MB4 Peds Hematology Forsythe
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When is Fe deficiency anemia most likely to happen in peds?
When is it rare?
Why these ages?
rare before age 4 yo 6 mo. b/c still have maternal Fe stores. These become depleted around age 9-24 months which is when Fe deficiency anemia is most often seen
What is the main consideration with Fe deficiency anemia?
compromised tissue oxygenation
Why is tissue oxygenation compromised in Fe deficiency anemia?
cannot make HgB -> RBC are broken down -> exceeds ability of the body to make new RBC
What babies are at risk for IDA?
premature and low birth weight
they did not store as much Fe from the mothers
5 etiologies of IDA?
- 1. premature/low birth weight
- 2. excessive intake of cows milk
- 3. decreased Fe intake
- 4. increased Fe lost
- 5. periods of increased growth rate
Therapeutic prevention of IDA in babies?
start on Fe supplements around 2 months of age
Why does intake of cow's milk cause Fe deficiency anemia?
causes diarrhea and GI bleeding
Prevention of FE deficiency anemia r/t milk intake?
no cow's milk before 12 mo.
CH < 12 mo - no more than 24 oz per day
Who is most at risk for poor Fe intake?
adolescents - poor habits
What peds pt may be at risk for Fe deficiency anemia r/t iron loss?
adol. girls - periods
2 periods of increased growth rate that may increase risk for Fe deficiency anemia?
- 1. adolescents
- 2. premature and low birth weight babies
growing rapidly = increased need for Fe
Clinical manifestations of IDA?
- 1. skin - pallor, pale/porcelain skin tone
- 2. sclera pale
- 3. MM - pale
- 4. tachycardia
- 5. cardiomegaly
- 6. heart murmur (severe anemia)
- 7. poor school performance
- 8. koilonychias (spoon nail)
- 9. lethargy & fatigue
- 10. slow, strained movements
- 11. difficulty sucking/too tired to eat
- 12. sagging, limp posture
Why does tachycardia occur in anemia?
to compensate for RBC and decreased O2 to tissues
How is murmur r/t anemia Tx?
will reverse when anemia is treated
Tell parents murmur whould go away
Why may school performance suffer with anemia?
no energy...no energy to eat
How to assess a peds pt for fatigue r/t anemia?
ask the pt if they act tired a lot or take a lot of naps or play differently or not at all
How to assess anemia fatigue in an infant?
ask parent about the infant's movements and how much it sleeps
6 Dx eval. for IDA?
- 1. 24 h dietary Hx
- 2. HgB
- 3. RBC
- 4. reticulocyte (immature RBC)
- 5. Fe
- 6. Fe binding capacity (Fe carrying proteins)
What 2 things are included in a 24 h dietary Hx for Dx of IDA?
- 1. foods eaten
- 2. description of CH activities during that time
Lab tests for Dx of IDA include those that measure _____, ________, ________.
- 1. HgB
- 2. morphologic changes in RBC
- 3. Fe concentration
HgB level in IDA?
RBC appearance in IDA?
microcytic and hypochromic (small and pale/decreased HgB content)
What will they be like in IDA?
normal or slightly reduced due to decreased Fe stores
may be elevated in severe anemia r/t hypoxia -> erythropeietic response
Fe-binding capacity lab test?
What will it be like in IDA? Why?
binding capacity/measurement of all proteins available for binding Fe
increased in IDA b/c decreased Fe levels
2 Tx of IDA?
- 1. increase Fe in diet
- 2. Fe supplements
Why are supplements used along with diet in IDA?
b/c absorption of Fe from foods is unreliable
Foods that inhibit Fe absorption?
- 1. spinach
- 2. sweet potatos
- 3. coffee
- 4. tea
7 iron rich foods?
- 1. egg yolks
- 2. dried beans
- 3. cream of wheat
- 4. dried fruit
- 5. liver
- 6. iron-fortified cereal
- 7. green leafy veggies
4 examples of green leafy veggies rich in Fe?
- 1. broccoli
- 2. romaine lettuce
- 3. collard greens
- 4. mustard greens
5 considerations for Fe supplement admin?
- 1. admin tid
- 2. use a straw, dropper, or syringe
- 3. place at back of mouth and away from the teeth
- 4. brush teeth or wipe with wash cloth
- 5. Admin with Vit C
Nursing consideration when giving Fe supplements to children?
stains the teeth
3 juices high in vitamin C?
- 1. orange
- 2. tomato
- 3. grapefruit
SCA and SCD?
sickle cell anemia
sickle cell disease
2 ways SCA and SCD are different?
- 1. SCDs are broad category of hemoglobineamias
- 2. SCA is one of the groups of SCDs
a genetic disease caused by or ass. with abnormal HgB
3 common forms of sickle cell disease?
- 1. sickle cell anemia - homozygous HgBS
- 2. sickle C disease - HbC
- 3. sickle beta-thalassemia syndrome
2 general char of all 3 forms of SCD?
1. inherited autosomeal recessive - 2 copies of an abnormal gene present
2. life-long disease
Abnormal HgB in SCA?
Inheritance of SCA?
6 races that get SCA most?
- 1. AA
- 2. mediteranean-Italians & Greeks
- 3. East Indian
- 4. Middle Eastern
- 5. Caribbean
- 6. Central and South American
_____ in 1000 AA have SCA and ______ have the trait.
3 in 1000
1 in 12
Patho of SCA?
sickled or elongated RBC -> stasis of RBC & increased blood viscosity -> obstructed blood flow -> tissue ischemia DISTAL to obstruction -> acute pain & cell infarction/death
Where does blood flow obstruction r/t SCA usually occur?
at bifurcations in BV
Dx tests for SCA?
- 1. HgB electrophoresis
- 2. sickledex (sickle solubility test)
- 3. reticulocyte count
Definitive test for SCA?
HgB electrophoresis - shows have SCA trait
Purpose of Sickledex test?
detects presence of HgBS but not the trait that causes SCA
What will reticulocyte count be like with SCA?
normal RBC lives for 120 days/4months. Sickled RBC dies withing 12 days so need increased RBC production
S/S of SCA are primarily the result of what 2 things?
1. obstruction caused by the sickled RBCs and tissue ischemia/infarction distal to obstruction
2. increased RBC destruction
What is a sickle cell crisis?
a broad term for several acute or exacerbating conditions occurring with sickle cell anemia
Etiologies of SCA crisis?
- 1. infection
- 2. dehydration
- 3. hypoxia
- 4. trauma/phys stress
- 5. general stress
3 types of SCA crisis/complications?
- 1. vaso-occlusive crisis
- 2. acute sequestration crisis
- 3. aplastic crisis
Char of carrier state in SCA?
- 1. normally has no s/s
- 2. under extreme stress may have s/s: extreme vigorous exercise, high altitudes
Most common SCA crisis type?
5 s/s & complications of vaso-occlusive crisis?
- 1. bone and joint pain
- 2. dactylitis
- 3. acute chest syndrome
- 4. priapism
- 5. CVA
Most serious complication of a vaso-occlusive crisis?
3 char of pain r/t vaso-occlusive crisis?
- 1. in bone and joints
- 2. can occur anywhere in body
- 3. mild to severe
inflammed/red & edematous hands and feet
4 S/S of acute chest syndrome r/t vaso-occlusive crisis?
- 1. chest pain
- 2. fever
- 3. cough if occluded in chest area
- 4. severe abd pain
erection that won't go down
7 S/S of a CVA r/t vaso-occlusive crisis?
- 1. altered LOC
- 2. vision changes
- 3. headache
- 4. hemi or monoparesis
- 5. aphasia/dysphasia
- 6. ataxia
- 7. seizures
weakness on one side of the body
one limb is very weak but not paralyzed
2 S/S of acute sequestration crisis?
- 1. splenic enlargement
- 2. hypovolemic shock
What is the main concern with acute sequestration crisis?
blood volume r/t risk for hypovolemic shock
Hemolytic anemia HgB level?
Potential causes of hemolytic anemia?
6 to 9 g/dL
abnormal decrease in reticulocytes
- 1. pallor
- 2. fatigue
- 3. lethargy
- 4. fainting
- 5. dyspnea/breathlessness
- 6. HA
12 Tx for SCA crises?
- 1. monitor V/S q 4 h or more prn
- 2. bed rest
- 3. maintain O2 sat >95% (96 or above)
- 4. PAIN CONTROL
- 5. age-app. non-pharmacologic pain relief
- 6. apply heat to painful joints prn
- 7. HYDRATION
- 8. strict I&O
- 9. perform passive ROM
- 10. Meds
- 11. admin of RBC
- 12. instruct in foods high in folic acid
Rationale for checking V/S q 4 h in SCA crises?
CH with SCA more at risk for infection r/t splenic damage due to crisis of acute sequestration
CH with SCA may have splenic dysfunction by age _____.
Why are SCA crisis pt put on bed rest?
to reduce O2 expenditure
Rationale for keeping O2 sat at 96 or above in SCA crisis?
ease work of breathing & facilitate tissue oxygenation
prevent more sickling - hypoxia can cause crisis
Pain control in SCA crisis?
Morphine around the clock
maintain cont analgesic blood level & reduce met. demand for O2 to help end sickling
Why use non-pharm pain measures in SCA crisis?
Why apply heat to painful joints in SCA crisis?
vasodilation - increase blood flow & O2 to the tissues
Fluids used for SCA crisis?
D5W1/2NS 1 to 1/2 X the maintenance rate
reduces viscosity of blood (water will go into cells) & provides glucose and fluid
alleviating sites of vascular occlusion and preventing further sickling caused by dehydration
D51/2NS is a _______-tonic solution.
3 functions of D51/2NS in SCA crisis?
cells hydrated, blood hydrated with free water, and gives glucose for energy
Why monitor I&O in SCA crisis?
Maintain UO at ______.
How to assess I&O of a baby/child?
monitor for kidney failure r/t infarction of renal artery
weigh diapers or tell parents to catch it in hat/urinal
3 meds that may be used in SCA crisis and their functions?
- 1. hydroxyurea - increases HgB levels
- 2. folic acid - needed for RBC production
- 3. RBC transfusion - last resort
Why admin RBC in SCA crisis?
to maintain HgB and increase O2 carrying capacity and decrease further sickling & microvascular ischemia
What drug may given to SCA crisis pt prior to a blood transfusion?
more at risk for transfusion reactions b/c receive so many of them & may dev hypersensitivity
8 foods high in folic acid?
- 1. green leafy veggies
- 2. broccoli
- 3. oranges, canteloupe
- 4. nuts
- 5. peanut butter
- 6. dried beans/peas
- 7. enriched breads & breakfast cereal
- 8. enriched pasta and grain
- 9. probably need a suppement
2 interventions to decrease blood transfusion reactions in SCA crisis pt?
- 1. give immunosuppressants before
- 2. give leukocyte-poor/washed RBC to decrease reactions
Major risk for _____ with SCA r/t ______.
What age does this start and when may there be total dysfunction?
infection r/t splenic dysfunction
starts as early as 6 mo.
5 years old
Tx for immunocompromised state of SDA?
daily penicillin starting at 2 months and ont. until at least 5 years or throughout childhood
3 immunizations that should be give to SCA child?
- 1. pneumococcal
- 2. meningococcal
- 3. influenza
12 things to teach parents for home care of child with SCA?
- 1. encourage fluids & increase for hot weather etc
- 2. expect frequent urination
- 3. provide rest
- 4. avoid cold (crisis) & extreme heat(dehydration)
- 5. avoid infectin
- 6. avoid prolonged sun exposure
- 7. monitor body temp & notify MD if increases - do not use antypyretics without talking to MD
- 8. Admin penicillin qd
- 9. avoid use of aspirin - acet/ibu alternatives
- 10. avoid high altitudes
- 11. call primary caregiver if s/s of infection
- 12.know MD phone #
autosomal recessive disorder char by abnormal HgB synthesis
3 races that have high incidence of BT?
mediterranean, Asian, AA
Onset of BT?
late infancy or early toddlerhood & lasts whole life
Cause of death in BT?
usually causes heart and/or liver failure by 20 to 30
4 S/S of BT?
- 1. severe anemia
- 2. growth maturation retardation
- 3. facial features changed
- 4. hepatosplenomegaly
Why does growth maturation retardation occur in BT?
5 char. of BT facial features?
- 1. enlarge head
- 2. frontal & parietal bossing(enlargement)
- 3. severe maxiallary hyperplasia (top bucked)
- 4. malocclusion - teeth not aligned
- 5. bronze skin tone
3 therapeutic management Tx for BT?
- 1. erythrocyte transfusion
- 2. chelation therapy
- 3. splenectomy
Why do BT need chelation?
have build up of Fe from blood transfusions
Xplinked autosomal recessive disorder with a dificiency in one of blood coagulation factors - lifelong and genetic
Hemophilia A & B?
Other factor that may be missing?
- A - factor VII deficient
- B- factor IX deficient
- factor XI
3 bleeding classifications of hemophilia?
- 1. mild - bleeding occurs with severe trauma
- 2. moderate - bleeding occurs with minor trauma
- 3. severe - bleeding occurs with NO traum. Bleeds spontaneously!
Who gets hemophilia?
Can a man with hemophilia pass it to his son?
no it is X-linked
Clinical manifestations of hemophilia in a newborn? (2)
- 1. abnormal bleeding from umbilical cord
- 2. prolonged bleeding after circumcision
3 s/s of hemophilia?
- 1. bruise easliy
- 2. epistaxis
- 3. bleeding anywhere
Most common sites of bleeding with hemophilia?
1. muscles and joints - hemarthrosis
4 Dx evals for hemophilia?
- 1. Hx of bleeding in infants/toddlers
- 2. test of hemostasis
- 3. test blood clotting
- 4. test clotting factor function
2 ways to test hemostasis?
What will be the results in hemophilia?
1. platelet aggretation- will be prolonged
2. bleeding time - increased
2 ways to test blood clotting?
results in hemophilia?
- 1. PT - increased
- 2. PTT - increased
How is clotting factor function tested?
fatory assays - will tell which clotting factor is affected
Most definitive test for hemophilia?
factory assays that show if clotting factors are affected
What vitamin increases the absorption of Fe?
Therapeutic management of hemphilia?
- 1. classification of bleeding
- 2. type of deficient clotting factor
Severe bleeding in hemophilia char & Tx?
- 1. factor replacement w/ human blood or recombinant clotting factors
- 2. RICE
How is factor replacement with hemophilia given?
When may a child admin own med?
admin IV over 5 minutes
8 years old
RICE in hemophilia?
- rest 24-48 hours
Tx of mild-mod bleeding in hemophilia?
Route and action?
DDAVP/desmopressin - stimultes release of factor VIII
Teaching for hemophilia child?
- 1. Avoid toys with sharp edges, sharp scissors, straight razors, IM/IV meds, aspirin/ibup/finger sticks
- 2. use soft toothbrusha dn water irrigating device
- 3. use elc razor
- 4. wear helmet with bike
- 5. wear joint pads when playing
- 6. padded crib sides
- 7. med ID bracelet or necklace
- 8. keep on hand at all times bags of ice or cold packs
What may be used in place of finger sticks for hemophiliacs?
venipuncture b/c causes less bleeding
Immune thrombocytopenic purpura?
low platelet count
idiopathic thrombocytopenic purpura or hemorrhagic disorder
5 char of ITP?
- 1. thrombocytopenia - <150,000
- 2. pupuric rash, petechiae, echymoses
- 3. normal bone marrow
- 4. absence of identifiable cause
- 5. autoimmune reesponse to disease
any rash in which blood cells leak into the skin or MM
pinpoint purpuric lesions
larger hemorrhages into the skin=- bruising
When is ITP usually seen?
after upper resp infections or viral infections: measles, rubella, mumps, chickenpox, 5th disease or parvovirus B19/slapped face
2 classifications of ITP?
1. acute - self-limiting course (1 to 3 months)
2. chronic - lasts longer than 6 months
2 Dx evals for ITP?
1. thorough Hx including: fam Hx, recent meds, recent vaccination w/ liv virus, recent viral infections o upper resp infection
2. low serum platelet count - below 150,000
3 clinical manifestations of ITP?
How will they begin?
- 1. bruising, petechiae and/or ecchymosis
- 2. bleeding gums
- 3. heavy menstrual flow
2 goals in management of ITP?
- 1. prevent rare, life-threatening bleeding events: intracranial bleeding
- 2. restore platelet count to above 200,000
4 nursing interventions for ITP?
- 1. assess for s/s of bleeding from any body system
- 2. monitor platelet count
- 3. no IM injections, aspiring or aspirin containing products, NSAIDS
- 4. restrict activity at onset: no bikes, contact sports, etc
Medical treatement of ITP?
- 1. acute ITP: watch and wait - monitor bleeding and monitor platelets
- 2. oral or IV corticosteroids : block autoimmune destruction of platelets
- 3. IV admin of immunoglobulins (IVIG)
Tx of chronic ITP that doesn't respond to meds?
will try to wait until at least 5 years old
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