hemolytic/oncology Flashcards
(97 cards)
1
Q
What are the 5 types of sickle cell crisis?
A
- vaso-occlusive
- splenic sequestration crisis
- aplastic or hyper hemolytic crisis
- acute chest syndrome
- cerebral vascular accident {stroke}
2
Q
how many mg should they receive of iron per day?
4 months?
6-12 months?
1 to 3 years?
A
1mg/kg/day
11mg/kg/day
7mg a day.
3
Q
therapeutic management of iron deficiency anemia?
A
ferrous iron supplement vitamin c (orange juice)
GIVE ON AN EMPTY STOMACH
have them drink through a straw or a syringe to avoid teeth stains.
if you give IM z track, DONT MASSAGE AFTERWARDS
4
Q
What do you educate in iron deficiency anemia?
A
limit quantity of milk when to introduce solids (4-6 months) overweight doesn't mean good health give between ,meals / doses with citrus fruit juice use straw and brush teeth after iron NO IRON WITH TEA stools will turn tarry green or black
and foods high in iron RED MEATS LIVER WHOLE GRAINS GREEN LEAFY VEGETABLES PEANUTS POTATOES
5
Q
what is sickle cell anemia?
A
inherited autosomal recessive defect in the hemoglobin synthesis. a loss of oxygen happens and the red blood cell becomes sickle shaped.
6
Q
what is the normal lifespan of RBC and in sickle cell?
A
normal 120 days
sickle cell: 15 days
7
Q
how do you diagnose Hgb electrophoresis blood test?
A
by a Hgb electrophoresis blood test
8
Q
what exacerbates sickle cell anemia?
A
- trauma
- infection
- cold weather
- physical and emotional stress
- increased blood viscosity [DONT LET THEM GET DEHYDRATED]
- ## hypoxia from high altitude, plane rides, hypoventilation
9
Q
what are children given prophylactically in sickle cell anemia?
A
antibiotics like penicillin 2 months - 5 years
to prevent strep and pneumonia infection
10
Q
Tell me about vaso-occlusive thrombotic crisis?
A
Most common type of crisis
PAINFUL
they’ll have stasis of blood with clumping of cells which leads to ISCHEMIA and INFARCTION
11
Q
Tell me about Splenic sequestration crisis?
A
life threatening
blood pools [sickling] in the spleen and liver, the blood cannot leave because its OCCLUDED
12
Q
Signs of vaso-occlusive thrombotic crisis?
A
signs: fever, pain, tissue engorgement
also dactylitis- swelling of hand/feet in toddler…. often times an early sign of vaso-occlusive
13
Q
signs of splenic sequestration crisis?
A
profound anemia
hypovolemia
SHOCK
swelling of the spleen
14
Q
tell me about aplastic anemia or hyperhemolytic anemia?
A
- diminished production and increased destruction of RBCs
- triggered by viral infection or depletion of folic acid.
15
Q
Signs of hyperhemolytic or aplastic anemia?
A
profound anemia, pallor
16
Q
Tell me about acute chest syndrome
A
Pulmonary infiltrate
CRISIS IN THE CHEST
Capillaries in the lungs become OCCLUDED
These patients will be in the ICU with
- oxygen, deep breathing, blood transfusion, antibiotics- SUPPORTIVE CARE
17
Q
Signs and symptoms of acute chest syndrome?
A
chest pain fever cough wheezing tachypnea hypoxia
18
Q
Tell me about Cerebral Vascular Accident [stroke]?
SIGNS
A
Sickled cells that block major vessels to brain - can be sudden and severe
results in infarctions
signs: neurological impairment.
[ headache, vomiting, seizure, ataxia, speech, vision changes etc ]
19
Q
Does oxygen therapy help for sickle cell anemia?
A
if the child is hypoxic you can give them oxygen. it doesn’t help reduce sickling but it helps prevent further sickling.
20
Q
What do you monitor for Priapism?
A
Continuous intermittent painful erection that won’t subside. KEEP ELEVATED AND PUT HEAT PACKS ON GROIN AREA.
21
Q
If hypoxia in sickle cell disease is present, what can it cause?
A
metabolic acidosis which leads to increased sickling.
22
Q
How to prevent and treat pain in sickle cell anemia?
A
Heat helps with pain
pca pumps
iv narcotics
mild pain starts NSAIDs or codeine
Demerol short term . long term leads to the breakdown of the metabolite nomeperidine which causes seizures and loss of pain control
GOLD STANDARD: morphine
monitor reticulocyte count regularly to evaluate bone marrow function
methylprednisone is effective in short term use to help with pain control.
23
Q
Sickle cell treatment that is not for pain
A
Hydroxyuria - increases the production of fetal Hgb-F [ approved in <18 years]
splenectomy[ for splenic sequestrian]…will need long term antibiotics
bone marrow transplant/ stem cell transplant like hematopoietic stem cell transplantation (ONLY CURE)
RBC blood transfusion as needed to reduce ischemia. [ infusions increase hemosiderosis an increase in iron levels then we give DEFEROXAMINE/ VITAMIN C IV OR SUBQ to bind with the excess iron and be excreted through the kidneys.
24
Q
nursing management for sickle cell anemia?
A
monitor child’s growth
immunizations: pneumococcal and meningococcal vaccines and yearly influenza vaccines must be given and are especially important for children who don’t have a spleen
possible prophylactic antibiotics from 2 months-5 years
how to look for infection
prevent conditions for sickling- keep hydrated.
25
Aplastic anemia? what is it
what's hypoplastic anemia?
Bone marrow failure where all formed elements of the blood are simultaneously depressed aka pancytopenia. [LEUKOPENIA, ANEMIA, THROMBOCYTOPENIA]
hypoplastic anemia: depression of RBCs but normal WBCs and platelets.
confirm with bone marrow aspirate.
26
treatment for aplastic anemia?
Immunosupressive treatment
( administered through a central line over 4 days for 12-16 hours a day)
Tylenol and steroids prophylactically to decrease s/e of hypersensitivity, fever, chills
oral cyclosporin in organ transplants for those that don't respond to ALG or ATG
- colony stimulating factor
- BONE MARROW TRANSPLANT. do stem cell transplant before multiple transfusions that are given.
27
What is hemophilia?
hereditary disorder that results in deficiencies of anti hemophiliac factor like clotting factors. factors 8 or 9 deficient
28
what are the types of hemophilia?
hemophilia A
| hemophilia B
29
What is hemophilia A?
What is hemophilia B?
A: more common
deficient in factor 8
you'd rather want this one
B: aka Christmas disease
caused by deficiency in factor 9
15% of cases
the bad one
30
manifestations of hemophilia ?
bleeding. the platelet plug forms , but fibrin doesn't solidify.
mobility leads to injuries from falls and accidents
hemarthrosis - bleeding into joint spaces
ecchymosis - bruising
epistaxis
BLEEDING AFTER PROCEDURES. remember bleeding into neck chest mouth can compromise airway
31
how do you diagnose hemophilia?
decreased factor 8 or 9
DNA testing to confirm
can be through amniocentesis
child will have normal platelet , pT, fibrinogen count BUT PTT is what is prolonged.
32
medical management of hemophilia?
DDAVP- increases production of factor 8 so it's good for H. A
IV infusion- the parent is trained to give it at home 3x a week
replace missing clotting fa tors
NSAIDS be careful cause they inhibit platelets
steroids - could cause hematuria
transfusions- with factor 8 from pooled plasma
(8-10 children can do it)
can also be given prophylactically up to 3x a week
33
What should you tell the family about hemophilia in family support?
treatment is expensive
need to wear a medical bracelet
need to avoid contact sports
GENETIC COUNSELING- so parents know if they're likely to pass it on
34
management of hemophilia in an active bleed?
during bleeding, elevate and immobilize joint.
RICE ! rest, ice, constriction, elevation
apply pressure for at least 15 minutes if bleeding occurs.
analgesics if needed.
ROM after bleeding stops to prevent contractures.
AVOID OBESITY TO MINIMIZE JOINT STRESS
35
what is idiopathic thrombocytopenic purpura? when can it happen?
acquired chronic hemorrhagic disorder characterized
- thrombocytopenia
- purpura (discoloration caused by petechia beneath the skin)
- normal bone marrow.
it can happen between 2-10 years of age.
acute self limiting. can be autoimmune, can follow uri or other infections.
36
Diagnostics for idiopathic thrombocytopenic purpura?
platelet count less than 20,000
37
medications for idiopathic thrombocytopenic purpura?
```
rest
prednisone
IV Ig (gamma globulin)
prophylactic antibiotics
Tylenol for pain
monitor vital signs
```
38
what do you assess in children who have or possibly have cancer?
- increased signs of tiredness
- nightsweats
- pain
- bruising
- headache
- decreased appetite
39
nursing implementation for cancer?
prepare child and family for procedures
pain management
prevent complication of myelosuppresion
40
what causes cancer?
external factor (chemical) genetic mutation
immune system- gene abnormalities
chromosomal abnormalities
reoccurrence can happen after txt or years later and can be a new cancer different from first.
41
general treatment for cancer?
- chemotherapy
- surgery
- radiation
then if no luck with any of those 3,
- bone marrow transplant
- stem cell transplant
- biological response modifiers
- target cell therapy - gene therapy
42
is chemo given in combo to hit cells at all phases of the cell cycle?
YES
43
how can chemo be given?
IM, IV, subQ, PO, IT (thecal), or even via Cath
44
what is a Nadir scale?
The time the drug will cause the greatest suppression, neutrophil count with be LOW. this is when you protect child. (Usually within 7-10 days post infusion.)
45
When can children with cancer who are in the process of treatment or in-between rounds of treatment .... go back to school?
how do you calculate it?
when ANC absolute neutrophil count Is greater than 500.
look at segments and bands multiplied by WBC counts
so if WBC count is 1000, set 15%, bands 5%
15%+5% = 20%
0.20 x 1000 = 200 ANC
46
what kind of diet should cancer kids be on?
high in protein and calories
47
what should you watch for in kids with cancer?
HEMORRHAGE
- limited invasive procedures
- no rectal temps, IMs, fingersticks
- limit contact sports and activities
WATCH FOR TEMP AND SKIN BREAKDOWN
- the most skin breakdown will be stomatitis (mouth) and bottom
VACCINES ARE HELD WHEN THEY'RE IN THIS IMMUNOCOMPROMISED STATE
48
what kind of meds can you give for nausea and vomiting in cancer?
zofran- ondanesetron
dexamethasone also an anti-emetic
Aloxi- 2nd gen anti-emetic that lasts 7 DAYS
49
What do you do with cancer patients who experience anorexia?
let them eat whatever they want
alternate NG tube or TPN....(draw liver panels because TPN affects liver)
cold food or cold drinks are painful to their mouth. taste buds change and everything tastes metallic.
50
mouth care in cancer?
```
soft toothbrushes
saline mouth rinse
stay away from lemon swabs
milk of magnesia dries up the mucosa
hydrogen peroxide breaks down proteins
```
51
symptoms cancer patients experience besides n/v?
neuropathy. vincristine can cause this
hemorrhagic cystitis- bleeding in the bladder
[ mesna antidote to help coat bladder]
alopecia - wear hats in sun
mood changes - drug induced depression, irritability
moon face- from the steroids
constipation
52
# Define terms:
Vesicant
Extravasation
Tumor lysis Syndrome
vesicant- an agent that causes blistering and sclerosis ( tissue becomes hard from overgrowth of fibrous tissue)
extravasation-accidental administration of drugs from iv into extravascular space/tissue around infusion sites by leakage , previous stick, or mispositioned venous access device.
tumor lysis syndrome:
- chemo destroys cells fast and puts metabolites into circulation
the patient becomes hyperuricemic so they'll get allopurinol
53
Surgery and diagnostic interventions for cancer?
- biopsy
- removal of solid tumor or biopsy
- may get chemo or radiation before surgery
- CVC device. can be a central line
- chemo needs to be given in a large vein with fast moving blood
- PORT A CATH [intravenous access device]
can swim with it, take a bath, unlike central lines they don't need to be covered. 90 degree huber needle access, port is in subs and is flushed once a week. nothing shows from outside
54
Radiation?
can be curative, or palliative
total body before transplants
non selective - attacks all cells
s/s : fatigue
brain radiation need to be careful in kids cause brain still developing
- protect skin thats being radiated from sun , heat, cold, sometimes tattooed
55
Bone marrow or stem cell?
high dose of chemo to destroy own marrow
- infuse healthy marrow or stem cells IV
bone marrow aspirate isn't put into bone but through IV ***
- before this ur body is zapped without any cell count and you're put on isolation. also changes your blood type ****
56
# define for bone marrow or stem cell
- syngeneic
- autologous
- allogeneic
identical twin
own marrow
donor who matches
57
side effect of bone marrow or stem cell?
- rejection, graft vs host disease so give anti--rejection medication (these meds are life long)
- swelling and petechiae
- positive pressure room. patients are in reverse isolation from us.
NO LIVE PLANTS OR FLOWERS IN ROOM
58
what is target cell therapy?
- gene therapy
- antibodies that are tumor specific to certain cancers
- produces in response to antigens from cancer cells
59
what are late effects of cancer and treatment?
general intelligence, academic performance, memory, attention span
- vision and hearing loss
- short stature and loss of bone growth
- gonadal dysfunction
- scoliosis, kyphosis, asymmetry
- LVD, CHF, CAD, arrhythmia, risk of sudden death
- interstitial pneumonitis and pulmonary fibrosis
60
Leukemia?
stem cell or blast cell leukemia
" Unrestricted proliferation of immature WBCs in blood forming tissues of body. Not a tumor"
61
Acute lymphoid leukemia ? ALL
Acute non lymphoid leukemia ? ANLL or AML?
ALL:
most comment in children and easier to cure in kids. opposite for adults
you can give them ROUNDS of chemo, if it doesn't work u can do a transplant
ANLL:
outcomes aren't good in children, but better in adults.
if they don't respond to FIRST or SECOND chemo round, then transplant.
62
what does leukemia cause?
- anemia
- infection
- bleeding tendencies
- spleen, liver, lymph glands show enlargement, fibrosis, and infiltration.
- CNS- increased ICP lumbar punctures needed and IT meds like METHYLTREXATE to prevent CNS disease
- Bone pain
- Bone marrow aspirate GOLD STANDARD TO DIAGNOSE LEUKEMIA
63
What are the four phases of therapy for Leukemia?
Induction therapy: first 4-6 weeks
CNS prophylactic therapy, intrathecal chemo if theres a high chance of it spreading to brain. so its done even if spinal fluid doesn't show cancer
Intensification therapy: eradicate residual leukemic cells and prevent resistant leukemic clones
Maintenance therapy: to preserve remission
REINDUCTION: with alternative drugs as needed if relapse
64
What can you tell me about stem cell transplantation?
donors can be relatives or not
antigen matches or mismatched
peripheral stem cells can be used
stem cells from umbilical cord and bone marrow
risk for death
graft vs host disease
INFECTION
severe organ damage
cure after HSCT up to 60-70%...if relapse after HSCT ---> dismissal prognosis
65
what do you need to diagnose leukemia?
- WBC count
- age at time of diagnosis. if 2-9 better prognosis
- type of cells involved
- gender. girls live more
- karyotype analysis
66
what type of lymphomas are there?
Hodgkins disease (15- 19 years of age)
Non Hodgkins lymphoma (NHL) more in children younger than 14 years of age.
67
Define Hodgkins disease?
| Where does it metastasize to?
Neoplastic disease originating in lymphoid system
Spreads to the spleen , liver, bone marrow, lungs and other tissues.
most common in boys, adolescents, and young adults.
68
What are the classifications of Hodgkins disease?
Class A: asymptomatic - BETTER PROGNOSIS
Class B: Temp 38 c or higher for 3 + days, night sweats, unexplained weight loss of 10% or more over previous 6 months.
69
Therapeutic management for Hodgkins disease?
Radiation
| Chemo [ alone or with radiation ]
70
Clinical appearance for Non Hodgkins
Therapeutic management?
-Disease usually diffuses rather than nodular
cell type undifferentiated or poorly differentiated
-Dissemination occurs early, often and rapid
-Mediastinal involvement and invasion of meninges
-Can see and palpate an enlarged gland
Therapeutic management? Chemo and radiation
71
What is Wilms Tumor?
what are the clinical manifestations?
what is the treatment?
Renal Tumor
usually in children less than 5 years old
abdominal swelling or mass, fatigue, weight loss
surgery and chemo and radiation
SURVIVAL IS GOOD =]
DON'T PALPATE ! IF U TOUCH IT IT WILL SPREAD.
72
CNS tumors
brain tumors and neuroblastoma come from neural tissue.
| these are hard to treat and often result in death.
73
brain tumors
most common solid tumors in kids...
60% in posterior third of brain cerebellum or brainstem
- 40% occur in anterior 2/3 brain mainly in cerebrum
74
what is included in diagnostic eval?
neuro eval. LOC, weakness on one side or the other, behavioral changes, VS changes, seizures....
MRI, CT, EEG, LP, biopsy to see what kind of cancer it is
75
therapeutic management for brain tumors.
surgery
radiotherapy [ not always done in kids less than 3 ]
chemo- water soluble agent needed to cross BBB
76
What is neuroblastoma? it can be the silent tumor.....
most common malignant extra cranial solid tumor of childhood
adrenal glands, abdominal area, head, neck , chest, pelvis
77
what type of bone tumors are there?
osteosarcoma and Ewing sarcoma
occur more in males with higher incidence during accelerated growth rate.
78
diagnosis of bone tumors?
CT scans
Bone scans
Bone biopsy
MRI to evaluate neuromuscular and soft tissue extension
LABS: elevated alkaline phosphatase with some bone tumors
79
What is Osteogenic sarcoma/ Osteosarcoma?
most frequent bone cancer in children
usually 10-25 years old
mainly in metaphases of long bones like legs
Greater than 50% in legs
also in humerus, tibia, pelvis, jaws.
80
therapeutic management for osteosarcoma?
- radical surgical resection or amputation
- limb salvage: resection of bone with prosthetic replacement
- chemo along with surgical treatment
81
What is Ewing sarcoma?
second most common cancer in bone tumor in children and adolescents.
arises in bone marrow in:
femur, tibia, ulna, humerus, vertebrae, pelvis, scapula, ribs and skull
82
Treatment of Ewing sarcoma?
Radiation most common FIRST approach
Chemo as adjunct to radiation
Surgical resection in some cases
83
When is prognosis best?
when there is no spreading during diagnosis. Distal lesions have the best potential for cure also.
84
What is Rhabdomyosarcoma?
- Soft tissue tumor
- Malignant neoplasm originating from undifferentiated cells in muscle, tendon, bursa, and fascia or in fibrous connective, lymphatic or vascular tissue.
name reflects area.
myosarcoma- myo=muscle
rhabdomyosarcoma-rhabdo= striated muscle
85
potential sites of rhabdomyosarcoma?
- eye orbit - common
- nasopharynx
- paranasal sinuses
- middle ear
- retroperitoneal area
- perineum
86
tumor treatment for rhabdomyosarcoma?
- complete removal of tumor if possible
- radiation therapy for most tumors
- chemotherapy to shrink tumor ..may precede radiation
- long term chemo [1-2 years]
87
BLOOD TRANSFUSION THERAPY
probably on test select all that apply
verify identify of recipient and verification of donors blood group
monitor vs
use appropriate filter
use blood within 30 minutes of arrival
infuse over 4 hours maximum
88
what kind of reactions can happen during a transfusion?
Hemolytic: MOST SEVERE
Hypothermia
Allergic reaction ....urticaria, pruritus, laryngeal edema
Air emboli
Febrile reactions: fever, chills
Electrolyte disturbances: hyperkalemia from massive transfusions or pt with renal problems
89
Nursing responsibility during transfusions?
- identify donor and recipient blood types
- transfuse slowly for first 15-20 minutes
- observe carefully for patient response
- stop transfusion if reaction s/s
90
Delayed hemolytic reaction?
- Destruction of RBCs and fever 5-10 days after transfusion
| - Observe for posttransfusion anemia
91
Define Apheresis
Removal of blood from an individual; separation of the blood into tis components
92
what is given with Hematopoietic stem cell transplant? HSCT
ablative therapy: high-dose combo chemo [with or without radiation] to eradicate unhealthy cells and suppress immune system to prevent rejection of transplanted marrow.
93
Where do stem cells come from?
bone marrow, peripheral blood, umbilical vein of placenta
94
What is Allogenic bone marrow transplant?
- involves bathing histocompatible donor with recipient.
| - limited by presence of suitable marrow donor.
95
What is umbilical cord blood stem cell transplantation?
rich source of stem cell for children.
benefit: umbilical cord blood is blood's relative immunodeficiency at birth allowing partial matched cord blood transfusions to be successful.
96
what is autologous BMT?
uses patient's own marrow collected from disease free tissue, frozen and sometimes treated to remove malignant cells.
used to treat : neuroblastoma, Hodgkins, NHL, films tumor, rhabdomyosarcoma, Ewing sarcoma.
97
What is peripheral stem cell transplants? PSCT
A type of autologous transplant
stimulates production of high number os tea cells then collects stem cells by an aphaeresis machine.
remaining blood cells and plasma returned to patient after aphaeresis.
stem cells frozen for later transfusion to patient.
