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Fall - Pediatrics > Hematology > Flashcards

Hematology Flashcards

1
Q

What symptoms suggest iron deficiency anemia (IDA)?

A

Pallor
Irritability
Poor feeding
Lethargy

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2
Q

What is the function of RBC? What is the lifespan?

A

Transport hemoglobin and oxygen and carry CO2 back to the lungs

90 days

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3
Q

Where are RBC formed? What is reticulocyte? Do RBC differ from each other?

A

Formed from bone marrow

Reticulocyte: immature RBC

Abnormal size, shape, or heme component

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4
Q

What s/s would make you suspect anemia? (7)

A

Pallor
Fatigue
Activity intolerance
Murmur
Tachycardia
Poor growth
Learning delays

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5
Q

If anemia is not severe, what would be the first clue that a patient is anemic?

A

CBC results

Decreased RBC
Decreased Hgb/Hct
Increased reticulocyte count

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6
Q

What are nutritional anemias?

A

Iron deficiency (IDA)
Lead poisoning

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7
Q

What are two disorders cause chronic anemia?

A

Autoimmune disease
Renal failure

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8
Q

What are three congenital hemolytic anemias

A

Alpha-thalassemia
Beta-thalassemia
Sickle cell disease

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9
Q

What is IDA caused by? Is it preventable?

A

Inadequate supply or loss of iron

Preventable

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10
Q

What needs to be in an infants diet to avoid IDA? When would they develop IDA if they did not have this?

A

Fortified iron

Between 9-24 months would develop IDA if not fortified iron

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11
Q

What are the risk factors for IDA? (5)

A

Decreased supply of iron (excessive milk intake, poor eating habits, feeding problems)
Impaired absorption of iron (iron inhibitors, malabsorption IBD, chronic diarrhea)
Increased need fir iron (prematurity, LBW, adolescence)
Blood loss - mensuration
Low SES, lead exposure, exclusively breasting feeding over 12 months)

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12
Q

What would cause a decreased supply of iron?

A

excessive milk intake, poor eating habits, feeding problems

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13
Q

What would cause impaired iron absorption?

A

iron inhibitors, malabsorption IBD, chronic diarrhea

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14
Q

What would cause an increase need for iron?

A

prematurity, LBW, adolescence

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15
Q

What is the management of IDA?

A

Prevention and screening is primary goal
Only breast milk or iron fortified formula for first 12 months
Iron supplement of 1mg/kg/day by 4-6 months in breastfed babies
Infants and toddler should have iron fortified cereal and solid foods containing iron
Toddlers formula intake should be limited to 20-24 ounces/day

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16
Q

What are nursing diagnoses for anemia?

A

Impaired gas exchange
Alteration in perfusion
Fatigue
Activity intolerance

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17
Q

What are nursing interventions that could help with activity intolerance r/t anemia?

A

Decrease tissue/metabolic oxygen needs by conserving energy and clustering nursing care
Supplemental oxygen
Nutritional support

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18
Q

What is hemophilia? Is there a cure?

A

X linked hereditary bleeding disorder

No cure

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19
Q

Who carries hemophilia? Who does it affect?

A

Mom carried the hemophilia A gene and passed it to baby boy

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20
Q

What is the most common type of hemophilia? What factor? Why is that needed?

A

Hemophilia A

Factor VIII

Protein that is necessary for thromboplastin in clot formation

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21
Q

How does hemophilia A present? (7)

A

Hemorrhage from minor trauma
Can be spontaneous
Hemathroses
Intracranial hemorrhage
Nose bleeds
Hematuria
IM hematoma –> compartment syndrome

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22
Q

What is the medical management for hemophilia A?

A

Replace factor VIII clotting factor vis DDAVP/desmopressin or factor VIII infusion

PRBC if H&H is decreased

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23
Q

What does DDAVP/desmopressin do for hemophilia A? When is it used?

A

Directly increased factor VIII

OKAY for mildly affect individuals

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24
Q

What are the types of factor VIII infusion?

A

Synthetic/recombinant formulation

Plasma based

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25
Q

What should you monitor in hemophilia A to determine if replacing the factor VIII for effective?

A
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26
Q

What drugs should be avoided in hemophilia A?

A
27
Q

What are the nursing diagnoses r/t hemophilia A?

A

Risk for injury d/t bleeding
Acute pain - w/ bleed (joint) (RICE)
Risk for hypovolemic shock r/t hemorrhage

28
Q

How do you decreased to risk for injury d/t bleeding in hemophilia A?

A

Prevention
Soft toys
Avoid contact sports
Protective pads/helmets
Early identification of the blood
Hold pressure for increase time after injections

29
Q

What medical management needs to occur at home for a patient with hemophilia A?

A

Taught to administer factor VIII at home
Daily infusion via port
Sliding scale dose for acute bleeds
Education on s/s of hemorrhage (looking for a change in LOC)
Post injury care
Watching for neuro changes –> CVA
Educate on s/s that would indicate needing to come in for care

30
Q

What are changes or safety precautions that should be taken at home for a patient with hemophilia A and ITP?

A

Avoid high risk behaviors such as contact sports, riding a bike, roller skating
Avoid medications that can affect bleeding such as NSAIDS and aspirin
Use an extra-soft bristle toothbrush
Establish age appropriate safe home environment such as pad table corners, pad crib rails, extra joint padding on clothes

31
Q

What is the most effective test to diagnose hemophilia A?

A

PTT - partial thromboplastin time
Monitors bleeding times

32
Q

Do you see petechia/purpura in hemophilia A?

A

NO, because platelets are not involved
Hemophilia A will just go straight to bleeding

33
Q

What is the worst case scenario regarding hemophilia A?

A

Hemorrhage

34
Q

If a joint is injured with hemophilia A what should be done for pain? Should they use their joint?

A

Tylenol
RICE to vasoconstrict and decrease bleeding

PROM for joint and weight bearing later on

35
Q

What are platelets? What are their function?

A

Thrombocyte

Coagulation and capillary hemostatic

36
Q

What is ITP? What does it usually follow?

A

Acquired hemorrhagic disorder
Autoimmune –> excessive destruction of platelets

Usually follows 1-3 weeks after a viral illness such as viral exanthema

37
Q

Who does ITP affect most commonly? When is the recovery?

A

Most common bleeding disorder in childhood - ages 2-5

Most recover in 6 months

38
Q

How do you diagnose ITP?

A

< 100,000 platelets is diagnostic

39
Q

What are the clinical manifestations of ITP?

A

Mucocutaneous bleeding such as ecchymosis, petetchiae, purpura
Muscosal bleeding like oral, nasal, GI tract or GU tract
Hemorrhage

40
Q

Does ITP go away on its own?

A

Usually self limiting - treatment is usually symptomatic

41
Q

What is the treatment of ITP?

A

IV or oral corticosteroids (prednisone) initially
IVIG (intravenous immunoglobins) initially
Splenectomy if initial treatment ineffective and for chronic forms

42
Q

When would you do a platelet transfusion in ITP?

A

Given only when active uncontrolled bleeding occurs

43
Q

When should a patient with ITP begin to get treatment? What are you monitoring before getting to this point?

A

Less than 20,000 platelets

Monitoring platelets and H&H

44
Q

What are some nursing consideration regarding ITP?

A

Avoid administering intramuscular injection (instead subQ if possible)
NO ASA or NSAIDS
NO rectal temps
Any invasive procedures need extreme caution
Defer Vaccines after patient is given IVIG

45
Q

What should occur after a splenectomy r/t ITP?

A

Administer pneumococcal and HIB vaccine
Prophylactic PNC

46
Q

What occurs to RBC in sickle cell?

A

Destruction of RBC
Loss of RBC
Malformation of heme portion of RBC

47
Q

What is normal Hbg? What is sickle cell Hbg?

A

Normal Hbg: A or F
Sickle cell: S Hbg

48
Q

Is sickle cell inherited? Is it apparent at birth? What is fetal Hbg?

A

Genetic mutation that is inherited and hemoglobin sickles

Usually not apparent until later infancy d/t fetal hemoglobin so difficult to see in newborn which is why use newborn screening nationwide

Fetal hemoglobin is a protective factor that does not sickle

49
Q

What is the epigenetic adaptation regarding sickle cell?

A

Malaria protects against

50
Q

What is the pathology of sickle cell?

A

Hemoglobin sickles under physiologic stress (generally asymptomatic unless under stress) such as exercise, illness, increase oxygen needs, emotional
Sickle cells obstruct blood vessels
Increased RBC destruction
Decreased tissue perfusion and oxygen to tissues

51
Q

When do you see acute pain in sickle cell?

A

There is decreased perfusion so part of the body is deprived of oxygen and nutrients –> acute pain

52
Q

Where do you usually see pain in sickle cell crisis? Why do you see this pain?

A

Abdominal pain
Long bone pain
Hands and feet
Joint pain (arthralgia)
Acute chest syndrome
Priapism (prolonged ejection w/o sexual arrousal)

Ischemia causing mild-severe pain

53
Q

What are the complications of a sickle cell crisis?

A

Infections
Stroke (silent stoke occurs when a sickle cell gets stuck in brain and cuts off circulation)
URIs
Leg ulcers
Splenomegaly

54
Q

What is a vaso-occulsive or sickle cell crisis? How long does it last?

A

Sickle shaped RBC block blood flow through the tiny blood vessels to chest, abdomen and joints –> pain

Lasts minutes to days

55
Q

What is the acute management of sickle cell disease?

A

H- Hydration – aggressive to break sickle clots apart and restore perfusion (2-2.5 times maintenance)
O- Oxygenation
P- Pain control
Bedrest
Antibiotic if there is an infection (broad spectrum)

56
Q

What is the chronic management of sickle cell disease? (6)

A

Enrollment into a SCD program
Prevent sickling
Immunize!!
PNC prophylactically daily and lifelong
Recognize early s/s of crisis
Any fever over 38.5 in an emergency

57
Q

What is medical management of sickle cell disease?

A

Monthly blood transfusion w/ or w/o aphersis
Splenectomy
Daily hydroxyurea
Stem cell transplant (only potential cure and very high risk)

58
Q

Why do monthly blood transfusions help? What is aphersis? When do you get blood transfusions?

A

Shuts down patients bone marrow from producing sickle Hbg. Not a cure b/c still very low Hbg

Apheresis cleanses the blood by removing sickled RBC

Used if hydroxyurea doesn’t work and still having several vaso-occulsive crisis in a year OR brain scan reveals infarct

59
Q

What is a complication of receiving so many blood transfusions? How do you monitor for this? What is the treatment?

A

Hemosiderosis - iron storage in tissues b/c RBC that are transfused are packed with iron

Monitor by MRI of head, heart, kidney, and liver 1-2 times per year to look for overload. If overloaded –> failure of that organ

Treat by giving medication that binds to iron and poop it out

60
Q

How does a daily hydroxyurea help with sickle cell?

A

Works in bone marrow to stimulate detal Hbg –> decreased sickled Hbg production

61
Q

What are the s/s of acute chest syndrome? What should you start on these patients?

A

Presents like pneumonia

Cough, SOB, fever, retractions, increase RR, increase WOB, hypoxic, crackles

Draw cultures and start broad spectrum antibiotics until s/s improve or cultures come back negative

62
Q

What should patients with sickle cell be educated about?

A

Avoid temp extremes
Hydrate!!
Recognize fatigue and any joint pain –> need rest
Avoid alcohol, smoking(increase risk of infections and clots), and drugs

63
Q

Why are immunizations and prophylactic PNC important for patients with sickle cell?

A

Immunizations - high risk for infections b/c chronic anemic state

PNC daily - develop autosplenectomy b/c clogged up d/t so many damaged RBC –> stops functioning as immune support

Fall - Pediatrics (15 decks)

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