more than females,
lifelong
47
What causes the cells to sickle?
stress
48
Sickle cell anemia causes
occlusion of small vessels (pile up)
leads to ischemia and damage cells
49
HbS changes the molecular structure of an RBC to form what?
elongated crystals
- Cresent and distorted
50
Sickled RBC lifespan
less than 40 days
- leads to chronic anemia
51
Sicle cell anemia s/s are not usually seen until
after 1st 6 months of life
52
Fetal hemoglobin does not
sickle
- reason to continue giving in later years
53
Triggers to sickling
Dehydration
Acidosis
Hypoxia/Hypoxemia
Temperature changes (winter cold)
Emotional stress
Infection
54
Effects of Sickling
Abnormal adhesion, entanglement, and enmeshing of rigid sickle-shaped RBCs
↓
Inflammatory process
↓
Intermittent **blocking of microcirculation**
↓
Vaso-occlusion
↓
Absence of blood flow to adjacent tissues
↓
Local hypoxia
↓
Tissue ischemia and infarction
55
Infarctions can occur
anywhere
56
Dx of Sickle cell prenatally
Chorionic villus sampling from prenatal tissue
Amniocentesis
57
Dx of Sickle cell after birth
1st = Newborn screening – heel stick
If positive
2nd = Sickledex (sickle turbidity test)
3rd = **If positive, hemoglobin electrophoresis = confirms** (electricity to the RBCs to see sickling
58
S/S of Sickle cell anemia
asymptomatic until 6 months
general chronic hemolytic anemia s/s
- Frequent infections
- Fatigue
- Delayed physical growth – smaller ht and wt than peers
59
Sickle Cell CRISES
acute exacerbations triggered
- Vaso-occlusive
- Splenic Sequestration
Aplastic
Hyper hemolytic
60
Vaso-occlusive crisis (VOC) in sickle
observed with 4-6 days
- peripheral
- pain crisis by anemia
61
Splenic sequestration crisis in sickle cell
6 months to 5 y/o
Pooling of large amount of blood in the spleen and liver causing drastic decrease of blood volume
Hypovolemic and life threatening with death within hours without treatment
62
Aplastic crisis in sickle cell
Child has a viral illness
Causing Decreased RBCs
63
Hyper hemolytic crisis in sickle cell
Body unknown = Accelerates destruction of RBCs
64
Vaso-occlusive crisis (VOC) classic s/s
**Acute Pain – 15/10**
Fever
Severe abdominal pain
Painful **edematous** hands and feet
Hand-Foot Syndrome (infants)
**Arthralgia** = pain In joints
**Leg ulcers (adolescents) – low circulation**
Cerebrovascular accident **(CVA)**
65
What could mask as appendicitis if the pain is in the stomach area?
Vaso-occlusive crisis
66
Vaso-occlusive crisis **chronic signs**
**Splenomegaly** (enlarged spleen) → auto splenectomy (infarction of the spleen killing it)
**Hepatomegaly → liver failure**
Kidney abnormalities → **hematuria, inability to concentrate urine, enuresis, possible renal failure**
Bone changes → **osteoporosis, skeletal deformities**
Retinal detachment → **blindness**
67
When the spleen is in failure (auto splenectomy), the person has an increase of
infection
68
Additional complications of sickle cell in Chest Syndrome s/s
Fever ≥ 101.3°
Cough
Chest pain
Tachypnea
Dyspnea
Wheezing
Decreased O2 saturations
69
Acute Chest Syndrome
blockage of blood flow to the lungs
70
If the acute Chest Syndrome is untreated, then
respiratory distress and death
71
Acute Chest Syndrome tx with
Antibiotics, O2, and blood transfusion
72
VOC Mgmt
Hydration
**Electrolyte replacement
Strict I & O**
Monitor kidneys
Analgesics – PCA pumps and narcotics
- **Warm compresses**
Blood transfusion – RBCs are in to treat
Antibiotics if infection
Hydroxyurea
- Increases fetal hemoglobin production bc it does not sickle
73
What is contraindicated in VOC management?
cold compresses - vasoconstriction and extreme cold is a trigger for crisis
74
How do you reverse sickling of sickle cell anemia?
prevent dehydration with hydration
75
VOC Pain mgmt
mild to moderate = tylenol and ibuprofen
severe = opioids (morphine, hydromorphine, hydrocodone and ketorolac)
76
ORAL AND IV pain management for VOC need to be administered when
schedule with PCA if appropriate
77
Meperidine is contraindicated in children due to
seizures
78
Education to prevent hypoxia in Sickle cell anemia by avoiding
Avoid strenuous exercise = dehydration
Avoid high altitudes = cold environment
Avoid being around anyone who is sick and **seek care at first sign of infection**
79
Education to prevent hypoxia in Sickle cell anemia by
**prophylactic penicillin** if prescribed
Prevent strep
If allergic then azithromycin
Keep child well hydrated **(do not withhold fluids at night)**
Up-to-date immunizations
Refer family for genetic counseling
Provide emotional support
80
Prognosis of Sickle cell anemia
depending upon **severity of disease**
Typically live to 50
Greater risk is < 5 with crises
- Death is related to overwhelming infection
Stem cell transplantation
81
Beta Thalassemia **Major** aka
“Cooley’s Anemia”
**Autosomal Recessive Disorder**
- both parents are carriers
82
Beta Thalassemia **Major** is deficiency (partial or complete)
Synthesis Of The β Chain Of The Hemoglobin Molecule
83
Beta Thalassemia **Major** results in
**Life-Threatening Anemia Requiring Lifelong Blood Transfusions That Lead To Iron Overload**
84
Why do transfusions not work for Beta Thalassemia **Major**?
Transfusions = RBCs breakdowns = iron overload
- fetal hemoglobin into adulthood
85
What si the Tx for Beta Thalassemia **Major**?
Keylation
86
Beta Thalassemia **Major** Patho
Dramatically decreased or complete deficiency
in the synthesis of β-chain in Hgb molecule
▼
An unbalanced polypeptide unit is very unstable
▼
The Hgb molecule disintegrates
and damages the RBC
▼
The RBC prematurely breaks down
▼
Causing severe hemolytic anemia
87
Hemolytic Anemia life span of RBC is
30-60 days
88
Beta Thalassemia **Major** s/s
Severe anemia (pale, fussy, won't eat, and infection)
Chronic hypoxia
Small stature - FFT
In some adolescents – delayed puberty
Bronzed skin tone, possibly = High Bilirubin
Hepatosplenomegaly (liver –stores extra iron and spleen = pooling of cells and stuck))
Cardiomegaly
Bone Changes – frontal boxing, maxilla, and jaw bones (untx older children) - general osteoporosis
89
Beta Thalassemia **Major** s/s is shown within how many years of life?
2 y/o
90
Labs of Beta Thalassemia **Major**
High retic count
hypoxia (low O2 sat)
91
Beta Thalassemia **Major** Dx prenatal
Chorionic villus sampling from prenatal tissue
Amniocentesis
92
Beta Thalassemia **Major** Dx after birth
1st Newborn screening
2nd CBC
3rd **Hemoglobin electrophoresis**
X-rays for bone involevement
93
The goal for Beta Thalassemia **Major** is to
maintain adequate Hgb via transfusion program
94
What is the goal level of hemoglobin for Beta Thalassemia **Major**
> 9.5
checked every 3-5 weeks for PRBCs
95
Tranfuse to do what in a patient with Beta Thalassemia **Major**?
Improve well being (physical/mental)
Normal activity
Decrease cardiomegaly/hepatosplenomegaly
Prevent bone changes
Promote normal or near-normal growth and development
Decrease infections
96
Hemosiderosis
iron overload secondary to transfusion
- deposit to tissues
97
What could possible go wrong during a transfusion?
Hemosiderosis
chelation
Hypersplenism
- Splenectomy
98
Chelating agents
**Desferal (deferoxamine)**
Exjade (Deferasirox)
99
**Desferal (deferoxamine)**
IM or IV at infusion center
SQ
Portable infusion pump for 8 hours
4 - 6 nights a week
Bind with iron and excrete in urine **(rusty colored)**
100
Exjade (Deferasirox) FOR CHILDREN AGE
PO to children ≥ 2 years of age
101
Chelation can cuase what problems and need yealry check ups/
hearing and vision
102
What is given with iron
Vitamin C
103
Splenectomy
removal of spleen
- Treats severe splenomegaly and **increases life span of transfused RBCs**
- Increases susceptibility to **overwhelming infection**
104
Splenectomy Interventions
Prophylactic antibiotics lifelong**
Up to date Routine immunizations**
s/s of infection call HCP immediately
105
Prognosis of Beta Thalassemia Major with tx
children living into adulthood with decrease life span
- **death is usually heart disease, post splenectomy sepsis, or multi-organ failure
secondary to hemochromatosis**
Usually have a heart attack or failure by 35 y/o
106
Beta Thalassemia Major possible cure
stem cell transplant
107
Lead poisoning have what amount of lead in their system?
>5 mcg
108
What is the safe level of lead in the blood system?
NONE in children
109
Lead exposure and elevated levels have been linked to
low IQs
110
Lead causes
anemia
111
Lead exposure
Ingestion – most common route
Inhalation
112
Ingestion routes of lead
Crib rails
Windowsills
Older plumbing (water source)
Contaminated soil
Paint chips (sweet taste)
And many other sources
113
Inhalation routes of lead
renovation of old homes in nearby areas
114
Risk factors of Lead Poisoning
Children < 6 years old (oral) PICA
Living in poverty
Living in urban areas
Living in older homes
Children with anemia
Lead binds to hemoglobin and retains the lead
115
Who retains more lead?
children due to WT TO BSA ration
116
Lead poisoning s/s
Anemia + s/s
**Cramping, abdominal pain**
**Vomiting**
Constipation
Anorexia
Headache
**Lethargy**
Impaired growth
117
Early (toddlers) CNS S/S of lead poisioning
Hyperactivity
Aggression**
Impulsiveness**
Decreased interest in play
Irritability
Short attention span
118
Late CNS S/S of lead poisoning
Mental retardation= decreased IQ
Paralysis
Blindness
Convulsions
Coma
Death
119
Testing for lead poisoning
BLL TEST (Blood lead level)
120
Universal screening of BLL
All children at ages 1 - 2 years
Any child between 3 -6 years if never screened
PRN if at high-risk environment
121
Target screening for lead poisoning in
If reside in high-risk geographic areas
If in “at risk” group
If family cannot respond “no” to personal risk questions
- low IQs
122
Lead poisoning Interventions
Identify sources of lead in the child’s environment
Then rid them of the source
- no vacuum windows or sill - wet mop
- wash and dry hands, toys, and pacifiers
123
Occupations with the most exposure to lead poisoning?
construction
paint
ceramics
pottery
124
When BLL is >45, what Tx needs to be done?
What is the chelator used?
Chelation → Protect Kidneys
**Succimer (Chemet)
PO for 19 days**
Capsule or sprinkle
**Calcium disodium (EDTA) IM**
**Very painful**
Atraumatic care with rotation of site, EMLA,
Multiple treatments if necessary, after blood level tests
125
The Tx for BLL over 45, does not counteract the
CNS effects already in place
126
Hemophilia
Inherited **X-Linked Recessive Bleeding Disorder**
- maternal with males having the disease
127
Hemophilia is not passed to
females do to another X chromosome to cancel out the disease one
128
Hemophilia results in
Deficiency, Dysfunction, Or Absence Of
**Coagulation Factor VIII Or Factor IX**
129
Where is the bleeding of a hemophilia pt?
anywhere in the body
- mild to severe
130
Hemophilia A
Aka?
Deficiency type?
classic neoplastic
Factor 8 Deficiency (75%)
131
Hemophilia B
Aka?
Deficiency type?
Christmas disease
Factor 9 deficiency (25%)
132
Mild Hemophilia factor production
6-50%
133
Moderate Hemophilia factor production
1-5%
134
Severe Hemophilia factor production
less than 1% most common
135
Dx of Hemophilia
hx of bleeding episodes
genetic testing
136
Labs for Hemophilia
PTT less than 25%
137
Hemophilia Assessment
**Male** child: prolonged bleeding (Vit. K, umbilical cord, post-circumcision)
**Hemarthrosis** (most frequent site of bleeding)
Knees, elbows, ankles - loss of motion in joints
**“Target joints”**
- tingling sensation
**Spontaneous bleeding** subcutaneous tissues and muscles
- brusing excess, pooling hematoma
**Spontaneous hematuria or blood in stool**
Coffee grounds in vomiting
**Frequent and no stop nosebleeds**
138
What is the most frequent site of bleeding in hemophilia?
**Hemarthrosis**
Knees, elbows, ankles - loss of motion in joints
“Target joints”
- severe pain
139
How do you stop hemophilia nosebleed?
head down with pressure for at least 10 mins with cotoon or tissue in nostril – not back) – ice to constrict vessels try to remain calm of the child
140
If repeated bleeding episodes in the joints, then
, synovial thickens and becomes target joints leading to chronic arthritis
- Joint immobility and crippling deformities
141
How to Recognize & Control Bleeding in hemophilia
Believe child (feel pressure of tingling but not outward s/s bc faster = better outcome)
RICE then medical tx - help joints only
Factor replacement per bleeding episode and prophylactically
142
Factor replacement per bleeding episode and prophylactically
how many times per week?
2-3 times
143
Factor replacement starts when
2-3 y/o done by family after a while
- more often if severe
- impanted port with edu when older
expensive only good for 2 hours after reconstituted
144
Factor Replacement is
Genetically engineered recombinant factor VIII and IX
Recommended over factor concentrate from pooled plasma
145
When the hemophilia pt is 8-12 y/o then they are able to
self-administer factor by themselves
- port-a-cath
146
DDAVP (synthetic vasopressin is used for
mild Fcator 8 deficiency
and RA
147
How to prevent bleeding in hemophilia?
Regular exercise
Soft bristle toothbrush under warm water
**Water pick** instead of floss
**Med ID bracelet**
Avoid IM injections if possible
Venipunctures instead of heel sticks/finger sticks
Educate to recognize early signs and symptoms of hemorrhage
Medication to avoid Aspirin and aspirin mixed meds
(Ibuprofen) (give acetaminophen)
148
Complication of hemophilia
Inhibitors risk
Factor specific **antibodies**
Give slower
Develop after the child has received factor replacement
149
Prognosis of Hemophilia
no cure
average life expect
150
Activity acceptable for hemophilia
Swimming, golf, cross country, bowling, band, archery, discuss
No contact sports (football, basketball, hockey)
151
Immune Thrombocytopenia (ITP)
Autoimmune Disorder
Characterized By Thrombocytopenia
152
Immune Thrombocytopenia (ITP) occurs when
**Immune System
Mistakenly Attacks And Destroys Platelets**
153
Immune Thrombocytopenia (ITP) caused by
Unknown Etiology** But Most Often
**Presents After Viral Infection**
Etiology Unknown – Often Presents After A
Viral Infection
154
Thrombocytopenia
low platelets
155
ITP bone marrow aspiration (conscious sedation) results in
normal
if abnormal then leukemia
156
ITP S/S
spots on skin
Petechiae (spider spots)
easy Bruising
Bleeding from mucus membranes
Nosebleeds, bleeding gums
Prolonged bleeding from abrasions
157
ITP peaks
2-6 y/o
158
s/s of ITP disappear within
3 weeks
159
If ITP is chronic then persists for
years
160
Testing for ITP
No definitive tests due to mimic of other disease
HMP
CBC
**Platelets < 20,000/mcL**
161
What is the platelet count of ITP
**Platelets < 20,000/mcL**
162
Normal platelet count
150,000-200,000
163
Suportive Care of ITP
**Restrict activities while platelets < 50,000**
Monitor for s/s bleeding (increase HR, RR, drop in BP if enough)
Everywhere for bleeding sites
Prednisone, immune globulin (**IVIG**), Anti-D Antibody (Wintho)
Decrease activity destroying the platelets
164
A ITP pt should restrict activity while platelets are
< 50000
165
Chronic ITP can cause what in over 5 y/o
Splenectomy
If > 5 y/o bc of bacterial infection with sepsis
Penicillin prophylaxis
166
Vertical Transmission of HIV
Late in pregnancy
Labor and delivery
Breastfeeding
ART
for pregnant mothers with HIV
167
Horizontal Transmission of HIV
Sexual contact
HIV-infected blood products (transfusion)
Adolescents - high risk behavior
Drug use
Sharing needles
168
Dx of HIV
**Maternal antibodies persist up to 18 months**
Infants born to HIV mothers will test positive
Even if they don’t have HIV,
169
If an HIV pregnant mother has a baby and the baby tests positive for HIV, does the baby have HIV?
NOT ALWAYS
170
When are extra tests done to verify if the baby is HIV positive?
18 months
Elisa or wesern blot test
171
If the baby is less than 18 months what test is run to see if they are HIV +
HIV polymerase chain reaction test
172
HIV S/S
Failure to thrive (low wt, no growth)
Lymphadenopathy (swollen nodes)
Hepatosplenomegaly (enlarged)
Neuropathy (nerve pain)
Cardiomyopathy (enlarged heart)
Chronic/recurrent infections
Oral thrush (white fuzzy in mouth)
Unexplained fevers
173
HIV Tx goals
Slow growth of HIV virus
Prevent and treat opportunistic infections
Provide nutritional support
Promote normal growth
174
Medication therapy of HIV
Antiretroviral medications
Prevent opportunistic infections (avoid crows, prophylactic antibiotics)
IV gamma globulin (IVIG)
Antibiotics = Bactrum combination : trimethoprim – sulfamethoxazole
175
Interventions of HIV
**Standard** precautions
Nutritional support
HIV can lead to FTT
Protect privacy (HIPAA)
Immunizations – **no live vaccines**
176
Childhood CA typically grow
Faster
177
T/F: Lifestyle-related behaviors have little to no effect on childhood cancer
True
not smoking, drinking, or bad diet
178
Classic s/s of childhood CA
**Unusual mass or swelling, enlarged lymph nodes**
Unexplained paleness & loss of energy
**Sudden tendency to bruise**
Persistent, localized pain or limping - bones
**Prolonged, unexplained fever or illness**
Frequent headache, often with vomiting-brain tumor
Sudden eye or vision changes – brain or eye
**Excessive, rapid weight loss**
179
Leukemia charcterized by
an **unrestricted proliferation of immature white blood cells in the blood-forming tissues**
Bone marrow
180
What is the ost common form of CA?
LEUKEMIA
181
What are the most common type of Leukemia
Acute lymphocytic leukemia (ALL)
AML as well
182
Tx of Leukemia
primary chemo
183
Leukemia Patho
Competition for nutrients, infiltration, replacement in bone marrow
▼
Decreased RBCs, WBCs, platelets **(bone marrow suppression)**
▼
Anemia, risk for infection, bleeding, bruises, bone & joint pain, weakening of the bone, physiologic fractures
184
Leukemia **blast** cells migrate to what other organs
Spleen
Liver
Lymph glands
CNS
areas with high blood flow
185
S/S of Leukemia
**Minor infection that fails to completely disappear**
Infection, fever (**r/t neutropenia**)
Pallor, fatigue, weakness, lethargy (r/t anemia)
Petechiae, bleeding, bruising (r/t thrombocytopenia)
Bone joint pain (r/t leukemic infiltration of bone marrow)
Enlarged nymph nodes, hepatosplenomegaly
Anorexia, weight loss
Headache, vomiting (if CNS involvement)
186
Leukemia is supected if
History and physical manifestations
Labs including CBC with differential
Low WBC, RBC, and platelets
187
Dx of leukemia
Bone marrow biopsy and aspiration (BMA)
Positive leukemia
Lumbar puncture
188
Higher leukocyte count at diagnosis =
worse prognosis
189
what percentage of children relapse with leukemia within the year
15-20%
190
what is the 5 year survival rate of leukemia
85-90%
191
what may occur later in life in all CA and other cells being affected by the chemo therapies?
secondary malignancies
192
When are children usually dx with leukemia
1-9 y/o
193
High WBCs count means what for tx
higher dose of chemo and higher dosage cycle
194
Neuroblastoma
**Solid tumor that forms in the developing nerve cells**, or neurons, of the **sympathetic** nervous system
Most develop in the adrenal glands, abdomen or nerve cell next to the spinal cord
195
Neuroblastoma can be seen in
head
neck
chest
pelvis
196
What is the Most common extracranial solid tumor of childhood?
neuroblastoma
197
What is the Most diagnosed cancer in children younger than 1 year of age?
neuroblastoma
1-2 y/o dx median age but can be 0-14 y/o
198
In a CT scan the neuroblaoma look like
Big dark and crosses the midline of the body (different from other tumor)
in abd
199
S/S of Neuroblastoma
Firm nontender irregular mass in the abdomen
**“Silent” tumor**
grow rapidly and space in the abdomen to grow
200
Survival depends on what in neuroblastoma
age
stage
201
If the child is under 1 y/o, the chance of survival is
80%
202
if the child is greater than 1, there chance of survival is
50%
203
If the child is in the 1st stage of neuroblastoma, then curative rate is
90%
204
____ already metastasized by found (lymph nodes, bone marrow, skeletal, and liver)
Typ. found late and prognosis is bad
70%
205
Tx of Neuroblastoma
chemo
radiation depending on where and stage
206
Wilms Tumor
Nephroblastoma
207
Wilms Tumor is
Fast growing, asymptomatic, firm, encapsulated mass located to one side of abdomen
- 1 kidney RARE ON BOTH
208
Wilms Tumor occurs most frequently in
2-5
209
Wilms Tumor grows _______ before being noticed because of
LARGE; ABD space
210
Wilms Tumor is typically found before
metastasis
211
DO NOT ___________ Wilms Tumor or what could happen?
palpate or manipulate the abd
- rupture and CA cells go everywhere
212
Wilms Tumor is what type of mass
encapsulated mass
213
Wilms Tumor Tx
surgery, chemo, radiation depending on protocol
Tumor, kidney and adrenal gland are removed in surgery
- can live on 1 kidney
214
Retinoblastoma
Cancerous tumor arising out of the retina
Usually unilateral
May be inherited
215
Retinoblastoma primarily in children
<5 y/o
216
Retinoblastoma s/s
Opaque white area in pupil* (Leukocoria, Cat’s Eye Reflex, White Pupil with flash)
the other eye has a red dot
Strabismus (cross eye)*
Decreased vision in one eye
Eye pain
Larger than normal eyeball*
Lazy eye*
Redness of the eye
217
Retinoblastoma is seen by and dx by
Ophthalmology scope stages
Ophthalmologist sees the pt as soon as possible with s/s even if unrelated to retinoblastoma
218
Retinoblastoma Treatment
Laser treatment
Cryotherapy
Chemotherapy
Radiation
Enucleation surgery (removal of the eye)
- Fitted for ocular implant and later artificial eye
- Cleaning eye (take out with warm water and mild soap and put back)
- Parent education
219
Tx of Retinoblastoma priority
Save child life
Save the eye
Save the vision
220
CA Dx
History
Physical examination
Lab tests
Imaging (Xray, CT, MRI, PET)
Procedures
Surgical pathology - Bone Marrow Aspiration and LP)
221
Spinal Tap/LP To Collect CSF Or
To Give
Intrathecal Chemotherapy
222
Phase 1 of Chemotherapy
**Goal – remission**
Time of **high risk** because medications cause **myelosuppression**
Risk of septic
**CNS prophylaxis**
Goal – kill or prevent leukemic cells in CSF
**Intrathecal administration (tx or prevent CNS)
Cranial radiation reserved for high-risk patients**
223
Chemo Phases
Induction
Consolidation
Maintenance
224
In the induction phase they want to kill what % of leukemic cells in the 1st month
99.9
225
Phase 2 of Chemotherapy
Consolidation (Intensification) (1-2 months)
Goal - destroy any residual leukemia (last 100 million left)
combo of chemos
After completion of remission therapy
226
Phase 3 of Chemotherapy
Miantenance (2-3 years)
Goal - remain in remission
After successful completion of induction & consolidation therapy
- boys have a high chance of remission
227
Risk For Injury Related To Malignant Process & Treatment
Chemotherapy - suppression infections
Radiation Therapy damage to tissue and decrease immune system
Skin breakdown and infection and bleeding
Procedures
228
Chemotherapy is used through
Central line (Broviac or Implanted Port)
229
Extravasation
(chemo leaking out into surrounding tissue)
- Pain, swelling, itchy, necrosis
230
Anaphylaxis
Signs and Symptoms
of chemo
Cyanosis
Hypotension
Wheezing
Urticaria
emergency ready - ambu bag anf suction
231
If reaction with the chemo isuspected then
stop and call
232
You need to watch chemo for how long during tx
1st 20 minutes
233
Radiation
Uses high-energy beams to destroy cancer cells
Changes DNA in neoplastic cells so that it cannot reproduce
Usually scheduled over 1 - 6 weeks
234
Radiation Checks and cautions teachings
- change linen daily
- internal (potect visits)
Targeted area/site is marked with indelible ink
No soaps, creams, lotions or powders on area
Irritate skin and misdirect the radiation
Wear soft, loose cotton clothing
No rubbing
Keep area/site protected from the sun
235
Neutrophils
fight bacterial infections
236
Each chemotherapy agent that causes bone marrow suppression has a point of
NADIR
237
NADIR is
Bone marrow suppression at its greatest, neutrophil count at its lowest
- weakest point of immune system
7-14 days
238
WBC calculation for 9000-10000
highe the infection
239
Absolute neutrophil count (ANC)
Measures the body’s ability to fight infection
240
Segs
mature neutrophils
241
Bands
immature neutrophils
242
Calculate the ANC formula
Neutrophils (Segs + Bands) x WBC x 10
243
If the ANC is less than 1500 then
evaluation
244
If the ANC is less than 500
greatest risk for infection
245
Remember that when we place a patient with a low ANC in isolation
, we are protecting them from us, not us from them.
246
Normal ANC
1500
247
Infection Prevention
Monitor for signs and symptoms of infection
Private room with **restricted visitors**
**Reverse/protective isolation** is prescribed
**No fresh fruits or vegetables**
**NO Pets at home or flowers**
No live vaccines - immunosuppression
Administer prescribed prophylactic medications
Antibiotics
Antifungals
248
Nurse considerations when taking care of a CA t
Nurse can not be pregnant
Other patients should not be infectious
249
T/F: Family members should get live vaccines to protect the child
True
250
Risk for bleeding Preventions
Monitor for signs of bleeding
Avoid unnecessary skin punctures
Use aseptic technique
For fingersticks, venipunctures, IM injections
Meticulous mouth care
Soft toothbrushes, toothette, no alcohol or listerine
Avoid activities that might cause injury or bleeding
No contact sports
Nosebleeds (hold pressure 10 mins, head forward, ice)
Platelets transfusion
Monitor for HA (brain bleed)
For active bleeding episodes
No ibuprofen or aspirin
251
Normal platelet count
150000-450000
252
N/V treatment
Serotonin-receptor antagonists before chemotherapy
Ondansetron (Zofran)
Granisetron (Kytril)
Aprepitant (Emend)
Administered up to 1 hour before chemo
Often combined with Dexamethasone
253
Anxiety Tx for chemo
Adivan or benadryl
254
With chemo and CA pts you should avoid food with
strong odors
255
Anorexia/Loss Of Appetite Interventions with CA complications
**Avoid Pressure** To Eat
Do Not Let Food Become A Control Issue
Small, Appealing Meals With Increased Calories and Protein
- Sores in mouth
Tube feeding
- Gives options and frequent eating
- ice cream and chicken nuggets are okay if that’s what they want
- but not no fruits and veggies
Dietary help and high protein shakes
256
Preventing Mucosal Ulceration
Offer bland, soft foods
Use a soft sponge toothbrush (toothette)
Provide frequent mouthwashes (coat)
For discomfort
Local anesthetics or nonprescription preparations without alcohol
To prevent or treat mucositis
Moisten lips
Things to avoid
Rectal ulcers (stool softeners not suppositories)
- no rectal temps
Meticulous perineal hygiene
Barrier cream
257
Do not give __________ bc suppress gag reflex avoid hydrogen peroxide and lemon glycerin swabs
Lidocaine
258
If they have mucousal ulceration then it is
althoughout there GI tract mouth to anus
259
Alopecia in child CA
Hair falls out in clumps and regrows in **3 - 6 months**
May be darker, thicker, curlier
different than when fell out
Cotton cap, scarf, hat, or wig
Protect scalp from cold and sun
No burn and lose heat that way
Scalp hygiene – soap and water
260
Alopecia in child CA hair will grow back
3-6 months with different than fell out
261
Infants and Toddlers Developmental View Of Death
No concept of death (temp separation and death are the same)
Toddlers = Recognizes death in terms of immobility often viewed as reversible, temporary, or foreign
262
Infants primary fears
"Separation
Strangers"
263
Toddlers primary fears
"Separation
Loss of control"
264
Early school-age child primary fears
"Bodily injury and mutilation
Loss of control
The unknown
The dark
Being left alone"
265
school-age child primary fears
"Loss of control
Bodily injury and mutilation
Failure to live up to expectations of important others
Death"
266
Adolscent primary fears
"Loss of control
Altered body image
Separation from peer group"
267
Preschoolers Developmental View Of Death
Death as temporary, a departure, a kind of sleep
- "Recognizes death in terms of immobility often viewed as reversible, temporary, or foreign
Begins to question and develop a mature concept
"
268
School Age Developmental View Of Death
Death is irreversible but not necessarily inevitable and may **fear mutilation**
- Recognizes all the components of irreversibility, universality, nonfunctionality, and causality
269
Adolescent Developmental View Of Death
Death is **inevitable, irreversible**
- "Speculates on the implication and ramifications of death
Understands effect of death on other people and society as a whole
Future-oriented, difficult to understand reality of death as a present possibility"
270
Infant spiritual interventions of death
"Provide consistent caregivers
Minimize separation from significant others
Decrease parental anxiety
Maintain crib/nursery as ""safe place"" no procedures
encourage parental presence
Facilitate spiritual support system for family"
271
Palliative Care Principles
Guide **realistic goal** setting that includes the **physical, emotional, social, and spiritual distress**
Multidisciplinary approach
Child life, pastoral, social service
**Child and family are the unit** of care
272
Nursing Care in end of life
Whatever they want and encourage parental involvement
Assess level of pain/administer pain med
minimal
Provide soothing surroundings, avoid excessive light
Music desired, aroma therapy
Limit care to essentials
Last hour or day
Ensure pleasant smell, touch, temperature
Unusual Smell to death so aromatherapy
Encourage family to continuous touching the child and assist
Encourage child to talk about feelings (questions on death) - dreams talk through them
Explain all procedures/therapies
Structure hospital/home environment to allow for max self control/independence
- spiritual and privacy
Stay with the family
Family becomes close to nurse (TMIs)
Accept family's grief reactions
Anger, denial, crying, depressed, betrayal
Avoid artificial consolation
Do not say outloud
Allow parents to be with the child at the moment of death
Accept if don’t
Remember siblings (developmental)
273
Warn about the surge
phenomenon comatose and wake up say goodbye then go back into coma
- get excited family
274
Toddler spiritual interventions of death
"Minimize separation from significant others
Keep security objects at hand
Provide simple, brief explanations
explain and maintain consistent limits
Encourage participation in daily care
Provide opportunities for play and play therapy
Reassure the child that disease is not punishment"
275
Early school-age child
spiritual interventions
"Don't underestimate level of comprehension
Provide simple, concrete explanations
Use pictures, models, actual equipment, medical play when providing explanations
When appropriate, initiate discussion of love and caring from Higher Power to relieve anxiety and loneliness
Show behavioral qualities of love, trust, respect, caring and setting of firm limits"
276
school-age child spiritual interventions
"Provide choices whenever possible
Stress contact with school, sports, religious peer groups
Use diagrams, pictures and models for explanations
Emphasize the ""normal"" things the child can do
Reassure child he/she has done nothing wrong (Hospitalizat6ions isn't a punishment)
be alert to anxiety about being punished by deity
Provide appropriate concrete explanations in response to questions regarding spiritual beliefs
Continue spiritual rituals when appropriate
Model behaviors that show forgiveness and acceptance"
277
adolescent spiritual interventions
"Allow Adolescent to be an integral part of decision making regarding care
Give information sensitively
Allow as many choices and as much control as possible
Be honest about treatment and consequences
Stress what the adolescent can do for themselves
Stress the importance of cooperation and compliance
Assist in maintaining contact with peer groups
Provide answers without bias and enable participation in discussion of illness in terms of philosophical or spiritual beliefs
Encourage contact with friends and use of spiritual rituals if appropriate
Observe and document verbalizations of adolescent's values and beliefs"
278
Which statement best describes iron-deficiency anemia in infants?
A. Clinical manifestations result from a decreased intake of milk and the preterm addition of solid foods.
B. It is easily diagnosed because of an infant’s emaciated appearance.
C. Preterm infants are particularly at risk.
D. It is caused by depression of the hematopoietic system.
C. Preterm infants are particularly at risk.Iron is transferred from the mother to the fetus during the last trimester of pregnancy. Maternally derived iron stores are usually adequate for the first 5-6 months in a full-term infant, but only 2-3 months in preterm infants. In iron-deficiency anemia, the child’s clinical appearance is a result of the anemia, not the underlying cause. Usually the hematopoietic system is not depressed in iron-deficiency anemia. The bone marrow produces red cells that are smaller and contain less hemoglobin than normal red cells. Children who are iron deficient from drinking excessive quantities of milk are usually pale and overweight. They are receiving sufficient calories, but are deficient in essential nutrients. The clinical manifestations result from decreased intake of iron-fortified solid foods and an excessive intake of milk.
279
Which clinical manifestation would the nurse expect when a child with sickle cell anemia experiences an acute vasoocclusive crisis?
A. Cardiomegaly
B. Painful swelling of hands and feet
C. Hepatomegaly
D. Circulatory collapse
B. Painful swelling of hands and feetA vasoocclusive crisis is characterized by severe pain in the area of involvement. If in the extremities, painful swelling of the hands and feet is seen; if in the abdomen, severe pain resembles that of acute surgical abdomen; and if in the head, stroke and visual disturbances occur. Circulatory collapse results from sequestration crises. Cardiomegaly, systolic murmurs, hepatomegaly, and intrahepatic cholestasis result from chronic vasoocclusive phenomena.
280
What is the purpose of chelation therapy in the treatment of beta-thalasemia?
A. Manages nausea and vomiting
B. Eliminates excess iron
C. Treats the disease
D. Decreases risk of hypoxia
B. Eliminates excess ironA complication of the frequent blood transfusions in thalassemia is iron overload. Chelation therapy with deferoxamine (an iron-chelating agent) is given with oral supplements of vitamin C to increase iron excretion. Chelation therapy treats the side effect of the disease management. Decreasing the risk of hypoxia and managing nausea and vomiting are not the purposes of chelation therapy.
281
Parents of a hemophiliac child ask the nurse, “Can you describe hemophilia to us?” Which response by the nurse is descriptive of most cases of hemophilia?
Y-linked recessive inherited disorder in which the red blood cells become moon-shaped
B. X-linked recessive inherited disorder in which a blood-clotting factor is deficient
C. Autosomal dominant disorder causing deficiency in a factor involved in the blood-clotting reaction
D. X-linked recessive inherited disorder causing deficiency of platelets and prolonged bleeding
B. X-linked recessive inherited disorder in which a blood-clotting factor is deficient. The inheritance pattern in 80% of all of the cases of hemophilia is X-linked recessive. The two most common forms of the disorder are factor VIII deficiency, hemophilia A or classic hemophilia; and factor IX deficiency, hemophilia B or Christmas disease. The inheritance pattern is X-linked recessive. The disorder involves coagulation factors, not platelets, and does not involve red cells or the Y chromosomes.
282
Which describes the pathology of immune thrombocytopenia?
A. Diffuse fibrin deposition in the microvasculature
B. Deficiency in the production rate of globin chains
C. Bone marrow failure in which all elements are suppressed
D. An excessive destruction of platelets
D. An excessive destruction of plateletsITP involves the evolution of antibodies against multiple platelet antigens and cytotoxic T cells that cause platelet destruction in blood and spleen and/or inhibition of platelet production in the bone marrow. Bone marrow failure is associated with aplastic anemia. Deficient production of globin chains is associated with Thalassemia. Diffuse fibrin deposition in the microvascular is associated with disseminated intravascular coagulation.
283
Which is caused by a virus that primarily infects a specific subset of T lymphocytes, the CD4+ T cells?
A. Wiskott-Aldrich syndrome
B. Acquired immunodeficiency syndrome (AIDS)
C. Idiopathic thrombocytopenic purpura
D. Severe combined immunodeficiency disease
B. Acquired immunodeficiency syndrome (AIDS)AIDS is caused by the human immunodeficiency virus (HIV), which primarily attacks the CD4+ T cells. Wiskott-Aldrich syndrome, idiopathic thrombocytopenic purpura, and severe combined immunodeficiency disease are not viral illnesses.
284
A toddler with leukemia is on intravenous chemotherapy treatments. The toddler’s lab results are WBC: 1000; neutrophils: 7%; nonsegmented neutrophils (bands): 7%. Which is this child’s absolute neutrophil count (ANC)?
(Record your answer in a whole number.)
The correct answer is: 140
To calculate an ANC for a WBC = 1000; neutrophils = 7%; and nonsegmented neutrophils (bands) = 7%, the steps are
Step 1: 7% + 7% = 14%
Step 2: 0.14 × 1000 = 140
285
Which immunization would not be given to a child receiving chemotherapy for cancer?
A. Tetanus vaccine
B. Measles, rubella, mumps
C. Pertussis
D. Inactivated poliovirus vaccine
B. Measles, rubella, mumpsThe vaccine used for measles, mumps, and rubella is a live virus and can result in an overwhelming infection. Tetanus vaccine, inactivated poliovirus vaccine, and diphtheria, pertussis, tetanus (DPT) are not live virus vaccines.
286
The nurse is admitting a child with a Wilms tumor. Which is the initial assessment finding associated with this tumor?
A. Abdominal distension
B. Weight gain
C. Increased urinary output
D. Hypotension
A. Abdominal distension The initial assessment finding with a Wilms (kidney) tumor is abdominal swelling. Weight loss, not weight gain, may be a finding. Hypertension occasionally occurs with a Wilms tumor. Urinary output is not increased, but hematuria may be noted.
287
What does chelation do?
binds with iron to be excreted in the urine (rusty)
Used in Iron overload
288
Which chelator is extremely painful? A traumatic care for it?
**Calcium disodium (EDTA) IM**
**Very painful**
Atraumatic care with rotation of site, EMLA,
Multiple treatments if necessary, after blood level tests
