Peds - Exam 5 - Hem/Onc

hematology

the study of blood and blood producing tissues

blood cells

RBC, WBC, plt

plasma

liquid portion of the blood that helps the body stop bleeding with clotting factors

bone marrow

creates 95% of blood cells

spleen

stores blood in the body, acts as a filter to remove cellular waste and old/damaged blood cells, helps to make the WBC and antibodies

red blood cell production

begins at 8 weeks, created in the liver of the fetus then the bone marrow takes over after birth

hemoglobin

birth to 6 mo: fetal hgb (hgb F) that is replaced with adult hgb (hgb A) - many disorders start at this point

iron

when the baby is born ___, comes from the mom through the placenta in the third trimester. stores from mom are depleted at 6 mo

maintaining oxygenation

all blood disorders impact the body's ability to deliver oxygen to the cells, so we must maintain adequate oxygenation. if the child does not receive enough oxygen, they will compensate by making more RBC (polycythemia) which thickens the blood and increases the work load of the heart -- inc HR

Give oxygen sooner rather than later

maintaining hydration

volume helps to transport oxygen to the cells.
normally 0.5-2 mL/kg/hr but in hem/onc kids we want 1-3mL/kg/hr

infection prevention

higher risk of infection and harder time fighting off infections that do occur. promote handwashing, restrict visitors (hospital and home), monitor temp freq, hospital asepsis when accessing lines

prevent and control bleeding

impaired clotting, so RICE for all bleeding (internal or external) and give blood products

anemia

RBCs and Hgb are lower than normal

most common disorder of infancy and childhood

NOT a disease itself but an indication or manifestation of an underlying problem

4-5.5

normal pediatric RBC range

11-14

normal Hgb range in peds

30-45

normal Hct in peds

decreased production of RBC, abnormal loss of RBC

two main causes of anemia

decreased production of RBCs in anemia

due to nutritional deficits, alterations in cell structure (as in sickle cell), or malfunction of cell producing tissue (kidneys make erythropoietin)

abnormal loss of RBCs in anemia

due to toxic exposure (lead poisoning), surgery or trauma, or genetic or cellular development disorders

iron deficient anemia

body lacks enough iron to adequately produce Hgb

iron deficiency anemia

assessment: weakness, fatigue, decreased activity, tachycardia, tachypnea, linked to cognitive delay and behavioral changes, OH, inadequate sleep, > 32 oz of milk intake daily
long term delays in milestones

12-36 mo, adolescents

age group at risk for iron deficiency anemia

IDA in toddlers

due to excessive cows milk intake (no more than 32 oz!) or not eating enough iron fortified foods

iron supplements for breastfed babies starting at 6 mo and iron fortified foods

IDA in adolescents

due to rapid growth rate, poor diet, onset of menses, strenuous activity

preterm infants

high risk for IDA since iron stores are transferred from mother to fetus during the last trimester of pregnancy

iron deficiency anemia

nursing care: educate parents, iron fortified infant cereal, no cows milk before 12 mo, iron rich foods, iron supplement, return appointments (reassess Hgb for inc), WIC supports iron-fortified foods, will not routinely result in child services involvement

polyvisol

iron supplement for breastfed babies. this should be given in a dropper in the back of the mouth (can stain teeth), but do not give with milk. this may cause constipation, so contact HCP for stool softener

lead poisoning

common in children 1-5 yo. this is toxic to the bone marrow, nervous system, kidneys, and erythroid cells

lead in the blood stream interferes with biosynthesis of heme, resulting in hypochromic (pale RBC), microcytic (small RBC) anemia

sources of lead exposure

-paint in homes before 1978 - toddlers can eat a few flakes and be poisoned
-glazed pottery
-lead pipes supplying water to homes - do not drink water from pipes
-stained glass products
-old painted toys/furniture (wooden toys with lead paint)

complications of lead poisoning

-behavior problems
-learning difficulties
-seizures
-encephalopathy (nervous system issues)

45

chelation therapy for lead levels > ___ mcg / dL

chelation therapy

treatment for lead poisoning levels > 45
medication that binds to lead so body can eliminate it without cellular damage
eliminated through kidneys, so encourage hydration and monitor I/O

thalassemia

genetic disorder that affects those of african america, caribbean, middle eastern, SE asia, and mediterranean descent due to REDUCED HGB

thalassemia

autosomal recessive disorder included in most newborn screens where an abnormal piece of hgb chain causes unstable RBC that break down rapidly and release free iron into bloodstream and damages tissues. this results in large degree of anemia and cell damage due to iron buildup

thalassemia

this disease has 3 classifications that range from mild to moderate (blood transfusions) to major (freq med intervention, blood transfusion, and iron chelation)

iron chelation

binds to the iron to release it from the body - does not affect RBC themselves, only the free floating iron

thalassemia

assessment: pale, FTT, weakness, jaundice, hepatosplenomegaly (RBC entrapment), frontal or maxillary BOSSING or enlargement, brozed skin, kidney and liver failure

thalassemia

labs:
dec H/H
inc iron and bili due to RBC breakdown

thalassemia

nursing management: administer packed RBCs for anemia (these contain iron!!), chelation therapy, family education (genetic DO, iron toxicity, compliance)

sickle cell anemia

autosomal recessive hemoglobinopathy where HgbA is replaced largely/entirely by HgbS causing hemolytic anemia where cells break down more quickly

often seen at 6 mo

sickle cell anemia

S/Sx: pain, ischemia, acidosis (due to occlusions, esp in microvascular areas)

stress

sickling occurs during times of ___ such as infection, fever, dehydration, extreme temp

sickle cell anemia

assessment:
routine newborns screening (usually asymptomatic until 3-4 mo)
protruding abd - enlarged liver and spleen
jaundiced sclera
labored breathing - acute chest syndrome

acute chest syndrome

vaso-occlusive crisis in the pulmonary vessels in a child with sickle cell due that causes chest pain, SOB, hypoxemia, cough, wheezing, fever

presents like PNA with infiltrates on xray

vaso-occlusive crisis

in children with sickle cell - clogging of microvessels that can occur in any vessel of the body and causes pain and swelling distal to the vessel.

splenic sequestration

in a child with sickle cell, there is a risk of infarct in microvascular structure and ______

vaso-occlusive crisis in the spleen where the spleen is overworked and becomes fibrotic, so blood pools and causes blood vol to be depleted

acute surgical intervention is spleenectomy

spleenectomy

surgical intervention for infarct in microvascular structure and splenic sequestration

***INFECTION RISK -- spleen helps remove bacteria

7-10

hgb baseline in a child with sickle cell

reticulocyte

increased level in sickle cell since the body is making a large number of RBC that never reach maturityl

labs in sickle cell anemia

low Hgb
inc reticulocyte, plt, ESR, bili
sickle shaped cells

penicillin

for sickle cell, we give daily oral ___ to prevent infection

stress

____ can cause sickling (infection, extreme temps, dehydration, stress) so interventions prevent exposure

sickle cell anemia crisis prevention

administer daily oral PCN
encourage routine vax
stay warm in winter and cool in summer
avoid overexertion/stress
hydration in school (water bottle all times) and before surgery (admit night before for IVF)
education on when to see PCP or ED - fever or URI (acute chest crisis?)

hydration, oxygenation, pain

3 priorities during sickle cell crisis

hydration - sickle cell crisis

fluid support oral or IV is #1 priority for acute chest since the cause is occlusion!!!!!
strict I/O

oxygenation - sickle cell crisis

O2 therapy IF hypoxia is present

give O2 much sooner than normal. we do not want to wait until they decrease to 92%; we give for any decrease (even 98 to 95%)

pain relief - sickle cell crisis

prophylactic, daily pain meds (ibuprofen)
scheduled pain pains - narcotics such as hydrocodone
sickle cell anemia pts are not addicts
take meds at home, but if unrelieved come into hospital or PCP bc this could be vaso-occlusive crisis

hemophilia A (classic hemophilia)

factor VIII deficiency that accounts for 80% of cases, affects males only

hemophilia B (christmas disease)

factor IX deficiency that affects males only

vonwillebrand disease

absence of protein necessary for clotting that affects both males and females

hemarthrosis

hemorrhage into the joint cavity where the joint becomes swollen, red, and stiff. Multiple injuries can lead to joint deformities, so we need to do ROM exercises despite pain

muscle mass

increased ___ causes less bleeding in the joints and is considered a protective factor. we encourage physical activity to increase this protective layer with sports that do not cause injury (no contact sports, gymnastics, etc)

longer

bleeding is _____ not faster in hemophilia hemorrhage

bleeding

bony changes and crippling deformities occur after repeated ____ episodes in hemophilia

spontaneous hematuria

bleeding in the urinary tract that needs further investigation since they will continue to bleed. there is no way to monitor internal bleeding. educate caregivers to watch for blood in the urine and stool

black, tarry stools

side effect of iron supplements

hemophilia management

replacement of missing clotting factor IV
Tylenol for pain (NOT ibuprofen)
reasonable physical activity
prophylactic replacement therapy prior to medical tx (procedures, dentist)

DDAVP

synthetic vasopressin for MILD hemophilia that constricts BV and minimizes bleeding

FACTOR AND RICE

treatment for acute bleeding episodes of hemophilia

Priority: administer factor
parent trained to administer IV with butterfly or port if recurrent
Take to ER if you cannot get the IV!
RICE after giving factor

when to come to the ER for hemophilia

1. any injury to the head
2. prolonged bleeding (not treated right away)
3. chest or abd pain or injury

blood in stool or urine
unable to administer factor

bleeding prevention

baby/toddler: fall risk, so use helmets, pad hard surfaces, knee pads, soft flooring
adolescents, no contact sports or ATVS

preventing long term bleeding effects

increase muscle strength, active ROM

home care - hemophilia

contact school nurse and keep factor in the office
med alert bracelet
educate adolescents about when to seek medical care or go to the ER
special summer camps for hemophilia

50,000

idiopathic thrombocyopenic purpura is immune thrombocytopenia when plt < ______

idiopathic thrombocytopenia purpura

acute or chronic condition of unknown cause with development of antibodies. petechiae, purpura, and excessive bruising seen on assessment. this is self limiting, but there is a huge risk of bleeidng due to low plt

nursing care of hemophilia

supportive care for self limiting condition

education: avoid activities that increase bleeding risk, no rectal temps, no NSAIDs, delay procedures

control bleeding

restrict activity when plts are low

10,000

medical therapy for plt < _____

Prednisone, IVIG, antibody D

3 medical therapies for platelets under 10,000

prednisone

administering ____ for plt < 10,000 -- supresses immune system. high dose given and tapered down 2-3 weeks

IVIG

given to boost immune system when plt < 10,000

antibody D

aka WinRhoD - given when plt

platelets

do not give ___ in thrombocytopenia unless there is a life threatening emergency since the body will destroy them. focus on reversing response!

acute lymphocytic leukemia

unrestricted proliferation of immature, nonfunctioning WBC. Bone marrow is working on overdrive, but WBC being produced are nonfunctioning (lymphoblasts)

leukemia assessment

anemia - pallor, fatigue, weakness
neutropenia - fever
thrombocytopenia - petechiae, bruising, oozing of gums
bone pain

CBC, bone marrow aspiration

how to diagnose leukemias

lymphoblasts

what do you see on CBC that cues into leukemia

bone marrow aspiration

confirms diagnosis of leukemia after CBC. looking for lymphoblasts in the bone. sedate beforehand!

lumbar puncture

evaluates for CNS involvement in leukemias. are the lymphoblasts in the CSF?

induction, consolidation, maintenance

3 phases of chemotherapy

induction therapy

stage of chemo that achieves complete remission for 4-6 weeks. we give high doses of strong meds c

consolidation phase

phase of chemo that includes systemic chemotherapy and CNS prophylaxis (if not already involved) for 6-9 mo to eradicate anything leftover in the body

maintenance therapy

stage of chemo that is a continuation of therapy and serves to maintain remission. there are several years with meds that keep leukemic cells in the body suppressed and prevent remission

body surface area

chemo dosing is based on ___, so obtain accurate height and weight to calcula

OSHA chemo guidelines

nurse wears chemo certified gown and gloves
dispose of PPE and administration materials in specific chemo waste site

IV site

must be patent to ensure there is no leak and have fresh site in case first one dysfunctions or emergency

emergency equipment for chemo

each exposure to chemo increases adverse reaction risk to chemo
drug sheet, ambu bag, oxygen, PRN orders for epi
chemo is a double check med

supportive therapy for chemo

administer antiemetics
benadryl for itching or discomfort
eat whatever they want
monitor for infection

(% bands + % segs) x total WBC

absolute neutrophil count formula

nursing considerations for leukemia

ANC, anorexia, stomatitis (mouth wash benedryl + maylox), neuropathy, hemorrhagic cystitis (flush out bladder with IVF, oral), alopecia, body image, anticipatory grief, infection

1500

absolute neutrophil count should be ___ or more
500 or less = severe risk for infection of blood