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Module 5- PEDI > UNIT 3 HEMATOLOGY/IMMUNOLOGY > Flashcards

UNIT 3 HEMATOLOGY/IMMUNOLOGY Flashcards

1
Q

What is anemia?

A

Reduction in RBC mass and/or hemoglobin concentration

Decrease in the Hgb available to carry o2 to cell.

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2
Q

What are the 3 main causes of anemia?

A
  1. Inadequate production of RBC’s
  2. Increased destruction of RBC’s
  3. Excessive loss of RBC’s (trauma related)
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3
Q

What are the s/s of anemia?

A
  1. Tired
  2. Cold
  3. Increased HR
  4. Tachypnea
  5. Cool
  6. Poor circulation (blue extremities)
  7. Prolonged cap refill
  8. Pale
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4
Q

What is the last stage of development for a RBC?

A

Reticulocyte

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5
Q

What is the life span of a RBC?

A

120 days

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6
Q

What is the “homeostatic balance” of a RBC?

A

RBC production = RBC destruction

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7
Q

RBC transports …..

A

Hemoglobin–> o2 to cells

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8
Q

What lab can be drawn to look at what the body is doing during an anemic state?

A

Retic count

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9
Q

An increased Retic count tells us what?

A

There is an increased production of RBCs

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10
Q

What should we know about fetal hgb?

A
  1. Fetal hgb and oxygen has a super magnegtic charge
  2. This is important because as you age your body stops producing fetal hgb. However, in some disease like sickle cell and beta thalassemia major the body may continue to produce fetal hgb into adulthood to help with chronic anemia
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11
Q

What regulates the production of RBC’s

A
  1. Tissue oxygenation
  2. Renal production of erythropoietin
    • Erythropoietin is produced by the kidneys. If kidney damage occurs erthropoietin which is an extra layer on top of anemia
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12
Q

What happens as a RBC ages?

A
  1. The membrane ruptures
  2. Hgb is boken down
    • Fe containing pigment (hemosiderin)–> most reused in bone marrow for RBC production
  3. Bile pigment (bilirubin)
    • Excreted by the live in bile
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13
Q

What is a sign of too many RBC’s being broken down?

A

Free flow iron in the blood– too much can lead to organ dysfunciton with a direct impact on the brain.

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14
Q

Where is excess iron stored?

A

Liver

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15
Q

What might we see on our assessment of a child with anemia?

A
  1. Pallor
  2. fatigue
  3. weakness
  4. tachycardia
  5. increased resp. rate
  6. Dizziness (not enough o2 carried by hgb)
  7. headache (brains way of signaling the need for o2 carried by hgb)
  8. irritability

In severe anemia– hyperventilation

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16
Q

Children with anemia are prone too…..

A
  1. Infection
  2. Infections made worse by anemia
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17
Q

What are normal hgb ranges for a newborn?

A

14-24

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18
Q

What are normal hgb ranges for an infant?

A

10-17

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19
Q

What are normal hgb levels for a child?

A

9.5-15.5

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20
Q

What are normal hgb levels for a adolescent?

A

12-18

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21
Q

What is iron deficiency anemia (IDA)?

A

Hgb levels below normal range because of body’s inadequate supply, intake, or absorption of iron

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22
Q

What do they believe is the cause of IDA?

Iron Deficiency Anemia

A

May be caused by
1. inadequate iron stores during fetal development, 2. deficient dietary intake
3. Chronic blood loss,
4. Poor utilization of iron by body

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23
Q

What happens in the 3rd trimester of preganancy that is related to iron?

A

The third trimester is when moms iron storage is transferred to baby.

If a baby is born preterm they will not recieve the iron storage from mom putting them at risk for being anemic

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24
Q

What children are at highest risk of developing IDA?

A
  1. Preterm infants, multiple births
  2. Mother has iron deficiecny
  3. infants 6-24 months
    • Growth spurt
  4. toddlers
    • Picky Eaters
  5. Female adolescents
    • Menses, growth spurt, diet
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25
Q

What are the clinical manifestations of IDA?

A
  1. Pallor/paleness of mucous membranes
  2. Tiredness/fatigue
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26
Q

What are possible findings of a child with IDA?

A
  1. Overweight “milk baby”
    • taking too much cows milk (not a good source of Fe)
  2. Dietary intake low in iron
  3. Milk intake >32 oz. /day
  4. PICA habit (eating nonfood substances)
    • Body craves other things like dirt, chalk, clay.. trying to get other fe sources
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27
Q

How can we manage IDA with our diet?

A
  1. Iron fortified formula or cereal
  2. Dietary addition of iron rich foods
    • Green leafy veggies, Red meat, liver, fish, cereal with Fe
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28
Q

What should we know about oral iron supplements to treat IDA?

A

Ferrous Sulfate (Fer-in-Sol)
1. Can upset their stomach, cause stool color changes (black tarry/green), constipation (increase fluids & fiber)
2. In one month they can look at reticulite count and see if the body is regenerating more RBCs… If not changes w/in a month they will investigate further to r/o chronic bleeding or iron absorption issue

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29
Q

How might we manage severe anemia caused by IDA?

A

Blood transfusion
1. Packed RBCs to minimize chance of circulatory overload
2. Supplemental oxygen could be administered as well if tissue hypoxia is severe

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30
Q

What parent education should we provide about Iron Administration?

IDA

A
  1. Give on empty stomach
  2. Give with citrus juices (vitamin C)
    • Increases absorption of fe in the bloodstream
  3. Use dropper or straw as it can stain teeth
  4. Stools will be tarry
  5. Safety from overdose keep locked away
  6. Do not give with milk will decrease the Fe absorption
    7.Limit milk intake to less than 32 oz./day
    8.Dietary sources of iron
    - promote diet of fish, liver, whole grains, lagoons, grean leafy veggies
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31
Q

What is the prognosis of IDA if it is left untreated?

A

If anemia is severe and longstanding
1. Diminished cognitive function
2. Behavioral changes
3. Delayed growth and development.

In extreme cases can lead to death

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32
Q

What is sickle cell anemia?

Bolded most important

A

It is a autosomal recessive genetic disorder that results in the formation of abnormal hemoglobin chains

Normal adult hemoglobin A (hbA) is partly or completely replaced by abnormal sickle hemoglobin (hbS)

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33
Q

What is the likely hood of passing on sickle cell?

A

If both babys parents have trait there is a 25% chance the child will NOT have the disease/trait

50% chance that they will be a carrier and a 25% chance that they WILL have sickle cell anemia

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34
Q

True or false: Under certain circumstances, the abnormal RBC “sickles” resulting in occulusion of small blood vessels, ischemia, and damage to the affected organs

A

True

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35
Q

What happens with “sickling”? in Sickle Cell

A

Under certain conditions (triggers)
HbS changes molecular structure to form an elongated crystal

  1. RBC changes from pilable disc to cresent (sickle)
  2. Distorts RBC membrane
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36
Q

What is the life span of a “sickled” RBC?

A

Less than 40 days which leads to chronic anemia

Eventually the sickled RBCs will pile up and cause ischemia to an organ or peripherally

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37
Q

What are some triggers to “sickling”

A
  1. Dehydration
  2. Acidosis
  3. Hypoxia
  4. Temperature changes (esp. in winter months)
  5. infection
  6. emotional stress
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38
Q

At what age do s/s start to present in sickle cell anemia?

A

After 6m. of age

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39
Q

What is special about fetal hgb with sickle cell anemia?

A

It does not sickle

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40
Q

What are the effects of “sickling”

Sickle cell anemia

A
  1. Abnormal adhesion, entanglement and enmeshing of rigid sickle-shaped RBC
  2. Inflammatory process
  3. Intermittent blocking of microcirculaiton
  4. Vaso-occulsion
  5. Absence of blood flow to adjacent tissues
  6. Local hypoxia
  7. Tissue ischemia and infarction
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41
Q

What prenatal diagnositc testing is available for sickle cell?

A
  1. Chorionic villus sampling from prenatal tissue
  2. Aminocentesis
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42
Q

What diagnostic testing is available after birth for sickle cell?

A
  1. Newborn screening
  2. Sickledex (sickle turbidity test)
    • If postive, hemoglobin electrophoresis which sends electricity to RBC to see if it sickles
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43
Q

What are general signs/symptoms of sickle cell?

A
  1. Chronic hemolytic anemia
  2. Frequent infections
  3. fatigue r/t anemia
  4. delayed physical growth– smaller height/weight than peers
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44
Q

A sickle cell “crises” is an acute exacerbation characterized by….

A
  1. Vaso-occlusive crisis (VOC)
  2. Splenic sequestration crisis
  3. Aplastic crisis
  4. Hyperhemolytic crisis
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45
Q

What do we need to know about a vaso-occlusive crisis r/t a sickle cell crisis?

A

“Pain Crisis”
1. Ischdemia causing pain where cells arent getting oxygen
2. can last 3-4 days

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46
Q

What is a splenic sequestration crisis r/t a sickle cell crisis?

A

Pooling of a large amount of blood into the spleen/liver and will cause a drastic decrease in blood volume (hypovolemic) this is LIFE THREATENING and death can occur in hours in not treated.

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47
Q

What age group is most at risk of experiencing splenic sequestration crisis r/t a sickle cell crisis

A

6m-5 years

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48
Q

What is aplastic crisis r/t sickle cell crisis?

A

Viral illness will trigger a decrease production of RBC

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49
Q

What is hyperhemolytic crisis r/t sickle cell crisis?

A

Increased destruction of RBC

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50
Q

What are “classic” or acute signs of vaso-occlusive crisis (VOC)?

A
  1. Acute pain– rated extremely high
  2. Fever
  3. Severe abdominal pain
  4. Painful edematous hands and feet
    • Hand-foot syndrome (infants)
  5. Arthralgia (painful joints)
  6. Leg ulcers (adolecsents) due to imparied ciruculation
  7. Cerebrovascular accident (CVA)
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51
Q

What are some chronic signs of Vaso-Occlusive crisis

Sickle cell anemia

A
  1. Splenomegaly–> autosplenectomy
    - Repeated insults of the spleen lead to infarction and body eventually kills off spleen which puts you at risk for infection.
  2. Hepatomeglay–> liver failure
  3. Kidney abnormalities–> hematuria, inablility to concentrate urine, enuresis, possible renal failure
  4. Bone changes–> osteoporosis, skeletal deformities
  5. Retinal detatchment, Blindness
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52
Q

What are some additional complications of VOC?

Acute chest syndrome

Sickle cell anemia

A

Acute chest syndrome
1. Fever greater than or equal to 101.3
2. Cough
3. Chest pain
4. Tachypnea
5. Dyspnea
6. Wheezing
7. Decreased o2 saturation

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53
Q

Treatment of acute chest syndrome- VOC includes

Sickle cell anemia

A
  1. Antibiotics
  2. oxygen
  3. breathing exercises
  4. blood tranfusions
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54
Q

True or false: If Acute chest syndrome w/ VOC in sickle cell is impacting a small area it is usually self limiting but if it is left untreated or impacts a large area it can cause resp. distress and can lead to death.

A

True

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55
Q

How can we manage VOC “crisis”

A
  1. Hydration
    • Replace electrolytes
    • Strict I&O
    • watch for kidney impairment
  2. Analgesics
    • Warm compresses
    • Narcotics/PCA pumps
  3. Blood transfusions
    • More RBC in to tx chronic anemia
  4. Antibitoics for infections
  5. Hydroxyurea
    • Med to increase fetal hgb production
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56
Q

What medication can be used to increase production of fetal hgb?

A
  1. Hydroxurea
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57
Q

True or false: Cold compresses are recommened in the management of sickle cell anemia?

A

False– Cold compresses are contraindicated it can trigger a crisis and causes vasocontriction

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58
Q

How can we manage VOC pain

A

Mild and Moderate pain
1. Tylenol
2. Ibuprofen

Severe Pain
1. Opiods (morphine, hydromorphine, hydrocodiene)
2. Kytoralic

Give oral or IV and administer on a schedule. Use PCA if appropriate. Keep on schedule to minimize break threw pain

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59
Q

What medication is contraindicated in the treatment of pain r/t VOC in sickle cell anemia?

A

Meperdine can cause seizures

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60
Q

What education should we provide with Sickle Cell anemia?

A

Prevent Hypoxia
1. Avoid strenous excercise (can lead to dehydration)
2. Avoid high altitudes
3. Avoid being around anyone who is sick and seek care at first sign of infection
4. Use prophylactic penicillin if prescribed (prevent streptococcus phenomena…. if allergic use penicillin
5. Keep child well hydrated (do not withhold fluids at night)

In addition
1. Stay up to date on immunization
2. Refer to family for genetic counseling
3. Provide emotional support

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61
Q

What is the prognosis of sickle cell anemia?

A

Variable depending on the severity of the disease

Most patient will live to be in their 50’s greater risk for patients younger than 5

Death usually occurs due to overwhelming infection

Stem Cell transplantation

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62
Q

What is beta thalassemia major?

A

AKA Cooleys anemia

It is a autosomal recessive disorder. It is the partial or complete deficiency in the synthesis of the b chain of the hemoglobin molcule.

Results in a life-threatening anemia requiring life-long blood transfusions that lead to iron overload

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63
Q

What is the patho of Beta thalassemia major?

A
  1. Dramatically decreased or complete deficiency in synthesis of b chain in hgb molecule
  2. Unbalanced polypeptide unit is very unstable
  3. The hgb molecure disintegrates and damages the RBC
  4. The RBC prematurely breaks down
  5. Causing severe hemolytic anemi a
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64
Q

What are the clinical manifestations of beta thalassemia major

A
  1. Severe anemia
  2. Chronic hypoxia
  3. Small stature–> FTT
  4. In some adolescents– delayed puberty
  5. bronzed skin tone, possibly
    • Due to high biliruben
  6. Hepatosplenomeglay
    • spleen becomes hyperactive/large, liver enlarged
  7. Cardiomegaly
  8. Bone changes
    • In older children who are untreated.
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65
Q

What is the life span of a RBC in beta thalassemia major

A

30-60 days

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66
Q

When does beta thalassemia major typically present?

A

Within the 1st 2 years of life… child may be pale, fussy, poor appetitie and lots of infections and then they develope the life threatening edema

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67
Q

What might the the reticlic count look like in beta thalassemia major?

A

Increased

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68
Q

What prenantal diagnostic testing is available for beta thalassemia major?

A
  1. Chorionic villus sampling from prenatal tissue
  2. Aminocentesis
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69
Q

What diagnostic testing is available for beta thalassemia major after birth?

A
  1. Newborn screening
  2. CBC
  3. Hemoglobin Electrophoresis
  4. Xray
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70
Q

What is the of treatment in beta thalassemia major?

A

Maintain adequate hgb levels via transfusion progam

  1. Maintain hemoglovin about 9.5 gm/dl
  2. Transfuse every 3-5 weeks
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71
Q

What are the advantages of blood transfusions with the treatment of beta thalassemia major?

A
  1. Improve well wbeing (physical and mental)
  2. Decrease cardiomeglay/hepatomeglaly
  3. Prevent bone changes
  4. Promote normal or near normal growth and development
  5. Decrease infections
72
Q

What is Hemosiderosis & Chelation?

A
  1. Iron overload secondary to transfusion
    • Deposited in tissues
  2. Chelation is the treatment of hemosiderosis
73
Q

What are different types of chelating agents?

A
  1. Desferal (deferoxamine)
    Given IM or IV at infusion center
    SQ- portable infusion for 8 hours 4-6 nights a week
  2. Exjade (deferasiorox)
    PO to children over 2 years of age
74
Q

What education do we need to provide about chelating agents?

A
  1. These meds will bind to iron and ecrete through the urine so inform parents that urine may be orange/rusty
  2. Make sure they do yearly hearing vision screen because these meds can effect vision… contact hcp immediately if vision changes occur
  3. Vitamin C helps get the iron back into the blood so that these meds can find and attach to the iron and help get rid of it.
75
Q

What med is given in conjunction to cheletaing agents?

A

Vitamin c- helps get all the iron out of the tissues and back into the blood so chelating agents can attach to the blood and be excreted through the urine

76
Q

What should we know about hypersplenism & splenectomy in regards to beta thalassemia major?

A
  1. Hypersplenism
  2. Splenectomy
    • Treats severe splenomeglay and increases life span of transfused RBCs
    • Increase susceptibility to overwelming infection
    • NOTIFY HCP at any sign of infection
  3. Interventions
    • Prophylactic antibiotics- for the rest of their lives
    • ## Routine immunizations
77
Q

What is the prognosis of beta thalassemia major?

A
  1. With treatment- children liveing into adulthood.
  2. Death usually heart disease, or multi organ failure secondary to hemochromatosis (iron overload)
  3. Stem cell transplantation
78
Q

What do we need to know about lead poisioning?

A
  1. Approx. 1/2 million children between 1 and 5 years of age living in the U.S. have blood levels of lead more than 5mcg/dL
  2. There is “NO SAFE” level of lead for children
  3. Lead exposure and elevated levels have been linked to decreased IQ
  4. Lead causes anemia
79
Q

How are children exposed to lead?

A
  1. Ingestion– most common route
    - Crib rails
    - windowsills
    - older plumbing
    - contaminated soil
    - paint chilps
    - ANd many other sources
  2. Inhalation
    • REnovation of old homes (sanding and old paint)
80
Q

What are risk factors of lead poisioning?

A
  1. Children younger than 6 years old… Chilren absorb and retain more lead than adults
  2. Living in poverty
  3. Living in urban areas
  4. Living in older homes
  5. Children w/anemia
    • Correlation that led binds to hemaglobin = at risk for retaining more lead
81
Q

What are clinical manifestations/general signs of lead poisioning?

A
  1. Anemia
  2. cramping, abdominal pain
  3. vomiting
  4. constipation
  5. anorexia
  6. headache
  7. lethargy
  8. impaired growth
82
Q

What are some EARLY CNS manifestions of lead poisioning?

A
  1. Hyperactivity
  2. aggression
  3. impulsiveness
  4. Decreased interest in playing
  5. Irritability
  6. Short attention span
83
Q

What are some LATE CNS manifestations of lead posioning?

A
  1. Mental retardation (decreased IQ)
  2. Paralysis
  3. Blindness
  4. Convulsions/Seizures
  5. Coma
  6. Death
84
Q

How do we test for lead poisioning?

A
  1. Blood lead level (BLL) test
    • Universal screening
    • All children at ages 1 and 2 years
    • Any child between 3 and 6 years if never screened
  2. Target screening
    • If reside in high-risk geographic areas
    • If in “at risk” group
    • If family cannot respond “no” to personal risk questions
85
Q

What are interventions for lead poisioning?

A
  1. Identify sources of lead in the child’s envionment
    • If they live in an old home- avoid vacumming floors and window seals, wash childs hands requently, wash toys/pacis frequently, assure lead poisioning isnt coming from parents works (construction, pottery/paints)
  2. When BLL is over 45 mcg/dL
    • Chelation to help protect kidneys
  3. Equilibrium process- multiple treatment may be necessary

Test blood leels and repeate tx if needed

86
Q

What are types of chelation meds that might be used for lead poisioning?

A
  1. Succimer (chemet)
    • PO daily for 19 days
    • Capsule or sprinkle
  2. Calcium disodium (EDTA) IM
    • Very painful when administered IM
87
Q

What is hemophilla?

A

Results– deficiency, dysfunction, or absence of coagulation factor VIII or factor IX… effects is bleeding anywhere in the body externally/internally. Can be moderate, mild or severe.

  1. Inherited x-linked recessive bleeding disorder
  2. Mom Carries it and MALES have the disease
  3. Boys have the xy chromosome unlike girls who have the 2 x’s that would cancel each other out
88
Q

What is hemophilla A?

A

“Classic hemophilla”- factor 8 VIII (75%)

89
Q

What is hemophilla B

A

“christmas disease” Factor 9 IX (25%)

90
Q

The less ____ the body produces the more severe the hemophilla?

A

Factor

  1. In mild hemophilla the body makes 6-50%
  2. In moderate hemophilla the body makes 1-5% factor
  3. In severe hemophilla the body makes less than 1% (most of the population)
91
Q

In mild cases of hemophilla how much factor does the body produce?

A

6-50%

92
Q

In moderate cases of hemophilla how much factor does the body produce?

A

1-5%

93
Q

In severe cases of hemophilla how much factor does the body produce?

A

Less than 1%– this is most of the population who has hemophilia

94
Q

How is hemophilla diagnosed?

A
  1. History of bleeding eipisodes, genetic testing, PTT to test clotting factors if less than 25%
95
Q

On our assessment of a child suspected of having hemophilla what symptoms might we observe?

A
  1. Male child with prolonged bleeding
    • Seen possibly w/ umbilical cord, vit k injection, post circumcision
  2. Proloned bleeding anywhere from or in the body
  3. Hemoarthrosis (most frequent site of bleeding)
    • Knees, elbows, ankles– loss of motion in joints
    • swelling of joint, warmth and pain, tingling, fullness in joint, mod to severe pain, synvial joint thickens… these joins become known as “target joints” which will lead to joint immobility and dysformity
  4. Spontaneous bleeding subcutaneous tissues and muscles
    • Excessive bruising, even from a slight injury
    • pooling of blood in tissues may cause hematoma
  5. Spontaneous hematuria or blood in stool
  6. Frequent and hard to stop nose bleeds.
96
Q

True or false: Children with hemophilla bruise more easily?

A

True

97
Q

How do we teach parents to stop nosebleeds if there child has hemophillla?

A
  1. apply pressure for at least 15 mins .
  2. Can insert cotton/tissue into nostril
  3. Ice
  4. Keep child calm and head positioned down
98
Q

What are ways we can recongize and control bleeding in children who have hemophilla?

A
  1. Believe the child
    • esp. if they report pressure in joints, c/o tingling and pain.. the faster we tx the better the outcome
  2. RICE (dosent replace factor insusion)
  3. Factor replacement per bleeding episiode and prophylactically
    • 2-3 times per week… starting when they are very young.. can have them more often if they have more bleeding episodes.
99
Q

What should we know about factor replacement in the tx of hemophilla? GENERALIZED

A
  1. Given 2-3 times per week or more if they have more bleeding episodes.
  2. Starts when patients are young
  3. Most children will have a port and parents are taught how to access and then as the child ages they can be tought.
  4. Wont premix factor because it is expensive and only good for a couple hours
100
Q

What should we know about Factor replacement more specifically?

A
  1. Genetically engineered recombinant factor VIII and IX
    • Recommeded over factor concentrated from pooled plasma
    • Starting at 2-3 years of age- family to start venipuncture or access central line and admin factor replacement
    • 8-12 year-of-age- chid may be taught how to self admin factor and most will have a port-a-cath
101
Q

What drug can we use for mild factor VIII deficiency?

A

DDAVP (synthetic vasopressin)

102
Q

What are some ways we can prevent bleeding in a chlild with hemophilla?

A
  1. Strengthen muscles and joints
  2. Prevent oral bleeding
    • Soft bristle toothbrush wet with warm water prior to use, waterpick instead of floss,
  3. Avoid IM injections if possible
    • SQ over IM if possible
  4. Venipunctures instead of heel sticks/finger sticks
  5. Educate to recongize early s/s of hemorrhage
  6. Avoid asprin, excedrian and ibuprophen
103
Q

What are complications and the prognosis of hemophillia?

A
  1. Inhibitors
    • Factor specific antibodies
    • Develop after child has received factor replacement
  2. No cure
  3. Average life expectancy
104
Q

What sports can a child with hemophillia participate in?

A
  1. Track
  2. Swimming
  3. Golf
  4. Bowling
105
Q

What sports should a child with hemophillia avoid?

A

Contact sports such as
1. Football
2. basketball
3. hockey
4. tennis
5. baseball

106
Q

What is immune thrombocytopenia (ITP)?

A

An autoimmune disorder charaterized thromboxytopenia it occurs when immune system mistankenly attacks and destroys platelets

Typically acute and is self limiting

Peak 2-6 year olds… sumptoms come on suddleny but dissapear within a few weeks.

107
Q

What is the cause of immune thrombocytopenia?

A

Etiology unknown but most often presents after viral infection

108
Q

True or false: Acute ITP doesnt usually reoccur once the child is better

A

True

109
Q

ITP is more common in ____ than ____?

A
  1. Adult
  2. PEDI
110
Q

On our assessment of a child with immune thrombocytopenia what might we see?

A
  1. Easy brusing
  2. Petechiae
    3.Bleeding from muscus membrane
    • Nose bleeding from abraisions
  3. Prolonged bleeding from abrasions
111
Q

What tests can diagnose immune thrombocytopenia?

A

No definitive tests

CBC
- Platelets <20,000/mcl
Normal 150,000-450,000

112
Q

Supportive care of ITP includes?

A
  1. Restrict activities while platelets are under 50,000
  2. Monitor s/s of bleeding
    • Increased rr/hr/bp decreases, monitor entire body for bleeding and bruising
  3. Prednisone, immune globulin (IVIG), anti-d antibody
113
Q

Supportive care of chronic ITP

A
  1. Splenectomy
    if over 5
    Prophylactic penicillin
114
Q

How is HIV transmitted?

A

Vertical- When?
1. Late in pregnancy
2. Labor and delievery
3. Breastfeeding
Give ART to pregnant mothers w/HIV

Horizontal- How?
1. Sexual conduct
2. HIV infected blood products
3. Adolescents-high risk— risky behavior
4. sharing needles

115
Q

What diagnostic tools can we use to dx HIV?

A
  1. MOTHER HIV postive–
    • Maternal antibodies persist up to 18 months
    • Infants born to HIV mothers will test positive EVEN if they do not have HIV
  2. More specific tests are reuired in infants’ birth-18 months of age
    - 18m or older you can use the Elisa or wester blot test to determine HIV infection
    - If under 18m tested with the HIV polymerace chain reaction
116
Q

What are the clinical manifestations of HIV in children?

A
  1. FTT (low weight)
  2. Lymphadenopathy
  3. Hepatosplenomegly
  4. Neuropathy
  5. Cardiomyopathy
  6. Chronic/recurrent infections
    • Oral thrush- white fuzzy stuff in mouth
  7. Unexplained fever
117
Q

What are our goals of HIV treatment in children?

A
  1. Slow growth of HIV virus
  2. Prevent and treat opportunistic infections
  3. Provide nurtirional support
  4. Promote normal growth
118
Q

What medication treatment is available for HIV treatment in children?

A
  1. Antiretroviral medication (life long)
  2. IV gamma globulin (IVIG)
119
Q

What are some interventions we can provide for children with HIV?

A
  1. Prevent opportunistic infections
    • Avoid crowds, prophylactic antibiotics (trimethroprim sulfmethoxazole)
  2. Standard precautions
    • Hand hygiene gown and gloves
  3. Nutritonal support
    • HIV can lead to FTT ensure patient has nutritious meals
  4. Protect privacy (HIPPA)
  5. Immunizations– NO LIVE VACCINES
120
Q

What are cardinal symptoms of cancer in children?

A
  1. Unusual mass or swelling (enlarged lymph nodes)
  2. Unexplained paleness & loss of energy
  3. Sudden tendency to bruise
  4. Persistent, localized pain or limping
  5. Prolonged, unexplained fever or illness
  6. frequent headache, often w/vomiting- seen with brain tumor patients
  7. Sudden eye or vision changes
  8. Excessive, rapid weight loss
121
Q

What is leukemia?

A

Characterized by an unrestricted proliferaition of immature white blood cells in the blood-forming tissues

Most common form of cancer in children

Most common type
- Acute lymphocytic leukemia (ALL)

122
Q

What is the primary treatment for luekemia?

A

Chemo

123
Q

What is the patho of Leukemia?

A
  1. Competition for nutrients, infiltration, replacement in bone marrow
  2. Decreased RBCs, WBCs, platelets (bone marrow supression)
  3. Anemia, infection,bleeding, bone & joint pain, weakening of the bone, physiologic fractures
124
Q

What are s/s of Leukemia?

A
  1. Anemia
  2. Infection
  3. Bleeding
  4. Bone & Joint pain
  5. Weakening of the bone
  6. Physiologic fractures
125
Q

In leukemia blast cells migrate to what other organs?

A
  1. Spleen
  2. Liver
  3. Lymph glands
  4. CNS
126
Q

What clinical manifestations might you see with Leukemia?

A
  1. Minor infection that fails to completely disappear
  2. Infection, fever r/t neutropenia
  3. Pallor, fatigue, weakness, lethargy r/t anemia
  4. Petechiae, bleeding, brusising r/t thrombocytopenia
  5. Bone joint pain r/t leukemic infiltration of bone marrow
  6. Enlarged lymn nodes, hepatosplenomegaly
  7. Anorexia, weight loss
  8. Headache,vomiting (if CNS involvement)
127
Q

Leukemia is suspected from?

A
  1. Hx and physical manifestation
  2. Labs including CBC w/diffrential
  3. Hospital admission
    • Bone marrow biopsy and aspiration (BMA)
      • Lumbar puncture
128
Q

If a suspected leukemia patients lumbar puncture comes back negative how are they treated?

A

Treat as a ITP patient

129
Q

What is the prognosis of Leukemia?

A
  1. The higher the leukocyte count at diagnosis = worse prognosis
130
Q

When are kids typically diagnosed with Leukemia?

A
  1. 1-9 years old

Approximately 15-20% relapse within the 1st year

5 year survival rate= 85-90%

131
Q

What is a neuroblastoma?

A

It is a solid tumor that forms in the developing nerve cells, or neurons, of the sympathetic nervious system

Most develop in the adrenal glands, abdomen or nerve cell next to the spinal cord but can also be found in the head neck chest and pelvis

Most common extracranial solid tumor of childhood

132
Q

When are most kids diagnosed with a neruoblastoma?

A

Infancy- 14 years of age.. More rare to see in children over the age of 10

Most diagnosed cancer in children younger than 1 year of age. The median age at diagnosis is between 1-2 years old

133
Q

True or false: A neuroblastoma does not cross the midline?

A

False: it crosses the midline

133
Q

S/S of a neuroblastoma depend on what?

A

Location and stage

134
Q

Why is a neuroblastoma called the “silent tumor”

A

Typically found in the abdoment and because of that and the space the abdomen can hold it often grows undetected. By the time most tumors are found there is a 70% chance that it has metastasized somewhere else

135
Q

If a child has a neuroblastoma in the abdomen what symptoms might they present with?

A

Firm, non tender and irregular mass in the abdomen

136
Q

What is the prognosis of a neuroblastoma?

A
  1. Younger than 1- 80% chance of survival
  2. Older than 1 50% chance of surival
    The younger the better the prognoisis

If caught before it has metastized there is a 90% curitive rate.

Prognosis is typically poor due to the late dianosis.

137
Q

What is a Wilms tumor (nephroblastoma)

A

Fast growing, asymptomatic, firm encapsulated mass located on one side of the abdoment.

Will grow large before its noticed but they are typically found before spread to other parts of the body

138
Q

At what age does Wilms tumor (nephroblastoma) occur most frequently in?

A
  1. 2-5 years
139
Q

What is the prognosis of a wilms tumor (nephroblastoma)

A
  1. 90% cure if found in stage 1
140
Q

How is a wilms tumor (nephroblastoma) treated?

A

1.Surgery- during surgery the tumor, kidney and adrenal glands removed

141
Q

What cant we do to a wims tumor (nephroblastoma)?

A

DO NOT PALPATE
Since it is an encapulated mass it could rupture allowing the cancer to spread

142
Q

What is a retinoblastoma?

A
  1. Cancerous tumor arising out of the retina
    • Usually unilateral
    • May be inherested
143
Q

What age does Retinoblastoma commonly affect?

A

Children under 5 but most of the time under 2

144
Q

What are signs and symptoms of retinoblastoma?

A
  1. Opaque white area in pupil (leukocoria)
  2. Strabismus
  3. Decreased vision in one eye
  4. eye pain
  5. larger than norma eyeball
  6. Lazy eye
  7. Redness of the eye

ANY OF THESE SYMPTOMS CHILD WILL NEED TO SEE OPHTHALMOLOGIST– TUMOR WILL BE STAGED BY THEM BEFORE SURGERY

145
Q

What is the treatment for retinoblastoma?

A
  1. Laser treatment
  2. Cryotherapy
  3. Chemotherapy
  4. Radiation
  5. Enucleation surgery
146
Q

What are the 3 main goals that guide the tx of retinoblastoma?

A
  1. Anything to save the childs life
  2. anything to save the eye
  3. Vision last
147
Q

What parent education could we provide with enucleation surgery?

A
  1. This will be the removal of the eye and the child will be fitted for an occular implant. Later on they will get a artifical eye
  2. To clean the artifical eye: warm water and mild soap.
148
Q

What is phase 1 of chemotherapy?

A

Phase 1: Induction
Goal: Remission
TIme of high risk because medications cause myelosupression- increased risk of sepsis
Start with 100 billion leukemic cells

CNS prophylaxis
1. Goal-kill or prevent leukemic cells in CSF
2. Intrathecal administration
3. Cranial radiation reserved for high risk patients

149
Q

What is phase 2 of chemotherapy?

A

Consolidation (intesification)
Goal: Destroy any residual leukemia
After completion of remission theraoy

Kills the last 100 million cells we have… phase last about 1-2 months and is a combo of different meds

150
Q

What is phase 3 of chemo?

A

Maintenance
Goal- remain in remission
After successful completion of induction and consolidation therapy

last 2-3 years

boys at higher risk of prolapse than girls

151
Q

What are different risks for injury related to malignant process & treatment?

A
  1. Chemo– immunosuppressed
  2. Radiation therapy– damage to radiated tissue area and can also decrease the immune system
  3. Procedures… increased risk for skin breakdown, infection and bleeding
152
Q

What is extravasation in chemo?

A

It is when chemo is leaking out of the port into the tissue

Ex- redness, pain, swelling,itching

153
Q

What are s/s of anaphylaxis in chemotherapy?

A
  1. Cyanosis
  2. Hypotension
  3. Wheezing
  4. Urticaria
  5. Itching/rash

Watch child close for the 1st 20 mins of infusion

Have ambu bag, flow meter connected, suction and canister ready

154
Q

What do we need to know about radiation?

A
  1. Uses high-energy beams to destroy cancer cells
  2. Changes DNA in neoplastic cells so that it cannot reporduce
  3. Usually scheduled over 1-6 weeks
  4. Can be external or internal
155
Q

Radiation teaching should include?

A
  1. Targeted area/site is marked with indelible ink– try not to mess it up
  2. No soaps, creams, lotions or powders on area
    • Can irritate skin and some powders contain metals which would redirect radiation
  3. Wear soft, loose cotton clothing
    • So it doesnt rub skin
  4. Keep area/site protected from the sun

Most common side effect is radiation fatigue

156
Q

What is the function of neutrophils?

A

Fight bacterial infections

157
Q

What is the point of NADIR

A

Bone marrow suppression at its greatest, neutrophil count at its lowest. Each chemo agent that causes bone marrow supression has this

NDAIR is individual for each drug
- usually 7-14 days

158
Q

Normal WBC is between….

A

5-10k

The lower the count the hight the risk of infection can be as low as sero w/chemo

159
Q

What is a absolute neutrophil count (ANC)

A

Measures the body’s ability to fight infection

160
Q

What are segs?

A

Mature neutrophils

161
Q

What are bands?

A

Immature neutrophils

162
Q

Risk of infection is based on….

A

ANC

163
Q

How do we calculate ANC?

A

Neutrophils (segs + bands) x WBC X 10
1. ANC <1500 warrents eval
2. ANC <500 places child at greatest risk for infection

164
Q

What are some infection prevention tips for children with cancer?

A
  1. Monitor for s/s of infection
  2. Private room w/restricted visitors
  3. Reverse/protective isolation is prescribed
  4. No fresh fruits or veggies
  5. No pets at home
  6. No live vaccines– can cause severe reaction.. encourage family to get all immunizations
  7. Admin prescribed prolylactic medications
    • Antibiotics
    • Antifungals
165
Q

What things place children at risk for bleeding?

A
  1. Monitor for signs of bleeding
  2. Avoid unnecessary skin punctures
  3. use aseptic tecnique for fingersticks, venipunctures, im injections
  4. Meticulous mouth care
    • Soft toothbrushes, toothette
  5. Avoid activites that might cause injury or bleeding
    • Avoid contact sports
  6. Nosebleeds
  7. Platelets (low)
  8. No ibuprofen or asprin
166
Q

How can we manage n/v –r/t chemo tx

A
  1. Serotonin-receptor antagonists before chemo
    • ondansetron
    • Granisetron (kytril)
    • Aprepitant (emend)

Administer up to 1 hour before chemo often combined with dexamethasone

  1. Avoid foods with strong odors.
167
Q

How can we manage anorexia/loss of appetitie secondary to chemo?

A
  1. Avoid pressure to eat
    • Give pleny of options and opportunities to partcipate in eating (high protien and high calorie) but if they want ice cream and chicken nuggies let them have it.
    • They may have sores in their mouth and may need TPN
  2. Do not let food become a control issue
  3. Small, Appealing meals w/increased calories and protien

AVOID FRESH FRUITS AND VEGGIES

168
Q

How can we prevent mucosal ulceration?

A
  1. Offer bland, soft foods
  2. Use a soft sponge toothbrush
  3. Provide frequent mouthwashes– mayhave maylox to help coat oral cavities
  4. For discomfort
    • Local anesthetics or non prescription preparations without alcohol
  5. To prevent or treat mucositis
  6. moisten lips
  7. things to avoid (lemon glycerin swabs)
  8. Retal ulcers
    • Meticulous perineal hygiene: implement stool softners, barrier cream, avoid rectal temps and suppositories
169
Q

What do we need to know about alopecia?

A
  1. Hair falls out in clumps and regrows in 3-6 months
    • May be darker, thicker, curlier
  2. Cotton cap, scarf, hat or wig
  3. Protect scalp from cold and sun
  4. Sclap hygiene :soap and water
170
Q

How do infants and toddlers view death?

A
  1. No concept of death
171
Q

How do preschoolers view death?

A
  1. Death as temporary, a departure, a kind of sleep
172
Q

How do school-age children veiw death?

A

Death is irreversible but not necessarily inevitable and may fear mutiliation

173
Q

How do adolescents view death?

A

Death is inevitable and irreverible… angry until they accept

174
Q

What are palliative care principles?

A
  1. Guide realistic goal setting that includes the physical, emotional, social and spiritual distress… utilize resources
  2. Multidisciplinary approach
  3. Child and family are the unit of care
  4. Keep pts pain as minimal as we can.
175
Q

What is our nursing care consist of with a child on pallative care?

A
  1. Assess level of pain/admin pain meds
  2. Provide soothing surrounding, avoid excessive light
    • Play soft/loud music if thats what family wants, aromatherapy
  3. Limit care to essentials
  4. Ensure pleasant smell, touch, temp.
    • Encourage family to touch their child, put temp at whatever family wants.
  5. Encourage child to talk about their feelings
  6. explain all procedures/therapies
  7. Structure hospital/home environment to allow for max self control/independnece
  8. Encourage family assistance with care
  9. encourage family to be honest with childs question about dying
  10. frequent and timely updates to familyl on childs status
  11. provide privacy when communicating bad news
  12. Facilitate appropriate spiritual care
  13. Stay with family
  14. accept family’s grief reactions
  15. Avoid artifical consolation
  16. Allow parents to be with the child at the moment of death
  17. remember sibling
176
Q
A

Module 5- PEDI (25 decks)

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