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Pediatrics-MJ > T3-Blueprint: Blood Disorders > Flashcards

T3-Blueprint: Blood Disorders Flashcards

1
Q

What is key with sickle cell disease?

A

Hydration and oxygenation

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2
Q

Sickle cell disease is a genetic issue resulting ins ever reduction in ____

A

Circulating RBC

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3
Q

What is the average life span for sickle cell?

A

45

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4
Q

What is the hgb count in sickle cell? What is normal?

A

2-5 gm/dl

Norm is above 11

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5
Q

Sickle cell disease–RBC contain an abnormal form of HGB so what does that mean?

A

RBC are inflexible and rigid (sickle shape)

More fragile= hemolysis (death of cells)

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6
Q

Can sickle cell be cured?

A

No

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7
Q

What is treatment for sickle cell?

A

Controlling anemia and relieving pain

Blood transfusions

Ions and fluid replacement is needed

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8
Q

T/F: A small cold in a sickle cell patient will make them very ill

A

True

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9
Q

Nutrition: What do they need? What do they not need?

A

Need: folic acid, and can eat normal foods but don’t need lots of sweets and fast foods

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10
Q

Sickle cell patients in crisis is a HIGH PRIORITY!–What are the normal complaints they are telling if they come to ED?

A

Severe pain and fever

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11
Q

Screening tests for sickle cell only detect the prescence of an abnormal hb. What are they NOT specific for?

A

Trait or anemia

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12
Q

What is the definitive test for sickle cell?

A

Hgb electrophoresis

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13
Q

Sickle cell: What is the Hgb electrophoresis test?

A

Definitive test for sickle cell that separates and measures the various HGB aka differentiates between trait and anemia

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14
Q

Sickle cell: Ophthalmic complications. What does this mean

A
  • Vitreous hemorrhage
  • Retinal detachment
  • Blindness
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15
Q

Sickle cell: What is a vaso-occlusive crisis?

A

CVA
Chest syndrome
Dactylitis
Painful episodes

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16
Q

Sickle cell: Vaso-occlusive crisis

Chest syndrome: If they have had this in the past, will they have it again?

A

They probably will

It can cause life threatening issues

Chest syndrome= chest pain, fever, cough (can worsen with or result from pneumonia)

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17
Q

Sickle cell: Vaso-occlusive crisis. What may be the first symptom of vaso-occlusion?

A

Hand and foot syndrome (dactylitis)

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18
Q

Sickle cell: Vaso-occlusive crisis. What is the most frequent complication?

A

Painful episodes occuring in joints and limbs

*Pain is there bc there is obstruction of blood flow due to sickled RBC

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19
Q

What are some other complications of sickle cell disease?

A
  • Cardiomegaly & systolic flow murmurs
  • Abdominal pain, GU dysfunction
  • Enlarged spleen
  • Dilute urine
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20
Q

What is a main issue of sickle cell disease?

A

Enlarged spleen

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21
Q

SCD: Enlarged spleen is caused by picked blood. How is this treated? What can this cause?

A

Treat with blood transfusion

Can cause anemia, hypovolemic shock

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22
Q

SCD: What is the role of the spleen?

A

Filters out infection in the body

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23
Q

SCD: If the spleen is enlarged, there is risk for infection!!! Explain.

A
  1. Normal cells last 3 months; sickled cells only last 1 month
  2. Keep out of crowds
  3. Penicillin is DOC for this issue
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24
Q

What is sickle cell crisis triggered by?

A

Viral infection or depletion of folic acid

Signs: profound anemia and pallor

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25
Q

What is aplastic crisis of SCD caused by?

A

Human parvo virus leading to lowered Hb, retake count

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26
Q

SCD: How is aplastic crisis treated?

A

Observation and transfusion

27
Q

SCD: T/F: Once they’ve had one stroke, they will normally have another

A

TRUE

Stroke affects 10% of children with sickle cell

28
Q

What do we do to treat the SCD complications?

A
  • IV hydration
  • Analgesia: morphine
  • Transfusion/transplants
29
Q

When is RBC transfusion used for sickle cell complications?

A

Aplastic, hyperhemolytic, and splenic sequestration crisis

30
Q

SCD: Avoid heat or cold?

A

COLD!!!

31
Q

What is treatment for FE overload?

A

Chelating agent that binds with iron in the bloodstream and enhances its elimination in the urine and feces

32
Q

Who is more likely to get hemophilia: boys or girls?

A

BOYS

*this is an X linked recessive disorder–girls have 2x, boys only have 1

33
Q

Hemophilia A is a deficiency in factor ?

A

Factor VIII defiency

“classic hemophilia”–80% of cases

34
Q

Hemophilia B is a deficiency of what factor?

A

Factor IX

“Christmas disease”

35
Q

Hemophilia: What is there difficulty controlling bleeding?

A

Bleeding time is exteneded due to lack of factor required for blood to clot

36
Q

Hemophilia: When may difficulities become apparent?

A

Male circumcision…but may not become apparent until infant becomes more active and prone to injuries during toddler years

37
Q

Hemophilia: What are s/s?

A 
Active bleeding
Hematomas/bruising
Hemarthrosis
Headache
Slurred speech
Decreased LOC
38
Q

Hemophilia: Avoid hot or cold?

A

AVOID HOT!

39
Q

What are some tests for hemophilia?

A

aPTT
Factor specific assays
Platelet and prothrombin time
Whole blood clotting time

40
Q

Hemophilia: Ok to take rectal temps?

A

Avoid this, as well as skin punctures and use surgical aseptic technique

41
Q

What are Factor VIII and IX ?

A

Clotting factors necessary to begin clotting cascade

42
Q

Why do we give factor VIII and IX to hemophilia patients?

A

This is given via port-a-acth bc this is what hemophilia pts ar deficient in

43
Q

What is the key sign to prolonged bleeding to the joints in hemophilia pts?

A

Hemoarthritis

44
Q

What are s/s of prolonged bleeding to the joints in hemophilia pts.?

A
  • Joint pain stiffness
  • Warmth
  • Swelling
  • Redness
  • Loss of ROM
  • Deformitis
45
Q

Hemophilia: How do we treat prolonged bleeding to joints?

A

Must treat immediaely!

RICE

Active ROM after bleeding controlled

Maintain ideal weight to avoid stress to joints

Encourage regular exercise and PT

46
Q

Hemophilia: If we need to give injections or perform an invasive procedures, how should we pretreat?

A

Pretreat with their specific factor (VIII or IX)

47
Q

Hemophilia: How do we prevent bleeding at home?

A

Padded crib

48
Q

Hemophilia: How do we prevent bleeding for toddlers?

A

Environment free of clutter–padding corners of furniture

Dress toddlers in extra layers of clothing to provide padding

49
Q

Hemophilia: How do we prevent bleeding for preschool?

A

Supervision/diversion

50
Q

Hemophilia: How do we prevent bleeding in school aged?

A

Set activity restrictions

Low contact sports!!! (tennis, swimming)–use protective equipment

51
Q

Hemophilia basics–Whats important for client educatin?

A
  • Use soft bristle toothbrush
  • Up-to-date on immunizations
  • Med ID wristband or tags need to be worn
  • RICE
52
Q

Why is iron deficiency anemia common in infancy?

A

Ages 12-36 months are most at risk due to consuming a diet high in cow’s milk without adequate intake of foods high in iron

*Hgb requires iron…Low iron=low hgb

53
Q

Why are adolescents at risk for iron deficiency anemia?

A

Poor diets
Menses
Obesity

54
Q

How should we administer iron for iron deficiency anemia?

A

Give on empty stomach (with juice, NOT MILK)

Give with meals and start with reduced dose and gradually increase if GI distrubce occurs

Give with Vit. C to help increase absorption

55
Q

If we are given liquid iron, how should child drink it?

A

Through a straw

*brush teeth to prevent staining

56
Q

What should we tell parents about stools of children who are taking iron?

A

Stools with be tarry green–this is normal

57
Q

If we give iron IM, how should it be given?

A

z-track method–do not massage afterward!

58
Q

What is epistaxis?

A

Nose bleed

59
Q

How do you stop a nose bleed?

A

Sit up and slightly lean forward and pinch bridge of nose for 10 min

60
Q

A secondary disorder of coagulation that occurs as a complication of a number of pathologic processes (hoopla, acidosis, shock, burns)

A

Disseminated intravascular coagulation (DIC)

61
Q

What are the hallmarks of DIC?

A

Bleeding and clotting

62
Q

DIC: What is the patho and why is this bad?

A

Starts when the clotting process gets started abnormally–thrombin is made faster than body can neutralize it…bad bc child is susceptible to uncontrollable hemorrhage into vital organs

63
Q

S/S of DIC?

A
  • Petechia
  • Purpura
  • Bleeding form openings
  • Bleeding from umbilicus or trachea (newborn)
  • Bleeding evidence in GI
  • Hypotension
  • Organ dysfunction from infarction and ischemia
64
Q

Therapeutic management for DIC?

A

Control of underlying or imitating cause which usually stops problem spontaneously

Platelets and fresh frozen plasma may be needed

Pediatrics-MJ (61 decks)

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