pediatic Hematology Flashcards
New born normal range for Hgb
14-24
Infant norm range for Hgb
10-17
Child Hbg norm range
9.5-15.5
Adult Hgb norm ranges
12-18
Diet for infants with IDA (iron deficiency anemia)
Iron fortified formula or cereal
Dietary sources for an older child with IDA
Fish, liver, whole grains, lagoons, green leafy veggies, other cereals with iron
Oral iron supplement
Ferrous sulfate ( fer-in-sol )
Iron admin
Give on an empty stomach
Give w citrus juices (vit c)
Use a dropper or straw
Stools will be tarry
Safety
Do no give with milk
What do we want to teach parent about iron supplement ?
Can upset stomach
Cause black tarry or dark green stools, cause constipation, so increase fluids and fiber
How long does it take to increase RBC for IDA?
3 mo
(Takes 120 days for RBC to mature)
Why Do we give fruit juices (vit c ) with iron admin
Vit c increases absorption of iron into blood stream so blood can deliver it
Why Do we use a dropper or straw for iron admin
Stains teeth
What can we do to prevent over dose of iron supplement ?
Locked safely and out of reach for children
Why shouldn’t you give an iron supplement with milk?
Will decrease iron reabsorption
Sickles cell RBC life span
< 40 days
Why are we so concerned with anemia in children?
Diminished cognitive function
Behavioral changes
Delayed growth & development
Death can be a result
What populations are at highest for sick cell anemia
African American
Males > females
Triggers for sickle cell anemia
Dehydration
Acidosis
Hypoxia
Temp changes( cold)
Infection
Why don’t you see s/s of sickle cell in new borns
Born w 80% fetal Hgb and it does not sickle
When do you start seeing signs and symptoms of sickle cell in babies
At around 6 mo
What can a sickle cell crisis be caused by
Sickness , dehydration, cold weather w/o gloves or coat
PCA pump opoids for Vasooclusive crisis for severe pain
Morphine
Hydromorphone
Hydrocodone
Ketolorac
Ketorolac
Visceral pain in middle of body
Works as well as narcotic
Given for VOC pain management
Hydroxyurea
Increases fetal hemoglobin production
Given for VOC
Why is meperidine (Demerol) contra indicated for vasooclussive crisis
Causes seizures
Mild to moderate painVOC
Tylenol ibuprofen
Someone with a Beta thalassemia major RBC lifespan
30-60 days
Clinical manifestations of IDA
Pallor/ paleness of mucous membranes
Tiredness fatigue
Possible findings with a child with IDA
Over weight “milk baby”
Dietary intake low in iron
Milk intake over 32 ox a day
Pica habit
Pica habit
Body craving other sources of iron
Eating iron and clay
High risk populations of IDA
Preterm infants , multiple births
Mother has iron deficiency
Infants 6-24 mo
Toddlers
Female adolescents
how long does it take for oral iron to increase the RBC count in IDA?
3 mo for mature RBC to get out
Why would someone with IDA have a repeat h& h done in a month
To observe reticulate count to see generating of RBC
In a pt diagnosed with IDA who has to have a repeat H&H in a month and turns out it hasn’t improved what could that mean?
Will further investigate to see if there is an iron absorption issue or chronic bleeding
What do you give to pediatric pt with severe anemia and what also may you add IF tissue hypoxia is severe?
Blood transfusion of packed RBC to minimize chance of circulatory overload
Supplemental oxygen
Percentages of autosomal recessive emetic order sickle cel anemia
25% chance of not being a carrier or having disease
25% chance they will have disease
50% chance they will be a carrier
How to test prenatal for sickle cell anemia
Chorionic villas sampling from prenatal tissue
Amniocentesis
After birth diagnostic testing for sickle cell anemia
New born screening
Sickledex (sickle cell turbidity test) screening)
And if positive electrophoresis ( confirmative results)
General signs of chronic hemolytic anemia (sickle cell)
Frequent infections
Fatigue
Delayed physical growth
VOC
Vasoocculsive crisis
Peripheral
Pain crisis ischemia causing pain
(Cells crying for o2)
Most observed sickle cell crisis
Last 4-6 days
Splenic sequestration crisis
Will cause drastic decrease in blood volume
Can cause Hypovolemia
If not treated- within hours death can occur
Commonly seen @ ages 6 mo - 5 years
Aplastic crisis
Can be triggered by a viral illness = decrease production of RBC
Hyperhemolytic crisis
Something has made the body accelerate the rate of RBC destruction
Acute exacerbations of sickle cell crisis
Vasoocculsive
Aplastic crisis
Hyperhemolytic crissi
Splenic sequestration crisis
VOC classic or acute signs
Acute pain (15 out of 10)
Fever
Severe abdominal pain
Hand foot syndrome in infants
Arthralgia (painful joints)
Leg ulcers (adolescents)
Cerebrovascular accident
Why would someone with VOC be having severe abdominal pain
If vassoclusive happens in the abdomen and it may be masked as appendicitis
Hand foot syndrome in infants
If VOC happens in hands or feet - painful edema
Why would leg ulcers happen in an adolescent with VOC
Impaired circulation
Why would CVA happen in VOC
Due to blockage
Chronic signs of VOC
Splenomegaly
Hepatomegaly
Kidney abnormalities
Bone changes
Retinal detachment
When someone has constant VOC and splenomegaly occurs what can that eventually lead to
Autosplenectomy
When spleen enlarges =repeated insults of spleen= eventually body kills off spleen
When an autosplenectomy occurs after several VOC what does this put a child at risk for?
Increase risk infection
Hepatomegaly can occur with VOC what can this put a child at risk for
L risk for liver damage leading to liver failure
What kind of kidney abnormalities can occur with chronic VOC if damage to the kidneys happens
Hematuria
Inability to concentrate urine , enuresis, possible renal failure =
What kind of bone changes associated with chronic signs of VOC can happen
Osteoporosis
Skeletal deformities
Weakened bones
If retinal detachment happens due to chronic VOC what can it lead to
Blindness
Acute chest syndrome is a complication that can happen when occlusion happens within the chest (VOC). What are some s/s associated with acute heat syndrome ?
Fever >101.3
Cough (due to blockage flow2lungs)
Chest pain
Tachypnea (increase RR)
Dyspnea
Wheezing
Decrease O2 sat
What are the treatments for acute chest syndrome?
Antibiotics
Administration of o2
Breathing excercises
Blood transfusion
What is the goal for VOC management
Manage symptoms and reverse excess sickling
VOC crisis management
Hydration (prevent dehydration)
Electrolyte replacement
Strict I&O
Analgesics (for severe pain)
Warm compresses
Blood transfusions
Antibiotics
Hydroxyurea
Why do we want strict I&O during VOC management
Watching for kidney impairment
What does warm compresses benefit during VOC management and why dont we want to do cold compress
To vasodialate blood vessels and that will make sickle cells flow more easily
If we use cold it will vasoconstriction blood vessels and contraindicates because cold is a trigger of VOC
When teaching parents how to avoid triggers for VOC what are some teachings we can include
Avoid strenuous exercise (leads2dehydration)
Avoid high altitudes (cold places)
Avoid being around sick folks
Seek care at first sign of infection
Use prophylactic penicillin if prescribed
Keep child wheel hydrated
Why would prophylactic treatment be prescribed to avoid VOC
Prevents streptococcus
For VOC prevention if a person is allergic to penicillin what can we prescribe
Erythromycin
T or f do not withhold fluids from a child at night in regards to preventing VOC
True.
We want to ensure hydration
What are other things we can do to prevent VOC (3)
Up 2 date immunizations
Refer fam for genetic counseling
Provide emotional support
Prognosis of sickle cell
Most people live to be in their 50s
Greatest risk for pt under 5 to have a crisis
Death due to overwhelming infection
Beta thalassemia major
Life threatening anemia
Ultimately increase destruction of RBC
Also my produce fetal Hgb in adulthood
Beta thalassemia major RBC life span
30-60 days
Clinical manifestations of beta thalassemia at first
Pale, fussy , poor apetite and a lot of infections related to anemia
When does beta thalassemia typically show up
Within first 2 y of life
What lab signs will be increased with bet thalassemia
Retic count
What does severe anemia cause (beta thalassemia)
Chronic hypoxi
Small stature (FTT)
adolescents (delayed puberty)
Bronzed skin tone
Hepatosplenomegaly
Cardiomegaly
Bone changes
Why do you see bronzed skin tone in beta thalassemia ?
High bilirubin (bile)
Why is liver enlarged in beta thalassemia
Due to liver storing excess iron from chelation
Why is spleen enlarged in beta thalassemia
And the spleen is hyperactive due to pooling of blood RBC die faster spleen works harder. Also RBC gets stuck in spleen due to small and misshaped
In untreated children in beta thalassemia what kind of bone changes do we see?
Prominent frontal bossing
Changes to nose, and maxilla , jaw bones
Generalized osteoporosis
Prenatal Testing for beta thalassemia
Chorionic villus
Amniocentesis
After birth diagnostic testing for beta thalassemia
No screening if positive-
CBC (to see chronic anemia )
But ultimately hemoglobin electrophoresis
X-rays to see bone involvement
Goal of children with beta thalassemia major ?
To keep hemoglobin above 9.5 gm/dl
How often would a child with beta thalassemia major receive packed red blood cellls
Every 3-5 wks
Advantages of packed RBC of beta thalassemia major
Improve well being (phys&mental)
Decrease cardiomegaly/hepatosplenomegaly
Prevent bone changes
Promote normal or near normal growth and development (avg growth chart)
Decrease infection
Beta thalassemia major
Cooley anemia
What is the safe level of lead in the blood
There is no safe level
What can lead cause
Anemia
How can we get lead into our blood stream by ingestion
Crib rails
Window seals
Older plumbing(water source)
Contaminated soil(drop.)
Paint chips
Etc
How can we get lead into our blood stream by inhalation
Saying, lead pain in nearby vicinity
General sings of lead exposure
Anemia s/s
Cramping , Abdominal
What age is a risk factor for lead poisoning
Younger than 6 ( age when they put things in their mouth
What population is also at risk for lead poisoning
Living in poverty
living in Urban areas
Living in older homes
Children with anemia
Why are children with anemia more prone to lead poisoning
Led binds to hemoglobin = at risk for getting more led
Clinical manifestations of led poisoning
Anemia
Cramping , abd pain
Vomiting
Constipation
Anorexia
Headache
Lethargy
Impaired growth
Early CNS manifestations of led poisoning
Hyperactivity
Agggression
Impulsiveness
Decreased interest in play
Irritability
Short retention span
Late manifestations of lead poisoning
Mental retardation (decreased IQ)
Paralysis
Blindness
Convulsions
Coma
Death
Universal screening for lead poisoning
Blood lead level test BLL
When does a BLL get done
Children at ages 1 and 2
Any child who hasn’t got screened at ages 3 and 6 years if never screened
Target screening AD LIB)
Will always be screened during well checks
For high risk population
If in a high risk group
If fam cannot respond to no to personal risk questions
K
Identifying the sources of lead in the child’s environment
If they lie in old home
Assess where parents work (construction, pottery/ paints)
How can we eliminate lead sources when we identify them in the child’s environment
If in an old home teach to wet pop areas instead of vacuuming
Wash and dry child’s hands frequently especially before they eat
Wash toys and pacifiers frequently
Ensure the exposure isnt coming from parents occupation
Hemophilia
X -linked
Mom carries it
And male will have the disease ( males dont have second X chromosome to cancel it out)
Resulting of absence factor 8&9
Two type of hemophilia
A- Factor 8
B- Factor 9
Factor 8 deficiency
Hemophilia A classic (75%)
Factor 9 deficiency
Hemophilia b
Christmas disease
(25%)
Where can bleeding happen in hemophilia
Anywhere in the body
(Internal or external)
How can hemophilia be labeled?
Mild moderate or severe
Mild clotting factor
6-50%
Moderate clotting factor
1-5%
Severe clotting factor
Less than 1%
Diagnosis of hemophilia
History of bleeding episodes
Genetic testing , ptt to test clotting time
What will PTT ( partial thromboblastin time) come back as positive hemophilia
Less than 25 % clotting factor
Hemarthrosis
Hemophilia
Bleeding in the joints
