hemolytic/oncology Flashcards
What are the 5 types of sickle cell crisis?
- vaso-occlusive
- splenic sequestration crisis
- aplastic or hyper hemolytic crisis
- acute chest syndrome
- cerebral vascular accident {stroke}
how many mg should they receive of iron per day?
4 months?
6-12 months?
1 to 3 years?
1mg/kg/day
11mg/kg/day
7mg a day.
therapeutic management of iron deficiency anemia?
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
What do you educate in iron deficiency anemia?
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
what is sickle cell anemia?
inherited autosomal recessive defect in the hemoglobin synthesis. a loss of oxygen happens and the red blood cell becomes sickle shaped.
what is the normal lifespan of RBC and in sickle cell?
normal 120 days
sickle cell: 15 days
how do you diagnose Hgb electrophoresis blood test?
by a Hgb electrophoresis blood test
what exacerbates sickle cell anemia?
- trauma
- infection
- cold weather
- physical and emotional stress
- increased blood viscosity [DONT LET THEM GET DEHYDRATED]
- ## hypoxia from high altitude, plane rides, hypoventilation
what are children given prophylactically in sickle cell anemia?
antibiotics like penicillin 2 months - 5 years
to prevent strep and pneumonia infection
Tell me about vaso-occlusive thrombotic crisis?
Most common type of crisis
PAINFUL
they’ll have stasis of blood with clumping of cells which leads to ISCHEMIA and INFARCTION
Tell me about Splenic sequestration crisis?
life threatening
blood pools [sickling] in the spleen and liver, the blood cannot leave because its OCCLUDED
Signs of vaso-occlusive thrombotic crisis?
signs: fever, pain, tissue engorgement
also dactylitis- swelling of hand/feet in toddler…. often times an early sign of vaso-occlusive
signs of splenic sequestration crisis?
profound anemia
hypovolemia
SHOCK
swelling of the spleen
tell me about aplastic anemia or hyperhemolytic anemia?
- diminished production and increased destruction of RBCs
- triggered by viral infection or depletion of folic acid.
Signs of hyperhemolytic or aplastic anemia?
profound anemia, pallor
Tell me about acute chest syndrome
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
Signs and symptoms of acute chest syndrome?
chest pain fever cough wheezing tachypnea hypoxia
Tell me about Cerebral Vascular Accident [stroke]?
SIGNS
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 ]
Does oxygen therapy help for sickle cell anemia?
if the child is hypoxic you can give them oxygen. it doesn’t help reduce sickling but it helps prevent further sickling.
What do you monitor for Priapism?
Continuous intermittent painful erection that won’t subside. KEEP ELEVATED AND PUT HEAT PACKS ON GROIN AREA.
If hypoxia in sickle cell disease is present, what can it cause?
metabolic acidosis which leads to increased sickling.
How to prevent and treat pain in sickle cell anemia?
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.
Sickle cell treatment that is not for pain
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.
nursing management for sickle cell anemia?
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.
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.
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.
What is hemophilia?
hereditary disorder that results in deficiencies of anti hemophiliac factor like clotting factors. factors 8 or 9 deficient
what are the types of hemophilia?
hemophilia A
hemophilia B
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
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
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.
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
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
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
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.
Diagnostics for idiopathic thrombocytopenic purpura?
platelet count less than 20,000
medications for idiopathic thrombocytopenic purpura?
rest prednisone IV Ig (gamma globulin) prophylactic antibiotics Tylenol for pain monitor vital signs
what do you assess in children who have or possibly have cancer?
- increased signs of tiredness
- nightsweats
- pain
- bruising
- headache
- decreased appetite
